Use of vasodilators for the treatment of pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis: A systematic review

Respir Investig. 2019 Mar;57(2):183-190. doi: 10.1016/j.resinv.2018.10.004. Epub 2018 Nov 23.


Background: There are several medications available to treat pulmonary arterial hypertension (PAH): PAH-targeted drugs. However, in patients with pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis (PVOD/PCH), rare diseases that cause pulmonary hypertension, the effectiveness and safety of vasodilators, including PAH-targeted drugs, are unclear.

Methods: We searched English-language publications listed in three electronic databases (PubMed, Cochrane Library, and the Japan Medical Abstracts Society). Reports with efficacy outcomes (survival, improvement in 6-minute walk distance, and pulmonary vascular resistance) and data on development of pulmonary edema after administration of vasodilators to patients with PVOD/PCH were selected (1966 to August 2015).

Results: We identified 20 reports that met our criteria. No randomized controlled or prospective controlled studies were reported. The survival time ranged from 71 minutes to 4 years or more after initiation of vasodilators. Most of the reported cases showed an improvement in the 6-minute walk distance and pulmonary vascular resistance. Pulmonary edema was reported in 15 articles, some cases of which were lethal.

Conclusions: The present study demonstrates the potential efficacy and difficulties in the use of vasodilators in patients with PVOD/PCH; however, drawing a firm conclusion was difficult because of the lack of randomized controlled trials. Further research is needed to ascertain if vasodilator use is beneficial and safe in patients with PVOD/PCH.

Keywords: Hemodynamics; Pulmonary edema; Pulmonary hypertension; Treatment; Vasodilator.

Publication types

  • Systematic Review

MeSH terms

  • Databases, Bibliographic
  • Hemangioma, Capillary / complications
  • Hemangioma, Capillary / drug therapy*
  • Hemangioma, Capillary / mortality
  • Hemangioma, Capillary / physiopathology
  • Humans
  • Hypertension, Pulmonary / drug therapy
  • Hypertension, Pulmonary / etiology
  • Hypertension, Pulmonary / mortality
  • Hypertension, Pulmonary / physiopathology
  • Lung Neoplasms / complications
  • Lung Neoplasms / drug therapy*
  • Lung Neoplasms / mortality
  • Lung Neoplasms / physiopathology
  • Pulmonary Edema / chemically induced
  • Pulmonary Edema / epidemiology
  • Pulmonary Veno-Occlusive Disease / complications
  • Pulmonary Veno-Occlusive Disease / drug therapy*
  • Pulmonary Veno-Occlusive Disease / mortality
  • Pulmonary Veno-Occlusive Disease / physiopathology
  • Risk Assessment
  • Survival Rate
  • Vascular Resistance
  • Vasodilator Agents / adverse effects
  • Vasodilator Agents / therapeutic use*
  • Walk Test


  • Vasodilator Agents