Abstract
To analyze the pathophysiologic importance of the right heart in different types of congenital heart disease (CHD), summarize current diagnostic modalities, and discuss treatment options. The right ventricle (RV) plays a key role in disease progression and prognosis, either as the subpulmonary or as the systemic ventricle. Volume and/or pressure overload as well as intrinsic myocardial disease are the main factors for RV remodeling. Echocardiography and cardiac magnetic resonance imaging are important noninvasive modalities for assessing anatomy, size, and function of the right heart. Timely repair of related lesions is essential for preventing RV dysfunction. Few inconclusive data exist on conventional pharmacotherapy in CHD-related RV dysfunction. Cardiac resynchronization therapy and ventricular assist devices are an option in patients with advanced systemic RV failure. Right heart disease is highly related with adverse clinical outcomes in CHD. Research should focus on early identification of patients at risk and development of medical and interventional treatments that improve RV function.
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