Congenital Heart Disease
The Remarkable Journey From the “Post-Mortem Room” to Adult Clinics
The progress in congenital heart disease (CHD), since the first anatomic depiction of an anomalous pulmonary venous return by Leonardo da Vinci in 1513, has been unabated. The course has been decorated with occasional sparks stemming largely from the ingenuity of pioneering physicians and surgeons who departed from the prevailing notions and brought forth new brave ideas to diagnosis and management of patients with CHD (Table). The progress, however, was not without frequent disappointments. The extremely high mortality rate in the early days of open-heart surgery with the use of extracorporeal circulatory machine disappointed John Gibbons, the inventor of the first heart–lung machine, to the point that he called for a long-term moratorium on the use of his invention. Yet, the convictions of pioneering surgeons such as C. Walton Lillehei and John Kirklin led them to forge ahead with improved versions of the pump, ultimately changing the treatment of CHD. Advances in diagnostic methods and surgical treatment during the past several decades have transformed CHD malformations from being “hopeless futilities” in neonates, infants, and children to treatable conditions with >90% of patients surviving into the adult years.
Of the ≈125 million children born every year (www.unicef.org), it is estimated that about 1 000 000 to 1 500 000 are born with a CHD. Because of the scientific and technological advances during the past several decades, the vast majority of these children survive to adulthood, and those with less complicated lesions expect a life expectancy similar to that of the general population. Consequently, it is estimated that worldwide ≈50 million adults live with CHD, including ≈1.5 million in the United States alone, more than the number of children with CHD. As a result of an excellent survival rate, the landscape of CHD …