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.