Pulmonary veno-occlusive disease in an elderly man: case report and review of the literature

Jpn Circ J. 1984 Jun;48(6):602-9. doi: 10.1253/jcj.48.602.

Abstract

A 75-year-old man complaining of dyspnea and having sings of postcapillary pulmonary hypertension was diagnosed as pulmonary veno-occlusive disease and confirmed at autopsy. This is the oldest case ever reported. Almost all the small veins 2 mm or less in external diameter were partially or nearly completely occluded by intimal fibrous tissue, and the obstructive changes in the pulmonary arteries were much more limited. Pulmonary veno-occlusive disease is a rare, almost inevitably fatal disease of unknown etiology which has only recently been separated clearly from primary pulmonary hypertension as a distinct entity. Chest roentgenogram finding suggesting postcapillary pulmonary hypertension is a clue to a diagnosis and differentiates this from two other causes of clinical primary pulmonary hypertension, that is, recurrent pulmonary embolism and plexogenic pulmonary arteriopathy.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Closing Volume
  • Echocardiography / methods
  • Electrocardiography
  • Humans
  • Hypertension, Pulmonary / complications
  • Lung / diagnostic imaging
  • Male
  • Peak Expiratory Flow Rate
  • Prognosis
  • Pulmonary Veins* / pathology
  • Radionuclide Imaging
  • Serum Albumin
  • Stroke Volume
  • Technetium
  • Technetium Tc 99m Aggregated Albumin
  • Thrombosis / diagnosis*
  • Thrombosis / pathology
  • Thrombosis / physiopathology
  • Total Lung Capacity
  • Vital Capacity

Substances

  • Serum Albumin
  • Technetium Tc 99m Aggregated Albumin
  • Technetium