Partial Congenital Absence of the Pericardium

Abstract

A 45-year-old male was admitted to the hospital with recurrent, stabbing, nonexertional chest pain. Chest x-ray showed an increased cardiothoracic ratio (Figure 1A), and ECG demonstrated sinus rhythm with incomplete right bundle-branch block and poor R-wave progression (Figure 1B). Echocardiography showed normal left ventricular dimensions and function; however, the right ventricle appeared grossly dilated with moderate to severe tricuspid regurgitation with no evidence of intracardiac shunts. These findings were confirmed by right heart catheterization, which also showed normal pulmonary and right atrial pressures without a pressure gradient over the pulmonic valve. Catheterization of the left heart showed normal ventricular pressures and function as well as normal coronary arteries. The patient was referred for cardiac magnetic resonance imaging (CMR) with suspected right ventricular cardiomyopathy. CMR showed cardiac displacement into the left hemithorax (Figure 2A). There was severe tricuspid regurgitation with marked dilatation of both the right atrium (6×8 cm) and ventricle (end-diastolic volume, 343 mL). Right ventricular function was mildly reduced (ejection fraction, 46%) without localized wall motion abnormalities. Left ventricular volumes and …