A large pulmonary arteriovenous malformation causing cerebrovascular accidents

BMJ Case Rep. 2015 Apr 16:2015:bcr2014207786. doi: 10.1136/bcr-2014-207786.

Abstract

The incidence of pulmonary arteriovenous malformations (PAVMs) is 2.5 in 100,000. 80% are associated with Osler-Weber-Rendu syndrome or hereditary haemorrhagic telangiectasia. We report the case of a 70-year-old man with a 6 cm spherical mass incidentally found on chest X-ray. There was a localised systolic bruit over the right lower zone posteriorly; however, he was asymptomatic. He had suffered a stroke, affecting his right hand and his speech, from which he recovered. He experienced regular transient ischaemic attacks, on an average of every 2 months. He underwent a right lower lobectomy and on ligating the right lower lobe pulmonary artery the saturations of oxygen rose from 92% to 97%, demonstrating a significant right to left extracardiac shunt. Postoperative recovery was excellent and 1 year later the patient reports no further neurological symptoms. 40% of such lesions exhibit symptoms, however, only one-third are neurological. Treatment should be by percutaneous embolisation.

Publication types

  • Case Reports

MeSH terms

  • Activin Receptors, Type II / genetics
  • Activin Receptors, Type II / metabolism
  • Aged
  • Arteriovenous Fistula / complications
  • Arteriovenous Fistula / diagnosis
  • Arteriovenous Malformations / complications*
  • Arteriovenous Malformations / surgery
  • Humans
  • Male
  • Pulmonary Artery / abnormalities
  • Pulmonary Veins / abnormalities
  • Rare Diseases / diagnosis
  • Rare Diseases / etiology
  • Stroke / diagnosis
  • Stroke / etiology*
  • Treatment Outcome

Substances

  • ACVRL1 protein, human
  • Activin Receptors, Type II

Supplementary concepts

  • Pulmonary Arteriovenous Fistulas