Adolescents with congenital heart disease (CHD) are a rapidly growing population with complex medical needs and psychosocial challenges. Identity formation is an important developmental task accomplished during the teenage years. This article reviews different aspects of ongoing care that pertain to teenagers with CHD, with a particular focus on primary care issues and a summary of recommendations from various scientific societies. A successful smooth transition to the adult health care setting should achieve 2 important goals: to prevent loss of follow-up and to foster and encourage self-care behaviors.
Key points
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Adolescents with CHD are a rapidly growing population.
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They require lifelong cardiac and noncardiac care.
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Identity formation is a core developmental task during the teenage years.
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Special attention to psychosocial issues is required to prevent maladaptive outcomes during this vulnerable period.
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A seamless successful transition process is central to prevention of patients lost to follow-up and to foster self-care behaviors.
Introduction
Rapid advancements in medical and surgical sciences have resulted in an increased number of patients with congenital heart disease (CHD) entering teenage and adulthood. CHD is the most common birth defect in United States and the incidence of CHD is approximately 9 per 100,000 live births. Each year, 40,000 babies are born with CHD in the United States and 85% to 90% of these patients are surviving into adulthood; the total number of adults with CHD in the United States has exceeded 1,000,000. For the first time in the history of medicine, adults with CHD outnumber pediatric patients with CHD. A 400% increase in adult CHD outpatient workload was reported in Canada.
With these statistics in mind, it is inevitable that pediatricians will provide care to this special and complex group of patients during their teenage years. Because teenagers may be vulnerable in many different ways, even in the absence of a disease, the presence of a chronic disease such as CHD poses an extreme challenge for health care providers. The goal of care for patients with CHD during their adolescence is to best prepare them to deal with the issues that may arise now or in the future. Critical to this process is a successful transition from a pediatric to an adult health care setting, both in terms of primary care and specialized care. As stated by the American Academy of Pediatrics, “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.”
This article reviews the unique issues pertaining to teens with CHD that a pediatric health care provider should be familiar with when taking care of these patients to ensure a smooth transition into adulthood. Details of individual cardiac lesions are not discussed in this article. To understand how chronic illness such as CHD can affect developmental milestones, awareness of normal developmental milestones is necessary. All teenagers with CHD require primary care and ongoing specialized cardiac care. As defined by the American Academy of Pediatrics’ medical home policy statement, patients with chronic lifelong health care needs should have easy access to a “medical home” where they receive primary, family-centered care. This role can be played by pediatricians, family practitioners, or internal medicine-pediatric experts. When these patients are cared for by family practitioners or internal medicine-pediatric experts, it reduces the total number of physician transfers that they have to go through in their lifetime. Expertise in adolescent medicine might be beneficial in dealing with general teenage issues in addition to issues specific to CHD and allow for a smooth transition into adulthood.
Introduction
Rapid advancements in medical and surgical sciences have resulted in an increased number of patients with congenital heart disease (CHD) entering teenage and adulthood. CHD is the most common birth defect in United States and the incidence of CHD is approximately 9 per 100,000 live births. Each year, 40,000 babies are born with CHD in the United States and 85% to 90% of these patients are surviving into adulthood; the total number of adults with CHD in the United States has exceeded 1,000,000. For the first time in the history of medicine, adults with CHD outnumber pediatric patients with CHD. A 400% increase in adult CHD outpatient workload was reported in Canada.
With these statistics in mind, it is inevitable that pediatricians will provide care to this special and complex group of patients during their teenage years. Because teenagers may be vulnerable in many different ways, even in the absence of a disease, the presence of a chronic disease such as CHD poses an extreme challenge for health care providers. The goal of care for patients with CHD during their adolescence is to best prepare them to deal with the issues that may arise now or in the future. Critical to this process is a successful transition from a pediatric to an adult health care setting, both in terms of primary care and specialized care. As stated by the American Academy of Pediatrics, “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.”
This article reviews the unique issues pertaining to teens with CHD that a pediatric health care provider should be familiar with when taking care of these patients to ensure a smooth transition into adulthood. Details of individual cardiac lesions are not discussed in this article. To understand how chronic illness such as CHD can affect developmental milestones, awareness of normal developmental milestones is necessary. All teenagers with CHD require primary care and ongoing specialized cardiac care. As defined by the American Academy of Pediatrics’ medical home policy statement, patients with chronic lifelong health care needs should have easy access to a “medical home” where they receive primary, family-centered care. This role can be played by pediatricians, family practitioners, or internal medicine-pediatric experts. When these patients are cared for by family practitioners or internal medicine-pediatric experts, it reduces the total number of physician transfers that they have to go through in their lifetime. Expertise in adolescent medicine might be beneficial in dealing with general teenage issues in addition to issues specific to CHD and allow for a smooth transition into adulthood.
Approach to a teen with CHD: role of a primary provider
Teenagers with CHD may have various cardiac, noncardiac medical and surgical needs because of coexisting diseases or anomalies. Good record keeping and coordination of care with the various subspecialists is central to optimal primary care. It is crucial that there be a strong mechanism of communication between the cardiologist, the primary care provider (PCP), and any other specialists involved. The PCP should take charge of their patients’ care, prevent fractionated care, and involve the family members and the patient in medical decision making with ongoing education. Updated, accurate personal health information documented in the format of a passport is not only beneficial for the health care providers in various settings but may also improve patient knowledge and understanding about their heart disease. Providers should be aware of the patient’s diagnosis, anticipated and ongoing issues, current medications, baseline functional status, and current recommendations regarding exercise and endocarditis prophylaxis, which are discussed later in this article. This knowledge enables them to take care of their patients appropriately when they report any new symptoms or concerns. Information about current medications help to guard against potential drug interactions if they are on multiple medications or if a new drug is prescribed. The American Heart Association (AHA) Scientific Statement regarding “Best practices in managing transition to adulthood for adolescents with CHD” lists all of the health maintenance needs of a patient with CHD that a PCP may handle including vaccinations, cholesterol screening, hypertension screening, cancer screening at appropriate ages, assessment of tobacco, alcohol and or drug use, nutritional counseling, contraception, and sexuality issues. It is important that all primary care givers spend some time alone with adolescent patients discussing various issues unique to this age group. Lapse in cardiac care is likely to occur during the transition from adolescence to adulthood and may occur despite being in contact with health care. For compliant teenagers, the rate of retention by a pediatric cardiologist is surprisingly high. A primary provider can play an active role in transitioning this group of patients from the pediatric to adult care setting in a seamless manner as opposed to an abrupt transfer. During transition, it is important that there is a period of overlap of care between the 2 kinds of services (pediatric and adult). Ideally, the transition of primary care and subspecialty care should not occur at the same time.
Compliance Issues: Lost to Follow-Up
Most adults with complex CHD are either lost from specialized cardiac care or are taken care of by cardiologists with no CHD expertise and training. Less than 30% of adults with CHD are seen by specialized providers. In 2004, Reid and colleagues reported that 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 important for successful transition. Unfortunately, this component of discussion and patient education is often missing. The other likely reasons for lost to follow-up may be the false perception that the heart has been fixed. which is supported by a lack of symptoms or a lack of knowledge about the need for follow-up. This issue can be further aggravated by insurance barriers. Wacker and colleagues, in their study of 10,500 German patients, reported lack of follow-up at a specialized adult CHD center in a 5-year period in 76% of their patients. Yeung and colleagues cited the most common reason for lack of follow-up was the message “no need to follow up.” They also report an association between lapse of medical care and adverse outcome. The American College of Cardiology (ACC)/AHA 2008 Guidelines on the “Management of Adults with Congenital Heart Disease” recommends that the transition process for patients with CHD begin at 12 years of age to prepare the patient for transfer to adult care. The lost to follow-up concept is not only seen with cardiac care but also for primary care. As the need for immunizations and other health-related issues decline in this age group, it is easier for patients to fall out of the system. Some patients and families may perceive their cardiologist as the primary care giver potentiating noncompliance with PCP visits.
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All patients with significant CHD (repaired or unrepaired) need lifelong care and follow-up with a CHD specialist and primary provider
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Good record keeping and coordination of care with all the specialists involved is central to optimal care
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Adolescent patients are likely to fall out of the medical system at some time during the teenage years for several reasons
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Lack of symptoms, lack of knowledge about the need for follow-up, and lack of insurance results in high rates of lost to follow-up
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Ongoing patient education and discussion regarding the importance of follow-up might improve lost to follow-up rates
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Both the primary provider and cardiologist can be instrumental in improving compliance
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The ACC/AHA recommend that the transition process whereby patients are handed over from the pediatric to adult care setting should start at 12 years of age
Interventions
Cardiac interventions and reinterventions
The number of unrepaired adolescent patients with significant CHD is steadily declining particularly in developed countries. Late diagnosis may be encountered in some cases. However, most adolescents with moderate to severe CHD have already gone through a surgical intervention. Therefore, reoperations are more common than primary repair in this age group. Monro and colleagues report a 15% incidence of reintervention up to 26 years after the first operation with a 20-year survival rate of 86%. However, the incidence of reoperation varies based on the specific cardiac lesion, type, and age at repair. The perioperative issues encountered in adolescents and young adults during cardiac and noncardiac surgeries are discussed by Mott and colleagues. Optimal management of other coexisting conditions and patient education is central to prompt recovery. In those patients diagnosed with CHD for the first time, referral should be made to a tertiary care center after careful consideration of the expertise of the cardiac surgeon for that particular CHD and the adequacy of postoperative care at that particular institution.
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Reinterventions are more common during adolescence compared with primary repair
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For primary repair, referral to a tertiary care center with a congenital surgeon and CHD cardiologist is optimal
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Optimal management of other coexisting medical conditions is crucial for prompt recovery
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Sensitivity to cosmetic concerns regarding the surgical incision is particularly important in this age group
Interventional/Device therapy
Catheter-based interventional therapy has undergone tremendous advancement in the last 2 decades. Most of the simple shunts such as atrial septal defect, patent ductus arteriosus, and selected ventricular septal defect can be repaired primarily by the transcatheter route. Coarctation, valvular, conduit, or baffle stenosis and branch pulmonary artery stenosis can be repaired percutaneously in many instances. Transcatheter pulmonary valve therapy is now approved for use in children and adults who have failing pulmonary valve conduits with promising initial results. Arrhythmia is a frequent finding in postoperative patients during adolescence and adulthood. Serious arrhythmia may lead to heart failure, exercise intolerance, thromboembolic complications, or even sudden cardiac death. The risk of sudden cardiac death is higher in this group of patients compared with healthy adolescents. Silka and colleagues reported 25 to 100 times greater risk of sudden cardiac death in a postoperative patient with CHD compared with age-matched controls with a higher risk for cyanotic CHD and after the second decade.
With advances in electrophysiology, a range of treatment options are now available including ablations and device therapy. Contact sports such as tackle football, judo, karate, and wrestling should be avoided in anyone with a device or a pacemaker. Additional restrictions or limitations may be specific to the underlying CHD and should be discussed with the cardiologist. Patients with implanted metallic devices may require magnetic resonance imaging (MRI), which requires knowledge of the current recommendations. The AHA and the American College of Radiology (ACR) have issued guidelines stating that a careful risk-benefit ratio must be established and that cardiac implantable devices remain a relative contraindication to MRI. In 2011, the US Food and Drug Administration (FDA) approved the first cardiac pacemaker designed to be used safely, but conditionally, during MRI examinations. Keeping detailed records of the implanted metallic devices in individual patients is helpful for those who may require MRI in the future.
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The risk of sudden cardiac death is high in teenage patients with CHD compared with healthy adolescents
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Careful and judicious use of intervention and device therapy might lower this risk to some extent
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A variety of cardiac interventional procedures and device therapy are now available
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Contact sports should be avoided by those with devices
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Pacemakers are relative contraindications to an MRI study
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Newer pacing systems have been specifically designed that can safely be used in the MRI environment
Noncardiac surgeries
Teenagers and adults with CHD undergoing noncardiac surgery may be at increased risk of complications associated with the procedure. This risk varies by individual cardiac lesion and associated findings. The ACC/AHA 2008 guidelines for management of adults with CHD classify congenital cardiac lesions as high and moderate risk based on their perioperative risk during a noncardiac surgery. The risk of complication is highest when the procedures involve the respiratory or neurologic system. Careful preoperative planning is necessary involving the cardiologist, cardiac anesthesiologist, surgeon, and the PCP. The objective of a preoperative evaluation is to identify the major risk factors and then modify them in an attempt to reduce the surgical risk. A detailed history, including any previous experiences with anesthesia and surgery, is valuable and a thorough physical examination is warranted. Baseline laboratory parameters such as a complete blood count, coagulation profile including bleeding time, renal function tests, and liver function tests when indicated should be obtained. For female patients, a pregnancy test should be performed when applicable. Warner and colleagues report major perioperative risk factors such as cyanosis, treatment of congestive heart failure (CHF), poor general health, pulmonary hypertension, and younger age. Preoperative phlebotomy in certain cyanotic patients with hematocrit more than 65% may be beneficial. CHD patients undergoing surgery should receive antibiotics for prevention of infective endocarditis based on recommendations made by the cardiologist as suggested by the revised AHA guidelines. Pacemaker interrogations should be performed before and after the surgical procedure. Pacemakers can be programmed to an asynchronous mode during the procedure to avoid interference from cautery devices. Adequate fluid management before, during, and after the operation is crucial. Optimal mechanical ventilation, appropriate pain management, early mobilization, and pulmonary toilet are also important aspects of postoperative care. Patients should be restarted on cardiac medications at the earliest possible time. Patients on anticoagulation require substitution with unfractionated heparin depending on the underlying CHD, with prompt resumption when possible. In-line filters should be used for intravenous lines to decrease the risk of air embolism especially in patients with right to left shunts.
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There is increased risk of complications in patients with CHD during noncardiac surgery compared with healthy adolescents
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A team approach between primary provider, cardiologist, anesthesiologist, and surgeon with involvement of the patient is optimal
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Preoperative laboratory assessment and pregnancy test should be done when applicable
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Patients should receive endocarditis prophylaxis when indicated as per revised AHA guidelines
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Need for pacemaker interrogation should be assessed when applicable
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Oral anticoagulation should be substituted with unfractionated heparin based on the procedure and underlying CHD
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Cardiac medications should be restarted at the earliest
Psychosocial Issues
Adolescence is a vulnerable period in many different ways. During this time, teenagers develop self-esteem, self-identity, and autonomy, and hence their self-image. In patients with chronic illnesses such as CHD, the issues of adolescence such as puberty, autonomy, self-esteem, personal identity, sexuality, education, vocational training can be complicated because of their need to cope with their disease. Coping or living with CHD includes many psychosocial factors in addition to the ongoing cardiac surveillance, hospitalizations, medications, reinterventions, and many others. It is important that there is an understanding of the whole patient with CHD as implied by Kovacs and Verstappen. The medical demands of a chronic illness may disturb the adolescent’s self-image, self-esteem, and delay the completion of normal developmental tasks. The Society of Adolescent Medicine emphasizes the importance of addressing the medical, psychosocial, and educational or vocational needs during the transition process for adolescents with chronic illness. Many of these teens feel different from their peers because of their CHD and concerns about their body image play an important role in their social functioning. Their social functioning seems to strongly correlate with their psychological functioning. Social factors likely to affect the quality of life most in the future are family, friends, education, or career. Studies have shown that there is no relation between the severity of the cardiac defect and psychological adjustment or any other demographic factors. Data regarding the incidence of emotional and behavioral issues during the teenage years with CHD is conflicting. It varies from reports of no significant psychological issues to reports of increased incidence of anxiety, diminished self-esteem, depression, increased dependency, and poor social and emotional adjustment. A meta-analysis of 11 studies showed increased risk of internalizing problems such as depression and anxiety and externalizing behavior issues such as aggression and hyperactivity compared with healthy adolescents. Many studies report a higher likelihood of psychological issues in adults with CHD compared with their healthy peers. Psychiatric disorders such as mood or anxiety disorder may be frequent; as many as 1 in 3 patients with CHD meet diagnostic criteria for a psychiatric disorder. Only a small number actually receive mental health treatment. A single institution study published by Spijkerboer and colleagues looked at psychological outcomes before 1980 and a decade later and reported no improvement in psychological outcomes with improving medical outcomes and surgical techniques. The adolescents with CHD are less likely to smoke, drink alcohol, or use illicit drugs and are less likely to be sexually active. This might come as a good news because the negative implications of these high-risk behaviors particularly in this group are well known; however, it might highlight another problem: their social unacceptability, low self-esteem, fear of rejection, and fear of forming close relationships. Their social unacceptability is also reflected by the discrimination and bullying they experience from their peers and the reported feelings of anger, exclusion, and embarrassment.
This highlights an important aspect of care for adolescents with CHD. Rapid progress is being made with the diagnosis and interventions for CHD, but are the psychosocial issues that come as a package with these patients being recognized and dealt with? Lip and colleagues discuss the role of psychological interventions for this group. The clinicians providing care to this group of patients must be familiar with the effect and interplay of the various psychosocial factors in their lives. Primary providers are usually at the forefront and share the responsibility to recognize and manage these issues with the cardiologist, using all available resources.
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The development of self-identity, self-esteem, and self-image can be complicated in patients with chronic illness
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Coping with CHD may include several psychosocial factors in addition to the medical issues
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The Society of Adolescent Medicine emphasizes the importance of addressing the medical, psychosocial, and educational or vocational needs during the transition process for adolescents with chronic illness
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No relationship has been shown between the severity of the cardiac defect and psychological adjustment
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The primary provider and the cardiologist must be familiar with the various psychosocial issues and be able to recognize and manage these using all available resources
Neurodevelopmental issues
Children and adolescents with complex CHD are at increased risk for developmental disorders including learning difficulties, attention-deficit hyperactivity disorder, and delay in areas of language and fine and gross motor functions. The complexity of the CHD is directly proportional to the prevalence and severity of the developmental issues. Many genetic syndromes are associated with CHD and developmental issues. Teenagers 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. It is possible that a developmental disorder may manifest itself for the first time during adolescence. As the educational curriculum advances and the expectations from a teen rises, some of the developmental disorders may now be unmasked. It is crucial to monitor the developmental progress of adolescents with CHD closely during teenage years. In a recent Scientific Statement put forth by the AHA, and approved by the American Academy of Pediatrics (AAP), periodic developmental surveillance, screening, evaluation, and reevaluation are recommended for children with CHD who are at increased risk for a developmental disorder. A list of specific cardiac lesions at high risk for developmental issues and an algorithm for surveillance, screening, and management is included in this Scientific Statement. The use of such an algorithm may result in improved identification of developmental issues, allowing for appropriate therapies and education to improve their academic, behavioral, psychosocial, and adaptive functioning. A systematic assessment for risk factors and age-specific developmental progress should be incorporated into the annual well-child visits. Based on this algorithm, adolescents suspected to have developmental issues during screening should be referred for further evaluation and care by a behavioral or mental health specialist, along with ongoing care by the PCP in the primary care medical home.
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Children and adolescents with CHD are at increased risk for developmental disorders including learning difficulties, attention-deficit hyperactivity disorder, and delay in areas of language and fine and gross motor functions
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A teenager with CHD may exhibit signs of a developmental disorder for the first time during the teenage years
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The AHA Scientific Statement (2012) proposes an algorithm for health care providers involved in taking care of patients with CHD in an attempt to optimize the neurodevelopmental outcomes
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A systematic assessment of risk factors for developmental issues should be incorporated in the routine preventive visits for patients with CHD
Knowledge About Heart Disease
Educational intervention
Adolescents are encouraged to take charge of their health and life. Ongoing education is central to this process of transfer of responsibility from parents to the patients themselves. They should receive detailed information regarding their cardiac diagnoses and surgical repair with the help of a diagram or a heart model. They should be familiar with their medications, need for endocarditis prophylaxis, preferred lifestyle, and recommendations about contraception and child bearing. The Alliance for Adult Research in Congenital Cardiology (AARC) investigators along with Adult Congenital Heart Association (ACHA) recently demonstrated the benefits of a focused educational intervention and an improvement in the knowledge gap of adult patients with CHD. Because all educational topics cannot be discussed in 1 visit, Kovacs and Verstappen suggest an educational checklist to be placed in the patient’s chart so that all topics are covered at some point during adolescence. Parental education should emphasize the benefits of transition of responsibility because it is not unusual to see parental overprotection.
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Patients and families should be aware of their diagnosis and details of the repair and anticipated issues
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Need for lifelong care for patients with moderate and complex CHD should be emphasized by the PCP and cardiologist
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An educational check list can be placed in the chart and used to keep track of the various topics that require discussion during adolescence
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Parental education should occur with emphasis on transferring responsibility to the teenager
Exercise recommendation
Obesity is a major problem in the pediatric age group and the problem is further intensified in patients with CHD. According to the Scientific Statement published by the AHA “exercise is an important component of quality of life (QOL) and health maintenance.” Physical activity is beneficial for overall good health including cardiovascular health and particularly in adolescents with CHD. Good exercise habits developed in adolescence can help promote these benefits. Despite the potential benefits, exercise and activity levels are often not discussed by the cardiologist and primary provider. Patients with CHD may even be discouraged from exercise and may lead a sedentary lifestyle because of personal, parental, or school personnel fears. To allay these fears, the recommended level of activity should be discussed and patients with CHD should be encouraged to carry out activities within that range. It is the responsibility of the PCP and the cardiologist to provide patients and families with a clear message regarding exercise. The AHA has published guidelines to assist specifically with the recommendations for sports participation in patients with CHD and those with genetic heart disease. The Association for European Pediatric Cardiology recommends “a daily participation in 60 minutes or more of moderate-to-vigorous physical activity” for all children with CHD.
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Health benefits of being active should be discussed with the teen during each visit
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Adolescents with CHD should be encouraged to stay active within a prescribed range of activities
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Patients should receive a clear message regarding their recommended level of activity
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The AHA has published guidelines for sports participation for patients with CHD
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