Pulmonary veno-occlusive disease - A case series and new observations

Study objectives: The aim of this study was to describe our experience at o ne 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 PV OD at our institution were retrospectively studied. Included were all avail able 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, w as found in 5 patients, rales in 6, and increased interstitial markings on chest radiograph in 10. Half of the 10 patients who underwent acute vasodil ator testing exhibited a decrease in pulmonary artery pressure of > 20%, al though one patient died shortly after receiving IV calcium-channel blockers . Three patients have demonstrated sustained clinical improvement with ther apy, which includes calcium-channel blockers, epoprostenol, and lung transp lantation 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. Howev er, both physical examination findings and radiographic studies often provi de clues to the diagnosis, which may obviate the need for lung biopsy in th e 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 transpl antation remains the only proven therapy.