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AHA Scientific Statement
Best Practices in Managing Transition to Adulthood for
Adolescents With Congenital Heart Disease: The Transition
Process and Medical and Psychosocial Issues
A Scientific Statement From the American Heart Association
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Craig Sable, MD, FAHA, Co-Chair; Elyse Foster, MD, FAHA, Co-Chair;
Karen Uzark, PhD, PNP, FAHA, Co-Chair; Katherine Bjornsen, BSN, ARNP;
Mary M. Canobbio, RN, MN, FAHA; Heidi M. Connolly, MD; Thomas P. Graham, MD, FAHA;
Michelle Z. Gurvitz, MD, MS; Adrienne Kovacs, PhD, CPsych; Alison K. Meadows, MD, PhD;
Graham J. Reid, PhD, CPsych; John G. Reiss, PhD; Kenneth N. Rosenbaum, MD;
Paul J. Sagerman, MD, MS; Arwa Saidi, MB, BCh; Rhonda Schonberg, MS; Sangeeta Shah, MD;
Elizabeth Tong, MS, RN, CPNP, FAHA; Roberta G. Williams, MD, FAHA;
on behalf of the American Heart Association Congenital Heart Defects Committee of the Council on
Cardiovascular Disease in the Young, Council on Cardiovascular Nursing, Council on Clinical
Cardiology, and Council on Peripheral Vascular Disease
any children born with complex childhood illnesses
that historically caused early death are now surviving
into adulthood with the expectation of leading meaningful
and productive lives. They will ultimately need to transition
their care from pediatric to adult-centered care. Unfortunately, in the absence of structured programs to guide this
transition, there is often delayed or inappropriate care, improper timing of the transfer of care, and undue emotional and
financial stress on the patients, their families, and the healthcare system. At its worst, and as frequently happens now,
patients are lost to appropriate follow-up. In fact, the number
of adults with congenital heart disease (CHD) in the United
States is rising exponentially and now exceeds 1 000 000.1–7
At least half of these patients may have complex CHD. Fewer
than 30% of adults with CHD are seen by appropriate
specialized providers. Fewer than 15% of these patients, who
are seen in specialty adult CHD (ACHD) clinics, have CHD
that is classified as severe.8 Thus, adolescents with CHD
constitute a growing population of individuals for whom a
well-planned and well-executed “transition process” is
The goals of a formal transition program are to prepare
young adults for transfer of care. It should provide uninterrupted health care that is patient centered, age and developmentally appropriate, flexible, and comprehensive. It should
include age-appropriate education about medical conditions
and promote skills in communication, decision making,
self-care, and self-advocacy.9 –13 It should foster greater
personal and medical independence and a greater sense of
control over health, healthcare decisions, and psychosocial
environment. The ultimate goal of a transition program is to
optimize the quality of life (QOL), life expectancy, and future
productivity of young patients.14
We acknowledge that the development of ideal transition
programs is a laudable goal that may not be achievable in the
current healthcare environment. With the recognition that
The American Heart Association makes every effort to avoid any actual or potential conflicts of interest that may arise as a result of an outside
relationship or a personal, professional, or business interest of a member of the writing panel. Specifically, all members of the writing group are required
to complete and submit a Disclosure Questionnaire showing all such relationships that might be perceived as real or potential conflicts of interest.
This statement was approved by the American Heart Association Science Advisory and Coordinating Committee on September 3, 2010. A copy of the
statement is available at⫽3003999 by selecting either the “topic list” link or the “chronological
list” link (No. KB-0107). To purchase additional reprints, call 843-216-2533 or e-mail [email protected]
The American Heart Association requests that this document be cited as follows: Sable C, Foster E, Uzark K, Bjornsen K, Canobbio MM, Connolly
HM, Graham TP, Gurvitz MZ, Kovacs A, Meadows AK, Reid GJ, Reiss JG, Rosenbaum KN, Sagerman PJ, Saidi A, Shah S, Tong E, Williams RG; on
behalf of the American Heart Association Congenital Heart Defects Committee of the Council on Cardiovascular Disease in the Young, Council on
Cardiovascular Nursing, Council on Clinical Cardiology, and Council on Peripheral Vascular Disease. Best practices in managing transition to adulthood
for adolescents with congenital heart disease: the transition process and medical and psychosocial issues: a scientific statement from the American Heart
Association. Circulation. 2011;123:●●●–●●●.
Expert peer review of AHA Scientific Statements is conducted at the AHA National Center. For more on AHA statements and guidelines development,
Permissions: Multiple copies, modification, alteration, enhancement, and/or distribution of this document are not permitted without the express
permission of the American Heart Association. Instructions for obtaining permission are located at
identifier⫽4431. A link to the “Permission Request Form” appears on the right side of the page.
(Circulation. 2011;123:00-00.)
© 2011 American Heart Association, Inc.
Circulation is available at
DOI: 10.1161/CIR.0b013e3182107c56
April 5, 2011
adolescents and young adults receive their care from a diverse
group of providers, this document is intended for pediatric
and adult cardiologists who are likely to care for this group of
patients. We have addressed diverse topics that pertain to the
care of adolescents and young adults with CHD, beginning
with issues directly related to transition. The American Heart
Association (AHA) classification of recommendations and
levels of evidence for practice guidelines were used when
applicable. The classification and levels of evidence are
shown in Table 1.
Concepts of Transition Care
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In the United States, ⬇14% of children are identified as
having a special healthcare need.15 More than 1 in every 5
households has at least 1 child with some special needs. This
number represents a slight increase from data reported in
2001, perhaps because of increasing diagnostic capability,
increased medical access, and an increasing survival rate for
many congenital or chronic conditions that historically were
associated with high childhood mortality. Advances in medical and surgical care for children with congenital cardiovascular disease have dramatically improved their life expectancy, resulting in 85% to 90% of these children surviving to
Transition is defined by the Merriam-Webster dictionary as
“a movement, development, or evolution from one form,
stage, or style to another.” Medical transition is the process of
moving from a pediatric medical system to an adult one.
Transfer refers to the actual point in time at which responsibility for patient care is “handed off” to the adult provider.
Developmental transitions occur at specific milestones
throughout the life spectrum.
The American Academy of Pediatrics states, “The goal of
transition in health care for young adults with special health
care needs is to maximize lifelong functioning and potential
through the provision of high-quality, developmentally appropriate health care services that continue uninterrupted as
the individual moves from adolescence to adulthood.”17
However, for children with congenital or long-term healthcare needs, transition to an adult lifestyle or environment is
often made more difficult by the inability to obtain quality
health care as they leave their pediatric providers. The
American Academy of Pediatrics’ Medical Home Initiative
states that 90% of children with special needs reach their 21st
birthday, yet 45% of them lack access to a physician who is
familiar with their condition(s). In addition, 30% of all young
adults 18 to 24 years of age lack a payment source for their
health care.18 The Maternal Child Health Bureau, a division
of the Health Resources and Services Administration, US
Department of Health and Human Services, in response to
Healthy People 2010 national health objectives and the
President’s New Freedom Initiative, includes “transition to
adult health care, work, and independence” in 6 core systems
changes that are deemed necessary to improve the overall
health of children with special needs.19
To date, transition outcomes overall have been shown to be
suboptimal, particularly among ethnic minorities. Better outcomes are seen for older teens and those receiving care within
a “medical home.”20 Several steps have been identified that
are paramount to the successful transition of a child with
special needs from a family-centered pediatric system to a
patient-centered adult long-term care system, including the
1. Comprehensive care that is coordinated and managed
through a medical home (usually a primary care provider but in some instances a tertiary care center or a
subspecialty practice)
2. Access to healthcare financing
3. Education of adult providers in managing chronic conditions previously limited to the pediatric population
4. Ongoing, coordinated communication between patients,
families, and pediatric and adult healthcare providers to
facilitate transition and transfer
Reid et al21 reported in 2004 that only 48% of adolescents
with CHD underwent successful transition. Their data suggested that ongoing care during adolescence with continued
discussion of the importance of transition was very important
to successful transition. The standard of transition as defined
by the Society of Adolescent Medicine is “a purposeful,
planned process that addresses the medical, psychosocial and
educational/vocational needs of adolescents and young adults
with chronic physical and medical conditions as they move
from child-centered to adult-oriented health care systems.”9
The aim of the present statement is to provide guidelines for
clinicians to promote early and high-quality care and support
for their patients with CHD for successful transition of care.
Timing of Transition
The transitional process depends on the patient’s medical and
developmental status and should be individualized.22 The
developmental status of the patient should be considered
relative to normal developmental milestones (Table 2),4 with
special considerations for how chronic illness (Table 3)23 and
congenital heart defects (Table 4) impact these milestones.4
The American College of Cardiology task force and the
recent “ACC/AHA Guidelines on the Management of Adults
With Congenital Heart Disease” recommended that the transition process start at 12 years of age16 to prepare the patient
for transfer to adult care.
“Envisioning a future” is an important first stage in the
transition process and occurs at the time of diagnosis of a
chronic condition.24 This emphasizes starting the discussion
early and includes prompting parents to consider future
expectations for their child’s education, employment, and
independent living.24 Just as patients are prepared to take
increased responsibility for their health care, parents also
benefit from education and support as they gradually share
and then hand over the primary responsibility for healthcare
management. From initial diagnosis, discussion with parents
should reference transitional topics to help parents prepare for
this shift in responsibility. Preparing the patient for self-care
is an ongoing process that should begin in early childhood
and continue into adulthood25 to allow the development of the
necessary self-care skills.26 Knowledge of the developmental
stages in adolescence and the impact of chronic illness is
essential for a successful transition.27 During childhood, the
patient should become increasingly involved in direct discussions about his or her diagnosis, medications, and exercise
Sable et al
Table 1.
Best Transition Practices for Adolescents With CHD
Applying Classification of Recommendation and Level of Evidence
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*Data available from clinical trials or registries about the usefulness/efficacy in different subpopulations, such as sex, age, history of diabetes, history of prior
myocardial infarction, history of heart failure, and prior aspirin use. A recommendation with Level of Evidence B or C does not imply that the recommendation is weak.
Many important clinical questions addressed in the guidelines do not lend themselves to clinical trials. Although randomized trials are unavailable, there may be a
very clear clinical consensus that a particular test or therapy is useful or effective.
†For comparative effectiveness recommendations (Class I and IIa; Level of Evidence A and B only), studies that support the use of comparator verbs should involve
direct comparisons of the treatments or strategies being evaluated.
limitations. In the teenage years, discussions about heart-healthy
behaviors and the risks of smoking, alcohol, and drugs should be
introduced and reviewed regularly. Vocational and employment
advice should continue into early adulthood,25 and birth control,
pregnancy, genetics, and long-term prognosis should also be
emphasized. Table 3 describes practical steps that can be
undertaken in the designated age group, with the assumption of
appropriate developmental status. In patients with developmental delay, this timeline will have to be altered as appropriate.
Self-care education should not culminate with transfer to adult
care but is an ongoing educational process to address life
changes. Even patients who continue to see the same healthcare
providers for CHD care should undergo a process of transition
similar to that provided by family practitioners and internal
medicine/pediatric specialists who do not transfer patient care.28
Before transfer to ACHD care, ensure that any ongoing
medical or surgical issues are addressed. A portable and
accessible summary of medical history allows for effective
collaboration among healthcare providers.17 A transfer-ofcare letter with relevant medical and psychosocial information and a care plan should be provided to the regional ACHD
center in a timely fashion, with a copy for the patient to keep
in case the ACHD provider changes.
The simultaneous transfer of care of the young adult to
adult general care and subspecialty care during a period of
significant life changes can be overwhelming. This simulta-
Table 2.
April 5, 2011
Typical Life Span Developmental Tasks After Childhood for Individuals Without a Chronic Illness,4 by Age Group
Mid Adolescence
(14 –16 y)
Late Adolescence
(16 –19 y)
Young Adulthood
(19 –35 y)
Middle Adulthood
(Mid 30s–Mid 40s)
(Mid 40s–Mid 60s)
Completing puberty
Positive body image
Maintaining physical
Positive body image
Maintaining physical
physiological changes
physiological changes
and onset of common
illnesses of adulthood
Adjusting to
decreased physical
health and/or illness
Social and
Establishing peer
independence from
Establishing peer
independence from
Separation from family
of origin
Selecting a
Starting a family
Finding a social group
Assisting teenage
children in developing
Adjusting to aging
Coping with loss of
Competence in
Competence in
Maintaining emotional/
mental health
Maintaining emotional/
mental health
Maintaining emotional/
mental health
emotional/ mental
Completing high
Higher education and/or
entering work force
Establishing and/or
maintaining an
Maintaining standard
of living
Satisfaction with
Appropriate exercise
and diet
Avoiding health-risk
appropriate weight
Appropriate exercise
and diet
Avoiding health-risk
Maintaining appropriate
Appropriate exercise
and diet
Avoiding health-risk
appropriate weight
Appropriate exercise
and diet
appropriate weight
Obtaining screening
for health problems as
recommended for age
and sex
Appropriate exercise
and diet
appropriate weight
Obtaining screening
for health problems as
recommended for age
and sex
Appropriate exercise
and diet
appropriate weight
Obtaining screening
for health problems
as recommended for
age and sex
Sex-role development
independence and
interdependence with
family and friends
independence and
interdependence with
family and friends
independence and
interdependence with
family and friends
Acceptance of one’s
life history
Acceptance of death
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neous transfer can be avoided by transitioning the adolescent
with chronic health needs to a family practice or an internal
medicine/pediatric provider or to a medical care home for
primary care. This provider can then follow the patient into
adulthood while the young adult is transferred to ACHD care.
Consider referring patients with complex medical conditions
to a regional “medical home,”29 because that facilitates better
medical transition.20
Social/Family Dynamics: Impact of Chronic
Illness as Related to CHD
CHD is a chronic illness, and as such, the need for long-tem
medical monitoring, medication adjustments, repeated hospitalizations, and illness-related absences from school can
affect the adolescent’s self-image and self-esteem, as well as
delay completion of normal developmental tasks.27,30 During
the transition process, it is important for providers to understand how the individual adolescent and his or her family
interpret the diagnosis and prognosis. Research consistently has
shown that psychological adjustment is not proportionally related to the severity of the cardiac defect.31–36 Healthcare
providers must also take care not to place their own judgment
value on an adolescent’s subjective experience and coping
process. Cultural and family dynamics and past experiences
both within and outside of the healthcare environment are
other important factors to consider when preparing adolescents and their families for the transition process.
Old Age (Mid 60s⫹)
Early research reported conflicting results about the emotional and behavioral problems in adolescents with CHD.
Although some studies reported anxiety, diminished self-esteem,
depression, increased dependency, and poor emotional and
social adjustment among adolescents with CHD,37– 40 others
reported no significant psychopathology.41,42 This discrepancy
has been attributed to differences in methodology, types of
defects, and small sample size.43 A recent meta-analysis of 11
studies of the psychological problems of children and adolescents with CHD revealed that older children and adolescents had an increased risk of internalizing problems (eg,
depression, anxiety) and externalizing behavior problems (eg,
aggression, hyperactivity) compared with healthy control
subjects.35 One study examined patients at a single institution
before 1980 and a decade later to ascertain whether newer
treatment modalities and improved surgical outcomes provided more favorable psychosocial outcomes. No differences
were found; improvements in psychological outcomes did not
track with medical outcomes.44 On balance, the percentage of
adolescents and adults with CHD who have emotional and
behavioral problems is slightly greater than in the general
Qualitative research using semistructured interviews has
recently provided insight into the social experiences of
adolescents with CHD. Studies suggest that although adolescents with CHD may try to live a normal life, these teens
describe feeling “different” and set apart from their peers
because of their heart disease.46 Adolescents speak of com-
Sable et al
Table 3.
Best Transition Practices for Adolescents With CHD
Transition Timeline for Adolescents With Special Healthcare Needs: Chronic Illnesses/Physical Disabilities23
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Birth to 3–5 y, or
according to your child’s
developmental ability
• Begin keeping a record
of your child’s early
intervention, educational,
and medical history,
including immunizations.
• Attend support groups
and get to know other
parents who have
children with a chronic
illness. Learn from their
experiences, be
encouraged, and find out
about helpful resources.
By ages 3–5 y, or
according to your child’s
developmental ability
• Assign your child chores
appropriate for his/her
ability level.
• Encourage
decision-making skills by
offering choices.
• Teach natural
consequences of your
child’s behaviors and
• Continue involvement in
community and
recreational activities that
include children with and
without special needs.
• Begin teaching your child
self-care skills: general
skills and those related
to his/her special
healthcare need.
• Begin helping your child
interact directly with
doctors, nurses,
therapists, and teachers.
• Begin teaching child
about relationships,
personal space, and their
body (including their
heart condition).
By ages 6 –11 y, or according to
your child’s developmental ability
• Assess your child’s perception and
basic knowledge of his/her heart
condition. Build on their
• Continue teaching your child
general self-care and health skills,
as well as skills related to his/her
special healthcare need.
• Discuss relationships and personal
safety with your child.
• Determine whether reasonable
accommodations are needed to
ensure equal access to school
programs; if so, ask if your child
qualifies for a 504 plan.
• Encourage hobbies and leisure
activities; include exploring
community and recreational
activities, clubs, 4-H, Scouts,
Campfire, YMCA, sports, and so on.
• Continue to encourage
decision-making skills by offering
• Continue assigning your child
chores appropriate for his/her
ability level.
• Take your child shopping whenever
possible so he/she can help in
• Let your child choose how to
spend some or all of his/her
• Teach your child the consequences
of his/her behaviors and choices.
• Allow your child to experience the
consequences of a poor choice as
well as a good choice.
• Begin teaching your child
self-advocacy skills.
• Begin asking “What do you want to
do when you grow up?”
By ages 12–18 y, or according to your
child’s developmental ability
• Assess your teen’s perception and
basic knowledge of his/her heart
condition. Fill in gaps in understanding.
• Continue teaching your teen general
self-help and health skills, as well as
skills related to his/her special
healthcare need.
• Begin helping your teen keep a record
of his/her medical history, including
conditions, operations, treatments
(dates, doctors, recommendations) and
504 plan if he/she has one.
• Encourage your teen to meet with the
doctor alone for at least part of the
visit and to ask questions.
• Begin helping your teen take
responsibility for making and keeping
his/her own medical appointments,
ordering his/her own supplies, etc.
• Begin exploring future healthcare
coverage at age 17 y. Check eligibility
for SSI. At age 18 y, the teen’s
financial resources are evaluated, not
the parents’/guardians’, and medical
criteria differ.
• If your teen has a 504 plan, encourage
him/her to participate in any 504
• Discuss relationships, sexuality and
personal safety with your teen.
• If your teen is interested, introduce
him/her to age-appropriate support
• Explore possible career interests with
your teen.
• Help your teen find work and volunteer
• Continue to encourage hobbies and
leisure activities.
• Help your teen identify and be involved
with adult or older teen role models.
• With your teen, begin looking for an
adult healthcare provider.
• Encourage your teen to contact
campus services to request
accommodations, if needed, if he/she
will be attending college.
By ages 18 –21 y, or according
to your child’s developmental
• Act as a resource and support
to your young adult.
• Encourage your young adult to
participate in support groups
and/or organizations relevant to
his/her special healthcare need.
• Finalize healthcare coverage
with your young adult.
• With your young adult, finalize
transfer of medical care to an
adult provider.
• If your young adult is attending
college, encourage continued
contact with disabled student
services as needed for
• Encourage your young adult to
investigate services provided by
the Department of Vocational
Rehabilitation if he/she has not
already done so.
SSI indicates Supplemental Security Income.
mon dilemmas and concerns, including whether or not to
disclose their heart disease to others; the fear of rejection and
other effects of disclosure on social relationships; and the fear
of forming close relationships. Adolescents may have recognized the need to take a more active role in managing their
health care, but they were unsure how to begin.46 In the report
by Tong et al,46 adolescents were concerned about planning
for the future given their uncertain longevity. In another
study, both adolescents and young adults tended to have
unrealistic expectations of their life expectancy, and qualitative data suggested a lack of awareness and understanding of
their future health risks.47 Compared with their healthy peers,
adolescents with CHD are less likely to smoke, drink alcohol,
or use illicit drugs and are less likely to be sexually active.48,49
Table 4.
April 5, 2011
Life Span Developmental Tasks and Issues for the Adolescent and Adult With Congenital Heart Defects,4 by Age Group
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Mid Adolescence (14 –16 y)
Late Adolescence (16 –19 y)
Young Adulthood (19 –35 y)
Middle Adulthood
(Mid 30s–Mid 40s)*
Coping with body image
and limitations in physical
Coping with body image
and limitations in physical
Gradual or acute decreases
in physical functioning;
burden/complications with
onset of common illnesses
of adulthood
Gradual or acute decreases
in physical functioning;
burden/complications with
onset of common illnesses
of adulthood
Social and family
Peer acceptance of physical
coping with stigmatization;
lack of social support for
CHD issues
Decisions about dating;
increasing independence
from family; lack of social
support for CHD issues
Decisions regarding life
partner and reproduction;
coping with loss of
normative family life cycle;
finding a social
Addressing the impact of
premature death on
partner, any children, and
extended family
Managing anxiety-provoking
medical procedures;
maintaining emotional
adjustment during period of
critical transitions
Managing anxiety-provoking
medical procedures;
maintaining emotional
adjustment during period of
critical transitions
Managing anxiety-provoking
medical procedures;
avoiding arrhythmia-related
anxiety/ phobic reactions;
avoiding despair,
depression, or anxiety;
emotional/mental health
anxiety-provoking medical
procedures; avoiding
arrhythmia-related anxiety/
phobic reactions; avoiding
despair, depression, or
anxiety; maintaining
emotional/mental health
Education and
Coping with possible
intellectual and/or learning
Selecting educational and
vocational goals appropriate
to present/future abilities
in obtaining employment;
maintaining employment
during medical crises
employment and/or career
goals with decreases in
physical functioning
Taking some responsibility
for medical care; learning
appropriate health
Increasing responsibility for
medical care; transition to
adult care; knowledge of
diagnosis, prognosis, and
associated health behaviors
Primary responsibility for
medical care; knowledge of
prognosis; reoperation(s);
CHD complications; coping
with medical procedures
and hospitalization; coping
with procedure-related pain
Primary responsibility for
medical care; knowledge of
prognosis; reoperation(s);
CHD complications; coping
with medical procedures
and hospitalization; coping
with procedure-related pain
Health behaviors
Avoiding initiation of risky
health behaviors;
maintaining appropriate
weight and getting
exercise; maintaining oral
hygiene and preventing
Regular medical follow-up;
avoiding risky health
behaviors; maintaining
appropriate weight; getting
appropriate exercise;
maintaining oral hygiene
and preventing endocarditis
Regular medical follow-up;
avoiding risky health
behaviors; maintaining
appropriate weight; getting
appropriate exercise;
maintaining oral hygiene
and preventing endocarditis
Regular medical follow-up;
avoiding risky health
behaviors; maintaining
appropriate weight; getting
appropriate exercise;
maintaining oral hygiene
and preventing endocarditis
Personality and
Integration of CHD into self;
acceptance of being
different and unique
Lack of control over health
outcomes; increasing
Balancing independence and
interdependence with family
and friends
Resolving loss of typical
life achievements; facing
prospect of premature
CHD indicates congenital heart disease.
*Life expectancy varies with lesion severity and increases with improved medical care.
Birks et al50 found that although the participants in their study
coped well, concerns related to being unable to physically
keep up with their peers or consume alcohol as freely as their
peers (because of medication side effects) were especially
challenging and isolating. Not engaging in common risky
behaviors during adolescence is appropriate given the negative health implications; however, this creates additional
challenges for social acceptance and integration for adolescents with CHD. Adolescents in another qualitative study
developed strategies to deal with the physical limitations and
decreased stamina imposed by the disease, but the discrimination and bullying they experienced from peers left them
feeling particularly angry and excluded.51
Evaluation of QOL in adolescents with CHD by use of the
Pediatric Quality of Life Inventory revealed significantly
lower overall perceived QOL, including physical, psychosocial, and school functioning, with the greatest difference in
the domain of school functioning. Specific psychosocial
issues included feeling angry and being worried about the
future, and these were unrelated to the severity of the cardiac
defect. In contrast, Culbert et al52 used the Child Health
Questionnaire to examine health status or QOL in adolescents
with transposition of the great arteries 11 to 15 years after
their initial repair and concluded that health status as perceived by this specific cohort was excellent and showed no
significant difference from the published normative data. In
addition, a more positive outcome was found for subjects who
had the arterial switch procedure versus the atrial (Senning/
Mustard) repair.52 Although patients undergoing atrial switch
may have had longer periods of cyanosis, they were born
Sable et al
during the same time period as the arterial switch patients
Overall, the combined quantitative and qualitative data on
the QOL of teenagers with CHD suggest that they face unique
challenges in addition to those typically confronted during
adolescence. Clinically, it is important to understand the
reasons behind adolescent perceptions of their health status
and QOL to provide appropriate education and support during
the transition process.
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Transition planning for adolescents with CHD cannot occur
without a tandem evaluation of the impact of the diagnosis on
their parents. During their formative years, children and
adolescents learn and take cues from their parents on how to
manage and adapt to their illness. Although parents may be
expected to take an active role in the transition process, some
parents find this difficult. Because of the chronic nature of
CHD, many parents have devoted substantial time and energy
to monitoring their child’s medical care to ensure optimal
health and survival. Many studies suggest that parents of
children with CHD are more likely to experience elevated
symptoms of depression, anxiety, stress, and anger, as well as
poorer QOL.53–57 Research has shown that parental anxiety
about raising a child with CHD is more directly related to the
diagnosis per se and not the severity of the defect.31,32,53,54,56,58 In addition, the quality of the parent-child
relationship, and not disease severity, holds the key to
successful transition, with perceived parental acceptance
having a more positive effect on adolescent psychological
Parental anxiety can lead to overprotection and may also
explain parents’ reluctance to shift greater responsibility for
illness management to their adolescent.27,60 Many adults with
CHD recall parental overprotection during their teenage
years.32,51,61,62 Parenting styles can either hinder or support
the appropriate integration of illness into lifestyle.63 Furthermore, even after parents have relinquished control over their
adult child’s healthcare management, control might shift back
to the parents in situations when there is dissatisfaction with
medical communication or competency of care.62 McDonagh64 highlighted the importance of “negotiating” parental
involvement throughout the transition process.
Parent-adolescent perceptions of QOL may not always be
in agreement. Casey et al65 found that parents underestimated
their child’s physical exercise tolerance in 80% of cases, and
in their study on QOL in children with heart disease, Uzark et
al36 found that parent-adolescent agreement about QOL was
consistently lower for the adolescent group than for their
younger counterparts, with the greatest difference occurring
in the domain of social functioning. Other studies, however,
have found that children and adolescents with CHD reported
more behavioral/emotional problems and poorer QOL than
their parents reported about them.66,67 To fully understand the
impact of CHD on adolescents, therefore, it is important to
gather information from both teens and their parents during
the transition process. Optimal transition involves collaboration; in addition to patients and healthcare providers, parents
Best Transition Practices for Adolescents With CHD
and other family members must be integrated into the
There are limited data on the impact of CHD on other
family members. Janus and Goldberg68 looked at the impact
on healthy siblings. High treatment intensity was associated
with high accommodation of illness and elevated behavior
problems among patients. In contrast, siblings in families in
which treatment intensity was low but accommodation of
illness was high were at most risk for behavior problems.68
On the basis of data from a parental survey, Wray and
Maynard69 reported that a having a child with CHD was
perceived to have a negative impact on many areas of family
life for approximately one fifth of the sample, particularly in
those families in which the child was perceived to be more ill.
Family relationships, however, were affected in a very
different way, with 43% reporting that family members had
become closer, and only 8% reporting that they had been
“pulled apart” by the condition of their child.69
For Adolescents
1. The timing of transition should be guided by emotional maturity and developmental level (as opposed
to chronological age) for transition planning (Class
I; Level of Evidence C).
2. The adolescent should be engaged in transition
planning (Class I; Level of Evidence C).
3. The adolescent should be asked about their understanding of their disease in relation to their current
health status, restrictions on activities, and future
goals (Class I; Level of Evidence C).
4. The adolescent should be encouraged to share concerns about QOL issues (physical restrictions,
school, peers, social relationships) (Class I; Level of
Evidence C).
5. The adolescent’s fears and concerns should be acknowledged in an empathetic, nonjudgmental manner (Class I; Level of Evidence C).
6. The pediatric cardiology provider should initiate
and work together with the adolescent on a transition plan using a transition resource binder and/or
health “passport” (Class I; Level of Evidence C).
7. Providers should begin to direct health discussions
more toward the adolescent than the parent (Class I;
Level of Evidence C).
8. QOL issues should be discussed privately with the
adolescent (Class I; Level of Evidence C).
9. Be flexible (Class I; Level of Evidence C).
For Parents
1. The pediatric cardiology provider should initiate
discussions on transition planning and partner with
parents in the process (Class I; Level of Evidence C).
2. The pediatric cardiology provider should solicit information about parental perceptions of their child’s
QOL (Class I; Level of Evidence C).
3. The pediatric cardiology provider should encourage
discussion of parental understanding of their child’s
disease and concerns in relation to future goals
(illness management, education, career) (Class I;
Level of Evidence C).
April 5, 2011
4. The pediatric cardiology provider should be nonjudgmental and empathetic when acknowledging
fears70 (Class I; Level of Evidence C).
Health Supervision Issues
Primary Care and Medical Follow-Up Needs
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The primary care provider can play an important role in the
process of transition. Nearly all individuals born with congenital heart defects require both primary medical care and
routine cardiac follow-up care. Many types of providers,
including family practice physicians, general pediatricians,
adolescent medicine specialists, internists, social workers,
nurses, and nurse practitioners, can function in the role of
primary care provider. Ideally, the number of transfers among
primary care providers should be minimized. The coordination of care between primary and specialty providers is
particularly important during adolescence, when new medical
and social issues arise that interact with the CHD and can
affect health status. Expertise in adolescent medicine is
important, including sexuality, contraception and pregnancy,
drug and alcohol use and abuse, smoking, mental health, and
exercise and physical activity. Although discussions should
involve the patient and the parents, time must be allotted to
discuss all issues with the adolescent alone. Once a patient
reaches adulthood, planned transition of primary care to an
internist must occur without gaps in primary care. Some
period of overlap of care between pediatric/adolescent specialists and internal medicine specialists is ideal, with consideration of not having the patient transfer to adult primary
and cardiac care providers at the same time.
Role of the Primary Care Provider
All individuals with chronic lifelong healthcare needs should
have access to a “medical home” as defined by the American
Academy of Pediatrics’ medical home policy statement.71
According to this policy statement, the primary physician
must provide primary, family-centered care. The primary care
physician should also maintain an accessible, comprehensive,
central record that contains all pertinent information about the
patient, preserving confidentiality. The coordinating physician should transmit appropriate primary care and subspecialty reports to appropriate consultants and to the patient and
the patient’s family. It is critical to avoid having multiple
specialists functioning independently without knowledge of
each other’s activities. Patient and family involvement and
education act as a safeguard against fractionation of medical
care. Careful attention to an accurate and up-to-date patient
“passport” is an important component of this. The primary
care provider must coordinate care with the CHD expert on a
wide variety of noncardiac issues. This need for coordination
of services is especially important for patients with multiple
medical problems, which is often the case in adolescents and
adults with CHD.
The population of adolescents with CHD is widely heterogeneous. They have residua and sequelae of their disease that
at the same time may be lesion specific but also variable in
severity and manifestations among individuals with similar
diagnoses. To appropriately triage a patient with new symptoms or clinical findings, the primary care physician must
know the diagnosis, the surgical history, and the current
cardiac status, as well as current medications. The primary
care provider should be aware of the patient’s baseline
functional status and recommended restrictions and have an
understanding of the cardiac prognosis. In addition, the
primary care provider should recognize what signs or symptoms are potentially related to the cardiac condition and what
might represent worsening of the patient’s condition. The
primary care provider should be encouraged to contact the
cardiologist for any potentially concerning findings.
Medical Follow-Up
Many of the specific issues of medical follow-up, including
noncardiac medical problems and surgical needs, psychosocial issues, anesthetic issues, medications and drug interactions, anticoagulation, antibiotic prophylaxis and dental care,
exercise and sports participation, contraception and pregnancy, new symptoms or acute illness, travel, education,
employment, insurance, diet, and genetic counseling, are
addressed in this scientific statement.
Patients with CHD may have other medical problems and
may be taking medications with potential drug interactions.
For such patients, the primary care physician should be aware
of all medications and should be alert to any potential
interactions. Examples include drugs that raise serum levels
of other drugs or that alter the activity of another drug (eg,
warfarin). Some drugs have direct cardiovascular effects (eg,
tricyclic antidepressants prolong the QT interval) and may be
contraindicated in some cardiac patients.
There are published recommendations about the need for
and administration of prophylactic antibiotics to prevent
bacterial endocarditis.72 The cardiologist should inform the
pediatrician, dentist, and family of the need for such prophylaxis. Nonmedical procedures, such as body piercing and
tattoos, that engender a risk of endocarditis73 should be
discussed with the patient, family, and pediatrician. In general, such procedures should be discouraged.
Role of the CHD Expert in Medical Follow-Up
To ensure continuity of care, the cardiologist should prepare
and maintain an up-to-date, comprehensive cardiac record
and transmit that record to the coordinating physician and the
patient.7,17,74 The record should include stratification of risk
and complexity, as well as identification of specific issues
that place the patient at increased risk. This information
should also be available to the parents and the adolescent
patient. The appropriate cardiac information should be updated at the time of each cardiology encounter, and new
information should be transmitted to the coordinating physician and the patient.
For successful transition to adult congenital cardiac care,
the pediatric cardiologist should prepare a written adolescent
transition plan that includes a cardiac destination. The plan
should be shared with the coordinating physician and eventually the adult congenital cardiologist. Each pediatric cardiologist should identify the ACHD center to which the transfer
of patients will be made.9,21,75,76 In addition to provision for
adult cardiac care, the pediatric cardiologist should coordinate transition care with the primary care physician to ensure
continuity of primary medical care into the adult healthcare
Sable et al
system. In cardiology encounters during adolescence, the
pediatric cardiologist should address medical issues specific
to adolescence as they apply to cardiovascular disease. The
natural and postoperative history of CHD changes with each
decade; thus, even for seemingly simple issues, management
evolves with the availability of new outcome data. The
cardiologist must be responsible for the ongoing education of
the primary care provider and the patient, including education
and oversight of the transition process. The cardiologist
should also provide guidance to other subspecialists who may
have limited experience with CHD to avoid unnecessary
testing and misunderstanding
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1. The patient’s “medical home” should reside with the
primary care provider, who should provide familyand patient-centered care (Class I; Level of Evidence
C). The primary care physician should maintain a
confidential central record that contains all pertinent information about the patient (Class I; Level of
Evidence C).
2. The pediatric cardiologist should prepare a written
adolescent transition plan that includes a cardiac
destination (Class I; Level of Evidence C).
3. The ACHD expert should prepare and maintain an
up-to-date comprehensive cardiac record and transmit that record to the primary care provider (Class
I; Level of Evidence C).
Residual Surgical Issues
General Considerations
Many patients with repaired CHD have residual hemodynamic derangements. The transition process must include education about the likelihood of progression and the potential need
for future medical, catheter-based, and surgical intervention.
Patients should be alerted to signs and symptoms that suggest
worsening status and the need for routine and nonroutine
diagnostic assessment.2 This discussion should focus on the
present state of the art and on future developments that may
expand available therapeutic options. The patient should be
referred to appropriate resources to allow them to remain
informed on the latest diagnostic and therapeutic options
available to them.
Another consideration is the provision of inpatient care,
which may depend on whether the service functions within a
stand-alone children’s hospital or a combined medical center.
Where to best provide care for the older adolescent or young
adult should take into account the resources of the institution,
maturity of the patient, specific procedure being performed,
and the patient’s comorbidities.77
Medical Considerations
A center that provides services to the ACHD population must
have, in addition to experienced surgeons, an active electrophysiology/cardiology team that can provide diagnosis and
medical, catheter-based, and intraoperative expertise in the
treatment of arrhythmias.78 The cardiologist and surgeon
must recognize the risk of embolic events from thrombus
formation within previously placed synthetic systemic-to–
pulmonary artery shunts or synthetic patches, recognize the
Best Transition Practices for Adolescents With CHD
need for bridging of anticoagulation/antiplatelet medications
at the time of procedures, and anticipate the presence of
acquired heart disease (congestive heart failure, coronary
artery disease, valvular disease) over and above the substrate
of existing CHD.
The decision to proceed with surgery for a residual or
recurrent lesion in an adolescent or young adult with CHD is
often based on the assessment of myocardial and valvular
function, which is not always straightforward, especially for
right and single ventricles. Comprehensive assessment can
include echocardiography with newer modalities, cardiac
magnetic resonance imaging, and cardiopulmonary exercise
testing and arrhythmia monitoring, as well as a detailed
history and physical and biomarkers.
Surgical Considerations
Special consideration must be given to adolescents and young
adults with CHD who present for surgical intervention.79 In
all cases, catheter-based and surgical options should be
discussed thoroughly with the patient. Operative approaches
may be altered to address cosmetic concerns without sacrificing optimal visualization of the defects to be repaired.
Sequelae of CHD that may impact management and surgical
options include hematologic disorders from chronic cyanosis,
aortopulmonary collateral development, volume overloading
or ventricular failure from intracardiac shunting, and arrhythmias.80 There are also inherent risks associated with reoperation and resternotomy, that is, chamber, vessel, or conduit
entry and phrenic nerve injury. Synthetic pericardial membrane closure during initial surgical interventions may aid in
reduction of adhesion formation.81 Elective procedures
should be discussed with the adolescent, and the patient
should be given the opportunity to participate in the decision
about scheduling, taking into consideration the patient’s
academic, athletic, and social schedules. This also enables the
patient to participate in medical decision making and develop
necessary skills for the future.
An important consideration is the optimal environment for
perioperative care, which may require transitioning from an
inpatient pediatric service to an adult service. It is important
to individualize these decisions depending on the patient’s
physiology, the type of operation, and the emotional maturity
of the patient.77
Specific Lesions Requiring Intervention
The majority of adolescent and young adults going through
the transition process have already undergone reparative
intervention.82 The number of patients with unrepaired or
palliated CHD has diminished as corrective cardiac surgery in
infancy has become the standard for many lesions. For
example, single-ventricle pathways are often completed before the age of 3 years. Interventional cardiac catheterization
techniques have also advanced. Most atrial septal defects,
patent ductus arteriosus, and some ventricular septal defects
are now closed primarily by use of transcatheter interventions. Initial surgery in the adolescent and young adult
population is therefore most likely to be required in those
with lesions that are not amenable to percutaneous repair,
such as sinus venosus atrial septal defects or partial anomalous pulmonary venous drainage, or in those with progressive
April 5, 2011
disease such as aortic root dilatation or progressive stenosis
or insufficiency in patients with bicuspid aortic valve. Reoperation is common as patients require new implants (valves,
conduits, or pacemakers) or replacement of existing implants
because of deterioration or growth of the patient.
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1. Follow-up studies, both noninvasive and invasive,
should be performed by providers with expertise in
the performance and interpretation of the data
specific to CHD (Class I; Level of Evidence C).
2. Regular (at least annual) follow-up is required to
ensure appropriate hemodynamics are maintained
and to prevent secondary complications (Class I;
Level of Evidence C). This will reinforce to the patient
that regular follow-up is essential indefinitely.
Follow-up schedules specific to each disease are discussed in the “ACC/AHA 2008 Guidelines for the
Management of Adults With Congenital Heart
3. Nonsurgical and surgical interventions must be tailored to the individual patient and should be performed in centers with specific expertise (Class I;
Level of Evidence C).
Electrophysiology/Pacemaker Issues
In adults with repaired CHD, the development of symptomatic arrhythmias is the most frequent cause of hospital
admissions and is associated with significant morbidity,
including exercise intolerance, heart failure, and thromboembolic complications including stroke and syncope, as well as
mortality. Although late ventricular arrhythmias have received the most attention from medical personnel, more
recent data show that the development of late atrial arrhythmias is far more common and important because of atrial
thrombus formation.80,83
Atrial septal defects, both repaired and unrepaired, have a
high incidence of developing supraventricular tachycardias
over time, especially among those with late repair.80,83 Such
patients and others who have undergone atrial surgery, such
as Senning- and Mustard-type repairs for transposition of the
great arteries, may be treated successfully with catheter
ablation procedures84 or pacemakers with antitachycardia
pacing capability. Atrial tachycardia is common in patients
with single-ventricle repairs and is poorly tolerated. In these
patients, ablation is challenging and has a lower success rate.
Ventricular arrhythmias, including premature ventricular contractions and nonsustained ventricular tachycardia, occur in
as many as 35% of patients with repaired tetralogy of Fallot
or double-outlet right ventricle.85 The risk of sustained
ventricular tachycardia or late sudden death approximates 6%
to 7% with long-term follow-up.86
Advances in device therapy provide a wide spectrum of
treatment options for patients with CHD and arrhythmias that
require pacing. Abdominal generator implantation may lessen
the external appearance of pacemaker implantation in young
patients, which poses a psychological issue for adolescents.
Activity restrictions are relatively minor but include avoidance of blunt contact sports such as tackle football, judo,
karate, and wrestling. Limitations of venous access and the
Table 5. Congenital Cardiac Lesions and Perioperative Risk
for Noncardiac Surgery16
High risk
Pulmonary hypertension, primary or secondary
Cyanotic CHD
New York Heart Association class III or IV
Severe systemic ventricular dysfunction (ejection fraction ⬍35%)
Severe left-sided heart obstructive lesions
Moderate risk
Prosthetic valve or conduit
Intracardiac shunt
Moderate left-sided heart obstruction
Moderate systemic ventricular dysfunction
CHD indicates congenital heart disease.
need for lead and generator replacement with age favor an
epicardial approach for pacemaker implantation.
Noncardiac Surgery
The presence of CHD places most patients at increased risk
for noncardiac surgery (Table 5).16 In the transitional period,
several additional issues arise in perioperative management.
Adolescence and young adulthood may be a period when
there is an increased need for surgical interventions. Examples include worsening orthopedic conditions, such as progressive scoliosis, and removal of impacted molar teeth. Even
relatively minor surgeries such as tooth extraction may place
the complex cyanotic patient at risk for complications.
During the transitional period from the care of a pediatric
cardiologist to an adult congenital cardiologist, critical issues
for intraoperative management may be overlooked. As in the
child or the adult, high- or moderate-risk surgeries in adolescent patients with complex CHD, including those with
pulmonary vascular disease, should be performed in hospitals
with specialized centers for their care whenever possible. The
need to transfer the patient to a tertiary facility may be faced
by community physicians who do not have the unique
resources required for the perioperative care of these patients.
In tertiary centers, the pediatric cardiology and adult cardiology inpatient services may be physically separated, and a
decision about the optimal location for the procedure will
need to be individualized.
Ideally, elective surgery will be preceded by preoperative
planning that includes providers with representation from the
adult congenital or transitional heart disease, anesthesiology,
and surgical services. The purpose of this planning session
should be to address the preoperative evaluation and plan the
intraoperative and postoperative management, with an emphasis on the risk factors for adverse surgical outcome. The
level of risk for noncardiac surgery depends on the patient’s
underlying congenital lesion and ventricular function, as well
as the presence and severity of cyanosis and pulmonary
hypertension. The urgency and complexity of the surgical
procedure also impact risk.
The Surgical Procedure
There are no available publications describing the most
common noncardiac operations performed in the adolescent,
Sable et al
Best Transition Practices for Adolescents With CHD
Table 6. Important Historical Features in
Preoperative Evaluation
Table 7.
The nature of the original congenital lesion
Signs of congestive heart failure
Previous cardiac surgery, including palliative (eg, shunts) and total repairs
(copies of previous operative reports are essential)
Prolonged periods of mechanical ventilation or prior tracheostomy
Outcome of previous noncardiac surgery
Previous nonsurgical cardiac interventions
Functional class of the patient based on reported exercise tolerance
History of syncope or significant arrhythmias
Cardiac and noncardiac medications
History of phlebotomy requirements and possible symptoms of
hyperviscosity syndrome
History of bleeding disorders
Associated pulmonary disease
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and although there may be overlap with adults, they may
differ in frequency. The most common procedures are likely
to span a number of different surgical subspecialties and are
likely similar to those in the adult populations.87,88 Some of
these procedures are related directly to the patient’s CHD (eg,
orthopedic procedures in a patient with Marfan syndrome or
cholecystectomy in a cyanotic patient), but others are
The risk for the patient is related not only to the severity of
the illness but also to the nature of the surgery being
performed.89 Urgent surgery is nearly always associated with
higher risk, in part because unanticipated problems are more
likely to occur. The more extensive fluid shifts associated
with intra-abdominal surgery are associated with higher risk,
especially in patients with greater preload dependence, including those with congenital aortic stenosis or other forms of
left ventricular outflow tract obstruction and pulmonary
hypertension. In a series from the Mayo Clinic, procedures
involving the respiratory tract or neurological system were
associated with the highest risk of complication.90
Preoperative Risk Assessment
There are very few studies addressing the outcomes of
noncardiac surgery in patients with CHD. In 1 study,90 the
major perioperative risk factors included cyanosis, treatment
for congestive heart failure, poor general health, pulmonary
hypertension, and younger age. The overall risk of complications was low at 5.8%, and there was only 1 death.
The goals of preoperative evaluation are to identify the
major risk factors and, when possible, modify them to
minimize surgical risk. The key elements of the preoperative
evaluation specific to this patient population are included in
Tables 6 through 9. Cardiac catheterization is rarely indicated
specifically for a preoperative evaluation in the patient with
planned noncardiac surgery but should be considered if the
noninvasive studies are inconclusive. Pacemakers and defibrillators should be interrogated to ensure proper functioning.
Preoperative Management Issues
There are a number of preoperative management issues that
need to be addressed in the patient with CHD. Consultation
should be obtained from a cardiologist experienced in the
Physical Examination in the Preoperative Patient
Presence of clubbing and cyanosis (including differential cyanosis)
Jugular venous distention
Pulmonary crackles
Peripheral edema
Evidence of residual disease— examples include
Diastolic murmur of aortic insufficiency in patient with previous aortic
valvotomy for congenital aortic stenosis
Diastolic murmur of pulmonary valve insufficiency in patient with previous
surgery for tetralogy of Fallot
Holosystolic murmur due to residual VSD in patient with a VSD patch leak
Holosystolic murmur of mitral regurgitation in a patient with a repaired
atrioventricular septal defect (also known as endocardial cushion defect)
due to persistence of cleft leaflet
Accentuated P2 sound due to pulmonary hypertension
VSD indicates ventricular septal defect.
care of adolescents with CHD. In general, most cardiac
medications should be continued until the time of surgery and
resumed as soon after surgery as possible. Patients may
require substitution of warfarin with heparin depending on
the indication for anticoagulation. In patients with mechanical
prosthetic valves or classic Fontan surgery (direct right
atrium–to–pulmonary artery connections), the thrombotic risk
is high, and the period without anticoagulation should be
minimized. In the cyanotic patient with marked degrees of
secondary erythrocytosis, a bleeding diathesis has been recognized. Preoperative phlebotomy has been recommended to
reduce bleeding risk in some cyanotic patients with secondary
erythrocytosis and hematocrit greater than 65%.16,91 Procedures likely to be associated with bacteremia require endocarditis prophylaxis according to the most recent AHA
guidelines, “Prevention of Infective Endocarditis.”92
Intraoperative Management Issues
The anesthesiologist must pay careful attention to the physiological consequences of mechanical ventilation and the
fluid balance that is required in patients with complex
CHD.93,94 For example, in patients with single ventricles and
Fontan physiology, the pulmonary blood flow is passive and
preload dependent. The use of positive end-expiratory pressure and high tidal volumes may actually reduce oxygen
saturation. Adequate hydration is important in these patients
Table 8. Baseline Laboratory Evaluation in the
Preoperative Patient
Hematologic evaluation, including CBC and coagulation profile, as well as
bleeding time and serum iron and ferritin levels if mean corpuscular volume
is low
Liver function tests (especially in the presence of right-sided heart failure
and in patients with Fontan-type surgery), serum albumin levels
Renal function
Arterial blood gases
Pregnancy test (in appropriate females)
CBC indicates complete blood count.
Table 9.
April 5, 2011
Preoperative Cardiac Evaluation
ECG for evidence of conduction system disease and sinus node dysfunction.
At-risk groups for bradycardia include
Congenitally corrected transposition of the great vessels (l-TGA)
Transposition of the great vessels (d-TGA) after atrial switch procedures
(Mustard or Senning operations)
Atrioventricular septal defects (endocardial cushion defect)
Holter monitor or event monitor in patients with symptoms suggestive of
Echocardiogram to evaluate
Ventricular size and function
Systemic right ventricular function in patients with l-TGA and d-TGA
after atrial switch
Residual shunts
Severity of valvular disease
Pulmonary hypertension
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Cardiac catheterization: Indicated when noninvasive studies are inconclusive
with regard to pulmonary arterial pressure, ventricular function, and severity
of valvular obstruction
Pacemaker/defibrillator interrogation
l-TGA indicates levo-transposition of the great vessels; d-TGA, dextrotransposition of the great vessels.
and in cyanotic patients with elevated hematocrit. Urine
output, central venous pressure, and intraoperative blood loss
must be monitored carefully in these patients. In certain
cases, a pulmonary artery catheter should be considered for
monitoring left-sided filling pressures. Intraoperative transesophageal echocardiography is an alternative method for the
evaluation of ventricular filling and function.
Particular care must be exercised to avoid introduction of
air into intravenous lines and central catheters to minimize
the risk of air embolism, especially in patients with Eisenmenger physiology. The choice of anesthetic agent should be
individualized, with consideration of the cardiodepressant
effects of some of the inhalation agents and the vasodilating
properties of other agents. Intraoperative management of
pacemakers and defibrillators requires consultation from the
electrophysiologist. If at all possible, use of electrocautery
should be avoided. When it is required, the pacemakerdependent patient should be programmed to the asynchronous
mode to avoid interference.95 Other precautions include the
use of a bipolar cautery system and assurance that the current
path for the cautery does not include the pacemaker. Laparoscopic procedures may be especially hazardous in the
patient with CHD. Insufflation of the abdomen may cause a
decrease in venous return with a reduction in cardiac output.
There is also a potential for paradoxical gas embolism in
patients with residual shunts.
Postoperative Management Issues
Fluid management may require postoperative invasive monitoring in the patient with tenuous hemodynamic status.
Abdominal surgery may be associated with considerable
third-space losses that persist for several days postoperatively. With continued postoperative mechanical ventilation,
the intensivist needs to be aware of the potential adverse
effects of positive end-expiratory pressure and high tidal
volumes. Once the patient is extubated, early mobilization
and attention to pulmonary toilet may reduce the risk of
postoperative pulmonary infection. In general, most cardiac
medications, including anticoagulants, should be started as
soon after surgery as possible. Occasionally, in the postoperative patient, equivalent doses of parenteral drugs may be
substituted for the oral preparation.
Appropriate pain management is important for patient
comfort and to minimize the hemodynamic demands placed
by tachycardia and hypertension associated with pain. On the
other hand, hypoventilation associated with excessive narcotic use may lower oxygen saturation and increase pulmonary vascular resistance. Adolescents and young adult patients with CHD may have lower thresholds for pain
tolerance, influenced by childhood experience. Preoperative
evaluation of the patient’s perception of pain tolerance may
be helpful in the postoperative period.
1. High- or moderate-risk surgeries in adolescent patients with complex CHD should be performed in
hospitals with specialized centers for the care of
patients with CHD (Class I; Level of Evidence C).
Depending on a number of factors, the choice of
setting (pediatric or adult inpatient) should be individualized (Class I; Level of Evidence C).
2. Elective surgery should be preceded by preoperative
planning that includes ACHD experts, as well as
anesthesiology and surgical services (Class I; Level of
Evidence C). Original operative and catheterization
reports should be obtained and reviewed (Class I;
Level of Evidence C).
3. Intraoperative management should be performed by
an anesthesiologist familiar with the physiology imposed by the patient’s CHD (Class I; Level of Evidence C).
Follow-Up Needs for Diagnosis/Repair
As noted in the 32nd Bethesda Conference,1 there are nearly
1 million adults with congenital heart defects in the United
States. Despite extremely successful diagnostic and treatment
strategies, more than half of these adults have complex
conditions or residual issues that require follow-up and
treatment by physicians with experience and expertise in the
care of these patients. These adults should have their care
coordinated with a regional ACHD center, as defined in the
guidelines from the 32nd Bethesda Conference, and each
patient should have a primary care physician who has
24-hours-a-day, 7-days-a-week backup from a regional center
to provide optimal ongoing care.2 If the ACHD center is not
within 1 to 2 hours’ travel, a cardiologist with experience in
CHD who is in the patient’s local area and who can help with
urgent patient issues should be involved. A detailed approach
to each lesion is provided in the ACHD guidelines.16
General principles of coordinating care are depicted in
Tables 10 and 11. Table 10 includes health maintenance
needs of particular importance to CHD patients. Common
medical issues in cyanotic patients of relevance to both the
primary care physician and the cardiologist are listed in Table
11. Thorough review of both prescribed and over-the-counter
Sable et al
Best Transition Practices for Adolescents With CHD
Table 10. Health Maintenance Needs Usually Handled by
Primary Care Provider
Cholesterol screening
Hypertension screening
Cancer screening
Assessment of tobacco, alcohol, and drug use
Nutritional counseling
Contraception, sexuality issues
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medications is important to assess for drug interactions and
potential adverse reactions.
Nearly all patients with repaired defects or mild defects can
engage in moderate- to high-intensity exercise. Patients with
complex defects, cyanosis, ventricular dysfunction, or symptomatic arrhythmias require evaluation by a specialist in
ACHD for an individualized exercise prescription. The report
from the 36th Bethesda Conference96 and ACHD guidelines16
give detailed descriptions of individual lesions and recommendations for sports participation and can be used as
Anticipatory Guidance
Genetic Counseling
Although approximately 1 in 100 infants is born with a
congenital heart defect, the genetic cause and recurrence risk
vary. As the adolescent approaches the reproductive years,
the cause of the congenital heart defect and inheritance
patterns should be addressed if these issues have not been
addressed previously. These investigations can potentially
define the risk to the adolescent’s offspring, and the information can be provided at an appropriate developmental stage. A
genetics evaluation, with a thorough clinical evaluation and
newer diagnostic tests, may offer diagnostics previously
unavailable to families and their care providers.
CHD can occur in isolation (nonsyndromic) or as a
component of an underlying genetic or cytogenetic anomaly
(syndromic). It may also occur secondary to environmental
factors97 such as diabetes mellitus or prenatal exposure to
alcohol, or it may be the result of multifactorial inheritance.
Table 11.
Medical Issues in Cyanotic Patients
Compensated (stable hematocrit, iron replete, symptoms of hyperviscosity
mild or absent)
Decompensated (rising hematocrit, iron deficient, symptoms of
hyperviscosity severe)
Abnormal hemostasis
Renal dysfunction
Hyperuricemia and gout
Hypertrophic osteoarthropathy
Approximately 10% to 15% of newborns with CHD will have
an underlying genetic basis for the disease. The majority of
cases are considered to be a result of multifactorial
For these adolescents, the process of delineating the cause
of the heart disease and its impact on their offspring can be
instrumental in their understanding of risks for their children.
In some cases, the risk to their offspring will be no higher
than that for the general population; in other cases, it may be
as high as 50%. For some affected individuals, the risk may
be not only for recurrence of a congenital heart defect but for
a range of birth defects or disabilities associated with an
underlying genetic syndrome.99,100
To assist the clinician in evaluating the mode of inheritance
for a specific family, a number of items should be taken into
consideration. A checklist recommended for the adult patient
with CHD can also be implemented in the adolescent years.99
Recommendations include an evaluation for other medical
conditions; assessment of noncardiac malformations and
facial dysmorphism; determination of cognitive ability; confirmation of prior genetic studies; and completion of a
detailed family history. The information obtained can be used
to identify families who can benefit from referral to a genetic
specialist. Genetic evaluation of an adolescent with CHD
requires a multifaceted approach that often entails the input of
multiple specialists. Elements of the process should include
the following:
Targeted 3-generation family history, including individuals
with CHD, other malformations, pregnancy loss, or developmental delay
Genetic evaluation: Syndromic versus nonsyndromic
Cytogenetic and/or molecular testing if indicated
For some adolescents, further counseling about risk of recurrence and reproductive counseling about prenatal screening
and other diagnostic options may need to be delayed until
there is appropriate emotional maturity, but these should be
offered to the pregnant adolescent. The ACHD specialist
should assess the patient’s understanding of the information
provided by the genetic counselor and its emotional impact.
Counseling should be individualized, with consideration
given to the patient’s emotional maturity and family dynamics. In many cases, it may be preferable to include a
parent/guardian or other family member of the patient’s
An important resource that has been beneficial to geneticists
is available to all practitioners. GeneTests (
is a service that provides information about laboratories that
offer specific testing for specific disorders. Reimbursement for
these tests varies by test and insurance carrier. Gene reviews on
important syndromes can also be found at this Web site. Another
helpful tool is Online Mendelian Inheritance in Man
(⫽omim), which now
lists 363 genetic disorders that include a congenital heart defect
as a presenting finding. Many other resources are available that
describe the increasing number of cardiac defects associated
with single gene disorders/cytogenetic syndromes and multifac-
Table 12.
April 5, 2011
Empirical Recurrence Risk for Isolated CHD102
One Sibling
6 –10
3– 6
Pulmonary stenosis
Coarctation of aorta
Aortic stenosis
CHD indicates congenital heart disease; VSD, ventricular septal defect; ASD,
atrial septal defect; and TOF, tetralogy of Fallot.
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torial inheritance.101 Empirical risk tables have been developed
to assist practitioners with offering the most accurate recurrence
risks both to affected individuals and to their parents (Table
Once a patient has had a child with CHD, clarifying the
cause will help them understand recurrence risks for their
future offspring to have a CHD. Although some CHD occurs
in isolation, if an association and correct diagnosis can be
made, recurrence risks and prenatal diagnosis options can be
addressed accurately. Determination of the inheritance pattern allows for provision of the most accurate genetic
Counseling Issues
Although an adolescent may not be thinking about immediate
childbearing, access to available information about the likelihood of having an affected child and prenatal screening and
diagnostic options may plant a seed for future reference.
Often, the perceived risk is higher than the actual recurrence
risk. A meeting with a genetic counselor and a genetic
specialist can help diagnose a child who has a CHD secondary to a genetic disorder, be it a chromosome abnormality or
a single gene disorder. Identification of a dysmorphic syndrome may lead to molecular testing now available that was
not available when the child was an infant or when the initial
diagnosis was made.
Ethical considerations for the adolescent should not be
ignored, because potential stigmatization can be a concern
when discussing genetic disorders. In some cases, additional
support may be beneficial, including social workers and
psychologists. It may be difficult for adolescents to understand and incorporate how information about their genetic
makeup can affect their future family. The approach to these
topics should take place in an environment where psychological counseling or other support is available, if needed.
1. All patients should have a targeted 3-generation
family history that includes individuals with CHD,
other malformations, pregnancy loss, and developmental delay (Class I; Level of Evidence C).
2. Genetic evaluation with cytogenetic and/or molecular testing should be performed if indicated (Class I;
Level of Evidence C).
3. Recurrence risk and reproductive counseling, including prenatal screening and/or diagnostic op-
tions, should be presented to adolescent patients who
are pregnant (Class I; Level of Evidence C).
4. Counseling should be individualized, with consideration of the adolescent patient’s emotional maturity
and potential impact of counseling (Class I; Level of
Evidence C).
Sexuality, Pregnancy, and Reproductive Issues
Although the primary responsibility for discussion of sexuality and reproductive issues should fall to the primary care
physician, the cardiologist may have more frequent visits
with certain patients, especially those with complex lesions,
who require frequent monitoring. Therefore, the cardiologist’s awareness of these important issues is critical to
patients’ well-being. Although some studies have shown that
adolescents with CHD are less likely to be engaged in sexual
behavior,48,49 they may be taking greater risks because of a
lower level of knowledge about safe practices, with a lower
prevalence of contraceptive use than among their counterparts.103 It may be that their parents are more reluctant to
discuss issues of sexuality and reproduction,104 and as a
result, these patients may enter adulthood with many misconceptions and fears about their sexuality and their ability to
conceive and bear children.104,105 Therefore, it is important
for transitional care programs to initiate routine sexual health
risk screening and preventive counseling that includes topics
such as sexuality, contraception, and reproduction for adolescents with CHD.
Adolescent Sexual Activity
The majority of teenagers report having had sexual encounters by mid to late adolescence.106 The National Survey of
Family Growth provided data that indicated ⬇47% of female
teenagers and ⬇46% of male teenagers had engaged in sexual
intercourse at least once.107 Approximately one fourth of all
youth reported having had intercourse by 15 years of age,107
with the average age for first sexual intercourse encounter
being 17 years for girls and 16 years for boys.108,109 One study
reported higher rates of early sexual encounters among youth
with chronic conditions than among those in a comparison
group.110 Emotional distress and depression, which are more
prevalent in adolescents with chronic disease, constitute a
recognized risk factor for engaging in high-risk behaviors104,111; however, these studies were not specific to patients
with CHD. Although the importance of addressing sexual
practices, contraception, and pregnancy has long been recognized, little is known about the sexual and contraceptive
practices of adolescents with CHD.4 In 1 study of older teens
16 to 20 years of age, however, the overall rates of sexual
activity were lower than in a comparison sample. These lower
rates were attributed to delays in psychosocial development
reported in these patients.112 Among sexually active patients,
72% of the 16-to-18-year-olds were likely to have engaged in
other risky sexual behavior, such as having had ⱖ2 partners
in a 3-month period, using questionable birth control, or using
drugs or alcohol before sex.49
Despite a trend for increased use of some form of birth
control among teens at the time of first intercourse, 50% of
Sable et al
Best Transition Practices for Adolescents With CHD
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adolescent pregnancies still occurred within the first 6 months
of the first sexual encounter.109 The most common method of
contraception among female teens is the “pill.” Today, oral
contraceptives contain a combination of a low dose of
estrogen (20 to 35 ␮g) with varying amounts of progestin.
The low doses of estrogen in the various forms of contraceptives have less effect on platelet activation and clotting than
the higher doses of estrogen used previously.113 However,
because of the risk of thromboembolic complications,
estrogen-based oral contraceptives are considered contraindicated in females who are cyanotic, those with right-to-left
shunts, and those with pulmonary vascular disease. Oral
contraceptives are relatively contraindicated in patients who
have prosthetic valves or conduits.114
Progestin-only contraceptives inhibit ovulation and have
no measurable impact on clot formation or platelet function,
thus offering an acceptable alternative to estrogen-based
contraception. Some of the progestin-related side effects
include breakthrough vaginal bleeding, amenorrhea, and a
diuretic response.113,115 The only oral form available in the
United States is the “minipill,” but because of its short
half-life, it must be taken at the same time each day, which
makes it a poor choice for many adolescents. A backup form
of contraception must be used for 48 hours if a pill is missed.
Depot medroxyprogesterone acetate (DMPA), known as
Depo-Provera, is a long-acting progesterone-only injectable
that is administered every 12 weeks; it is a preferred method
of artificial birth control in adolescents for whom compliance
is a concern. Unfortunately, the associated side effects
(breakthrough bleeding, weight gain, and headache), most of
which occur in the first year, contribute to a high discontinuation rate (60%) among teens.116 Additionally, the risk of
osteoporosis associated with amenorrhea with long-term use
is of concern.117,118 Although there are no specific guidelines
for teens, it is generally recommended that females who use
this method for ⬎2 years or who smoke or are extremely thin
should be monitored regularly for bone density loss and be
prescribed calcium supplements (1200 to 1500 mg/d). The
contraceptive implant Implanon is a favorable choice for
teens with compliance issues or for patients for whom
pregnancy is contraindicated but who are not yet ready to
undergo permanent sterilization. Implanon consists of a
single small plastic rod that is effective for 3 years and has
lower risk with insertion.119
Although an effective contraceptive method, the intrauterine device may not be considered an appropriate first choice
for adolescents, primarily because of the risk of sexually
transmitted disease.120,121 With proper counseling, however,
this may be a viable method in selected patients. Barrier
methods have the highest failure rate because of inconsistency of use and the discomfort young people may feel when
applying the device. The diaphragm is the most commonly
recommended barrier method of female contraception in
adolescents. Used with spermicide, it is reported to be 80%
effective.122,123 Regardless of the contraceptive method selected, the addition of a male condom with spermicide must
be stressed to ensure protection against sexually transmitted
diseases, HIV, and hepatitis B.
Emergency contraception is used to reduce the risk of
pregnancy after unprotected vaginal intercourse. The 2 most
commonly used methods are the combined estrogenprogestin (Preven) pill, referred to as the Yuzpe regimen, and
the progestin-only regimens of oral contraceptive pills, referred to as Plan B.124 –126 Both consist of 2 doses of
contraceptive steroids taken 12 hours apart. They can reduce
the risk of pregnancy up to 120 hours after unprotected
vaginal intercourse but are more effective when the first dose
is taken within 72 hours. Plan B, which is available from
drugstores without a prescription for females who are ⱖ17
years of age, is the preferred method for patients at risk for
thromboembolic complications.
Because of potential emotional consequences, permanent
sterilization is generally not recommended for adolescents.
For adolescent females, it is preferable to postpone tubal
ligation until they are sufficiently mature to participate in the
decision. When there is a decision to proceed with permanent
sterilization, a transcervical procedure that uses coils that lead
to fallopian tube occlusion is now available.127–129 Clinical
trials of this device report the fallopian tubes are closed
within 3 to 6 months, and thus, additional contraception is
required early after the procedure.
Regardless of an adolescent’s previous sexual history,
abstinence can always be presented as an option. Because
peer pressure plays a key role in the onset of sexual activity,
adolescents need to be encouraged to avoid engaging in sex to
please others.
Selecting a Contraceptive Method
The selection of a contraceptive method for the adolescent
female with CHD must be individualized by taking into
account the primary cardiac defect, any related surgical
intervention and medication, postoperative residua and sequelae, and the adolescent’s maturity. The World Health
Organization has recommended that females who have undergone successful surgical repair for simple lesions such as
atrial septal defects, patent ductus arteriosus, and ventricular
septal defects with no sequelae have no restrictions in their
choice of contraception. Patients with lesions for which
estrogen-based contraceptives are contraindicated are outlined in Table 13.114
Adolescent pregnancy is considered high risk from psychological, medical, and sociological perspectives.130,131 Psychologically, pregnancy interrupts normal developmental tasks
by forcing teens to assume adult responsibilities before they
are prepared to do so. Adolescents are at higher risk for
complications of pregnancy because they frequently fail to
seek early prenatal care and are often noncompliant with
medical recommendations, rendering them at risk for anemia,
pregnancy-induced hypertension, preterm births, and lowbirth weight infants.132 For the adolescent with CHD, there is
the additional maternal and fetal risk posed by her cardiac
condition. The adolescent should be counseled about the
teratogenic effect of medications. Social issues such as access
to care, health coverage, and ambivalence about informing their
parents create barriers to early prenatal health care. Transitional
care programs for adolescents should emphasize the importance
Table 13.
April 5, 2011
Recommendations for the Use of Combined Hormonal Contraceptives in Women With Cardiac Disease
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Always Useable
Condition with no
restriction for the use of the
contraceptive method
Broadly Useable
Condition for which the advantages of
the method generally outweigh the
theoretical or proven risks
Caution in Use
Condition for which the theoretical or
proven risks usually outweigh the
advantages of using the method
Do Not Use
Condition represents an
unacceptable health risk if the
contraceptive method is used
● MVP with trivial MR
● Bicuspid aortic valve with
normal function
● Mild PS
● Repaired coarctation with
no hypertension or
● Simple congenital lesions
successfully repaired in
childhood and with no
sequelae (atrial or
ventricular septal defect,
patent ductus arteriosus,
or total anomalous
pulmonary venous
● Most arrhythmias other than atrial
fibrillation or flutter
● Uncomplicated mild native mitral
and aortic valve disease
● Tissue prosthetic valve lacking any
of the features noted in WHO 3 or 4
● Surgically corrected CHD lacking
any of the features noted in WHO 3
or 4 columns
● Small left-to-right shunts not
reversible with physiological
maneuvers (ie, small ventricular
septal defect, small patent ductus
● HCM lacking any WHO III or IV
● Past cardiomyopathy, fully
recovered, including peripartum
● Uncomplicated Marfan syndrome
● Small left-to-right shunt not
reversible with physiological
● Atrial fibrillation or flutter on warfarin
● Bileaflet mechanical valves in the
mitral or aortic position taking
● Atrial septal defect with left-to-right
shunt that may reverse with
physiological stress (eg, Valsalva
● Repaired coarctation with aneurysm
and/or hypertension
● Marfan syndrome with aortic dilation
● Previous thromboembolism
● Atrial fibrillation or flutter, if not
● Bjork-Shiley or Starr-Edwards
valves even taking warfarin
● Dilated left atrium ⬎4 cm
● Fontan heart even taking
● Cyanotic heart disease even
taking warfarin
● Pulmonary arteriovenous
● Past thromboembolic event
(venous or arterial) not taking
● Prior LV dysfunction due to any
cause (eg, dilated
cardiomyopathy), LVEF ⬍30%
● Coronary artery disease
● Coronary arteritis (eg, Kawasaki
disease with coronary
WHO indicates World Health Organization; MVP, mitral valve prolapse; MR, mitral regurgitation; CHD, congenital heart disease; PS, pulmonary stenosis; HCM,
hypertrophic cardiomyopathy; LV, left ventricular; and LVEF, left ventricular ejection fraction.
Reprinted from Silversides et al114 with kind permission from Springer Science⫹Business Media, © 2009.
of early counseling and medical evaluation should an unplanned pregnancy occur despite guidance and education on
For the pregnant adolescent, the laws about parental notification and parental consent for termination vary by state. In several
states, a pregnant adolescent is considered an emancipated adult,
requiring no parental consent to terminate the pregnancy.
Knowledge of the state laws governing a minor’s rights is an
important component of the transitional program.
Medical termination is not without its own risks, particularly for the patient with complex CHD. It is therefore
important to act swiftly, because the choice of termination
procedure is determined by the duration of the pregnancy.128
The emotional impact of pregnancy termination should not be
underestimated, and appropriate personnel should be available for counseling, which must be offered in a supportive,
nonthreatening environment. For the adolescent who chooses
to continue the pregnancy, a well-coordinated pregnancy and
delivery plan must be developed, one that includes not only
the obstetrician and cardiologist but clinical social work and
psychological services personnel to assist and support the
patient in making the decision about keeping the baby or
placing the baby into adoptive services.
Counseling the Adolescent: Role of the
Healthcare Provider
In addition to the topics of contraception and pregnancy,
discussions with adolescents should stress prevention of sexually
transmitted diseases and HIV. To facilitate these discussions, an
environment must be established that is supportive and nonjudgmental and that establishes a level of trust and confidence.
Additionally, it is important to encourage parents to discuss
sexuality and contraception in a manner consistent with the
family’s attitudes, values, and beliefs. Timing of these discussions will vary. Health education materials should be available to
preadolescents and their parents. With the onset of puberty, a
comprehensive history should include an assessment of the
patient’s knowledge about sexuality, sexual feelings, and other
high-risk behaviors, including use of alcohol and nonprescription drugs. Additionally, handouts to reinforce safe sex practices
and responsible decision making should be available in the
office or clinic. Once menarche begins, a gynecologic history
should be obtained routinely during cardiac follow-up visits. For
adolescents who are contemplating becoming sexually active or
who demonstrate high-risk behaviors, referral to a contraception
clinic or to gynecologic services should be provided.
1. Introduce age-appropriate reproductive health information on sexual health, contraception, and pregnancy beginning early in adolescence (age 12 years)
(Class I; Level of Evidence C).
2. Healthcare practitioners who are knowledgeable in
both adolescent sexual and reproductive health and
CHD should provide clinic-based teaching and counseling on contraception and pregnancy (Class I;
Level of Evidence C).
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Best Transition Practices for Adolescents With CHD
3. Young teens should be assessed for evidence of
high-risk behaviors that could expose them to sexually transmitted diseases, HIV, and pregnancy, and
this information should be included in the patient’s
history (Class I; Level of Evidence C).
4. Providers should document prescribed interventions to
modify high-risk behavior (Class I; Level of Evidence C).
5. Providers should document prescribed contraception for females and recommended use of condoms
for males (Class I; Level of Evidence C).
6. Comprehensive adolescent reproductive services
that include adolescent gynecology should be provided (Class I; Level of Evidence C).
7. Educational materials and local resources on sexuality and contraception should be provided for adolescents and their parents, and for females with
CHD, the importance of planning all pregnancies
should be discussed (Class I; Level of Evidence C).
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Exercise Prescription/Rehabilitation
Because more CHD patients survive to adulthood, it is also
important to focus on QOL with aging and the onset of adult
comorbid conditions. Exercise is an important component of
QOL and health maintenance. Although studies of functional
capacity have not been undertaken on a large, multicenter
scale, a number of smaller studies have been performed. The
ages of patients and methodologies used in these studies vary,
but most report benefits of exercise and that at least some
level of exercise is safe. In general, they also suggest that
exercise tolerance is not likely to be “normal” for many
patients but that many are also capable of performing some
level of regular exercise. Many children are not encouraged to
exercise because of parental, school, or personal fears about
their underlying heart condition, and thus, the patients do not
exercise much as they enter adulthood. This is a cause for
concern for overall health as they enter adulthood, because a
sedentary lifestyle can contribute to other comorbid conditions such as obesity, coronary artery disease, hypertension,
diabetes mellitus, dyslipidemia, and osteoporosis.
Exercise Assessment and Benefits
Exercise is beneficial in overall health for prevention of
adult-onset diseases, as well as in recovery from chronic
conditions, including coronary artery disease and congestive
heart failure.133 Despite the potential benefits of exercise, it is
often not discussed as part of a routine clinic visit. In a study
of 99 adults with CHD,134 exercise was discussed by the
physician in only 28 cases, and discussions generally focused
on exercises to avoid rather than encouraging appropriate
activity. On the other hand, 44% of patients thought all
exercise was safe. The most common barriers to exercise
described by patients included symptoms and fears.134
In general, exercise in the CHD patient population has been
evaluated by either subjective patient interpretation or objective exercise testing. Subjective studies usually include questionnaires about QOL or interest in exercise participation.
The objective measures include those assessed by routine
exercise treadmill testing, cardiopulmonary exercise testing
including ventilatory parameters, or a 6-minute walk test.
These tests provide measurable data such as standardized
exercise time, maximal oxygen consumption, or distance
walked, respectively. Adverse outcomes, such as electrocardiographic evidence of ischemia or strain, arrhythmias, oxygen desaturation, and hypotension, can be detected. Although
important for research, this information may also assist in
clinical decision making and risk assessment for sports
participation or may be used as a basis for a recreational or
rehabilitation exercise prescription. The results can also be
reassuring for patients or families who may be afraid to
exercise because of their CHD.
Regular exercise among adolescents and young adults
with CHD has been evaluated in small, single-center
analyses. Small studies of pediatric and adult patients with
CHD have shown that exercise or exercise training programs may improve functional capacity and some measures of QOL. In a study by Gratz and colleagues,135
adolescent and adult CHD patients (14 to 73 years old)
reported decreased QOL scores in physical function and
general health domains compared with peers, but they did
not show decreased scores in psychosocial aspects. Interestingly, they also significantly overestimated actual exercise capacity based on exercise test results. So, although
these CHD patients may have limitations, it did not appear
to affect their psychosocial function or estimation of their
own exercise capacity. In another study, structural and
pathophysiological diagnoses were related to exercise
ratings, but they were poorly predictive of exercise capacity; thus, other factors likely play a role.136
Short-term exercise training programs have been evaluated. Although exercise training or rehabilitation programs
for children and adults with CHD are thought to be safe
and likely to be effective at improving exercise parameters,
the results of small studies of exercise training in children
and adults with CHD have shown mixed results. One study
using home exercise and dyspnea thresholds in children
did not show any improvement.137 Another study evaluated
a 12-week cardiac rehabilitation program in a small
number of teenagers with complex heart disease. Even in
this short time, improvements in peak oxygen consumption
and heart rate recovery were noted.138 Fredriksen and
colleagues139 showed an increase in peak oxygen consumption and improvement in psychosocial measures after
exercise training, including decreased social withdrawal
and decreased somatic complaints. Finally, another study
that evaluated a 3-month exercise training program in adult
patients with repaired tetralogy of Fallot also found an
increase in peak oxygen consumption.140
Developing good exercise habits in childhood can provide
lifelong benefits. The health benefits are clear in terms of
decreased risk of obesity and adult-onset cardiovascular
disease. There also may be benefits in prevention of other
conditions, such as osteoporosis, with the use of mild weightbearing exercise. It is known that obesity is already a problem
in children and young adults in the United States but also
specifically in CHD patients. Adolescent lifestyle habits,
including exercise, related to atherosclerosis risk were assessed in children with CHD in Belgium. They found that
only 15% spent 30 minutes per day in physical activity.141
Although adolescents with CHD may not practice perfect
health-promotion behaviors, one study from Taiwan showed
Table 14.
April 5, 2011
Classification of Sports147
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MVC indicates maximum ventilatory capacity; Max O2, maximum oxygen consumption.
*Danger of bodily collision.
†Increased risk if syncope occurs. Reprinted with permission from American College of Cardiology Foundation.147
that they are no different from their peers in that country. The
consequences, however, may be greater for the CHD patients,
which makes lifestyle choices even more important.142 Physical activity was also assessed in adults with CHD in the
United Kingdom. Only those in New York Heart Association
class I performed similar activity to a sedentary reference
population, whereas groups in New York Heart Association
class II or III exercised less than half as much as those in New
York Heart Association class I. Despite this, patients reported
a desire to participate in exercise.143
In any exercise endeavor, it is important that the adolescent
CHD patient receive support to succeed and develop good
lifelong habits. A more sedentary lifestyle may contribute to
decreased exercise tolerance, and this lifestyle in children
may be a result of overprotective parents and teachers.144 In
another study that evaluated perceptions about exercise in a
small group of children with CHD, sports and exercise were
thought to be beneficial but not valued. The low self-efficacy
in this study appeared to be influenced by fear and exclusion.145 Interestingly, a study of teens 12 to 18 years of age
showed that belief in self-efficacy was more important than
severity of CHD in determining exercise or sports participation. The self-efficacy and attitude of the parent toward
exercise participation was significantly influenced by the
recommendation of the cardiologist.146 To encourage physical activity habits, it appears to be important for children and
teens to receive appropriate direction and encouragement
from the family and cardiologist, as well as coaches, teammates, and friends.
Type of Exercise
The majority of available information and opinion leans
toward the benefits of exercise for children and adolescent
CHD patients; however, determining the appropriate types
and levels of exercise remains very important. When determining appropriate and safe types of exercise, many recreational activities, such as walking, swimming, or biking to
various levels of exertion, may be appropriate for many
patients. Others may want to participate in higher-level or
more competitive sports, including at the high school and
college level. For this determination, the use of exercise
testing can be beneficial to help gauge safety and level of
exercise individually. To improve this decision-making process, the AHA has published 2 sets of guidelines about sports
participation and involving patients with congenital or genetic heart disease. The 36th Bethesda Conference recommendations outline different types of CHD and repairs and
discuss different types of competitive athletics that may or
may not be appropriate.96 This document also outlines the
differences between static and dynamic types of sports to help
determine what may be more appropriate for patients with
different types of CHD (Table 14).147 The AHA developed a
second statement on exercise participation for patients with
genetic cardiovascular diseases, including Marfan syndrome
and hypertrophic cardiomyopathy. These reports can be
helpful for both physicians and patients to guide activities to
achieve exercise in a safe and comfortable fashion.96
Although most CHD patients can perform some form of
exercise or sports and should be encouraged to do so, there
are those who will require even further consideration. All
CHD patients should be considered individually when it
comes to discussing exercise and exercise programs because
of the variability even within types of CHD. However, those
with heart rhythm devices, dilated aortas, and coronary
anomalies deserve special attention. Those with pacemakers
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Best Transition Practices for Adolescents With CHD
or implantable cardioverter defibrillators may have restrictions as to their maximal heart rates. They also may need to
avoid contact sports or have special equipment to protect the
device during activity. Those with dilated aortas or connective tissue disease of the aorta also may need to avoid contact
sports or high static activity.
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1. Given the general benefits of exercise for overall
health and prevention of obesity, at least some form
of routine exercise is advisable for most CHD patients (Class I; Level of Evidence C). Early activity
habits will also likely enlist the understanding, support,
and encouragement of parents, teachers, and coaches at
2. Objective exercise testing should be performed as a
baseline in all CHD patients to provide an exercise
prescription (Class I; Level of Evidence C). Periodic
repeat testing is advised in the presence of new
symptoms or other changes in the clinical status or
to assess the hemodynamic or arrhythmic response
to medication (Class I; Level of Evidence C).
3. On the basis of the results of objective testing, the
cardiologist should discuss exercise and physical
activity with the patient and family as early in
childhood as possible to encourage good habits
(Class I; Level of Evidence C). The level of recommended exercise should be reassessed during
follow-up and revised according to the patient’s
clinical status and the results of repeat testing (Class
I; Level of Evidence C).
4. The exercise prescription should include the type of
exercise (dynamic or static), heart rate goals and limits
(maximum rate if applicable), duration and frequency
of exercise, and a guide for advancing the level of
exercise (Class I; Level of Evidence C). The prescription
should also include exercise to avoid. The exercise
prescription should follow previously published guidelines, recognizing, however, that most of these guidelines pertain to competitive sports at a high school and
college level (Class I; Level of Evidence C).
5. For patients with limited exercise ability, adverse
hemodynamic response to exercise, or recent surgery, a medically supervised cardiac rehabilitation
program may be beneficial (Class IIb; Level of
Evidence C).
Education and Career Choices
An important developmental task for adolescents with CHD
is to assume more responsibility for their own health care;
however, studies have shown that adolescents and young
adults have a poor understanding of their cardiac disease,
treatment, and prognosis. Veldtman et al148 reported that only
22% of 7-to-18-year-olds (mean age 13 years) with CHD
interviewed could correctly identify their diagnosis by name,
and this was unrelated to age, sex, or severity of the defect. In
addition, only 30% had a good conceptual understanding of
their disease, with 36% having a wrong or poor understanding
of their illness. Moons and colleagues149 found that although
80% of patients 18 to 46 years of age (mean age 23 years)
with CHD were able to correctly identify treatment plans,
frequency of follow-up, proper dental practices, appropriate
vocational choices, pregnancy risks, and the appropriate use
of the contraceptive pill (women), only 50% to 80% were
able to correctly identify the name and anatomy of their
cardiac lesion; the risks, signs, and symptoms of endocarditis;
the genetic risks of CHD; the impact of smoking and alcohol
on their heart disease; and the appropriateness of the intrauterine device as a method of contraception (women). Similarly, Dore and colleagues150 reported that of 104 patients
with CHD (16 to 72 years of age) enrolled in their study, the
clinical diagnosis was unknown by 34.6% of patients, many
of whom had complex CHD. More importantly, although
79% knew about the need for antibiotic prophylaxis, 50% did
not know why it was needed.150
Rönning et al151 used semistructured open-ended interviews to ask 16 adults (19 to 55 years of age, mean age 28
years) about their experiences in learning about their heart
disease. The majority of subjects wished that they had
received developmentally appropriate education sessions beginning in early childhood, with added information and
continuing reinforcement through adolescence.151 Although
few could specifically describe what information should be
provided, the subjects did identify the lack of knowledge
about their heart disease as a barrier to taking a more active
role in decisions about their treatment and care.
Thus, it is common for adolescents and young adults to
have significant gaps in their knowledge about CHD, and
these deficits must be remedied before we can expect adolescents to become more independent regarding their health
care and more responsible for their long-term follow-up.
Neurodevelopmental Issues
Academic achievement, learning disabilities, behavioral
problems, and inattention/hyperactivity and other psychological issues must be taken into account when planning for
transition, because they can all significantly impact an adolescent’s ability to learn and assume responsibility for their
health care.
Improvements in the longevity and survival rates for
patients with CHD have been accompanied by an increasing
awareness of adverse neurodevelopmental outcomes for patients who required cardiac surgery as neonates or young
infants. Children with complex CHD such as d-transposition
of the great vessels, total anomalous pulmonary venous
return, hypoplastic left heart syndrome, and other functional
single-ventricle lesions have a significantly higher incidence
of problems with academic performance, behavioral abnormalities, inattention, hyperactivity, and the ability to perform
higher-order executive functions such as visual-motor
Shillingford and colleagues,153 in a study on inattention,
hyperactivity, and school performance, found that the number
of subjects in their study with high risk scores for inattention
and hyperactivity was 3 to 4 times higher than that in the
general population and that 49% of the 109 enrolled subjects
were receiving some type of remedial academic services.
Although early research focused on modifying intraoperative
technical and procedural factors to prevent and reduce neurological injury, such efforts have resulted in only moderate
success. More recent research has pointed to patient-specific
April 5, 2011
factors such as low birth weight, age at surgery, length of stay
in the intensive care unit, and socioeconomic status as
stronger determinants of adverse neurodevelopmental
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Education and Employment
Education and employment are crucial to financial security
and psychological well-being and therefore represent another
important concern for these adolescents. Although educational milestones are similar to those of healthy peers,
research has shown a significant difference with regard to
employment rate. Simko et al156 found full-time or part-time
employment in 71% of adults with CHD compared with 84%
of healthy subjects (P⫽0.011). The rate of employment tends
to be higher among those with milder and acyanotic forms of
CHD.156 –158
This high rate of unemployment for adults with CHD is
concerning. One possible explanation for this high unemployment rate is the misunderstanding and lack of knowledge
about CHD by employers. In a small survey of employers in
the United Kingdom, most (10 of 14, or 71%) said they would
employ these patients on the basis of current functional
assessment, with or without medical opinion, but 4 (29%) of
14 would exclude patients with CHD or would look at the
individual’s future health prospects before hiring them. Unfortunately, no consensus about hiring practices exists among
employers who hire these young adults, and no actuarial
survival statistics are available to help them with their
decision-making process.159 When job participation was evaluated, the patients with complex CHD had significantly more
job handicaps (primarily mobility related) than patients with
mild CHD. Those with complex CHD also tended to cease
employment because of physical problems/demands, emotional problems, or problems with transportation, and the
most frequent requests for adaptations to the work environment focused on more flexible work hours, reduced time
pressure, and increased freedom to organize one’s work.157
These findings underscore the importance of career counseling in these patients to help them find the best fit for their
physical and emotional capabilities.
Career Counseling
Numerous challenges await adolescents with CHD as they
prepare to enter the workforce. These include discrimination
in the workplace, difficulty in maintaining employment
during medical crises, and challenges in maintaining or
changing employment because of changes in functional
capacity.157 Career and education plans should be discussed
in early adolescence (13 to 15 years of age) to allow for
assessment of the patient’s mental, physical, and social
abilities.4 This assessment and education should involve not
only the healthcare provider and patient but also parents,
educators, and career advisors. The receipt of structured
career/employment advice has been shown to be associated
with a higher rate of employment (73%) than no career/
employment counseling (46%).158
Discrimination is prohibited in all aspects of the work
process, including hiring, advancement, compensation, and
job training. It is unlawful for the employer to question an
applicant about a disability or require a medical examination
unless the information is necessary for specific job-related
performance. Employers are also required, barring creation of
a substantial hardship, to accommodate and modify equipment and training policies to allow a disabled worker to
perform his or her job.160 The US Department of Education’s
“Pocket Guide to Federal Help for Individuals With Disabilities is a good resource for information on benefits and
There are 3 governmental legislative acts that protect
patients with disabilities with regard to seeking employment:
The Rehabilitation Act of 1973 and the subsequent amendment in 1978 prohibit employment discrimination based on
disability by any federal employer programs that receive
federal funding, federal executive agencies, and the US
Postal Service. It also requires that these agencies develop
plans for hiring, placement, and education/advancement
through vocational training.162
The second-injury section in worker’s compensation allows an employee to register a preexisting disability with
the local worker’s compensation facility. This allows the
disabilities or progression of disability to be reimbursed
from a special second-injury fund to ensure that employers
are protected from future losses.
The Americans With Disabilities Act is the most comprehensive legislation for individuals with disabilities. It
defines disability and extends the scope of protection for
these individuals to the private sector. It prohibits
disability-based discrimination by any employer who has
ⱖ15 employees for each working day in each of ⱖ20
calendar weeks in the current or preceding calender year.163
Parents and patients should be educated about any possible
restrictions that might affect ability to work that may develop
with age. This education could allow the adolescent to select
a career in which full employment can be maintained
throughout a lifetime. Motivation does not appear to be a
problem with patients with CHD, because more of them
proceed to higher levels of education than control subjects.158
Higher levels of education lead to higher levels of job
In 1986, the AHA published “Recreational and Occupational Recommendations for Young Patients With Heart
Disease” that can be used to objectively define and assist with
occupational counseling for patients with CHD (Tables 15
and 16).164 Occupational activities should also be assessed for
any additional patient-specific risks. For example, isometric
exercise increases systemic blood pressure, and activities
involving this type of work should be discouraged in patients
with left-sided heart lesions such as coarctation of the aorta,
aortic stenosis, bicuspid aortic valve, and aortic regurgitation.
Patients with left ventricular outflow tract obstructive lesions
and ventricular arrhythmias have an increased risk of dizziness and syncope, which may pose grave risks for certain
jobs, such as piloting a plane or boat, driving a bus or other
public vehicle, or working on high-rise construction projects.
A list of occupations and their physical demands is provided
by the US Department of Labor.165 Objective measures of
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Best Transition Practices for Adolescents With CHD
Table 15. Classification of Occupational Activity, for Use With
Occupational Recommendations164
Category 1
Very heavy work: Peak load of ⱖ7.8 cal/min. Involves lifting objects in
excess of 102 lb with frequent lifting or carrying of objects weighing
ⱖ50 lb.
Category II
Heavy work: Peak load of ⱖ7.8 cal/min. Involves lifting 100-lb maximum
and frequent lifting or carrying of objects weighing up to 50 lb.
Category III
Medium work: Peak load of 5–7.5 cal/min. Involves lifting 50-lb maximum
with frequent lifting or carrying of objects weighing up to 25 lb.
Category IV
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Light work: Peak load of 2.6 – 4.9 cal/min. Involves lifting 20-lb
maximum, with frequent lifting and carrying of objects weighing up to 10
lb. Although the weight may be negligible, a job is also in this category if
it requires considerable walking or standing, or if it involves sitting most
of the time with some pushing and pulling of arm or leg controls.
Category V
Sedentary work: Peak load of ⱕ2.5 cal/min. Involves lifting 10-lb
maximum and occasionally lifting or carrying such articles as dockets,
ledgers, and small tools. Although a sedentary job is defined as one that
involves sitting, a certain amount of walking and standing is often
necessary. Jobs are sedentary if walking and standing are required only
occasionally and other sedentary criteria are met.
cal/min indicates calories per minute.
exercise tolerance should be used to assist in counseling
whenever possible.
Once the occupational activity level, education capacity,
and social ability have been assessed, the highest level of
education should be encouraged. For patients who are handicapped physically or mentally, vocational rehabilitation
should be encouraged. Many states offer vocational educational and rehabilitation services. These vocational services
perform a full assessment of the individual and can suggest
types of employment the individual can be expected to
perform.166 The counselor can also suggest programs that
provide appropriate training, and if need be, the patient can
apply for financial assistance (“Pocket Guide to Federal Help
for Individuals With Disabilities”161).
1. Develop a structured educational/teaching plan
based on the adolescent’s individual academic abilities, educational level, and developmental maturity
(Class I; Level of Evidence C).
2. Begin counseling early in adolescence to identify
interests, as well as the need for additional educational or vocational training and guidance (Class I;
Level of Evidence C). Utilize exercise testing as
appropriate to assist in determining physical abilities (Class I; Level of Evidence C).
3. View every patient as employable, and tailor career/
employment counseling to the adolescent’s physical
capacity, as well as mental and psychological disposition16 (Class I; Level of Evidence C).
Insurance (Health and Life)
A significant proportion of patients with CHD in the United
States experience problems obtaining health insurance or
continuing health insurance coverage after reaching independence in adulthood, which will hopefully be addressed though
healthcare reform.160,167,168 For most children with CHD,
coverage is obtained through government programs or a
parent’s policy. Some patients may continue to have coverage
through the parent’s insurance company if they are students
and/or through Medicaid, State Child Health Insurance Programs, or state Title V Children with Special Health Care
Needs programs until their 21st birthday. Unfortunately, there
is a significant change in healthcare coverage at the very time
that transfer to the adult healthcare system traditionally
occurs. In the general population, the lack of insurance is
highest from 19 to 26 years of age.169,170 Nearly one third of
the population in this age group lacks health insurance. Those
with incomes ⬍200% of the federal poverty level are 2.6
times more likely to be uninsured than those with higher
incomes. Thirty percent of this age group lives below the
federal poverty level. Individuals in their early 20s are less
likely to have full-time employment or to have the type of
employment that offers insurance coverage. Only 28% of the
population from 19 to 26 years of age has access to sponsored
insurance from their employer or spouse’s employment.171
Likewise, only 13% of uninsured adults in this age range are
eligible for Medicaid or other public coverage under current
eligibility rules.171 Callahan and Cooper172 have shown that
uninsured young adults with a chronic condition are 8 times
more likely to have unmet medical needs and 6 times more
likely to have no access to routine care than insured young
adults. The likelihood of an unmet need due to cost among
those with a disability was 35% in this population. Thus,
poverty and presence of a disability are among the factors that
negatively influence healthcare coverage of young adults
with CHD at the most vulnerable time of transition to the
adult healthcare system.
Another explanation for lower insurance coverage relates
to attitudinal differences.173 Young adults may be less risk
averse than older adults, and the maturation of planning/
executive skills may be incomplete in the early 20s.174 At
particular risk are those patients who are not symptomatic or
disabled but have cardiac pathology that may progress to
permanent disability if not managed proactively. Some patients have elected to avoid clinic visits, cardiac catheterizations, or operations because of the personal financial
Late in adolescence, a transition must be made to ensure
coverage of cardiac and noncardiac care.175 Healthcare professionals should address the issue of insurability before
patients with CHD leave their parent’s policy or lose their
eligibility for children’s services. In fact, evidence points to
very early intervention (3 months before birth to kindergarten) having a positive effect on the likelihood of the individual in the urban poor population having insurance as a young
adult.176 This is consistent with previous long-term studies
that have shown a positive effect of early education on later
employment and health.177 Thus, preparation for successful
transition to adult health care should begin at first contact
with preparation of the family, early assessment of educational needs, and the initiation of appropriate interventions.
Vocational or career counseling of adolescents should con-
April 5, 2011
Table 16. Occupational Recommendations for Young Patients
With CHD
Aortic insufficiency
Table 16.
Ventricular septal defect
Mild to moderate PVOD
Moderate to severe PVOD
Other major defects
Other major defects
Aortic stenosis
(unoperated or palliated only)
Atrial septal defect
(postoperative intracardiac repair)
Cardiac arrhythmias
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Complete heart block
Pacemaker (artificial)
Mild to moderate PVOD
Premature atrial contractions
Moderate PVOD
Premature ventricular contractions with normal heart
Congenital or acquired heart disease
Congestive (dilated)
Supraventricular tachycardia
Ventricular tachycardia with normal heart
Congenital or acquired heart disease
Coarctation of aorta
Operated, normal BP
Wolff-Parkinson-White syndrome
CHD indicates congenital heart disease; PVOD, pulmonary vascular occlusive
disease; BP, blood pressure; ⫹/⫺, with or without; PA, pulmonary artery; and
RV, right ventricular.
*For occupational recommendations, the numbers (I, II, III, IV, and V)
correspond to the restrictions given in the categories listed in Table 15.
Unless otherwise noted, all restrictions apply regardless of whether the
patient has had surgery.164
Moderate or severe
Mitral insufficiency
Mild, no cardiomegaly
Severe (⫹/⫺ atrial fibrillation)
Mitral stenosis
Severe (⫹/⫺ atrial fibrillation)
Mitral valve prolapse
Mild, no symptoms
Patent ductus arteriosus
Mild to moderate PVOD
Moderate to severe PVOD
Pulmonary stenosis
Pulmonary hypertension (idiopathic)
PA pressure ⬍0.5 systemic
PA pressure ⬎0.5 systemic
Tetralogy of Fallot, postoperative
RV pressure ⬍50 mm Hg
RV pressure ⬎50 mm Hg or cardiomegaly
sider realistic physical abilities and the educational potential
of the adolescent with CHD. Education or job training should
be encouraged. An important avenue for obtaining insurance
is through group policies available through employers or
professional associations. Larger employers are more likely
to offer full coverage because of the larger size of their risk
pools. Although recent healthcare legislation, the Affordable
Care Act, will mandate that young adults be covered by their
parents’ health insurance until age 26, and the insurance
industry is already moving in this direction, a substantial
number of young adults whose parents do not have access to
employer-based insurance will remain uncovered.
Patients considered totally disabled according to Social Security disability guidelines (⬍10% of patients with CHD) may
apply for healthcare coverage under their state’s Medicaid
program and may be eligible for Supplemental Security
Income.176 It is particularly important to note that the criteria
for Social Security Disability Insurance are markedly different for children and adults with CHD; therefore, these
changes must be thoroughly understood by the patient and
parents well before the patient reaches the age of 18 years. A
comprehensive and up-to-date resource for employment and
insurance is available on the Healthy and Ready to Work
Web site ( A list of resources to assist
patients in confronting insurability issues is also provided by
the Adult Congenital Heart Association (
under information and resources.
Sable et al
Best Transition Practices for Adolescents With CHD
1. Counseling about health insurance issues before
patients with CHD leave their parents’ policy or lose
their eligibility for children’s services, including
information regarding the relationship between education/vocational choices and access to insurance
benefits, may be beneficial to facilitate continued
insurance (Class IIb; Level of Evidence C).
2. Discussion of the relationship between education/
vocational choices and access to insurance benefits
should occur early and guide educational planning
(Class I; Level of Evidence C).
End of Life, Mortality, and Advance Directives
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Long-term survival is an expectation for most individuals
with CHD; however, patients with complex CHD remain at
increased risk for medical complications and early mortality. It is not uncommon, however, for adolescents and
young adults with CHD to hold the misperception that they
have been “cured” or “fixed.”178 –180 For some patients, the
transition period might represent the first occasion when
they are confronted with mortality information. Furthermore, patient expectations are often inconsistent with data
on life expectancy among patients with CHD. Reid and
colleagues47 studied the self-reported life expectancies of
296 patients with moderate to complex CHD between the
ages of 16 and 20 years. Patients with CHD expected to
live to 75 years of age, only 4 years less than they expected
their peers to live; ⬎85% of the patients expected to live
much longer than was estimated by the study authors.47 If
discussions about life expectancy with healthcare providers were routine, developmentally appropriate, and incorporated as part of general discussion about psychosocial
issues, it should help young people have more realistic
expectations about their future.
When an adolescent confronts end-of-life issues, the notion
of a life cut short can be distressing for patients, their
families, and healthcare providers. Freyer noted that for
teenagers facing death, “adolescence is a paradox of emerging capabilities and diminishing possibilities.”181 Although it
is often difficult for healthcare providers to initiate end-of-life
discussions, research suggests that adolescents are often
prepared and willing to discuss these issues.182–184 Health
professionals should consider several factors when discussing
death and end-of-life issues with a young person, including
the patient’s developmental level and decision-making capacity, the patient’s conceptualization of death (including personal values), the family’s understanding of death (including
religious and cultural beliefs), the patient’s and family’s usual
ways of coping, and expectations for the actual death.185–187
These discussions need to be a routine part of care, because
patients’ thinking about death is likely to change markedly
during the developmental period of adolescence and early
Adolescents should be involved in decision-making
about palliative and end-of-life care to an extent that is
commensurate with their developmental level and prior
experience with medical decision making.181,188 Healthcare
professionals should consult the laws of their individual
states or provinces to determine the specific situations in
which adolescents are permitted to provide autonomous
consent for medical decisions. Advance care documents
allow patients to specify their preferences for medical and
palliative care and to identify a legal healthcare decision
maker. As such, they allow for the wishes of an individual
to be heard and respected when that individual is no longer
able to communicate these wishes. Although adolescents’
advance directives might not be legally binding, their
preparation can assist with the determination of the adolescent’s preferences and best interests. The process
should include patient education about the medical condition, testing, and treatment; clinical determination of the
patient’s comprehension of the situation; and solicitation
of the patient’s preferences about proposed care.188 McCabe and colleagues185 present a comprehensive list of
questions for the assessment of adolescents and families to
guide the process of effectively involving adolescents in
medical decision making.
The American Academy of Pediatrics advised that “as
children develop, they should gradually become the primary guardians of personal health and the primary partners
in medical decision-making, assuming responsibility from
their parents.”188 Adolescents will benefit from additional
decision-making support as they manage end-of-life issues. End-of-life decision-making can be particularly difficult for adolescents, who typically have limited experience in making choices of such magnitude. If the wishes of
the adolescent conflict with parental preference, the adolescent may require extra assistance and counseling, particularly if the parents have a history of being overly
protective. Although transition is characterized by a process of increased self-determination, the ongoing support
and participation of parents when confronting difficult
decisions must not be ignored. Many adolescents and
young adults will have siblings who would be affected by
their death. It is critical to attend to the emotional and
practical needs of the entire family during the illness
period, as well as after death. It is also important to start to
address medical power of attorney and long-term care
plans with families of patient with disabilities.
Special Issues for Patients With
Developmental Delay/Disabilities
As healthcare providers for adolescents and adults with CHD,
we often approach our patients with cognitive impairment
and developmental delay differently from those without
obvious disabilities. The assumption is that they are unable to
successfully transition to adult medical services because of
developmental delay, cognitive impairment, or lack of ability
to independently manage their own health care. Many pediatric cardiologists have continued to care for adolescents and
adults with developmental disabilities. A successful model of
healthcare transition for adolescents and adults with CHD
must include provisions for patients with developmental
disabilities and cognitive impairment.4
The goal of transition in health care for adolescents and
adults with special healthcare needs is to maximize lifelong
functioning and potential through the provision of high-
April 5, 2011
quality, developmentally appropriate healthcare services that
continue uninterrupted as the individual moves from adolescence to adulthood.17 To develop appropriate care for patients
with developmental disabilities and CHD, we must be committed to creating individualized transition plans, be flexible
when necessary, and utilize all available resources to achieve
successful transition.
Table 17. American Academy of Pediatrics Recommendation
for Patients With Trisomy 21190
Annual physical examinations and follow-up beyond adolescence include:
Complete blood count
Thyroid function tests
Audiologic and ophthalmologic evaluation
Assessment for symptoms of snoring or obstructive apnea
Practical Implications
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Although the transitional goal for most adolescents and
young adults is to eventually accept responsibility for their
own health care, this goal may not be realistic for some
developmentally delayed patients. ACHD healthcare providers, in conjunction with the primary care physician, must
be willing to work with families, vocational counselors,
and group home supervisors in situations in which an
individual is not able to live and attend to their healthcare
needs independently.
The Individuals with Disabilities Act amendment of 1997
mandated that transition planning would be a mandatory part
of the annual individual education plan for individuals receiving special education services beginning at age 14 years and
continuing through graduation from high school. Adolescents
with developmental disabilities and CHD should receive
anticipatory guidance at each clinic appointment during
adolescence in a manner appropriate for the adolescent’s
cognitive ability and level of function. Ideally, ACHD care
should be a part of the overall healthcare transition plan,
which considers issues such as transportation, community
resources, and the individual’s capacity for self-care.189 A
social worker who is familiar with the guidelines provided by
state and federal programs for individuals with developmental disabilities can assist in the development of the healthcare
transition plan.
ACHD healthcare providers should be familiar with genetic disorders that are commonly associated with both
congenital heart defects and developmental delay. Down
syndrome, Williams syndrome, and 22q11 deletion disorders
(also known as DiGeorge or velocardiofacial syndrome) are
the most common ones associated with moderately complex
congenital heart defects. Developmental delay, learning difficulties, mental retardation, and other multisystem medical
problems are also often present. The American Academy of
Pediatrics published health assessment guidelines for children
with Down syndrome in 2001190 that remain relevant to the
adult population. They provide straightforward recommendations on the frequency of medical surveillance for associated
health issues at various ages (Table 17). The ACHD healthcare provider should be familiar with these guidelines because the additional health issues associated with genetic
disorders can impact the health of their patients and may interact
with their patients’ congenital heart disorders. For example,
obstructive sleep apnea in an obese adult with Down syndrome
and an unrepaired ventricular septal defect may lead to worsening hypoxia, pulmonary hypertension, and decompensated polycythemia. Obesity can increase the risk for acquired heart
disease, diabetes mellitus, and hypertension.
Guidelines for healthcare supervision of children with
Down syndrome190 and Williams syndrome191 can be ac-
Assessment of skin problems
Anticipatory guidance issues to address at each clinic appointment include
Diet and exercise issues to maintain appropriate weight
School and vocational/work issues
Issues related to group home setting or independent living issues
Issues related to family relationships, financial planning, and guardianship
cessed through the American Academy of Pediatrics Web site
at Adolescents with
22q11 deletion syndromes often have learning disabilities,
developmental delay, and difficulty with speech secondary to
a velopharyngeal incompetence, cleft palate, and hearing loss.
It is important that patients with CHD, developmental delay,
and associated clinical features suggestive of DiGeorge or
velocardiofacial syndrome undergo fluorescent in situ hybridization analysis to confirm the diagnosis and for future
genetic and reproductive counseling.101
The Transition Clinic
Key Elements of the Transition Process
Regardless of the model of the transition program, there are
fundamental principles of transition that have achieved nearly
universal endorsement.10,12,175 These principles provide a
framework for both individual programs and institutions
whose goal is to improve the transition experience for young
adult patients with chronic health conditions. These principles
are well described in the context of a model that incorporates
3 phases of the transition process: Pretransition, transition,
and transfer.
Although a formal transition curriculum is most appropriately
initiated during adolescence, the concept of transition should
be introduced much earlier and continued throughout childhood. As youngsters, patients must be counseled that although they may have a normal or near-normal life, they do
not have a normal heart, and they will require lifelong
surveillance. Furthermore, they must be taught that CHD is
vastly different from other forms of heart disease, which
makes it imperative that they follow up with providers
specializing in ACHD.
The transition process is a family process; the family plays
a critical role that must be supported and encouraged. In this
pretransition phase, families should have the expectation that
their children will become independent in managing their
own medical care by young adulthood. Families should be
encouraged to help their children reach this goal.12,149,192
Ideally, using such a strategy of early preparation, the patient
and family will enter the transition period with an understand-
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Best Transition Practices for Adolescents With CHD
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ing of the patient’s heart disease and need for lifelong
specialized care.
One model for pretransition counseling uses the transition
program advanced practice nurse or physician assistant in this
educational role. This practitioner can meet the youngster at
ⱖ1 visits in the pediatric cardiology clinic. During these
visits, this practitioner can introduce the concept of transition
and transfer, discuss diet and exercise, address high-risk
behaviors, discuss contraception and pregnancy, and discuss
future educational and vocational goals. This same practitioner can provide continuity of care by following the young
adult through the formal transition program and ultimately
into the ACHD program. An alternative model uses the
pediatric cardiologist in this educational role. In addition,
during this pretransition phase, the transition team can coordinate preteen and parent workshops to introduce the concepts of transition and provide a forum in which families can
share their fears and concerns with the process.
When patients are developmentally mature enough, they
should enter the formal transition program and complete a
transition curriculum. This transition curriculum should ensure that these young people understand their diagnosis and
medical history. Ideally, they must understand the normal
heart (basic anatomy and physiology) and how the hearts they
were born with differ from normal. They can begin to
understand the risks of residual hemodynamic burdens and
arrhythmic complications. They should be taught to recognize
important and concerning signs and symptoms. The transitioning adolescent should have a good understanding of the
rationale for previous therapies and options for future medical, surgical, and catheter-based therapies. Finally, they need
to be taught to navigate the adult healthcare system; how to
access an adult congenital heart specialist; how often they
need follow-up; how to access routine health care; how to
access emergency health care; and how to navigate the
insurance process.
A standard core educational curriculum (Table 18) is an
important component of an organized transition process. This
education allows patients to gain control of their health and
serve as their own advocates. Completing the goals of this
curriculum can be accomplished by a variety of educational
modalities and should be tailored to individual patients. The
curriculum should be of appropriate breadth and depth but
concise enough to be completed before patients move into the
ACHD clinic. This may require 2 clinic visits for mature,
well-adjusted patients with mild disease, mild functional
limitations, and a strong support system or many visits with
intense peer support-group involvement for a young person
with learning disabilities, complex disease, significant functional limitations, and no support system. Flexibility is a key
to success.
The example of a transition curriculum outlined in Table
18 includes residual hemodynamic and arrhythmia considerations, noncardiac surgical and medical problems, contraception and pregnancy planning, vocational and insurance planning, and other lifestyle issues. The curriculum goals can be
accomplished in a number of ways. Much of the curriculum
Table 18.
Transition Curriculum Topics
Residual hemodynamic considerations
Hemodynamic issues
Symptoms and how to respond
Diagnostic tools in follow-up
Management options
Arrhythmia considerations
Signs and symptoms
Screening tools
Diagnostic tools
Management options
Endocarditis considerations
Risks, implications, recognition, and response
Contraception and pregnancy planning
Contraceptive options and risks
Risks of pregnancy to mother and to fetus
Management of pregnancy plan
Noncardiac surgery considerations
Location of surgery
Knowledge and skills of surgical team
Noncardiac medical problems
Access to appropriate care
Career, vocational, and insurance planning
Lifestyle issues
Marriage and family planning
Life and health insurance
Learning disabilities
Anxiety and depression
High-risk behaviors
Healthy eating
Physical fitness
Salt and fluid restriction (if warranted)
Relative safety of exercise and hobbies
End-of-life decisions
Skills training
Decision making
Creative problem solving
can be addressed at routinely scheduled clinic visits with the
participation of physicians, advanced practice nurses or
physician assistants, and other support services. In addition,
introductory seminars for patients and families, peer support
groups, and informational seminars can be used to address
some of the components of the curriculum.
April 5, 2011
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Timing of Transfer
Ideally, transfer of care from the pediatric to adult healthcare
system occurs at the successful completion of a thoughtful
transition process. When deciding on the timing of transfer, 2
important points need to be considered: There should be a
policy on timing, and this policy should be flexible.
First, there should be an institutional policy on timing of
transition to ensure that transition and transfer actually occur,
and in a predictable manner. When patients and families have
an explicit target age, they recognize that transition and
transfer will occur, appreciate their active involvement in the
process, and are prepared for the ultimate transfer of care.
Additionally, when transition is the “rule,” young people see
the process not as something they as individuals are forced to
go through alone but rather as a natural process that everyone
goes through; much as high school follows junior high, so
adult care follows pediatric care. Finally, a policy on timing
ensures that all patients have the opportunity to take full
advantage of the transition curriculum.
Second, a transition program must be flexible with respect
to timing and should tailor the process to the developmental
and psychosocial status of each young person.193,194 Transition should be completed only after the young patient has
accomplished the developmental tasks of adolescence and has
demonstrated the ability to manage his or her health care
independent of his or her family and pediatric provider.
Adolescents have many difficult tasks to accomplish before they leave this stage in development. They must begin to
develop a personal sense of identity, which normally occurs
in young adulthood; adjust to this new physical sense of self;
adjust to new intellectual abilities and the increased cognitive
demands placed on them at school and in society; develop
educational and vocational goals and a plan to meet these
goals; establish emotional and psychological independence
from their parents and begin to adopt a personal value system;
learn to manage their sexuality and begin to develop intimate
relationships; and develop increased impulse control and
behavioral maturity. It is important to recognize that chronic
illness, physical disability, and cognitive limitations can
disrupt the usual developmental trajectories. This makes the
accomplishment of such tasks more difficult and thus the
process of transition more challenging.13
Furthermore, the sometimes overprotective attitudes of
pediatric providers and patient families can diminish selfesteem and hinder the development of self-sufficiency. In
general, the adult healthcare system demands a higher level of
personal responsibility and autonomy than the pediatric
system. Thus, to be effective healthcare consumers in the
adult healthcare environment, young people should demonstrate the ability to meet their healthcare needs independent
from their families and pediatric providers before transfer;
make their own appointments, meet independently with their
healthcare providers; administer their own medications and
other treatments; understand their medical history; and recognize signs of clinical deterioration.
Finally, transfer of care during medical crises or periods of
psychosocial disequilibrium should be avoided.12 All too
often, patients are transferred when they become pregnant,
mentally ill, or noncompliant, or after they experience their
first “adult” complication. Transfer during these times precludes maximal utilization of the transition curriculum and
imposes a tremendous psychological burden on the patient.
Adult Provider Services
Although the needs of some patients are relatively straightforward, others have complex medical needs and may require
the involvement of a variety of subspecialty consultants, in
addition to capable primary healthcare providers willing to
coordinate and manage the complex care they need.10 Other
medical and nonmedical professionals critical to successful
transfer include advanced practice nurses or physician assistants, social workers, and those who specialize in vocational
and educational issues.4,7,12
Coordinated Transfer Process
A coordinated transfer process is the final component of a
successful transfer process.11,12,195 A carefully prepared
health summary allows seamless transfer of care and provides
a blueprint for the new healthcare team. It should comprise a
complete medical history that includes diagnoses and previous interventions, a medication list, laboratory values, and
other diagnostic studies, as well as information about the
patient’s functional status, the tempo of disease progression,
and the impact of other comorbidities. Information about
psychosocial concerns, end-of-life preferences, the extent of
family involvement, and adherence issues should also be
communicated if available. A comprehensive summary
avoids the need to reinvent the therapeutic wheel and helps to
prevent errors being made or mistakes being repeated. In
addition to the formal medical summary, it is vital that the
pediatric providers communicate directly with their counterparts in the adult healthcare system. The more integrated
pediatric and adult providers are, the more seamless the
transition process will be.
It is important that the adult providers respect the therapeutic plan that was established by the pediatric providers and
communicated to the young patients and their families. As
explained above, the immediate reevaluation and drastic
change in management that many new adult providers are
tempted to make after care transfer can often be overwhelming to patients and diminish their trust. Regardless, one must
also take advantage of the unique opportunity this transition
process provides to take a fresh look at the patients as
evolving adults and to reassess therapeutic options in the light
of new technologies. This process can be done in conjunction
with the pediatric providers and after a period of introduction
and relationship development in the transition process. In this
way, trust is not lost, and the best possible plan for the future
is made.
Many transition “tools” have been suggested to aid in a
smooth transition and transfer. For example, a central provider,
such as an advanced practice nurse or physician assistant, who
can assume responsibility for the entire process has been shown
to be helpful.11 The coordinator can share membership on both
the pediatric and adult teams and serve as a liaison between
them, as well as serving as a reassuring presence and advocate
for patients and families. In addition, to address the inevitable
Sable et al
Best Transition Practices for Adolescents With CHD
uncertainty felt by young people and their families about
impending transfer, many programs have created a transfer
“package” with information about the adult programs.195 Finally,
patients who have already transferred can play a part in welcoming young people who are transferring their care.
Because increasing numbers of young people with complex congenital illnesses are surviving into adulthood, there is
an urgent need for programs designed to facilitate their
smooth movement from pediatric to adult healthcare environments. It is hoped that in the near future, transition programs
will become the standard of care, making it more likely that
patients with complex chronic illness can achieve their full
potential under appropriate medical surveillance and live
meaningful and productive lives.
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Writing Group Disclosures
Writing Group
Expert Ownership Consultant/Advisory
Witness Interest
Craig Sable
Children’s National
Medical Center
Elyse Foster
University of California,
San Francisco
Karen Uzark
Cincinnati Children’s Hospital
Medical Center
Bjornsen Investment Corp
UCLA School of Nursing
Mayo Clinic
Children’s Hospital, Seattle
Evalve Inc†
Adult Congenital
Heart Association*
University of Virginia*
Cardiology Consultants*
Member of Medical
Advisory Board for
Adult Congenital
Heart Association*
Katherine Bjornsen
Mary M. Canobbio
Heidi M. Connolly
Thomas P. Graham
Michelle Z. Gurvitz
Adrienne Kovacs
Alison Knauth
Graham J. Reid
John G. Reiss
Kenneth N. Rosenbaum
Paul J. Sagerman
Arwa Saidi
Rhonda Schonberg
Sangeeta Shah
Elizabeth Tong
Roberta G.
University of Toronto
University of California,
San Francisco
University of Western Ontario
University of Florida
Children’s National Medical Center
Wake Forest University Baptist
Medical Center
University of Florida
Children’s National Medical Center
Ochsner Medical Center
University of California,
San Francisco
Children’s Hospital Los Angeles
This table represents the relationships of writing group members that may be perceived as actual or reasonably perceived conflicts of interest as reported on the Disclosure
Questionnaire, which all members of the writing group are required to complete and submit. A relationship is considered to be “significant” if (1) the person receives $10 000
or more during any 12-month period, or 5% or more of the person’s gross income; or (2) the person owns 5% or more of the voting stock or share of the entity, or owns
$10 000 or more of the fair market value of the entity. A relationship is considered to be “modest” if it is less than “significant” under the preceding definition.
April 5, 2011
Reviewer Disclosures
Robert W. Battle
Loren Hiratzka
Karen Kuehl
University of Virginia
Bethesda North Hospital
Children’s National Medical Center
Jeffrey Kuvin
Tufts Medical Center
Philip Moons
Katholieke Universiteit Leuven,
Dan Murphy
Stanford University
Gary Webb
Cincinnati Children’s Hospital
This table represents the relationships of reviewers that may be perceived as actual or reasonably perceived conflicts of interest as reported on the Disclosure
Questionnaire, which all reviewers are required to complete and submit. A relationship is considered to be “significant” if (1) the person receives $10 000 or more
during any 12-month period, or 5% or more of the person’s gross income; or (2) the person owns 5% or more of the voting stock or share of the entity, or owns
$10 000 or more of the fair market value of the entity. A relationship is considered to be “modest” if it is less than “significant” under the preceding definition.
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KEY WORDS: AHA Scientific Statements
adolescence 䡲 patient-centered care
heart defects, congenital
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Best Practices in Managing Transition to Adulthood for Adolescents With Congenital
Heart Disease: The Transition Process and Medical and Psychosocial Issues: A Scientific
Statement From the American Heart Association
Craig Sable, Elyse Foster, Karen Uzark, Katherine Bjornsen, Mary M. Canobbio, Heidi M.
Connolly, Thomas P. Graham, Michelle Z. Gurvitz, Adrienne Kovacs, Alison K. Meadows,
Graham J. Reid, John G. Reiss, Kenneth N. Rosenbaum, Paul J. Sagerman, Arwa Saidi, Rhonda
Schonberg, Sangeeta Shah, Elizabeth Tong and Roberta G. Williams
Circulation. published online February 28, 2011;
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