Staged bilateral thoracotomies for multiple pulmonary arteriovenous malformations complicating hereditary hemorrhagic telangiectasia

J Thorac Cardiovasc Surg. 1982 Feb;83(2):285-9.

Abstract

A patient with hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) with multiple bilateral pulmonary arteriovenous malformations was hypoxic, cyanotic, polycythemic, and severely limited by dyspnea on exertion. Following staged bilateral thoracotomies, with removal of 23 major fistulas (12 from the right lung and 11 from the left), marked improvement in symptoms, blood gases, and objective measurements of exercise tolerance occurred.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Adult
  • Arteriovenous Malformations / complications
  • Arteriovenous Malformations / diagnosis
  • Arteriovenous Malformations / surgery*
  • Blood Gas Analysis
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Pulmonary Artery / abnormalities*
  • Pulmonary Artery / surgery
  • Pulmonary Veins / abnormalities*
  • Pulmonary Veins / surgery
  • Respiratory Function Tests
  • Telangiectasia, Hereditary Hemorrhagic / complications*