Atrial septal defect with failure to thrive in infancy: hidden pulmonary vascular disease?

Atrial septal defects are usually asymptomatic, and are closed surgically or by a catheter implanted device in preschool age children. Rarely, they may cause symptoms in infancy, and management at this age is debated. We report our experience of six infants who underwent surgical closure, with variable outcomes. Five of these had significant extracardiac pathology. Our data suggest the need to exclude other causes of symptoms, both cardiac and non-cardiac. The question should be asked as to whether surgery is of benefit in these children, and particularly whether they may have underlying primary pulmonary vascular disease.