title: The influence of a restrictive atrial septal defect on pulmonary vascular morphology in patients with hypoplastic left heart syndrome.
abstract: Hypoplastic left heart syndrome (HLHS) with a restrictive atrial septal defect (ASD) is a form of congenital heart disease with considerable morbidity and mortality. This morphologic analysis assesses the pulmonary vasculature in this patient population. Pulmonary arteries, the persistence of high-resistance fetal arterioles, pulmonary veins, and lymphatics from multiple lung sections from each of five patients with HLHS and a restrictive ASD were compared to those of five patients with HLHS and nonrestrictive ASD. Lung sections from each patient were qualitatively graded in severity of pathology from 0 to 3 for each of the structures described previously, with the pathologist blinded to the status of the ASD. Patients with a restrictive ASD exhibited more significant pulmonary venous thickening and lymphatic dilatation (p = 0.02), with a tendency toward persistence of high-resistance fetal vessels (p = 0.2), compared to patients with a nonrestrictive ASD. These findings imply that patients with HLHS and a restrictive ASD possess pulmonary vascular abnormalities that place them at higher risk for the current surgical interventions available compared to patients with a nonrestrictive ASD.
authors.affiliation: Department of Pediatrics, Division of Pediatric Cardiology, University of Michigan, Ann Arbor, MI 48109, USA.
authors.forename: J N
authors.initials: JN
authors.lastname: Graziano
authors.forename: K P
authors.initials: KP
authors.lastname: Heidelberger
authors.forename: G J
authors.initials: GJ
authors.lastname: Ensing
authors.forename: C A
authors.initials: CA
authors.lastname: Gomez
authors.forename: A
authors.initials: A
authors.lastname: Ludomirsky
mesh_terms: D005260:Female; D006344:Heart Septal Defects, Atrial / Q000150:complications / Q000473:pathology*; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000150:complications / Q000473:pathology*; D007231:Infant, Newborn; D008208:Lymphatic System / Q000473:pathology; D008297:Male; D011651:Pulmonary Artery / Q000473:pathology*; D011667:Pulmonary Veins / Q000473:pathology*; D012189:Retrospective Studies; D014655:Vascular Resistance