Trends in Congenital Heart Disease

Abstract

Congenital heart disease (CHD) is the most common class of major congenital malformations. Although there is slight variation between many population-based studies, CHD occurs in ≈1% of live births, with similar prevalence throughout world,1–3 and in 10% of aborted fetuses.4 It is also the leading cause of mortality from birth defects.5 Approximately one-quarter of the 40 000 children born with CHD annually in the United States require intervention in the first year of life. In the 60 years since the first successful repair of a congenital heart defect using cardiopulmonary bypass in 1953, the accurate diagnosis and effective treatment of even the most complex congenital heart lesions have become standard practice. Critical CHD is often lethal in the absence of treatment, and effective surgical, catheter, and medical therapies have dramatically extended life expectancy. With advances in the survival of patients with CHD, there have been major demographic shifts, so that adult patients with CHD now outnumber children even with complex forms of CHD.6 The expected surgical outcomes for patients with simpler congenital cardiovascular lesions (eg, typical forms of septal defects) have continually improved7 and have long since reached a very high level of excellence. These procedures can now be reasonably viewed as a commodity, which is widely available in the developed world. In treatment of simpler lesions, research is increasingly focused on methods to increase the safety, efficiency, and value of therapeutic procedures, and to provide high-quality alternatives to children in nations with resource-constrained healthcare infrastructure and budgets, as well. On the other end of the spectrum of disease severity, outcomes continue to improve in patients with more complex lesions (Figure 1).8 Survival through childhood is now common even in the most complex and lethal malformations, such as hypoplastic left heart syndrome. …