Natural History and Prognosis of Atrial Septal Defect

A series of 128 consecutive cases of atrial septal defect in adult patients was analyzed from the standpoint of the course and prognosis. The ages of the patients ranged from 18 to 67 years and the ratio of men to women was 1 to 2. Age distribution when compared with life expectancy tables indicated reduced life expectancy. Clinical analysis revealed that three quarters of the patients were symptomatic, but symptoms were mild to moderate and usually nonprogressive. Hemodynamic analysis revealed significant pulmonary hypertension in 22% of the series, of which 15% had high pulmonary vascular resistance, and significant arterial hypoxemia in 14%. The most serious risk factor in atrial septal defect is severe pulmonary hypertension. This complication, which develops in about 14% of patients with atrial septal defect when they are between 20 and 40 years of age, may be rapidly progressive, leading to shunt reversal, disability, and death. Once established, pulmonary hypertension may progress even when the defect is surgically closed. Heart failure occurs usually in older individuals and is associated mostly with chronic atrial arrhythmias rather than pulmonary hypertension.