Vol.23 No.6

Original Article

Intravenous epoprostenol treatment of patients with connective tissue disease and pulmonary arterial hypertension at a single center

Authors

Yuichiro Shirai1 , Hidekata Yasuoka1 , Tsutomu Takeuchi1 , Toru Satoh2, 3 , Masataka Kuwana 1

  • Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan
  • Division of Cardiology, Department of Internal Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku, Tokyo 160-8582, Japan
  • Department of Cardiology, Kyorin University School of Medicine, Shinkawa 6-20-2, Mitaka, Tokyo 181-8611, Japan
Received:

26 September 2012

Accepted:

28 December 2012

Published online:

29 January 2013

Full Text

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Abstract

Objective To assess the efficacy of epoprostenol treatment in Japanese patients with pulmonary arterial hypertension (PAH) associated with connective tissue disease (CTD).
Methods Sixteen patients with PAH?CTD treated with continuous intravenous epoprostenol at a single center between 2000 and 2009 were enrolled. Baseline characteristics, short-term and long-term outcomes, predictors of mortality, and safety profiles were evaluated. For survival analysis, 16 controls were selected who matched the underlying CTD, World Health Organization functional class, and use of PAH drugs, except for epoprostenol.
Results Six patients had systemic lupus erythematosus, five had mixed CTD, four had systemic sclerosis, and one had primary Sjo¨gren’s syndrome. The mean pulmonary arterial pressure (mPAP), cardiac index (CI), pulmonary vascular resistance, and functional class were significantly improved during the first 6 months of epoprostenol treatment.
Cumulative survival rates at 1, 2, and 3 years in epoprostenol-treated patients were 69, 69, and 55 %, respectively, and were significantly better than those of the controls. Functional class, CI at baseline, and reduction of mPAP at 6 months were identified as predictors of survival.
Adverse events, including flushing and catheterrelated infection, were frequent, but all patients tolerated the treatment.
Conclusion Based on the improvements in both shortterm and long-term outcomes among our patient cohort, epoprostenol is an effective treatment for CTD patients with advanced PAH.

Key words

Epoprostenol, Functional class, Pulmonary arterial hypertension, Survival