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Pediatric Cardiology

, Volume 23, Issue 2, pp 146–151 | Cite as

The Influence of a Restrictive Atrial Septal Defect on Pulmonary Vascular Morphology in Patients with Hypoplastic Left Heart Syndrome

  • J.N. Graziano
  • K.P. Heidelberger
  • G.J. Ensing
  • C.A. Gomez
  • A. Ludomirsky
Article

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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Copyright information

© Springer-Verlag New York Inc. 2002

Authors and Affiliations

  • J.N. Graziano
    • 1
  • K.P. Heidelberger
    • 2
  • G.J. Ensing
    • 1
  • C.A. Gomez
    • 1
  • A. Ludomirsky
    • 1
  1. 1.Department of Pediatrics, Division of Pediatric Cardiology, University of Michigan, Ann Arbor, MI 48109, USAUS
  2. 2.Department of Pathology, University of Michigan, Ann Arbor, MI 48109, USAUS

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