Pulmonary veno-occlusive disease: a case series and new observations.

STUDY OBJECTIVES: The aim of this study was to describe our experience at one institution with pulmonary veno-occlusive disease (PVOD) during the past 10 years, with particular reference to new findings and long-term outcome.

SETTING: Tertiary care, academic medical center.

PATIENTS AND METHODS: Eleven patients who were evaluated and treated for PVOD at our institution were retrospectively studied. Included were all available clinical, radiographic, hemodynamic, and pathologic data.

RESULTS: All 11 patients in our series had at least one symptom or clinical finding that, in conjunction with known pulmonary hypertension, suggested the diagnosis of PVOD. Digital clubbing, not previously reported in PVOD, was found in 5 patients, rales in 6, and increased interstitial markings on chest radiograph in 10. Half of the 10 patients who underwent acute vasodilator testing exhibited a decrease in pulmonary artery pressure of > 20%, although one patient died shortly after receiving IV calcium-channel blockers. Three patients have demonstrated sustained clinical improvement with therapy, which includes calcium-channel blockers, epoprostenol, and lung transplantation in one patient each. However, outcome was generally poor, with a 72% mortality within 1 year of diagnosis.

CONCLUSION: The diagnosis of PVOD requires a high clinical suspicion. However, both physical examination findings and radiographic studies often provide clues to the diagnosis, which may obviate the need for lung biopsy in the majority of cases. Although there may be patients who respond to medical therapy, the use of vasoactive medications in patients with PVOD should be undertaken with great caution. Long-term survival is poor, and lung transplantation remains the only proven therapy.