Bilateral lung transplantation in a patient with Vascular Ehlers-Danlos syndrome

Ann Thorac Surg. 2014 May;97(5):1804-6. doi: 10.1016/j.athoracsur.2013.07.132.

Abstract

We describe the case of a 29-year-old woman with end-stage chronic obstructive pulmonary disease secondary to vascular Ehlers-Danlos syndrome. Because of critical deterioration, respiratory arrest, and complete lung failure, she required urgent implantation of a venovenous extracorporeal membrane oxygenator as a bridge to lung transplantation. After 6 days of extracorporeal life support, a successful bilateral sequential lung transplantation was performed. This is the first case of lung transplantation in a patient with a diagnosis of chronic obstructive pulmonary disease secondary to Ehlers-Danlos syndrome.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Disease Progression
  • Ehlers-Danlos Syndrome / complications*
  • Ehlers-Danlos Syndrome / diagnosis
  • Extracorporeal Membrane Oxygenation / methods*
  • Female
  • Follow-Up Studies
  • Graft Survival
  • Humans
  • Lung Transplantation / methods*
  • Respiratory Insufficiency / etiology*
  • Respiratory Insufficiency / physiopathology
  • Respiratory Insufficiency / surgery*
  • Risk Assessment
  • Time Factors
  • Treatment Outcome
  • Waiting Lists