Safety and efficacy of epoprostenol therapy in pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis

Circ J. 2012;76(7):1729-36. doi: 10.1253/circj.cj-11-0973. Epub 2012 Apr 5.

Abstract

Background: Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) are rare causes of pulmonary hypertension. There is no proven medical therapy to treat these diseases, and lung transplantation is thought to be the only cure. Administration of vasodilators including epoprostenol sometimes causes massive pulmonary edema and could be fatal in these patients.

Methods and results: Eight patients were treated with epoprostenol for 387.3±116.3 days (range, 102-1,063 days), who were finally diagnosed with PVOD or PCH by pathological examination. The maximum dose of epoprostenol given was 55.3±10.7 ng·kg(-1)·min(-1) (range, 21.0-110.5 ng·kg(-1)·min(-1)). With careful management, epoprostenol therapy significantly improved the 6-min walk distance (97.5±39.2 to 329.4±34.6 m, P<0.001) and plasma brain natriuretic peptide levels (381.3±136.8 to 55.2±14.4 pg/ml, P<0.05). The cardiac index significantly increased from 2.1±0.1 to 2.9±0.3 L·min(-1)·m(-2) (P<0.05). However, pulmonary artery pressure and pulmonary vascular resistance were not significantly reduced. For 4 patients, epoprostenol therapy acted as a bridge to lung transplantation. For the other patients who had no chance to undergo lung transplantation, epoprostenol therapy was applied for 528.0±216.6 days and the maximum dose was 63.9±19.0 ng·kg(-1)·min(-1).

Conclusions: This study data suggest that cautious application of epoprostenol can be considered as a therapeutic option in patients with PVOD and PCH.

Publication types

  • Multicenter Study

MeSH terms

  • Adolescent
  • Adult
  • Antihypertensive Agents / adverse effects
  • Antihypertensive Agents / therapeutic use*
  • Biomarkers / blood
  • Blood Pressure / drug effects
  • Child
  • Epoprostenol / adverse effects
  • Epoprostenol / therapeutic use*
  • Exercise Tolerance / drug effects
  • Female
  • Hemangioma, Capillary / blood
  • Hemangioma, Capillary / complications
  • Hemangioma, Capillary / diagnosis
  • Hemangioma, Capillary / drug therapy*
  • Hemangioma, Capillary / physiopathology
  • Humans
  • Hypertension, Pulmonary / blood
  • Hypertension, Pulmonary / diagnosis
  • Hypertension, Pulmonary / drug therapy*
  • Hypertension, Pulmonary / etiology
  • Hypertension, Pulmonary / physiopathology
  • Japan
  • Lung Neoplasms / blood
  • Lung Neoplasms / complications
  • Lung Neoplasms / diagnosis
  • Lung Neoplasms / drug therapy*
  • Lung Neoplasms / physiopathology
  • Lung Transplantation
  • Male
  • Natriuretic Peptide, Brain / blood
  • Pulmonary Veno-Occlusive Disease / blood
  • Pulmonary Veno-Occlusive Disease / complications
  • Pulmonary Veno-Occlusive Disease / drug therapy*
  • Pulmonary Veno-Occlusive Disease / physiopathology
  • Recovery of Function
  • Time Factors
  • Treatment Outcome
  • Vascular Resistance / drug effects
  • Vasodilator Agents / adverse effects
  • Vasodilator Agents / therapeutic use*

Substances

  • Antihypertensive Agents
  • Biomarkers
  • Vasodilator Agents
  • Natriuretic Peptide, Brain
  • Epoprostenol