Heart Failure in Adults with Congenital Heart Disease

Abstract

The number of adults with congenital heart disease (ACHD), especially those with complex CHD, is increasing substantially. The major clinical characteristics include (1) a high prevalence of right-sided heart failure (HF) due to systolic and/or diastolic dysfunction of the right ventricle (RV), which is frequently seen in repaired patients with tetralogy of Fallot (TF); (2) pressure and/or volume overload to the ventricles due to intra- and/or extracardiac shunting, atrioventricular or semilunar valve stenosis, and/or regurgitation; and (3) a morphologic RV as a systemic ventricle seen in those with congenitally corrected transposition of the great arteries or in those after an atrial switch operation. In addition, some CHD-specific conditions are also included in these pathophysiologic conditions, such as cyanosis (hypoxia) in unrepaired patients with complex CHD, Eisenmenger syndrome, and single ventricular physiology, i.e., Fontan circulation. The impaired function of the noncardiac organs may also be involved in some ACHD patients. Furthermore, surgery-related issues, including perioperative myocardial damage and electrical pathophysiology, can modify and make the ACHD HF pathophysiology more complex. The application of an established anti-HF strategy used in non-ACHD patients with left-sided HF to ACHD patients is questionable and possibly limited efficacy. Although most of the ACHD HF pathophysiology appears progressive, and determining the optimal timing for surgical interventions, such as pulmonary valve replacement, is often difficult because some of those procedures are prophylactic. Thus, a comprehensive understanding of ACHD HF, including noncardiac issues, is required to manage ACHD CHF and anticipate a better long-term outcome in these patients. In this review, the common ACHD HF pathophysiology and major specific ACHD conditions are discussed.