title: Pulmonary vascular disease in secundum atrial septal defect in childhood.
abstract: Pulmonary vascular structure was analyzed in the lungs of 10 patients with a secundum atrial septal defect (ASD) in whom pulmonary hypertension had developed. Four patients were aged 6 months or less, 5 were aged 2 to 9 years, and 1 was 21 years old. Pulmonary vascular structure was analyzed using lung biopsy tissue in 5 and autopsy material in the other 5. All the infants presented with heart failure and all had a marked increase in pulmonary arterial smooth muscle; only 1 infant survived surgery. Of the 5 older children, 1 presented with cyanosis, but in the rest the ASD was incidental to the presentation. Three patients had severe pulmonary vascular disease, similar to that seen in adults with a hypertensive ASD. Only 2 older children underwent successful surgery. In 1 child and in the 1 adult, the severity of the pulmonary vascular disease precluded surgery. The ASD was closed in 8 patients, but only 3 survived. Pulmonary hypertension develops rarely in secundum ASD in childhood.
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D005260:Female; D006344:Heart Septal Defects, Atrial / Q000150:complications* / Q000473:pathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000473:pathology; D007223:Infant; D007231:Infant, Newborn; D008168:Lung / Q000473:pathology; D008297:Male; D008854:Microscopy, Electron; D009131:Muscle, Smooth, Vascular / Q000473:pathology*; D011651:Pulmonary Artery / Q000473:pathology; D011652:Pulmonary Circulation; D011667:Pulmonary Veins / Q000473:pathology