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.
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.