Pulmonary Veno-Occlusive Disease Clinical Presentation

Updated: Aug 25, 2020
  • Author: Hakim Azfar Ali, MD; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
  • Print


The presenting signs and symptoms of pulmonary veno-occlusive disease (PVOD) lack specificity. Most of the symptoms mimic other pulmonary and cardiac entities. The most common presenting symptoms are exertional dyspnea, fatigue, and cough. Sometimes, a respiratory tract infection–like illness may be identifiable preceding the diagnosis. Chronic cough (either productive or nonproductive) is present in some individuals.

In the later stages of PVOD, symptoms attributable to right ventricular failure, including chest pain and dizziness with exertion, abdominal pressure and tenderness secondary to hepatic congestion, and exertional syncope, may be noted. Hemoptysis with diffuse alveolar hemorrhage has been reported as a presentation. [32]

The suggestion of occult alveolar hemorrhage in bronchoalveolar lavage findings, however, is not uncommon. [33] Postural dyspnea or orthopnea may be reported by patients with PVOD but these findings are unusual among patients with primary pulmonary hypertension.


Physical Examination

Physical examination findings may be normal in patients with early pulmonary veno-occlusive disease (PVOD) disease. As the disease progresses, findings attributable to pulmonary hypertension and right-sided heart failure supervene. These findings include jugular venous distention, a right ventricular heave, a loud pulmonic heart sound, pedal edema, and epigastric tenderness. Inspiratory crackles may be heard if pulmonary infiltrates are prominent. [34] Clubbing is an unusual feature, [35] but has been described. Sudden death has also been reported as a presenting feature of PVOD. [36]

PVOD, being a postcapillary process, leads to increased visceral pleural and pulmonary capillary pressures with transudation of fluid into the pleural space resulting in pleural effusions. Pleural effusions are very rare in patients with primary pulmonary hypertension. [37]