Early Onset of Pulmonary Vascular Obstruction With Atrial Septal Defect

TODAY the diagnosis of atrial septal defect (ASD) in childhood no longer engenders anxiety or even a sense of urgency. The repair of an uncomplicated ASD is associated with a low and quite generally acceptable mortality.^1-4 That there is no aspect of urgency is attributable to a preponderant evidence that ASD is benign in childhood.^5-10 Indeed, not only may there be a remarkable longevity associated with this lesion^11-16 but the patients attaining such longevity commonly do not demonstrate significant pulmonary hypertension.^12-16 This is not to say, however, that a certain morbidity and mortality has not been ascribed to ASD in infancy and childhood. Hastreiter et al¹⁰ described 13 cases in which congestive heart failure secondary to ASD was considered the primary cause of death. All of these patients developed a fulminant clinical picture prior to 18 months of age. Only three patients were older than