Heart Failure in Adult Congenital Heart Disease

Advances in medical and surgical care have resulted in significant improvement in survival of patients with congenital heart disease. Much of the definitive, corrective surgery was developed in the 1970s.With over 80% of children with congenital heart disease (CHD) expected to live beyond age 40, a large, population of adults with CHD has emerged. Some estimates are that there are more adults with CHD than children. Sudden cardiac death (SCD) is the leading cause of death in these patients but with effective approaches to prevent SCD, heart failure is emerging as a leading cause of morbidity and mortality. Heart Failure hospitalizations have increased by over 90% in the twenty-first century in adults with congenital heart disease (ACHD). Consequently, ACHD are increasingly being referred for advanced therapies for heart failure such as cardiac transplantation and mechanical circulatory support. To prevent progression to advanced heart failure, efforts ae being made to tailor conventional heart failure therapies to this population. Managing adults with single ventricular physiology who have had Fontan procedures and then have failure of these procedures represents unique challenges. Specific advanced fellowship programs have been developed to train cardiologists in the complex management of these patients.