Abstract
Review of a series of 122 patients with ostium primum defect revealed 27 who had reached the end of their natural history. The factors that caused their disability and death have been studied. The development of established arrhythmias is the commonest cause of deterioration and the importance of acquired complete heart block is stressed, Pulmonary hypertension uncommonly complicates an ostium primum defect but occasionally causes death with pulmonary artery thrombosis and right heart failure. Young children with severe mitral regurgitation and usually with large left-to-right shunts are liable to respiratory infection that may precipitate heart failure and death. Associated coarctation of the aorta may lead to heart failure in infancy.