Health Care Management for the Child with Cardiovascular Disease
Mark Cocalis
The number of people living with congenital heart disease is rising yearly. Approximately 1 in 300 children is born with significant heart disease that requires intervention in the first month of life. In the United States, there are approximately 35,000 children born with heart disease each year. Secondary to advances in surgery, medicine, and intensive care unit (ICU) care, most of these children live into adult life. There are now as many adults with congenital heart disease as there are children. This section will deal with some of the day-today issues that face these patients.
INSURANCE
The financial stresses and strains placed on families with children with congenital heart disease can be enormous. Health care costs vary internationally but in the United States these issues can be challenging and may negatively impact upon care. In the current system, children, adolescents, and some young adults are covered under their parents’ private insurance group contracts. Dependent care continues until age 18, 21, or 25, depending upon such things as academic or dependent status and the parent’s contract with his or her employer. Some insurance contracts will allow young adults with disability to continue under the parent’s coverage. Even with some form of private health insurance, families still face significant financial hardships with the costs of medicines and needed services. Parents and older patients may decide when, where, and how much to work to simply have access to a certain level of insurance. Families without private health insurance may qualify for state or federal programs. There are many regulations for both initially receiving and maintaining eligibility, and the bureaucratic intricacies can be overwhelming. 
One major problem relates to adolescents and young adults as they try to transition to independence. Some will simply need continuous financial help from their parents in regard to medical issues. Others, with more severe lesions who can be classified as disabled, may qualify for Medicare and Social Security Disability (SSI). A Web site that may be helpful is http: www.disabilitysecrets.com/advice.html.
PSYCHOSOCIAL ISSUE IN CONGENITAL HEART DISEASE
Most children with congenital heart disease can expect to live to be adults; as survival has increased, attention has been diverted towards some of the other important aspects of care, including neurodevelopmental, behavioral, emotional, and psychosocial issues. Several studies have shown that although IQ scores of children with congenital heart disease are generally within the average range, they tend to be lower than in age-matched controls. Term newborns with cyanotic heart disease and complex single-ventricle physiology have widespread brain abnormalities before they undergo heart surgery. These abnormalities are not associated with known genetic defects/disabilities that are common in children with congenital heart disease. Children with congenital heart disease tend to have learning difficulties with attention deficit disorder, visual spatial issues, expressive language, or behavior.
The main focus at the toddler/preschool age is to anticipate issues in preparation for school and physical activities. As the children enter school, questions regarding the type of learning strategies and the areas of education that need to be stressed become important. The use of medicines for attention deficit hyperactivity disorder (ADHD) must be scrutinized, as these medicines are sympathomimetic and carry a risk of arrhythmia. This is an excellent time to try to steer a child to the appropriate level of physical activities and sports. The concept of health-related quality of life (HRQOL) has been explored in several studies, and many children and parents rate their quality of life in a similar manner to age and gender-match controls. Interestingly, in some of these studies, the parents of children with congenital heart disease rated HRQOL lower than their children. As many of these children have been through frequent medical and/or surgical procedures, there is a tendency of parents to be overprotective. Although this is certainly a natural reaction as the child grows and develops, this can have psychologically deleterious effects. It is therefore important to solicit information on activities and to avoid unnecessary restrictions. As children age, body image is also a consideration, as surgical scars and other morphologic differences can be associated with a negative impact on self-esteem. Studies have also shown an increased incidence of depressive symptoms in children with surgically corrected congenital heart disease, compared to their age-matched controls.
As children grow into adolescence and adulthood, concerns other than school performance predominate. These include peer relationships, fear of death, employment, insurance, marriage, contraception, the ability to have children, and the risk of transmission of congenital heart disease. Adolescents becoming independent have their own challenges. A study of heart transplant patients, ages 15 to 31, revealed that more than half of respondents admitted to missing medications or follow-up appointments. In a study of young adults (ages 19 and 20) and adolescents (ages 16–18) with congenital heart disease, more than half of the young adults and more than one-quarter of adolescents reported that in the past 30 days they had either smoked cigarettes, used marijuana or other drugs, or had an episode of binge drinking. More than one-third of adults with congenital heart disease have met Diagnostic and Statistical Manual of Mental Disorders Fourth Edition criteria for either a depressive episode or general anxiety disorder. Adolescents and adults with congenital heart disease do not always tell their doctors the extent of their problems or limitations; in fact, they tend to be ambivalent. One study concluded that they have a strong wish to be healthy, and they may hide their symptoms from the healthcare personnel and sometimes even from themselves. 
Specific screening and testing for children with known heart disease will be covered under the section “Exercise Considerations in Congenital Heart Disease.” In addition, this section will focus more on the high school and college athletes including club sports, as sports participation at this age and level is truly competitive and training is pushed beyond normal endurance. The section will also be limited to cardiac issues and will not include hemoglobinopathies (sickle cell variants), clotting abnormalities, pulmonary causes, or neurologic causes such as cardiovascular accidents (CVAs), and seizures.
MANAGEMENT CONSIDERATIONS IN HEART DISEASE
PREGNANCY AND CONTRACEPTION
Risk stratification for maternal outcomes for pregnancy are limited. Table 500-1 provides our best current practical guidelines for counseling patients regarding risks. Collaborative management by a pediatric cardiologist, or adult cardiologist with experience in congenital heart disease, and a perinatologist is optimal.
Prevention of pregnancy with steroidal birth control increases the risk of thromboembolic events and is contraindicated in patients with residual right-to-left shunts, pulmonary hypertension, and single ventricles with passive pulmonary blood flow. Alternative approaches should be utilized.
EXERCISE
Physical exertion at some level is beneficial for almost all people, including those with congenital heart disease. The goal is to balance the beneficial effects of increased cardiac work associated with exercise with the risk of disease progression or serious injury and death, which may occur. Making recommendations for the type or extent of physical activity for children with congenital heart disease is quite difficult and secondary to the heterogeneity within defects. In addition, secondary to changes in surgical techniques and the timing of interventions, there is a lack of data in making recommendations for various lesions for which we have not necessarily had enough time to know the long-term risks.
The 36th Bethesda Conference, published in 2005, deals with eligibility recommendations for competitive athletes with cardiovascular abnormalities.1 As such, it does not deal with recreational activities or physical education at school. Generally, sports are broken down into categories based upon the amount of dynamic and static activity (eTable 500.1 
). Restrictions for patients with congenital heart disease vary depending on the actual increase on myocardial oxygen demands and the physiologic stress placed on the underlying cardiac conditions. Exercise activities are also classified as to whether there is a collision risk or increased risk secondary to dizziness or momentary loss of coordination. For instance, patients with pacemakers and patients who take anticoagulation agents such as Coumadin or enoxaparin will have restrictions that are not related to myocardial demands.
Patients with cyanotic heart lesions usually restrict themselves, because they become more cyanotic with increasing physical effort. They may participate in physical education at school but must be allowed to rest or stop, and they should not be forced or induced to do any competitive activity. Most children with repaired lesions can participate in all sports. The timing after repair will depend on the type of surgery or intervention. Patients with atrial and ventricular septal defects that are repaired surgically must wait 3 to 6 months before full participation in sports. They should have an echocardiogram and EKG to make sure that they do not have any arrhythmias, pulmonary hypertension, or ventricular dysfunction. Small atrial septal defects/patent foramen ovales and ventricular septal defects with QP/QS ratios of less than 1.5:1 are not always repaired. These patients are cleared for all activity, except for patients with atrial septal defects and patent foramen ovales who should not scuba dive, secondary to the risk of embolic stroke. Moderate-to-large atrial septal defects, ventricular septal defects, and patent ductus arteriosus are usually repaired either by surgery or interventional catheterization once they are found in children old enough to compete competitively (approximately age 12 years). They can participate in routine exercise, as long as they are allowed to rest and limit their exertion until they are repaired.
Table 500-1. Pregnancy Risk Stratification
