Augusta University
Cardiology

Lifelong strategy for patients with congenital heart disease

Anastasios C. Polimenakos, MD, FACS, FACC Director, Pediatric and Congenital Heart Surgery

Congenital heart defects are the most common birth defects. Of all congenital conditions, heart defects account for the highest resource utilization among U.S. pediatric hospitalizations. In the earliest era of cardiac surgery, operations on children with congenital cardiac malformations were landmark achievements that heralded the birth of pediatric heart subspecialties. What followed was a period of remarkable evolutionary steps in understanding the circulatory physiology, abnormal morphology and functional consequences of structural congenital heart disease.

Innovative interventions have saved and prolonged lives, enhancing quality of life as well for those with congenital heart disease. The rapid progress in surgical therapy has been just one element of a broader picture that also includes less-invasive and more-informative diagnostic modalities, catheter-based interventions, “hybrid” collaborative strategies, the emergence of pediatric cardiac critical care and the evolution of multidisciplinary efforts with cell biologists and translational research scientists.

Congenital heart defects are now surgically corrected as soon as possible after birth, avoiding the consequences of impaired circulation. Malformations once widely considered to be uniformly fatal (hypoplastic left heart syndrome and related anomalies, for instance) are now successfully addressed. An estimated 85 percent of patients born with congenital heart disease in the United States are projected to survive into adulthood. The number of adults with congenital heart disease now exceeds the number of children with the condition.

These astonishing improvements in saving and prolonging the lives of those born with congenital heart disease come with the price of the lifelong challenges that accompany the longevity. Clearly, both short-term and long-term outcomes have improved substantially and continue to improve for even the most challenging forms of congenital heart disease. It is nonetheless apparent that long-term survival may require not only multiple surgeries, but catheter-based interventions to relieve obstructions, implant devices, and treat dysrhythmias. Managing adults with congenital heart disease will require support at a national level.

The success story of saving and prolonging the lives of those born with congenital heart disease requires acknowledgment of lifelong implications for the patients’ well-being. We must adapt to ensure that congenital heart disease patients of all ages have access to appropriate professional expertise and care.

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