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J Am Coll Cardiol, 2005; 46:697-704, doi:10.1016/j.jacc.2005.01.066 (Published online 20 July 2005).
© 2005 by the American College of Cardiology Foundation
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PEDIATRIC PULMONARY HYPERTENSION

Effects of Long-Term Bosentan in Children With Pulmonary Arterial Hypertension

Erika Berman Rosenzweig, MD*,*, D. Dunbar Ivy, MD{dagger}, Allison Widlitz, MS, PA*, Aimee Doran, RN, MS, CPNP{dagger}, Lori R. Claussen, RN{dagger}, Delphine Yung, MD*, Steven H. Abman, MD{dagger}, Adele Morganti, PhD{ddagger}, Ngoc Nguyen, BS{ddagger} and Robyn J. Barst, MD*

* Division of Pediatric Cardiology, New York Presbyterian Hospital, New York, New York
{dagger} University of Colorado Health Sciences Center and Pediatric Heart Lung Center, Children's Hospital, Denver, Colorado
{ddagger} Actelion Pharmaceuticals Ltd., Allschwil, Switzerland

Manuscript received September 9, 2004; revised manuscript received January 6, 2005, accepted January 11, 2005.

* Reprint requests and correspondence: Dr. Erika Berman Rosenzweig, Division of Pediatric Cardiology, New York Presbyterian Hospital, 3959 Broadway, BHN 2-255, New York, New York 10032 (Email: esb14{at}columbia.edu).

OBJECTIVES: This study investigated the long-term outcome of children with pulmonary arterial hypertension (PAH) treated with bosentan therapy, with or without concomitant prostanoid therapy.

BACKGROUND: Bosentan, an oral endothelin ETA/ETB receptor antagonist, improves hemodynamics and exercise capacity in adults with PAH; however, limited data are available on its long-term effects in children.

METHODS: In this retrospective study, 86 children with PAH (idiopathic, associated with congenital heart or connective tissue disease) started bosentan with or without concomitant intravenous epoprostenol or subcutaneous treprostinil therapy. Hemodynamics, World Health Organization (WHO) functional class, and safety data were collected.

RESULTS: At the cutoff date, 68 patients (79%) were still treated with bosentan, 13 (15%) were discontinued, and 5 (6%) had died. Median exposure to bosentan was 14 months. In 90% of the patients (n = 78), WHO functional class improved (46%) or was unchanged (44%) with bosentan treatment. Mean pulmonary artery pressure and pulmonary vascular resistance decreased (64 ± 3 mm Hg to 57 ± 3 mm Hg, p = 0.005 and 20 ± 2 U·m2 to 15 ± 2 U·m2, p = 0.01, respectively; n = 49). Kaplan-Meier survival estimates at one and two years were 98% and 91%, respectively. The risk for worsening PAH was lower in patients in WHO functional class I/II at bosentan initiation than in patients in WHO class III/IV at bosentan initiation.

CONCLUSIONS: These data suggest that bosentan, an oral endothelin ETA/ETB receptor antagonist, with or without concomitant prostanoid therapy, is safe and efficacious for the treatment of PAH in children.

Abbreviations and Acronyms
  b.i.d. = twice daily
  CHD = congenital heart disease
  CI = cardiac index
  IPAH = idiopathic pulmonary arterial hypertension
  PAH = pulmonary arterial hypertension
  PAPm = mean pulmonary artery pressure
  PVRI = pulmonary vascular resistance index
  RAPm = mean right atrial pressure
  WHO = World Health Organization




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