How is pulmonary veno-occlusive disease (PVOD) treated?

Updated: Oct 16, 2018
  • Author: Hakim Azfar Ali, MD; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Answer

Specific PAH therapies may be associated with a risk of pulmonary edema at any time during the therapy. Extreme caution should be used while administering intravenous or subcutaneous prostanoids and dose escalation should be relatively slow.

No well-structured, prospective clinical trials have been performed to evaluate the effect of various nonsurgical interventions on the outcome of pulmonary veno-occlusive disease (PVOD). Currently, the information gained from clinical trials involving other forms of pulmonary arterial hypertension (PAH) is extrapolated from clinical experience, case reports, and case series in order to choose various therapies. Patients with PVOD are best served at a pulmonary hypertension specialty center.

No general consensus has been reached on the choice of first-line therapy for persons with PVOD. However, because PAH therapies (eg, continuous intravenous prostacyclin) are poorly tolerated and are perceived to have only a marginal effect on outcome, patients are offered the option of lung transplantation whenever possible. In the absence of this surgery, most patients do not survive beyond 2-3 years after diagnosis.


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