Spontaneous Fontan Physiology in Burnt-Out Endomyocardial Fibrosis

A 42-year-old man presented with congestive heart failure and atrial fibrillation. He had been diagnosed with endomyocardial fibrosis 8 years earlier and was stable on diuretics. He had congested neck veins, pedal edema, and massive ascites and a loud third heart sound. ECG confirmed atrial fibrillation. The chest radiograph showed massive cardiomegaly with a cardiothoracic ratio of 90%. Two-dimensional echocardiography revealed features of endomyocardial fibrosis with predominant right ventricular involvement (online-only Data Supplement Movie I; Figure, A). The right atrium was aneurysmally dilated with dense spontaneous echo contrast. The right ventricular cavity was obliterated. The inferior vena cava measured 28 mm with no respiratory variation, suggesting high systemic venous pressures. Hepatic vein flow showed loss of a wave reversal and increased antegrade flow in inspiration. Low-pressure tricuspid regurgitation was reflected in the …