Symptomatic atrial septal defect in infancy

Atrial septal defect of the ostium secundum variety (ASD) is uncommonly diagnosed in infancy and rarely results in congestive heart failure (CHF). Eleven patients with uncomplicated ASD who developed CHF between 6 days and 13 months of age are presented. The diagnosis of ASD was documented by cardiac catheterization in all 11 patients. Left-to-right shunt ranged from 52 to 80%. Right ventricular pressure was near systemic levels in two patients and at least somewhat elevated in the other nine. Total pulmonary resistance (TPR) was within normal limits in all 11. No left ventricular obstructive lesions were found. Although one patient had unusual thickening of the left ventricular myocardium, and the left ventricular cavity was enlarged in two other cases, no specific abnormality in left or right ventricular function could be defined. Spontaneous closure of the ASD did not occur in any of our patients. Since spontaneous closure even of a large ASD can occur, however, it seems advisable to defer elective surgical closure until after 2 years of age if successful medical management can be achieved. In those patients in whom early surgery is not necessary, the clinical course usually becomes that of a typical ASD by age 2 years.