With improved surgical and medical care of children with congenital heart disease over the past 50 years, an ever-increasing number of patients have reached adult life and require informed long-term medical follow-up and supportive services for their successful integration into the society of their peers
These adults represent a uniquely favored group who benefited from the dramatic advances in medical and surgical care during the 20th century and who were supported by the intensive response of family, physicians and educational and social organizations to their specific needs. Some of these adults had repeated hospitalizations in infancy and childhood, others overcame complications of infections or of genetic or non-cardiac abnormalities, still others may have mild abnormalities ,benign during childhood but beginning to be aggravated by growth and aging. Thus multidisciplinary challenges need to be met within newly organized specialty services for adults with congenital heart disease or grown-up congenital heart disease.

In the U.S and in Europe Task Forces were appointed by the professional societies which evaluated patient needs and made recommendations for the establishment of long-term multi-specialty care concerned with health, education, vocational preparation, health insurance and supportive programs.

The Task Forces of the Cardiology Societies in the US and in other developed countries made recommendations for future medical practices and research The International Society of for Adult Congenital Heart Disease (ISACCD) was formed and joined with the International Journal of Cardiology for an ongoing presentation of reports on medical and surgical advances, clinical trials, psychosocial, ethical and moral issues ( Coats 03). Success in the transition to adult care was shown to depend on the early emphasis in adolescence on appropriate beliefs about adult care well before the transfer occurs (Fernandez, 2003).

Medical Surveillance

A congenital abnormality of the heart constitutes an indication for close and competent medical care throughout the person's life. This is essential for every person including those in whom the abnormality is mild, requiring no surgery and in all those who have been successfully operated upon in the past. This is important for the following reasons:

• With time and with growth cardiac status may change and conditions, such as arrhythmias (irregular heart beat), heart muscle thickening, or heart failure may supervene.
• Long term post-operative alterations of cardiac tissues, artificial valves, may become aggravated.
• Changes in general health, such as obesity or hypertension may adversely affect cardiac functions.
• Psychosocial status may adversely affect quality of life.
• Changes in life style may involve excessive physical activity.
• Unhealthy habits, such as smoking, drinking or recreational drug taking may add new risks.
• Almost all patients with congenital heart disease are at risk of blood stream infection.
• All patients with congenital heart disease must receive informed counsel regarding reproductive care and the risk of congenital heart disease in their offspring.
• Consistent and unremitting follow-up may lead to early preventive interventions.

Bacterial endocarditis

In the intact circulation bacteria entering the bloodstream are rapidly eliminated and cause no permanent harm. However in the presence of intracardiac abnormalities, such as abnormal valves or septal defects , bacteria will become attached to these sites and cause inflammatory lesions, which in turn may initiate clots and infected emboli traveling to other parts of the circulation with grave consequences . Depending on the infective organisms such blood stream infections may spread rapidly and become fatal, while less aggressive organisms may persist for weeks and months causing progressive damage.

Blood stream infections are preventable . The routine practice of antibiotic therapy for dental work has been conscienciously practiced by dentists and dental technicians, who usually ask ,but are not always correctly advised, by the patient. Silent or undiagnosed abnormalities, such as mitral valve prolapse, are now predominant among patients with blood stream infections.

Special emphasis must be placed on adults with congenital heart disease , many of whom remain at increased risk even after successful surgery. The patients must take responsibility to ascertain their own risk status and advise their dentists and other practitioners accordingly.

Emphasis is usually placed on oral health but it is necessary to remember other potential sources of blood stream infection : some of these result from fad practices in the current young population, such as body piercings, especially when performed by ignorant practitioners. Intravenous drug use presents a specially notable problem .

The implications for the prevention of this grave complication of operated and unoperated congenital heart disease are obvious : the appropriate education of the parents, the adolescent and the continued emphasis on precautions in the adult by all care takers.

Selected References

Reproductive care

Concern with reproductive care of people with congenital heart disease has three components:

• the specific risks of certain contraceptive methods
• the risks of pregnancy in women with congenital heart disease
• the risk of recurrent congenital heart disease in the offspring of men and women with congenital heart disease

The high risks of unprotected sexual activity must be shared with patients as with all others, considering also the added burden of cardiovascular complications which have been experienced with certain contraceptive methods; there maybe increased risk of thrombotic episodes in the use of hormonal contraceptives, of bacterial endocarditis with intrauterine devices, and a special risk in patients with certain diagnoses, such as pulmonary hypertension.

Pregnancy is well tolerated in women with mild defects or in those with corrected defects, but remains elevated in those with persistent circulatory changes which may result in complications and even death of the mother and fetus.(Siu, 2001). A multicenter study in Canada, developed risk assessment criteria valid for the population with uniformly available health care in that country. The risks included rhythm disturbances, stroke, heart failure, hypertension which affected 13% of the 562 women followed (Siu, 2001).

Selected References

Selected Web Resources

• Genetic Counseling for Adults With Congenital Heart Disease - American Heart Association
  
  
• GUCH and Pregnancy - Grown Up Congenital Heart Patients Association (GUCH) - United Kingdom
  
  
• Sex, Pregnancy and Birth Control - American Heart Association
  

The experience of painful diagnostic and surgical procedures as well as exclusion from peer activities can have a lasting effect on many young patients and may extend their concerns into adulthood. Studies of young adults have indicated a great need for supportive vocational development and every effort to maximize their life potential in all possible ways. Regrettably, most patients and their families were unaware of the outstanding support programs mandated in national legislation for persons with disabilities. Consequently these extensive legislated resources were seldom utilized by young adults with congenital heart disease.

It was apparent that the quality of life of young adults with congenital heart disease was greatly dependent on the support received in their earlier years. It is clear then that counselling and social services support must be provided through the medical teams who should follow up each individual in a comprehensive manner, including the access to societal benefits.

As soon as the first generation of young patients with operated heart disease began to grow up their most important question concerned their future parenthood: could they have children and would their children be free of heart disease? This stimulated their physicians to conduct follow-up evaluations which gave cause for concern as the rate of congenital heart disease in the second generation was clearly in excess of that found in the general population (Rose, 1985, Whittemore, 1994). The many individual and even the systematically collected cases in clinic populations have, however, not yet permitted the generalization of results to specific focus groups of interest, by types of cardiac malformations or other predictive factors.

The rapidly advancing knowledge in genetics is beginning to clarify some of the clinical observations regarding the familial occurrence of congenital heart disease. Many more years of research will be needed before we understand the genetic contributions to embryonic development, and, specifically, to the formation of the heart. But it is important, now to conduct a careful genetic evaluation of adults with congenital heart disease, especially those in whom other members of the family have congenital heart disease or any other birth defects, in order to advise them of the risk of recurrence in their offspring. This recommendation is now enhanced with the recent release of the U.S. Surgeon General's Family History Initiative.

In recent years many genetic studies have identified specific genes associated with complexes of abnormalities including congenital heart disease. These horizons of knowledge will greatly expand and emphasize the ever greater need to evaluate the genetic background of adults with congenital heart disease to assess their risk of having children with birth defects. A careful history and genetic counseling must be advised for all patients.

The Surgeon General's Family History Initiative will provide a great impetus to the care and guidance of families with congenital heart disease and will in time unravel crucial predictive information.

Selected References

Success in life depends on education. Historically children with birth defects and other chronic diseases were exempt from going to school to be protected from excess activities and exposure to infections. This seemingly protective attitude imposed a major personal and societal disadvantage for many young persons. In recent years attitudes changed dramatically with supportive legislation for improvements in the life outlook of all children with disabilities.

In the United States legislation provided support for all persons with disabilities to achieve:

• Equal opportunity and civil rights
• Individually appropriate education
• Vocational preparation for successful entry into the work force

See Legislation in Support of Persons with Disabilities

History records prejudice against minority members of society including persons with handicaps who were also once considered as second-class citizens. However in recent years the diffusion of knowledge and understanding diminished this separation from the societal mainstream. In the United States supportive legislation has provided improved opportunities for persons with disabilities in achieving:

• Equal opportunities and civil rights
• Education
• Vocational preparation for successful entry into the work force

Children with congenital heart disease represent a very small proportion of persons in need, but they can greatly benefit from the advances achieved by others with disabilities. Legislation has opened up broad avenues toward normal living and promotes the successful transition from childhood to adulthood. Knowledge of their special status is essential for patients and parents, as well as for their physicians and all other caregivers in order to access the opportunities available.

The philosophy of equal opportunities in education and work was made evident in recent as well as earlier laws.

Americans with Disabilities Act (PL 101-336, 1990) assures equal opportunities and civil rights for all persons with physical and mental impairments. Removing barriers in man-made structures and barriers stemming from peoples' attitudes will make it possible for all persons with disabilities to access and experience all society has to offer.

Legislation concerned with Education

Brown vs. Board of Education 347 U.S. 483,493, (1954) was a historic decision of the U.S. Supreme Court directed toward racial minorities, which also set the stage for equal opportunities for others:

It is doubtful that any child may be expected to succeed in life if he is denied the opportunity of an education. Such an education is a right which must be available to all on equal terms .

Education for all Handicapped Children Act, (PL 94-142, 1975), has been called a BILL of RIGHT for handicapped children as it mandates an Individual Education Plan (IEP) for each student with a disability to participate in the educational programs in the least restrictive setting.

Individuals with Disabilities Education Act. (PL 105-17, 1997). EDLAW, , passed with the purpose of ensuring that the rights of children with disabilities are protected, and to assist the States in assuring the effectiveness of all efforts to educate children with disabilities.

Legislation concerned with Vocational Habilitation

Vocational rehabilitation began with the Soldiers Rehabilitation Act (1918), and the Civilian Rehabilitation Act ( 1920), which were replaced by a permanent commitment in the Social Security Act (1935). It was recognized that self-dependence in the world of work depended on physical restoration as well as education, training and guidance.

The Ticket to Work and Work Incentives Improvement Act (PL 106-170, 1999) provides specialists in every State and a wide range of resources to assist youths and young adults. Remarkable international experiences in study and work could enhance the status and the employability of each participant.

Selected Web Resources

• Americans with Disabilities Act
  
  
• Ticket to Work and Work Incentives Improvement Act
  
  
• Individuals with Disabilities Education Act (IDEA)
  

The challenges and difficulties imposed by chronic diseases is most apparent regarding the ability to obtain insurance:

health insurance: to cover the cost of medical care including physician visits, diagnostic and therapeutic evaluations, hospitalizations, and treatments

life insurance: to protect dependents, an important consideration in building a family

It is in this respect that patients with congenital heart disease, and those with other long-term disorders, encounter considerable disappointments and difficulties.

Health insurance is often tied to the benefits of employment, and employability is often unfavorably judged by employers afraid of increased risks of claims. During childhood and adolescence the patients may be adequately protected by the insurance plans of their parents and they may not expect to have major worries fall upon them as they become independent.

The Cardiology Societies have shared this concern and have helped to negotiate appropriate considerations for their patients. Since the early years of cardiac surgery, cardiologists have undertaken an active role in achieving favorable considerations for patients with mild and those with successfully operated heart disease. However, patients with severe malformations or post-operation complications often encounter difficulties. Social Security disability benefits are available for patients with significant heart disease, but receiving this assistance may prevent the patient from seeking even light employment. Thus, employment and insurability represent some major hurdles confronting many patients.

Selected References

Selected Web Resources

• Insurance, Legal Issues and Advance Directives - American Heart Association
  
  
• Consumer Guides for Getting and Keeping Health Insurance (There is a guide for each state.)- Georgetown University Health Policy Institute
  
  
• Centers for Medicare and Medicaid Services
  
  
• Office of Special Education and Rehabilitative Services - U.S. Department of Education

Adults with congenital heart disease constitute an ever-increasing proportion of patients in cardiac clinics. The life course of each patient represents a joint responsibility of pediatric and adult care givers and both must provide supportive educational, vocational and social support. Socio-economic and educational disadvantages which affect the lives of so many young persons impact with greater force on handicapped children including those with congenital heart disease. Recently a group of Dutch investigators have begun to quantify the problems of psychosocial development (Van Rijen, 2003)

Many cardiac abnormalities are complex deviations from normal and the explanations offered may not be easily understood by the patients' parents or the patients themselves. This may cause real as well as unfounded anxiety affecting the patients' self-perception.

In the Baltimore-Washington Infant Study a study of the parents understanding of their infant's congenital heart disease revealed favorable results for parents with biologic and health care occupations, but much misinformation among the others ( Kaden et al 1985). There is a need for comprehensive health education of the family to improve also the patients' understanding. This would encompass caregivers in virtually all phases of life. Although this would be an extensive challenge it must be viewed in the light of the great investment already made in medical and surgical costs. Optimal personal development must be valued as much as the achievement of an improved circulation.

We have much to learn from the patients. We must assemble specific information on their feelings and problems: cyanosis, the blue color of lips and nail beds due to hypoxia, makes the patient feel different and depressed; the joy on turning pink after surgery is hard to describe! Other physical changes such as puffy face, swollen legs, pacemaker, limitation in activity, may similarly affect the patient's self-esteem. Physicians may not have the answers to these concerns, but must not avoid discussing them and finding supportive care. Reassurance must be realistic and truthful. Outstanding support programs mandated in national legislation may not be known to patients and their families. A study of young adults showed that vocational rehabilitation programs were seldom used by young adults with congenital heart disease, but much more often used by patients with other disabilities such as mental retardation and blindness (Ferencz, 1976).