Adult Congenital Heart Disease: Importance of the Right Ventricle

The right ventricle (RV) is of lesser importance in acquired heart disease, but its role is of increasing importance in congenital heart disease. It may function as a subpulmonary ventricle or as a subaortic (systemic) ventricle in transposition complexes. The RV has a remarkable ability to adapt to pressure and volume load, but its size and function are often overlooked. Patients usually develop symptoms only after RV dysfunction has occurred, and in many diverse clinical scenarios, late referral is common. Advanced RV enlargement and dysfunction are responsible for not only impaired functional capacity but also lethal ventricular arrhythmias and sudden death. Appropriate imaging of the size and function of the RV are important because timely surgery on the pulmonary and tricuspid valves may preserve RV size and function. Adults with congenital heart disease should be followed at centers where there is an understanding of these problems so that valvular surgery can be considered when appropriate.