Lengthy diagnostic challenge in a rare case of pulmonary veno-occlusive disease: case report and review of the literature

Intern Med. 2011;50(12):1323-7. doi: 10.2169/internalmedicine.50.5035. Epub 2011 Jun 15.


Pulmonary veno-occlusive disease (PVOD) is a rare and usually survival poor disorder. We report a patient with a long history of progressive dyspnea of over 8 years, who with a diagnosis of chronic cor pulmonale confirmed elsewhere, was ultimately diagnosed as PVOD via histological analysis of a lung biopsy. After treatment with combined bosentan, diuretics and digoxin, his symptoms and function improved. This case highlights that PVOD is an under-recognised and often misdiagnosed disease, especially in its chronic form. Understanding its pathogenesis, its poor response to medical therapy and its dismal prognosis remain challenges for the treatment of PVOD.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Antihypertensive Agents / administration & dosage
  • Biopsy
  • Bosentan
  • Cardiotonic Agents / administration & dosage
  • Diagnosis, Differential
  • Digoxin / administration & dosage
  • Diuretics / administration & dosage
  • Drug Therapy, Combination
  • Dyspnea / etiology
  • Humans
  • Hypertension, Pulmonary / diagnosis
  • Male
  • Pulmonary Heart Disease / diagnosis
  • Pulmonary Veno-Occlusive Disease / diagnosis*
  • Pulmonary Veno-Occlusive Disease / drug therapy
  • Pulmonary Veno-Occlusive Disease / physiopathology
  • Sulfonamides / administration & dosage
  • Time Factors
  • Tomography, X-Ray Computed


  • Antihypertensive Agents
  • Cardiotonic Agents
  • Diuretics
  • Sulfonamides
  • Digoxin
  • Bosentan