Congenital Defect of the Pericardium

Abstract

A 20-year-old woman with partial absence of the parietal pericardium is described. Radiologically, there was unusual prominence of the pulmonary are segment and the entire heart appeared to be shifted to the left. The electrocardiogram showed abnormal right axis deviation and suggested right ventricular hypertrophy. Right heart catheterization showed no evidence of pulmonary hypertension. Following the induction of left pneumothorax, air appeared in the right pleural space and in the pericardial space. A diagnosis of partial absence of the parietal pericardium was made. The recognition of this disorder is of importance because pulmonary hypertension or pulmonary valve stenosis with post-stenotic dilation of the pulmonary artery may be simulated, both radiologically and electrocardiographically.