In developed countries the expanding scope of pediatric cardiology has achieved a dramatic improvement in outlook for infants with congenital heart disease.

Critical advances include:

• Continuing improvement of diagnostic capabilities with the use of ultrasound technologies in the fetus and newborn infant permitting early diagnosis and case-specific management
• High standards of infant care in regional hospitals with designated standards of resuscitative equipment
• Neonatal transport systems to take sick babies to defined institutions where cardiac therapies can rapidly be instituted
• A notable change in philosophy from the early fear that an infant was "too small" or "too sick" to move to prompt action in transfer to initiate diagnostic improvements with gratifying results (Rowe 1981)
• Surgery is performed in a high proportion of infants with severe cardiac anomalies:
  • "palliative" surgery offers relief of major circulatory disturbances
  • "definitive" surgery attempts to restore the cardiac anatomy to normal

In the Baltimore-Washington Infant Study infants born in the decade 1981-1989 were followed until their first birthday. In that time period one third of the cases (34%) underwent cardiac surgery. The need for re-operation during childhood remains a possibility for many.

In the developing world a great public health challenge still exists. Most infants with congenital heart disease do not survive the first weeks and months of life. Death comes as a result of hypoxia (blue babies), congestive heart failure aggravated by anemia and infections such as pneumonia superimposed in pulmonary congestion. In the absence of adequate diagnostic and treatment facilities these little patients succumb to complications for which treatments are available elsewhere sometimes even in nearby medical centers. The Association of Children with Heart Disease in the World began to confront this need establishing collaborative treatment facilities locally and by visiting consultants. (Giamberti, 2004)

Selected References

The neurologic and cognitive development of infants with congenital heart disease is receiving increased attention in recent years. Several concerns must be addressed:

• Are the abnormalities of the heart and circulation associated with abnormalities of the central nervous system?
• Are behavioral abnormalities the result of hypoxia or secondary to the invasive diagnostic and surgical procedures in early life ?
• Does the parental anxiety surrounding the infant during the crucial neonatal period result in undesirable consequences?

Until recently the methodologic problems in the behavioral evaluation of infants were not adequately developed or tested, so that these questions have only recently been systematically evaluated.The results of these systematic investigations suggest an effect of each of the above possibilities. However, we need to look to the future to assemble adequate data regarding the origin of neurobehavioral anomalies. There should however be no delay in recognizing the clincial and educational needs of these young patients.

Selected References

It is difficult to grow up as normally as possible and at the same time acquire a balanced view of oneself as a patient with a chronic condition who needs to be vigilant of many medical and life style constraints.

Public funding programs, such as the Crippled Children's Program, were the first to raise questions about the eventual outcome of early treatments in terms of patient behavior and disease prevention. An early study (Artis, 1974) was directed to 102 young men who had been patients in a Children's Hospital with rheumatic or congenital heart disease. It was distressing to find that they were quite ignorant of their own medical status and also failed to be aware of available supportive community programs.

Adult patients often report discrimination in employment and insurance. Was it possible that the patients themselves contributed to these difficulties by mistaken impressions of their own cardiac status? Another study of 131 patients, aged 14-21 years demonstrated serious gaps in their health knowledge with important implications for medical and social risks (Ferencz, 1980)

Now twenty years later new studies show the same uncertainty and lack of preparation for long term preventive behavior (Kantoch, 1997, Moons, 2001, Dore, 2002).

It is clear that the nature of one's own cardiac defect is not properly understood by the patients, nor, probably by their parents. This calls for more concentrated efforts in patient education: the intelligent guidance of patient and family has to become a clearly defined responsibility of the medical, genetic and rehabilitative staff who work to bridge the gap between pediatric, adolescent and adult services.

Selected References

Pediatricians have always been concerned about the loss of systematic medical attention to young patients as they enter adolescence and increasingly take it upon themselves to avoid the routine follow-up care they had received as children. For patients with congenital heart disease concern is greatly heightened for fear of the occurrence of preventable complications. Some adolescents are eager to prove their physical capabilities and engage in activities that were previously disallowed. Others discontinue the care of dental hygiene and may become at risk of bacterial endocarditis following dental procedures by new practitioners unaware of their medical history.

A major concern is depression and emotional problems that affect growing children with chronic diseases in the absence of intensive social support. Clinical experience has shown that adolescents rarely confide in their parents regarding their feelings of inferiority in their school and recreational settings. There are few systematic studies of the indicators of emotional problems and very little guidance on their management. Pediatric cardiologists tend to be very encouraging to their patients, but it has long been said that the patients soon find out that "doing well" is "not good enough."

A recent survey by the Cochrane Review (Lip, 2004) was unable to identify any systematic research in this important field in spite of the various forms of pharmacologic and counseling therapies that are available.

The outreach of the new clinics for adults and adolescents with heart disease must therefore have a strong emphasis on preventive guidance of the patients and their families.

Selected References