Congenital Partial Right Pericardial Defect Associated with Herniation of the Right Atrial Appendaqe

CONGENITAL pericardial defect is relatively rare. Recently, however, the anomaly has been more frequently diagnosed during life, as awareness of the lesion increases and advances in radiological diagnosis are made. In 1961, one of the authors (1) reported a case of congenital partial defect of the pericardium on the left associated with herniation of the left atrial appendage, and since that time 3 more similar cases have been reported (2-4). To the authors' knowledge, however, there is no reported case of congenital partial pericardial defect on the right associated with herniation of the right atrial appendage. The purpose of this paper is to report such a case and to describe certain characteristic radiological features of the anomaly. Case Report K. J., a 61-year-old white male, was referred by a local physician to the West Virginia University Hospital because of chest pain and an abnormal cardiac shadow on the chest roentgenograms. He was in good health until two months previously when the chest pain appeared. The patient was seen in the Outpatient Department of the West Virginia University Hospital Feb. 17, 1964. Cardiac fluoroscopy and chest roentgenograms disclosed abnormal cardiac configuration with a localized bulging of the right upper cardiac border (Figs. 1 and 2). Active pulsations in the protuberance were paradoxical to those in the right ventricle. These findings were strongly suggestive of partial right pericardial defect associated with herniation of the right atrial appendage. On physical examination the blood pressure was 150/95 mm Hg, the heart rate was 75, and the rhythm was regular. No cardiac murmur or cardiac enlargement was noted. The patient appeared to be well nourished and well developed. Past history and family history were noncontributory. The patient was admitted to the West Virginia University Hospital for angiocardiographic study on Feb. 20. The pressure in the right atrium was 5/0 mm Hg, and samples from the superior vena cava and right atrium showed no evidence of arterialization. The angiocardiograms (Fig. 3) revealed protrusion of the right atrial appendage through the partial defect of the pericardium, confirming the initial diagnosis. No complications followed angiocardiography, and the patient was discharged. Repair of the pericardial defect is not planned at this time. Radiological Findings The radiological diagnosis of congenital pericardial defect was previously reported in detail by Chang and Leigh (1). The characteristic features of the partial right pericardial defect associated with herniation of the right atrial appendage are: 1. On the routine chest roentgenogram, an abnormal cardiac configuration with localized bulging in the right upper cardiac border is demonstrated.