Current Physical Medicine and Rehabilitation Reports (2019) 7:67–80

https://doi.org/10.1007/s40141-019-00216-9

PEDIATRIC REHABILITATION MEDICINE (A HOUTROW AND M FUENTES, SECTION EDITORS)

Pediatric Cardiac Rehabilitation: a Review

Unoma Akamagwuna 1 & Daryaneh Badaly 2

Published online: 7 May 2019

# Springer Science+Business Media, LLC, part of Springer Nature 2019

Abstract
Purpose of Review In addition to concerns with physical health and activity levels, children with cardiac conditions can be at risk
of neurodevelopmental and socioemotional maladjustment. Children with congenital heart defects requiring surgery early in life
are at risk of developmental delays and cognitive impairments, and both children with congenital heart defects and those with
cardiomyopathies are at risk of socioemotional concerns. As a result, there is an increasing focus on rehabilitation efforts for these
patients, in order to improve both their physical well-being and their adjustment outcomes. However, there are no established
standards for rehabilitation programs applicable across children with cardiac conditions, in stark contrast to guidelines for adult
patients. The purpose of the present review is to summarize recent studies on pediatric cardiac rehabilitation and describe the
structure of our own program, in order to aid with the delineation of future guidelines.
Recent Findings Twenty programs for pediatric cardiac rehabilitation were identified and reviewed. We review inpatient, out-
patient, and home-based programs, most of which include two to three sessions of exercise training per week for 12 weeks with a
focus on improving exercise capacity. We also review emerging cognitive rehabilitation for children with cardiac disorders and
discuss a newly developed program at our own institution.
Summary A review of past findings, along with recent efforts at our institution, suggests that a structured cardiac rehabilitation
program can benefit children by increasing exercise response and physical activity as well as improving developmental, cogni-
tive, and psychosocial outcomes.

Keywords Pediatric cardiac rehabilitation . Congenital heart defects . Cardiomyopathies . Heart transplantation .
Neurodevelopmental outcomes . Socioemotional adjustment

Children with cardiac disorders are at risk of neurodevelopmental standards for rehabilitation programs applicable across children
and socioemotional maladjustment [1–4], on top of concerns with cardiac conditions, in stark contrast to guidelines for adults
with their physical health and activity levels. As a result, there [5]. In this review, we summarize recent studies on pediatric
is an increasing focus on rehabilitation efforts for these patients, cardiac rehabilitation and present the structure of our own cen-
in order to improve both their physical well-being and their psy- ter’s program, in order to aid with the delineation of future guide-
chosocial adjustment. However, there are no established lines for pediatric patients.

This article is part of the Topical Collection on Pediatric Rehabilitation

Medicine
Pediatric Cardiac Disorders
* Unoma Akamagwuna
[email protected]; [email protected] Congenital Heart Defects
Daryaneh Badaly Congenital heart defects (CHDs) are one of the most common
[email protected]
birth defects, affecting approximately 1.35 million newborns
1
Department of Physical Medicine and Rehabilitation, University of
worldwide every year [6] and 1 million children of all ages in
Pittsburgh School of Medicine, 4401 Penn Ave., the USA [7]. CHDs encompass a wide range of conditions in
Pittsburgh, PA 15224, USA which there is abnormal development of the heart and/or its
2
Learning and Development Center, Child Mind Institute, 101 E. 56th major blood vessels; they are broadly categorized into cyanot-
St., New York, NY 10022, USA ic defects (where oxygen-rich and oxygen-poor blood mix)
68 Curr Phys Med Rehabil Rep (2019) 7:67–80

and acyanotic defects (where the amount of oxygen delivered emphasis on increasing physical activity and providing rele-
throughout the body is unaffected). A summary of heart de- vant education.
fects is provided in Table 1, with abbreviated details on clin- Children with CHDs are also at increased risk of early devel-
ical presentation and management. opmental delays, later cognitive dysfunction, and poor quality of
Children with CHDs can have comorbid pulmonary and life, including speech and language delays, deficits in attention,
other medical complications hindering their ability to func- executive functioning and visuospatial skills, and emotional and
tion [8, 9], and they are at risk of delays in motor develop- behavioral dysregulation [1, 2]. Neurodevelopmental disruptions
ment and exercise intolerance [10, 11]. Impairments in mo- are thought to result from a combination of brain dysmaturation
tor functioning and exercise tolerance, in turn, have been [14–16] (which likely arises from genetic factors and teratogens
linked with perioperative morbidity and sedentary behaviors that concurrently impact cardiac and cerebral fetal development
(which can have negative health consequences) [12]. Along [17, 18]), altered cerebral perfusion (which can then affect func-
with their physical impairments, children with CHDs are tional and structural brain development) [19], and neurological
more likely to have parents, educators, and health providers injuries (i.e., cerebral hemorrhages, ischemic lesions,
who excessively limit their activity, predisposing them to periventricular leukomalacia, and other white matter injuries)
physical inactivity and exercise intolerance [13]. They may, [20, 21]. The complexity of the heart defect has been related to
therefore, benefit from cardiac rehabilitation with an the severity of neurodevelopmental concerns, with children with

Table 1 Congenital heart defects and their clinical presentation and management

Congenital heart defect % of cases Presentation Management

with CHDs

Acyanotic Defects
Atrial septal defects 7–10 Fatigue, but usually asymptomatic Repaired surgically via patch or suture closure
Ventricular septal 50–60 Symptoms of overcirculation within the Management of CHF, management of
defects pulmonary vasculature, congestive heart nutrition, and ultimately surgical patch
failure (CHF), cardiomegaly on chest radiograph, repair
and evidence of pulmonary vascular congestion
Atrioventricular septal 5 Features of CHF, failure to thrive, cardiomegaly on Nutrition monitoring and management,
defects chest radiograph, and evidence of pulmonary diuretics, and ultimately surgical repair
vascular congestion
Aortic and pulmonic Aortic, 5–8 Systolic ejection murmur Catheter-based balloon valvuloplasty, surgical
valve stenosis Pulmonic, valvuloplasty
8–10
Coarctation of the Coarctation Lower post-ductal saturations, blood pressure Prostaglandin infusion to keep the ductus
aorta and interrupted of the gradients in upper and lower extremities, poor arteriosus open in newborns, surgical repair
aortic arch aorta, feeding, and cardiogenic shock if in crisis
5–8
Interrupted
aortic
arch, 1.5

Cyanotic Defects
Tetralogy of Fallot 5 Hypercyanotic spells with agitation, and fever Surgical management, palliative procedures,
or illness complete repair including closure of
ventricular septal defect and
resection of right ventricle obstruction
Transposition of the 2 Cyanosis in the first 12 h of life, narrow Prostaglandin infusion, surgical repair
great arteries mediastinal silhouette on radiograph
Truncus arteriosus 2–5 Cyanosis symptoms of pulmonary overcirculation Diuresis and fluid management, surgical repair
in the first few hours of life
Total and partial 1 Respiratory distress and cyanosis, radiograph with Surgical repair
anomalous white out of the lung fields and small heart, partial
pulmonary venous anomalous return possible in late childhood and
drainage adolescence
Hypoplastic left heart 2–3 As the ductus arteriosus closes, infants show signs of Multi-stage surgical palliation including
syndrome shock with poor pulses and perfusion, poor urine output, Norwood, Glenn, and Fontan procedures
pulmonary overcirculation, and radiograph with vascular in infancy through 2–4 years of age
congestion and cardiomegaly

Summarized from Puri et al. [91]

Curr Phys Med Rehabil Rep (2019) 7:67–80 69

single ventricle lesions thought to be at particular risk [1]. Even Children with cardiomyopathies, particularly those who
with simpler defects, though, factors such as cardiac arrest [22] progress to heart failure, have an increased risk of anxiety,
and extracorporeal membrane oxygenation (ECMO) [23] can depression, and quality of life concerns [3, 31••]. In addition,
portend increased risk of neurodevelopmental delay. As such, those who require permanent ventricular assist devices or
children with CHDs may benefit from access to habilitation heart transplantation can have mild neurodevelopmental dis-
and rehabilitation services, during acute care following surgical ruptions [4, 32]. Among adults, cardiac rehabilitation pro-
interventions, cardiac arrest, and ECMO but also follow-up in- grams have yielded success in improving not only cardiac
terventions for functional impairments or developmental delays function and overall physical activity but also emotional
that emerge throughout childhood. well-being and quality of life. Children may similarly benefit
from rehabilitation programs.
Cardiomyopathies
Heart Transplantation
Cardiomyopathies occur in fewer than 2 per 100,000 chil-
dren [24, 25]. Cardiomyopathies are abnormalities of the Per the Registry of the International Society for Heart and Lung
ventricular myocardium that cannot be explained by ab- Transplantation [33], there are approximately 400 to 600 cases of
normal loading conditions or heart defects; they can result pediatric heart transplantation per year, worldwide. CHDs are the
from coronary artery abnormalities, tachyarrhythmias, ex- most common indication for transplant during infancy (55% of
posure to infection or toxins, or other underlying disor- cases), followed by cardiomyopathies (37%). However, for chil-
ders [26]. There are several subtypes of cardiomyopathies, dren over age 10, cardiomyopathies are the most common indi-
summarized in Table 2 along with their presentation and cation (43–54% dependent on age). The median post-transplant
management. survival is approximately 22 years for those transplanted in in-
In many children with cardiomyopathies, the disorder pro- fancy, approximately 18 years for those in early childhood (age
gresses to the point where medications and surgical options 1–5 years), approximately 14 years for those in middle childhood
are ineffective. Nearly 40% of children with symptomatic car- (age 6–10 years), and 13 years for those in adolescence.
diomyopathy require a heart transplant or die [27, 28]. The Cardiac transplantation results in postganglionic denerva-
time to transplant or death for children with cardiomyopathy tion, leading to the inability to respond to the parasympathetic
has notably not improved over the past several decades, and nervous system [34]. In turn, there are higher systolic and
the most economically advanced nations have no better sur- diastolic blood pressures, elevated heart rate at rest, lower
vival outcomes than developing nations [29]. Rehabilitation maximal myocardial oxygen consumption, lower heart rate
programs may be beneficial, not to cure the diseases, but to reserve, and decreased exercise duration. Because of these
potentially improve the cardiac function and the overall health physiologic changes, cardiac rehabilitation can be helpful in
of children for whom options for care are limited [30••]. reestablishing physical health and activity levels.

Table 2 Cardiomyopathies and their clinical presentation and management

Cardiomyopathy % of cases with Presentation Management Survival

cardiomyopathy

Dilated > 50 Ranges from asymptomatic to acute Medical therapies, mechanical 60–75% at
cardiomyopathy decompensated heart failure and support, and transplant 5 years for
cardiogenic shock; arrhythmias those without
heart transplant
Hypertrophic 42 Ranges from asymptomatic with or β-Blockade, calcium channel 97% at 5 years
cardiomyopathy without murmurs to exercise blockers, disopyramide, surgical
intolerance, chest pain, palpitations, myomectomy, and automatic
syncope, or cardiac arrest; increased implantable cardioverter-defibrillator
risk of sudden death with exercise
Restrictive 4.5 Ranges from asymptomatic to overt Diuretics, anticoagulation, antiarrhythmics, 68% at 5 years
cardiomyopathy heart failure, syncope, or sudden automatic implantable
death; arrhythmias cardioverter-defibrillator,
early consideration for transplant
Noncompaction 4.8 Benign or severe course with progressive Anticoagulation, consideration for transplant Death or
cardiomyopathy systolic or diastolic dysfunction, transplantation
life-threatening arrhythmias, or in 18–25%
thromboembolism

Summarized from Lee et al. [26]

70 Curr Phys Med Rehabil Rep (2019) 7:67–80

Heart transplantation in infancy and toddlerhood has been Given its potential benefit, exercise training has been
associated with mild delays in motor and cognitive develop- outlined as a key component of cardiac rehabilitation with
ment, with a similar pattern of deficits as seen with children adult patients [37–39], recommended in guidelines for certain
with CHDs requiring surgery other than transplantation early pediatric cardiac populations (e.g., those with heart failure),
in life [35]. Specifically, this group can display mild reduc- and has consistently been included in emerging programs with
tions in motor skills, intellectual abilities, language abilities, children [30••, 43–45]. From prior reviews of cardiac rehabil-
and visuospatial skills. Older children, who often undergo a itation programs for children with CHDs or cardiomyopathies
heart transplant for cardiomyopathy, can display mild cogni- [30••, 43, 44], it has generally been recommended that pro-
tive deficits and are, more importantly, at risk of internalizing grams have a duration of at least 12 weeks, with two to three
and externalizing distress [36]. Among children who undergo sessions per week, and sessions of at least 30 min (and up to
heart transplantation, cardiac rehabilitation may be particular- 90 min). Programs should include aerobic, resistance, and
ly important not only to help pediatric patients regain their flexibility training, with warm-up and cool-down periods.
strength and mobility in the short term but also to help max- Training should be individualized based on the results of met-
imize children’s quality of life across their reduced lifespan. abolic stress tests, cardiac biomarkers, echocardiograms, base-
line resistance-training capacity, and past medical history.
Notably, the intensity of aerobic exercise should be at a heart
rate approximately equivalent to anaerobic threshold. The pa-
Pediatric Cardiac Rehabilitation
tient’s progress should be reviewed at least weekly, and pro-
gressive increases should be made in the child’s exercise
Guidelines from the American Heart Association and the
workload as tolerated and when medically appropriate.
American Association of Cardiovascular and Pulmonary
Programs might also benefit from a 6-month maintenance
Rehabilitation recommended that cardiac rehabilitation pro-
period with two exercise visits per month, including a review
grams for adults and older adults include several core compo-
of exercise logs. Both center-based and at-home training pro-
nents including a baseline assessment, management of health
grams may be effective [30••].
risk factors (e.g., diabetes, hypertension, lipid levels) and nu-
More broadly, cardiac rehabilitation programs can provide
trition, exercise training and physical activity counseling, and
education on appropriate physical activity in children with
psychosocial management [37–39]. Similarly, primary goals
cardiac conditions. Physical activity has been promoted in
for pediatric cardiac rehabilitation are managing physical
guidelines from the American Heart Association and the
health and activity as well as socioemotional functioning.
Association for European Paediatric Cardiology for children
Given the key developmental tasks of childhood, such as
with CHDs [46••, 47]. Specifically, guidelines for physical
gaining basic academic skills, there may be an additional fo-
activity in children with CHDs underscore the need for at least
cus on mitigating developmental and cognitive disruptions,
60 min of daily activity that is developmentally appropriate
which would not be a primary concern during adult cardiac
and enjoyable. Vigorous activity is recommended at least
rehabilitation.
3 days per week, and exercises for strengthening bone and
muscle (e.g., high-impact and anaerobic burst exercises such
Addressing Physical Health and Activity as jumping) are recommended 3 days per week. However,
children with certain heart defects (e.g., transposition of the
Historically, there have been concerns regarding the adverse great arteries, single ventricle conditions) may need to limit
effects of physical activity in those with cardiac conditions. participation in the highest intensity activities, notably in com-
However, research has underscored that exercise is not neces- petitive sports [48]. As for all children, screen time should be
sarily contraindicated. For example, a large study among limited to no more than 2 h per day for children over the age of
adults with CHDs demonstrated that the majority of sudden 5 years, and children under the age of 3 years should not have
cardiac events in patients occurred at rest (69%), with 11% any screen time [46••, 47].
during sleep, and only 10% during exercise [40]. In fact, phys- Guidelines from the American Heart Association and the
ical activity can be beneficial for those with cardiac condi- European Society of Cardiology for individuals with cardio-
tions. There is overwhelming evidence that exercise training myopathies also underscore the benefit of physical activity
within adult cardiac rehabilitation promotes cardiorespiratory [49, 50, 51••]. Still, those with cardiomyopathies may have
fitness, strength, flexibility, and metabolic health; reduces more restrictions in their activity. Intensive exercise programs
morbidity, mortality, and hospital admissions; and improves and competitive sport may be contraindicated for certain indi-
quality of life [41•, 42•]. Emerging research with pediatric viduals depending on their subtype of cardiomyopathy, histo-
populations with cardiac conditions similarly suggests that ry of symptoms, and findings on cardiac testing. More gener-
exercise training can have beneficial effects, although there ally, patients should be advised to start exercise sessions with a
is some variability in effects [43, 44]. warm-up period and end with a cool-down period; avoid burst
Curr Phys Med Rehabil Rep (2019) 7:67–80 71

exertion, preferably avoid high-intensity free weight lifting adolescent [55–57]. Behavioral and cognitive-behavioral thera-
due to the risk of injury with syncope; avoid exercising in pies also offer models for chronic pain and poor sleep [58, 59].
adverse environmental conditions; and exercise only in envi- The ability to consult psychiatric colleagues within cardiac reha-
ronments equipped with an automatic defibrillator. bilitation programs further allows pediatric patients access to
In addition to exercise training and counseling on appro- specialized psychotropic medication management when
priate physical activity, pediatric cardiac rehabilitation pro- indicated.
grams can help patients and their families establish a nutrition Interestingly, psychological interventions might benefit
plan and better recognize and manage the symptoms of their physical health along with psychosocial health. In a meta-
cardiac condition. As such, programs can promote a healthy analysis of 23 randomized control trials involving adult pa-
lifestyle and decrease the risk and the severity of future car- tients with coronary heart disease, psychological interventions
diovascular disease. both reduced emotional distress and improved systolic blood
pressure, heart rate, and cholesterol levels [60]. Similarly, a
Addressing Cognitive and Socioemotional pediatric cardiac rehabilitation program with a stress manage-
Functioning ment component improved physiological measurements, al-
though the study did not separate out the effects of exercise
Cardiac rehabilitation programs can help not only mitigate the training, health education, and stress management [61].
physiological effects of cardiac conditions but also enhance Research has also documented associations between physical
the socioemotional and cognitive functioning of patients. By and emotional health among youths who have completed car-
doing so, cardiac rehabilitation programs can help children, diac rehabilitation [62•] and between daily physical activity
adolescents, and young adults transition back to their family and mental health among children with CHDs [63].
and home life, peer groups, and school and work settings Cardiac rehabilitation programs can furthermore offer an
following hospitalization. Although such efforts are standard opportunity to identify and address disruptions in the acquisi-
in programs with adult patients, recent reviews suggested that tion of developmental milestones and cognitive abilities.
the same cannot be said of pediatric programs, even though Developmental assessments can identify delays in motor, lan-
the omission of mental health providers may decrease the guage, and adaptive development, and neuropsychological
success of cardiac rehabilitation [43, 44]. evaluations can identify cognitive deficits [1]. Such testing
Psychologists, social workers, and other licensed mental can aid with treatment and transition planning (e.g., clarifying
health professionals can assess the psychosocial needs of pe- the need for speech and language therapy and outlining rec-
diatric patients and their families and monitor changes in ommendations for an Individualized Education Program upon
needs throughout their rehabilitation [52]. For example, al- return to school).
though patients have typically undergone a psychosocial eval- Importantly, families of children with cardiac disorders
uation prior to a heart transplant, their needs may change dur- have described the need for effective communication among
ing the waiting period for a heart and subsequent recovery health and mental health professionals, families, and schools
from surgery. Families may also be more likely to report sen- as critical within rehabilitation programs [64]. By incorporat-
sitive information once they no longer need to be concerned ing mental health providers, care coordinators, and school
that they may not be listed for a transplant because of their liaisons into comprehensive cardiac rehabilitation programs,
financial, social, or personal resources. They may also share the needs of pediatric patients can be better understood and
important information once they feel more comfortable with addressed by the treatment team.
their care team over time. As such, they may be more apt to
discuss difficulty coping with their illness and medical care,
negative mood, insufficient social support, and/or premorbid Rehabilitation Programs
history of mental health concerns.
Having identified patients’ needs, mental health providers can As summarized in Table 3, we identified 20 cardiac rehabili-
implement interventions to foster healthy behaviors and treat- tation programs described across 26 reports, with samples that
ment compliance and address the distress associated with critical included patients under age 18. Due to the limited number of
illness and lengthy hospital stays. For instance, motivational studies and consistency in findings, we did not exclude pro-
interviewing strategies can aid with building a desire to engage grams that had both pediatric and adult patients. Similarly, we
in adaptive behaviors, such as taking medications and following included both randomized control trials and investigations
nutrition and exercise plans [53, 54]. Strategies from behavioral without control groups, due to the emerging nature of the
management therapy can target the behavioral manifestations of literature. Programs described within case studies and unpub-
distress often seen in young children, and cognitive-behavioral lished manuscripts (e.g., theses, conference presentations)
and mindfulness-based therapies can effectively target anxious, were excluded from the review. We only included reports
irritable, and depressed moods among older children and written in English and papers published after 1990.
72 Curr Phys Med Rehabil Rep (2019) 7:67–80

Table 3 Cardiac rehabilitation programs including pediatric patients

References N Patient population Age (in Program protocol Program Measures Outcome/results
years) duration

Inpatient Acute Care Rehabilitation

Hollander 14 Ventricular assist < 1–12 • 4–5 1-h sessions per N/A Completion of pathway • 11/14 achieved all goals
[65] device week via a goals focused on • No adverse events
standardized care age-appropriate
pathway development and
• 8–10 therapeutic goals activities of daily living
• Physical and
occupational
therapies
McBride [66] 20 Hospitalized for Mean • 3 1-h sessions 2–18 months Exercise testing* • Peak work rate and VO2
heart 13 ± • Resistance and aerobic significantly below
failure (i.e., 3.2 exercises (treadmill or percentile for age and
cardiomyopathy cycle ergometer) sex
and failing
single
ventricle)

Outpatient Rehabilitation
Balfour [61] 7 CHDs or acquired 13–19 • 3 days per week in 12 weeks ECG, Holter monitoring, • Decrease in resting
heart conditions facility echocardiography, blood pressure and
• 1–2 days per week graded maximal increase in peak
home exercise exercise test oxygen consumption
• 30–40 min per session and treadmill time
Brassard [67] 7 History of Fontan 11–26 • 3 20–30-min sessions 3 8 weeks Pulmonary function • Reduction in ergoflex
procedure times per week evaluation, contribution to
• Aerobic and resistance neuromuscular function absolute values of
training evaluated via systolic blood pressure
ergoreceptor activity (suggested
monitoring, skeletal improvement in
muscle strength, skeletal muscle
endurance evaluation, function)
exercise testing
Dedieu [68] 33 CHD 8–40 • 2 days a week training 8 weeks Exercise testing • Increase in maximum
heart rate, exercise
duration and metabolic
equivalents,
improvement in
quality of life
Duppen 93 Tetralogy of Fallot 10–25 • 3 1-h sessions per week 12 weeks Exercise testing, All ages:
[69–72] and history of • Sports activities twice psychological • Peak load increased in
Fontan per week assessment, the intervention group
procedure • 10-min warm-up semi-structured • Ventricular systolic
(vs. healthy • 40-min aerobic training interview, Web-based parameters did not
controls) (brisk walking, health-related quality of change in the
dynamic play, life assessment intervention group
jogging/running/- • No adverse events
bicycle) Ages 10–15:
• 10-min cool down • Improved cognitive
• Heart rate monitor functioning
given workload • Self-report of improved
adjusted to heart rate motor functioning after
levels the sports-related
• Control: life as usual intervention
• Parent report of
improved social
functioning
Curr Phys Med Rehabil Rep (2019) 7:67–80 73

Table 3 (continued)

References N Patient population Age (in Program protocol Program Measures Outcome/results
years) duration

Ages 16–25:
• No differences in quality
of life
Frederiksen 129 CHDs 10–16 • 2-week intensive 2 weeks or Exercise testing • Improvement in uptake
[73] rehabilitation at sports 5 months of peak level of oxygen
center or twice per • Increase in physical
week program activity
• Decrease in internalized
distress
Opocher [74] 10 History of Fontan 7–12 • Twice per week lessons 8 months Exercise testing • Improved aerobic
procedure for 3 months capacity
• Once per week lessons • Increase in maximal
for 4 months oxygen consumption
• At-home training twice • Decrease in heart rate
per week for curve
30–45 min • Increased oxygen pulse
curve during
submaximal exercise
Rhodes [62•, 30 CHDs 8–17 • 2 1-h sessions per week 12 weeks Exercise testing • Improved peak oxygen
75, 76] • Aerobic and weight consumption, peak
resistance exercises work rate, ventilatory
• Recommended home anaerobic threshold,
program of twice per 1-min recovery rate
week • After 1 year, peak
oxygen consumption,
work rate, and 1-min
heart rate recovery
remained improved
Sklansky [7] 11 History of tetralogy N/A • 3 30-min sessions per 8 weeks Exercise testing • Decrease in submaximal
of Fallot repair week heart rate, submaximal
• Use of cycle ergometer cardiac output,
maximal oxygen
uptake, respiratory
rate, and maximal
treadmill time
Wittekind 10 History of Fontan 8–15 • 2 1-h sessions per week 12 weeks Cardiopulmonary exercise • Increase in peak indexed
[78•] procedure • 5- to 10-min warm-up testing, ECHO, heart oxygen consumption
and stretching rate, blood pressure, increased, peak oxygen
• 30-min aerobic training VO2, VCO2, minute pulse, and indexed
• 15-min low-resistance ventilation, perceived oxygen consumption at
and high-repetition exertion using the Borg ventilator anaerobic
strength training threshold
• 5-min cool down

Home-based Rehabilitation
Amiard [79•] 23 CHDs with history 15 ± 1.4 • 45-min sessions 8 weeks Exercise testing • No strong improvement
of surgical repair consisting of 10-min in aerobic capacity or
warm-up and 5-min ventilatory threshold
stretching, and use of
a home cycle
ergometer
Hedlund [80] 55 History of Fontan Mean • Individualized 12 weeks 6-min walk test, ergometer After intervention:
procedure (vs. 14.2 endurance training cycle test, and Pediatric • Increase in average
healthy controls) program. Quality of Life intensity on the Borg
• 2 45-min endurance Inventory scale for activities
training sessions per • Increase in 5-min walk
week test
74 Curr Phys Med Rehabil Rep (2019) 7:67–80

Table 3 (continued)

References N Patient population Age (in Program protocol Program Measures Outcome/results
years) duration

• Activities included • VO2 max did not

running, skiing, increase
jogging, skiing, • Quality of life improved
cycling, swimming, At 1-year follow up
riding, dancing, • Borg intensity back to
football average
• 6-min walk test
remained improved
• Quality of life was
sustained
Jacobsen 13 History of Fontan 8–12 • 45-min routine with 12 weeks VO2 max estimates, 20-m • 93% program
[81•] procedure dynamic and static shuttle test run, baseline completion
exercises on video height, weight, body • Improvements in VO2
and handout mass index, blood max, quality of life,
• 3–4 times per week pressure, heart rate, and exercise capacity
• 3 in-person sessions at ECHO
0-week, 6-week, and Primary outcome:
12-week marks physical function and
• Use of Fitbit Flex to quality of life
monitor adherence Secondary outcome:
change in exercise
capacity
Longmuir 61 History of Fontan5.9–11.7 • 2 models with a focus 2 years Tests of gross motor • No difference was noted
[82] procedure on increasing physical development, aerobic in secondary outcomes
activity by play. step test, grip strength, (VO2 max, exercise
• Both models included hamstring flexibility, fitness scores, etc.)
specific daily body mass index, • Improved gross motor
activities, were health-related fitness, skills
parent-led, and had exercise testing, and
at-home and activity attitudes
community-based assessment
design
• 1-year commitment to
2 activity hours per
week
Moalla [83, 31 CHDs in New York 12–16 • 2 groups with CHDs: 12 weeks Pulmonary function tests, • Improved walking
84] Heart untrained and control. exercise testing, and distance
Association • 3 sessions per week near-infrared • Power output, VO2,
Class II or III • 10-min warm-up spectroscopy heart rate, and
(vs. • 10-min work and assessment used to ventilation increased
healthy controls) 5-min recovery evaluate oxygenation of slightly at peak
intervals on a respiratory muscles exercise and
stationary bike significantly at
• 5-min cool down ventilatory threshold
• Intensity set a heart rate level
of ventilatory
threshold obtained
during
cardiopulmonary
testing
Minamisawa 16 History of Fontan 11–25 • Sessions 2–3 days per 8–12 weeks Exercise testing • Improved peak oxygen
[85] procedure week consumption and
• 5-min warm-up with workload
20–30 min jogging to
target heart rate
Morrison 143 CHDs 12–20 • 1-day structured group 6 months Exercise testing • Improved peak oxygen
[86] activity consumption
• 6 months of activity • Increased moderate to
based on written vigorous physical
exercise plans activity
Curr Phys Med Rehabil Rep (2019) 7:67–80 75

Table 3 (continued)

References N Patient population Age (in Program protocol Program Measures Outcome/results
years) duration

Stieber [87] 20 Single ventricle 1–2 • 5 2-week sessions 10 weeks Peabody Developmental • Expected rate of motor
physiology, • Play activities for a Motor Scale, 2nd development was
transposition of total of 10 min or Edition achieved in both
great arteries, more each day with a groups
history of goal of 20 min total
superior c (10 min per
avopulmonary development goal)
connection
procedure or
arterial
switch operation
Patel [88] 11 History of heart Mean • Aerobic exercise 12 weeks Exercise testing • Increase in peak oxygen
transplant 14.7 3 days per week consumption,
• Strength training endurance time,
2 days per week strength measurements

* Exercise testing typically included the following measures: electrocardiogram (ECG), blood pressure, maximal oxygen consumption (VO2), work
performed, maximal oxygen pulse, maximum including oxygen uptake, carbon dioxide production (VCO2), minute ventilation, maximal ventilator, and
metabolic equivalents heart rate

Below, we describe pediatric cardiac rehabilitation pro- evaluated effects of exercise in patients with CHDs. The dura-
grams designed for inpatient and outpatient settings as well tion of the programs ranged from 2 weeks to 8 months, with
as home-based training. Across the different settings, pro- structured activity typically two to three times per week, typi-
grams typically adopted a team approach with physicians, cally for 30 min to 60 min per session. The programs all in-
rehabilitation therapists, and other health providers. Indeed, cluded some form of warm-up and cool-down periods (typi-
it has previously been recommended that programs include cally 5 min to 10 min each). All of the programs included an
dieticians, physical therapists, and occupational therapists at aerobic component to the exercise training program, and some
minimum and be coordinated by a pediatric cardiologist and a also included strength training. All of the programs demon-
pediatric physiatrist [30••]. Staff should be trained to handle strated an improvement in maximal oxygen output. Studies
medical emergencies, and the staff-to-patient ratio should not also reported exercise duration, metabolic equivalents, and im-
exceed 1 to 4 at any time, per prior recommendations [30••]. proved quality of life. One of the programs also documented
self-reported improvements in cognitive functioning and
Inpatient Acute Care Rehabilitation parent-reported improvements in social functioning [69–72].

The literature is limited in regard to programs that include Home-Based Rehabilitation

rehabilitation during the acute care hospitalization period.
Still, we identified two reports describing inpatient acute care Nine programs, summarized across ten reports, described
rehabilitation, one featuring patients with a ventricular assist home-based rehabilitation programs [79•, 80, 81•, 82–88].
device and one focused on patients hospitalized for heart fail- Although the majority of studies focused on school-age and
ure [65, 66]. The programs demonstrated the safety and feasi- adolescent patients with CHDs, there was one program that
bility of an acute care model both in the intensive care setting focused on toddlers [87], one that included young adults [85],
and on a standard hospital floor, with no adverse events related and one that enrolled patients following heart transplantation
to physical activity. One of the rehabilitation programs addi- [88]. Programs often required 8 weeks to 12 weeks of home-
tionally documented that patients were able to achieve the based exercise and included initial and follow-up sessions in
therapeutic goals established for them [65]. person with health providers, so as to provide education on the
program and monitor progress. Progress could also be moni-
Outpatient Rehabilitation tored with digital tools, such as heart rate monitors, acceler-
ometers, and Fitbits. Echoing the design of outpatient pro-
We identified nine outpatient programs, described across 14 grams, home-based rehabilitation programs typically involved
studies [61, 62•, 67–77, 78•]. The programs included patients sessions of an hour or less with 5-min to 10-min warm-up and
as young as 8 years old; although three of the programs includ- cool-down periods and focus on aerobic exercise. Although
ed young adults and one included patients as old as 40 years, training was often associated with improved exercise capacity,
they all enrolled children and/or adolescents. All of the studies better cardiorespiratory outcomes, and improved quality of
76 Curr Phys Med Rehabil Rep (2019) 7:67–80

life and psychosocial functioning, one study did not find sig- Currently, our institution is targeting children on the path-
nificant changes in patients after the intervention [79•]. way to heart transplantation for participation in pediatric car-
Interestingly, the program with toddlers reported improve- diac rehabilitation; however, we hope to expand our program
ments in gross motor functioning, daily physical activity, more broadly to children with CHDs and cardiomyopathies
and family life up to 2 years later [87], and a program with not requiring heart transplantation. The program structure in-
school-age children documented improvements in exercise cludes providing a multidisciplinary evaluation prior to trans-
capacity and quality of life up to a year after the intervention plantation, including assessments from medical and therapy
[80]. None of the programs reported significant adverse providers. Based on the evaluation, the team provides a cohe-
events. sive set of recommendations, including a potential course of
therapies prior to transplantation. Following cardiac transplan-
Cognitive Rehabilitation tation, patients are typically engaged in a 12-week outpatient
course of physical therapy three times per week (with sessions
The vast majority of rehabilitation programs among children lasting between 30 and 60 min), with aerobic conditioning,
with cardiac conditions have focused on exercise training, as a resistance, and strength training. Sessions include warm-up
function of children’s complex medical needs. However, we and cool-down periods. As needed, patients are engaged in
mention one study underway to examine cognitive rehabilita- occupational therapy with a focus on adaptive skills following
tion among children with cardiac conditions. Newburger [89] surgery, speech and language therapy with a focus on cogni-
is investigating the effects of a computer-based training pro- tive restructuring, and nutrition and physical activity educa-
gram to enhance the executive functioning skills of children tion. These sessions can occur at facilities associated with the
with CHDs. hospital for families that live close by; otherwise, a prescrip-
tion detailing needed interventions is provided to the family to
take to a facility in their community. As needed, patients are
Current Directions at Our Institution engaged in psychotherapy to aid with their psychosocial ad-
justment. Patients may also be referred for neuropsychological
At the UPMC Children’s Hospital of Pittsburgh (CHP), we de- testing to determine their cognitive needs, as they return to the
veloped a cardiac rehabilitation program modeled after recom- school and home environment; evaluations are conducted fol-
mendations from previously developed programs, as described lowing guidelines for assessment in children with cardiac dis-
above. The program seeks to establish a reproducible and sus- orders [90••].
tainable protocol for children with pediatric-onset or congenital
cardiac disease, in an effort to systematically influence and mit-
igate the effects on physical health, neurodevelopmental out-
comes, and quality of life. Specifically, the goals of the program Conclusion
are to (1) improve exercise function and capacity; (2) improve
heart rate recovery and response to exercise; (3) decrease the Children can benefit from pediatric cardiac rehabilitation
effects of deconditioning; (4) improve physical function and programs from physical health and psychosocial perspec-
age appropriate level of play and adaptive living; (5) improve tives, and more importantly, these programs can be toler-
patient and family confidence in exercise and participation in age ated with minimal adverse events. Although center-based
appropriate activities; (6) promote global wellness, including an programs may allow for a greater degree of refinement in
active lifestyle and healthy nutrition choices; and (7) improve individual patient goals and monitoring of their health
overall adjustment and quality of life post transplantation. status, the limitation of proximity to a tertiary care insti-
With this framework in mind, we established multidisci- tution is not necessarily a barrier (particularly among chil-
plinary inpatient and outpatient cardiac rehabilitations pro- dren with chronic concerns, such as CHDs), as demon-
grams, which involve medical evaluation, prescribed thera- strated by the efficacy of home-based programs. The
pies, nutritional and physical education geared toward im- home-based program has the potential to limit the burden
proving the physiologic effects of long-term cardiac illness, of transportation and potentially time off work for parents
and consultation for developmental, neuropsychological, and who bring their child to a facility for training. Still, home-
psychological services. The team, headed by a pediatric car- based programs should continue to be monitored by both
diologist and pediatric physiatrist, also includes physical ther- a cardiologist and a pediatric physiatrist. Further research
apists, occupational therapists, speech and language patholo- is needed to demonstrate sustained long-term benefits of
gists, dieticians, neuropsychologists, and pediatric psycholo- interventions, to understand the impact on quality of life
gists. This team meets at a regularly scheduled time to review for the family system, and to explore long-term impact of
patients for clinic care and to advance the cardiac rehabilita- cognitive interventions within pediatric cardiac rehabilita-
tion program’s development. tion programs.
Curr Phys Med Rehabil Rep (2019) 7:67–80 77

Compliance with Ethical Standards long-term prognosis in adults with repaired tetralogy of Fallot. Am
J Cardiol. 2007;99(10):1462–7.
13. Casey FA, Stewart M, McCusker CG, Morrison ML, Molloy B,
Conflict of Interest The authors declare that they have no conflicts of
Doherty N, et al. Examination of the physical and psychosocial
interest.
determinants of health behaviour in 4–5-year-old children with con-
genital cardiac disease. Cardiol Young. 2010;20(5):532–7.
Human and Animal Rights and Informed Consent This article does not 14. Limperopoulos C, Tworetzky W, McElhinney DB, Newburger JW,
contain any studies with human or animal subjects performed by any of Brown DW, Robertson RL Jr, et al. Brain volume and metabolism
the authors. in fetuses with congenital heart disease evaluation with quantitative
magnetic resonance imaging and spectroscopy. Circulation.
2010;121(1):26–33.
15. Licht DJ, Shera DM, Clancy RR, Wernovsky G, Montenegro LM,
References Nicolson SC, et al. Brain maturation is delayed in infants with
complex congenital heart defects. J Thorac Cardiovasc Surg.
Papers of particular interest, published recently, have been 2009;137(3):529–37.
16. von Rhein M, Buchmann A, Hagmann C, Huber R, Klaver P,
highlighted as:
Knirsch W, et al. Brain volumes predict neurodevelopment in ado-
• Of importance lescents after surgery for congenital heart disease. Brain.
•• Of major importance 2013;137(1):268–76.
17. Bruneau BG. The developmental genetics of congenital heart dis-
1. Marino BS, Lipkin PH, Newburger JW, Peacock G, Gerdes M, ease. Nature. 2008;451(7181):943–8.
Gaynor JW, et al. Neurodevelopmental outcomes in children with 18. Clifford A, Lang L, Chen R. Effects of maternal cigarette smoking
congenital heart disease: evaluation and management: a scientific during pregnancy on cognitive parameters of children and young
statement from the American Heart Association. Circulation. adults: a literature review. Neurotoxicol Teratol. 2012;34(6):560–
2012;126(9):1143–72. 70.
2. Bellinger DC, Newburger JW. Neuropsychological, psychosocial, 19. Claessens NH, Kelly CJ, Counsell SJ, Benders MJ. Neuroimaging,
and quality-of-life outcomes in children and adolescents with con- cardiovascular physiology, and functional outcomes in infants with
genital heart disease. Prog Pediatr Cardiol. 2010;29(2):87–92. congenital heart disease. Dev Med Child Neurol. 2017;59(9):894–
3. Friess MR, Marino BS, Cassedy A, Wilmot I, Jefferies JL, Lorts A. 902.
Health-related quality of life assessment in children followed in a 20. Owen M, Shevell M, Majnemer A, Limperopoulos C. Abnormal
cardiomyopathy clinic. Pediatr Cardiol. 2015;36(3):516–23. brain structure and function in newborns with complex congenital
heart defects before open heart surgery: a review of the evidence. J
4. Brosig C, Hintermeyer M, Zlotocha J, Behrens D, Mao J. An ex-
Child Neurol. 2011;26(6):743–55.
ploratory study of the cognitive, academic, and behavioral function-
21. von Rhein M, Scheer I, Loenneker T, Huber R, Knirsch W, Latal B.
ing of pediatric cardiothoracic transplant recipients. Prog
Structural brain lesions in adolescents with congenital heart disease.
Transplant. 2006;16(1):38–45.
J Pediatr. 2011;158(6):984–9.
5. Dalal HM, Doherty P, Taylor RS. Cardiac rehabilitation. BMJ.
22. van Zellem L, Buysse C, Madderom M, Legerstee JS, Aarsen F,
2015;351:h5000.
Tibboel D, et al. Long-term neuropsychological outcomes in chil-
6. van der Linde D, Konings EE, Slager MA, Witsenburg M, Helbing dren and adolescents after cardiac arrest. Intensive Care Med.
WA, Takkenberg JJ, et al. Birth prevalence of congenital heart dis- 2015;41(6):1057–66.
ease worldwide: a systematic review and meta-analysis. J Am Coll 23. Brown KL, Ichord R, Marino BS, Thiagarajan RR. Outcomes fol-
Cardiol. 2011;58(21):2241–7. lowing extracorporeal membrane oxygenation in children with car-
7. Gilboa SM, Devine OJ, Kucik JE, Oster ME, Riehle-Colarusso T, diac disease. Pediatr Crit Care Med. 2013;14(5_suppl):S73–83.
Nembhard WN, et al. Congenital heart defects in the United States: 24. Lipshultz SE, Sleeper LA, Towbin JA, Lowe AM, Orav EJ, Cox
estimating the magnitude of the affected population in 2010. GF, et al. The incidence of pediatric cardiomyopathy in two regions
Circulation. 2016;134(2):101–9. of the United States. N Engl J Med. 2003;348:1647–55.
8. Cooper DS, Jacobs JP, Chai PJ, Jaggers J, Barach P, Beekman RH, 25. Nugent AW, Daubeney PE, Chondros P, Carlin JB, Cheung M,
et al. Pulmonary complications associated with the treatment of Wilkinson LC, et al. National Australian Childhood
patients with congenital cardiac disease: consensus definitions from Cardiomyopathy Study. The epidemiology of childhood cardiomy-
the Multi-Societal Database Committee for Pediatric and opathy in Australia. N Engl J Med. 2003;348:1639–46.
Congenital Heart Disease. Cardiol Young. 2008;18(S2):215–21. 26. Lee TM, Hsu DT, Kantor P, Towbin JA, Ware SM, Colan SD, et al.
9. Limperopoulos C, Majnemer A, Shevell MI, Rosenblatt B, Pediatric cardiomyopathies. Circ Res. 2017;121(7):855–73.
Rohlicek C, Tchervenkov C, et al. Functional limitations in young 27. Bilgiç A, Özbarlas N, Özkutlu S, Özer S, Özme S.
children with congenital heart defects after cardiac surgery. Cardiomyopathies in children: clinical, epidemiological and prog-
Pediatrics. 2001;108(6):1325–31. nostic evaluation. Jpn Heart J. 1990;31(6):789–97.
10. Holm I, Fredriksen PM, Fosdahl MA, Olstad M, Vollestad N. 28. Lipshultz SE. Ventricular dysfunction clinical research in infants,
Impaired motor competence in school-aged children with complex children and adolescents. Prog Pediatr Cardiol. 2000;12:1–28.
congenital heart disease. Arch Pediatr Adolesc Med. 2007;161(10): 29. Boucek MM, Edwards LB, Keck BM, Trulock EP, Taylor DO,
945–50. Mohacsi PJ, et al. The registry of the International Society for
11. Hovels-Gurich HH, Konrad K, Skorzenski D, Nacken C, Heart and Lung Transplantation: sixth official pediatric report—
Minkenberg R, Messmer BJ, et al. Long-term neurodevelopmental 2003. J Heart Lung Transplant. 2003;22(6):636–52.
outcome and exercise capacity after corrective surgery for tetralogy 30.•• Somarriba G, Extein J, Miller TL. Exercise rehabilitation in pediat-
of Fallot or ventricular septal defect in infancy. Ann Thorac Surg. ric cardiomyopathy. Prog Pediatr Cardiol. 2008;25(1):91–102. This
2006;81(3):958–66. article nicely outlines the benefits of each component of pediat-
12. Giardini A, Specchia S, Tacy TA, Coutsoumbas G, Gargiulo G, ric cardiac rehabilitation in addition to laying out an outline for
Donti A, et al. Usefulness of cardiopulmonary exercise to predict a program format.
78 Curr Phys Med Rehabil Rep (2019) 7:67–80

31. Hollander SA, Callus E. Cognitive and psycholologic consider- exercise training programmes in children and young adults with
ations in pediatric heart failure. J Card Fail. 2014;20(10):782–5. congenital heart disease. Int J Cardiol. 2013;168(3):1779–87.
32. Stein ML, Bruno JL, Konopacki KL, Kesler S, Reinhartz O, 45. Kirk R, Dipchand AI, Rosenthal DN, Addonizio L, Burch M,
Rosenthal D. Cognitive outcomes in pediatric heart transplant re- Chrisant M, et al. The International Society for Heart and Lung
cipients bridged to transplantation with ventricular assist devices. J Transplantation guidelines for the management of pediatric heart
Heart Lung Transplant. 2013;32(2):212–20. failure: executive summary. J Heart Lung Transplant. 2014;33(9):
33. Rossano JW, Cherikh WS, Chambers DC, Goldfarb S, Khush K, 888–909.
Kucheryavaya AY, et al. The Registry of the International Society 46.•• Longmuir PE, Brothers JA, de Ferranti SD, Hayman LL, Van Hare
for Heart and Lung Transplantation: twentieth pediatric heart trans- GF, Matherne GP, et al. Promotion of physical activity for children
plantation report-2017. J Heart Lung Transplant. 2017;36(10): and adults with congenital heart disease: a scientific statement from
1060–9. the American Heart Association. Circulation. 2013;127(21):2147–
34. Wilson RF, Johnson TH, Haidet GC, Kubo SH, Mianuelli M. 59. This paper describes physical activity recommendations in
Sympathetic reinnervation of the sinus node and exercise hemody- individuals with congenital heart disease and is an important
namics after cardiac transplantation. Circulation. 2000;101:2727– statement coming from the American Heart Association. The
273. article also makes suggestions for potential research protocols
35. Antonini TN, Dreyer WJ, Caudle SE. Neurodevelopmental func- to further knowledge in this realm.
tioning in children being evaluated for heart transplant prior to 2 47. Takken T, Giardini A, Reybrouck T, Gewillig M, Hövels-Gürich
years of age. Child Neuropsychology. 2018;24(1):46–60. HH, Longmuir PE, et al. Recommendations for physical activity,
36. Todaro JF, Fennell EB, Sears SF, Rodrigue JR, Roche AK. recreation sport, and exercise training in paediatric patients with
Cognitive and psychological outcomes in pediatric heart transplan- congenital heart disease: a report from the Exercise, Basic &
tation. J Pediatr Psychol. 2000;25(8):567–76. Translational Research Section of the European Association of
37. Balady GJ, Ades PA, Comoss P, Limacher M, Pina IL, Southard D, Cardiovascular Prevention and Rehabilitation, the European
et al. Core components of cardiac rehabilitation/secondary preven- Congenital Heart and Lung Exercise Group, and the Association
tion programs: a statement for healthcare professionals from the for European Paediatric Cardiology. Eur J Prev Cardiol.
American Heart Association and the American Association of 2012;19(5):1034–65.
Cardiovascular and Pulmonary Rehabilitation Writing Group. 48. Graham TP, Driscoll DJ, Gersony WM, Newburger JW, Rocchini
Circulation. 2000;102:1069–73. A, Towbin JA. Task force 2: congenital heart disease. J Am Coll
38. Balady GJ, Williams MA, Ades PA, Bittner V, Comoss P, Foody Cardiol. 2005;45(8):1326–33.
JM, et al. Core components of cardiac rehabilitation/secondary pre- 49. Maron BJ, Chaitman BR, Ackerman MJ, Bayes de Luna A,
vention programs: 2007 update: a scientific statement from the Corrado D, Crosson JE, et al. Recommendations for physical ac-
American Heart Association Exercise, Cardiac Rehabilitation, and tivity and recreational sports participation for young patients with
Prevention Committee, the Council on Clinical Cardiology, the genetic cardiovascular diseases. Circulation. 2004;109(22):2807–
Councils on Cardiovascular Nursing, Epidemiology and 16.
Prevention, and Nutrition, Physical Activity, and Metabolism, and 50. January CT, Wann LS, Alpert JS, Calkins H, Cigarroa JE, Conti JB,
the American Association of Cardiovascular and Pulmonary et al. 2014 AHA/ACC/HRS guideline for the management of pa-
Rehabilitation. Circulation. 2007;115(20):2675–82. tients with atrial fibrillation: executive summary: a report of the
39. Williams MA, Fleg JL, Ades PA, Chaitman BR, Miller NH, American College of Cardiology/American Heart Association
Mohiuddin SM, et al. Secondary prevention of coronary heart dis- Task Force on practice guidelines and the Heart Rhythm Society.
ease in the elderly (with emphasis on patients ≥ 75 years of age) an J Am Coll Cardiol. 2014;64(21):2246–80.
American Heart Association scientific statement from the Council 51.•• Pelliccia A, Solberg EE, Papadakis M, Adami PE, Biffi A, Caselli
on Clinical Cardiology Subcommittee on Exercise, Cardiac S, et al. Recommendations for participation in competitive and
Rehabilitation, and Prevention. Circulation. 2002;105(14):1735– leisure time sport in athletes with cardiomyopathies, myocarditis,
43. and pericarditis: position statement of the Sport Cardiology
40. Koyak Z, Harris L, de Groot JR, Silversides CK, Oechslin EN, Section of the European Association of Preventive Cardiology
Bouma BJ, et al. Sudden cardiac death in adult congenital heart (EAPC). Eur Heart J. 2018;40(1):19–33. This position paper of-
disease. Circulation. 2012;126(16):1944–54. fers a comprehensive overview of the most updated recommen-
41.• Anderson L, Oldridge N, Thompson DR, Zwisler AD, Rees K, dations for practicing cardiologists and sport physicians man-
Martin N, et al. Exercise-based cardiac rehabilitation for coronary aging athletes with cardiomyopathies and myopericarditis and
heart disease: Cochrane systematic review and meta-analysis. J Am provides pragmatic advice for safe participation in competitive
Coll Cardiol. 2016;67(1):1–12. In this systematic review and sport at professional and amateur levels, as well as in a variety
meta-analysis of cardiac rehabilitation programs with adults of recreational physical activities.
with coronary heart disease, 63 studies with 14,486 participants 52. Annunziato RA, Fisher MK, Jerson B, Bochkanova A, Shaw RJ.
are reviewed. The authors conclude that exercise-based cardiac Psychosocial assessment prior to pediatric transplantation: a review
rehabilitation reduces cardiovascular mortality and hospital and summary of key considerations. Pediatr Transplant.
admissions and improves quality of life. 2010;14(5):565–74.
42.• Dias KA, Link MS, Levine BD. Exercise training for patients with 53. Suarez M, Mullins S. Motivational interviewing and pediatric
hypertrophic cardiomyopathy: JACC review topic of the week. J health behavior interventions. J Dev Behav Pediatr. 2008;29(5):
Am Coll Cardiol. 2018;72(10):1157–65. This review discusses the 417–28.
evidence supporting the safety and efficacy of different intensi- 54. Gayes LA, Steele RG. A meta-analysis of motivational
ties of exercise training in hypertrophic cardiomyopathy and interviewing interventions for pediatric health behavior change. J
considers novel strategies to improve fitness in adults. Consult Clin Psychol. 2014;82(3):521–35.
43. Tikkanen AU, Oyaga AR, Riano OA, Alvaro EM, Rhodes J. 55. Thomas R, Abell B, Webb HJ, Avdagic E, Zimmer-Gembeck MJ.
Paediatric cardiac rehabilitation in congenital heart disease: a sys- Parent-child interaction therapy: a meta-analysis. Pediatrics.
tematic review. Cardiol Young. 2012;22(3):241–50. 2017;140(3):e20170352.
44. Duppen N, Takken T, Hopman MT, ten Harkel AD, Dulfer K, 56. Compton SN, March JS, Brent D, Albano AM, Weersing VR,
Utens EM, et al. Systematic review of the effects of physical Curry J. Cognitive-behavioral psychotherapy for anxiety and
Curr Phys Med Rehabil Rep (2019) 7:67–80 79

depressive disorders in children and adolescents: an evidence-based 73. Fredriksen PM, Kahrs N, Blaasvaer S, Sigurdsen E, Gundersen O,
medicine review. J Am Acad Child Adolesc Psychiatry. Roeksund O, et al. Effect of physical training in children and ado-
2004;43(8):930–59. lescents with congenital heart disease. Cardiol Young. 2000;10(2):
57. Klingbeil DA, Renshaw TL, Willenbrink JB, Copek RA, Chan KT, 107–14.
Haddock A, et al. Mindfulness-based interventions with youth: a 74. Opocher F, Varnier M, Sanders SP, Tosoni A, Zaccaria M, Stellin G,
comprehensive meta-analysis of group-design studies. J Sch et al. Effects of aerobic exercise training in children after the Fontan
Psychol. 2017;63:77–103. operation. Am J Cardiol. 2005;95(1):150–2.
58. Meltzer LJ, Mindell JA. Systematic review and meta-analysis of 75. Rhodes J, Curran TJ, Camil L, Rabideau N, Fulton DR, Gauthier
behavioral interventions for pediatric insomnia. J Pediatr Psychol. NS, et al. Impact of cardiac rehabilitation on the exercise function of
2014;39(8):932–48. children with serious congenital heart disease. Pediatrics.
59. Palermo TM, Eccleston C, Lewandowski AS, Williams AC, 2005;116(6):1339–45.
Morley S. Randomized controlled trials of psychological therapies 76. Singh TP, Curran TJ, Rhodes J. Cardiac rehabilitation improves
for management of chronic pain in children and adolescents: an heart rate recovery following peak exercise in children with
updated meta-analytic review. PAIN. 2010;148(3):387–97. repaired congenital heart disease. Pediatr Cardiol. 2007;28(4):
60. Linden W, Stossel C, Maurice J. Psychosocial interventions for 276–9.
patients with coronary artery disease: a meta-analysis. Arch Intern 77. Sklansky MS, Pivarnik JM, Smith EO, Morris J, Bricker JT.
Med. 1996;156(7):745–52. Exercise training hemodynamics and the prevalence of arrhythmias
61. Balfour IC, Drimmer AM, Nouri S, Pennington DG, Hemkens CL, in children following tetralogy of Fallot repair. Pediatr Exerc Sci.
Harvey LL. Pediatric cardiac rehabilitation. Am J Dis Child. 1994;6(2):188–200.
1991;145(6):627–30.
78.• Wittekind S, Mays W, Gerdes Y, Knecht S, Hambrook J, Border W,
62.• Rhodes J, Curran TJ, Camil L, Rabideau N, Fulton DR, Gauthier
et al. A novel mechanism for improved exercise performance in
NS, et al. Sustained effects of cardiac rehabilitation in children with
pediatric Fontan patients after cardiac rehabilitation. Pediatr
serious congenital heart disease. Pediatrics. 2006;118(3):e586–93.
Cardiol. 2018;39(5):1023–30. In this prospective study, pediatric
This study is one of few that demonstrated sustained long-term
patients with a history of the Fontan procedure completed a
effects of pediatric cardiac rehabilitation.
cardiac rehabilitation program for 12 weeks. There were sig-
63. Müller J, Christov F, Schreiber C, Hess J, Hager A. Exercise capac-
nificant improvements in both submaximal and peak exercise
ity, quality of life, and daily activity in the long-term follow-up of
performance, with no serious adverse events. The changes were
patients with univentricular heart and total cavopulmonary connec-
thought to be mediated, in part, by more efficient oxygen ex-
tion. Eur Heart J. 2009;30(23):2915–20.
traction and ventilation.
64. Kendall L, Sloper P, Lewin RJ, Parsons JM. The views of parents
79.• Hedlund ER, Lundell B, Soderstrom L, Sjoberg G. Can endurance
concerning the planning of services for rehabilitation of families of
training improve physical capacity and quality of life in young
children with congenital cardiac disease. Cardiol Young.
2003;13(1):20–7. Fontan patients? Cardiol Young. 2018;28(3):438–46. In this pro-
spective study, pediatric patients with a history of the Fontan
65. Hollander SA, Hollander AJ, Rizzuto S, Reinhartz O, Maeda K,
procedure completed an endurance training program. Patients
Rosenthal DN. An inpatient rehabilitation program utilizing stan-
improved in their submaximal exercise capacity and quality of
dardized care pathways after paracorporeal ventricular assist device
life, with gains maintained after 1 year.
placement in children. J Heart Lung Transplant. 2014;33(6):587–
92. 80. Amiard V, Jullien H, Nassif D, Bach V, Maingourd Y, Ahmaidi S.
66. McBride MG, Binder TJ, Paridon SM. Safety and feasibility of Effects of home-based training at dyspnea threshold in children
inpatient exercise training in pediatric heart failure: a preliminary surgically repaired for congenital heart disease. Congenit Heart
report. J Cardiopulm Rehabil Prev. 2007;27(4):219–22. Dis. 2008;3(3):191–9.
67. Brassard P, Poirier P, Martin J, Noël M, Nadreau E, Houde C, et al. 81.• Jacobsen RM, Ginde S, Mussatto K, Neubauer J, Earing M,
Impact of exercise training on muscle function and ergoreflex in Danduran M. Can a home-based cardiac physical activity program
Fontan patients: a pilot study. Int J Cardiol. 2006;107(1):85–94. improve the physical function quality of life in children with
68. Dedieu N, Fernández L, Garrido-Lestache E, Sánchez I, Lamas MJ. Fontan circulation? Congenit Heart Dis. 2016;11(2):175–82. In
Effects of a cardiac rehabilitation program in patients with congen- this prospective study, pediatric patients with a history of the
ital heart disease. Open J Inter Med. 2014;4(01):22–7. Fontan procedure completed a cardiac rehabilitation program
69. Dulfer K, Duppen N, Kuipers IM, Schokking M, van Domburg RT, for 12 weeks. There were improvements in exercise capacity
Verhulst FC, et al. Aerobic exercise influences quality of life of and parent-reported (but not self-reported) quality of life over
children and youngsters with congenital heart disease: a random- time.
ized controlled trial. J Adolesc Health. 2014;55(1):65–72. 82. Longmuir PE, Tyrrell PN, Corey M, Faulkner G, Russell JL,
70. Dulfer K, Duppen N, Blom NA, van Dijk AP, Helbing WA, McCrindle BW. Home-based rehabilitation enhances daily physical
Verhulst FC, et al. Effect of exercise training on sports enjoyment activity and motor skill in children who have undergone the Fontan
and leisure-time spending in adolescents with complex congenital procedure. Pediatr Cardiol. 2013;34(5):1130–51.
heart disease: the moderating effect of health behavior and disease 83. Moalla W, Maingourd Y, Gauthier R, Cahalin LP, Tabka Z,
knowledge. Congenit Heart Dis. 2014;9(5):415–23. Ahmaidi S. Effect of exercise training on respiratory muscle oxy-
71. Duppen N, Etnel JR, Spaans L, Takken T, van den Berg-Emons RJ, genation in children with congenital heart disease. Eur J Cardiovasc
Boersma E, et al. Does exercise training improve cardiopulmonary Prev Rehabil. 2006;13(4):604–11.
fitness and daily physical activity in children and young adults with 84. Moalla W, Gauthier R, Maingourd Y, Ahmaidi S. Six-minute walk-
corrected tetralogy of Fallot or Fontan circulation? A randomized ing test to assess exercise tolerance and cardiorespiratory responses
controlled trial. Am Heart J. 2015;170(3):606–14. during training program in children with congenital heart disease.
72. Duppen N, Kapusta L, de Rijke YB, Snoeren M, Kuipers IM, Int J Sports Med. 2005;26(09):756–62.
Koopman LP, et al. The effect of exercise training on cardiac re- 85. Minamisawa S, Nakazawa M, Momma K, Imai Y, Satomi G. Effect
modelling in children and young adults with corrected tetralogy of of aerobic training on exercise performance in patients after the
Fallot or Fontan circulation: a randomized controlled trial. Int J Fontan operation. Am J Cardiol. 2001;88(6):695–8.
Cardiol. 2015;179:97–104.
80 Curr Phys Med Rehabil Rep (2019) 7:67–80

86. Morrison ML, Sands AJ, McCusker CG, McKeown PP, McMahon pediatric neuropsychologist. Child Neuropsychol. 2018;24(7):
M, Gordon J, et al. Exercise training improves activity in adoles- 859–902. This review paper provides an evidence-based, clini-
cents with congenital heart disease. Heart. 2013;99(15):1122–8. cally oriented primer on CHDs for pediatric neuropsycholo-
87. Stieber NA, Gilmour S, Morra A, Rainbow J, Robitaille S, Van gists. The paper provides an introduction to current
Arsdell G, et al. Feasibility of improving the motor development standard-of-care guidelines for managing children with
of toddlers with congenital heart defects using a home-based inter- CHDs, an overview of brain development within the context
vention. Pediatr Cardiol. 2012;33(4):521–32. of CHDs, a summary of typical neuropsychological outcomes
88. Patel JN, Kavey RE, Pophal SG, Trapp EE, Jellen G, Pahl E. and factors influencing variability in outcomes, and a discus-
Improved exercise performance in pediatric heart transplant recip- sion of the implications of past findings and strategies for clin-
ients after home exercise training. Pediatr Transplant. 2008;12(3): ical care.
336–40. 91. Puri K, Allen HD, Quereshi AM. Congenital heart disease. Pediatr
89. Newburger JW. Improving neurodevelopmental outcomes in chil- Rev. 2017;38(10):471–86.
dren with congenital heart disease: an intervention study. Boston:
Children’s Hospital of Boston; 2018.
Publisher’s Note Springer Nature remains neutral with regard to jurisdic-
90.•• Cassidy AR, Ilardi D, Bowen SR, Hampton LE, Heinrich KP,
tional claims in published maps and institutional affiliations.
Loman MM, et al. Congenital heart disease: a primer for the