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SUMMARY AND CONCLUSIONS Pericardial disease complicating congenital lesions of the heart is uncommon. A study has been made of six patients having this combination. Two patients had an atrial septal defect and chronic constrictive pericarditis, and two had an atrial septal defect and pericardial effusion amounting to 1200 and 1500 ml., respectively. One had severe pulmonic stenosis and pericardial effusion of 2400 ml. The sixth patient had anomalous connection of the right pulmonary veins and fibrinous pericarditis. All the patients underwent cardiac catheterization and subsequent surgical repair, which substantiated the existence of the intracardiac and pericardial lesions. When a patient with congenital heart disease presents clinical manifestations of congestive heart failure, one should consider the possibility of coexistent pericardial disease, especially if there is increased venous pressure with normal oxygen saturation of the arterial blood. Thus, it is important to identify the association of pericardial compression and congenital heart disease, as both are usually correctable by present-day surgical technics.