The Influence of a Restrictive Atrial Septal Defect on Pulmonary Vascular Morphology in Patients with Hypoplastic Left Heart Syndrome |
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Authors: | JN Graziano KP Heidelberger GJ Ensing CA Gomez A Ludomirsky |
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Institution: | (1) Department of Pediatrics, Division of Pediatric Cardiology, University of Michigan, Ann Arbor, MI 48109, USA, US;(2) Department of Pathology, University of Michigan, Ann Arbor, MI 48109, USA, US |
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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. |
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