Giant single atrium

Abstract

A 20-year-old young Ugandan woman presented with progressive exertional dyspnea, labial cyanosis and severe digital clubbing. Visualization of the heart by standard transthoracic echocardiography was optimal from the apical views allowing to show a complete lack of any atrial septal tissue (common single atrium). Mitral and tricuspid valve attachments to the interventricular crux were identified in the same anatomical plane and no congenital cleft was observed. The right ventricle was markedly enlarged with high filling pressures, systolic dysfunction and severe pulmonary hypertension (sPAP 90–100 mm Hg close to systemic values) suggestive of Eisenmenger syndrome.