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Pulmonary vein stenosis

C C Sun1, T Doyle, R E Ringel

  • 1Department of Pediatrics, School of Medicine, University of Maryland, Baltimore, USA.

Human Pathology
|August 1, 1995
PubMed
Summary
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Pulmonary vein stenosis (PVS) is a rare condition. Autopsy revealed unexpected bilateral PVS in some cases, highlighting the need for thorough anatomical examination and potential biopsies in affected lung regions.

Area of Science:

  • Pediatric Cardiology
  • Cardiovascular Pathology
  • Thoracic Surgery

Background:

  • Pulmonary vein stenosis (PVS) is a rare disorder often requiring anatomical assessment for diagnosis.
  • Clinical diagnosis of PVS can be challenging, potentially underestimating disease extent.

Observation:

  • Four pediatric cases of PVS were analyzed, with autopsy findings revealing bilateral lesions in two patients initially diagnosed with unilateral disease.
  • Histopathological examination showed intimal and medial fibromuscular proliferation in both extrapulmonary and intrapulmonary veins, with some lesions mimicking organized thrombi.
  • Anastomoses between pulmonary veins and bronchial vessels or small pulmonary arteries were observed in one case.

Findings:

  • Autopsy confirmed PVS in a third case where it was not diagnosed clinically.

Related Experiment Videos

  • Intrapulmonary venous lesions were identified in lobes without clinically detected PVS, indicating potential underdiagnosis of bilateral disease.
  • Thrombosis within stenotic pulmonary veins was found to potentially worsen obstruction.
  • Bilateral hypertensive arteriopathy was present in patients with both unilateral and bilateral PVS.

    • Implications:

    • Histological examination of contralateral lung during unilateral PVS surgery may be crucial for detecting occult bilateral disease.
    • Thrombosis is a significant factor in the progression of pulmonary vein obstruction.
    • The study contributes to understanding the pathogenesis of pulmonary vein stenosis and associated arteriopathy.