ALLSCHWIL, Switzerland, Aug. 16, 2005 (PRIMEZONE) -- Actelion Ltd. (Other OTC:ALIOF) (SWX:ATLN) announced today that the Journal of the American College of Cardiology (JACC) has published a new study that investigated the long-term outcome of children with pulmonary arterial hypertension (PAH) treated with the dual endothelin receptor antagonist Tracleer(r) (bosentan) with or without concomitant prostanoid therapy.
Commenting on the publication, lead investigator Dr. Erika Berman Rosenzweig, New York Presbyterian Hospital, New York, USA, said: "In the present study, clinical experience from two PAH centers involving 86 paediatric patients with PAH who received bosentan therapy was pooled to retrospectively assess the safety and long-term effects of bosentan treatment on functional capacity, hemodynamics and survival in children with PAH. The concomitant administration of bosentan and prostanoid therapy was also evaluated."
The authors conclude that in this study bosentan, with or without concomitant prostanoid therapy, was shown to be efficacious for the treatment of PAH in children. In addition, this study also indicated that the safety profile appears similar to that in adult PAH patients.
Dr. Rosenzweig added: "Considering the limited clinical paediatric data currently available, the present retrospective study, which involves a large cohort of children with PAH, provides a fairly extensive experience of long-term safety and efficacy of bosentan for the treatment of paediatric PAH."
Details on study publication and results:
The study, "Effects Of Long-Term Bosentan in Children with Pulmonary Hypertension," by E.B. Rosenzweig, is published in the August issue of JACC (Rosenzweig E.B. et al. Volume 46 Issue 4 Pages 697-704).
In this retrospective study, 86 children with PAH (either idiopathic, associated with congenital heart disease or connective tissue disease) started bosentan with or without concomitant intravenous epoprostenol or subcutaneous treprostinil therapy. Median exposure to bosentan was 14 months. In 90 percent of patients, World Health Organisation (WHO) functional class was improved (46 percent) or unchanged (44 percent). The one- and two-year Kaplan-Meier survival estimates were 98 percent and 91 percent, respectively. At cut-off date, 68 patients (79 percent) were still treated with bosentan, 13 (15 percent) were discontinued and 5 (6 percent) had died.
New bosentan formulation for children under clinical evaluation:
Considerable data exists showing that bosentan improves hemodynamics and exercise capacity in adults with PAH, but so far there has been limited data available on its long-term effects in children.
In the past three years, Actelion worked on a children-specific formulation of bosentan that recently entered clinical evaluation.
About Tracleer(r) in Pulmonary Arterial Hypertension (PAH)
Tracleer(r), the first oral dual endothelin receptor antagonist, is approved for the treatment of pulmonary arterial hypertension (PAH) and made available by Actelion subsidiaries in the United States, the European Union, Japan, Australia, Canada, Switzerland and other markets worldwide.
In clinical trials leading to the marketing approval of the drug, approximately 11 percent of PAH patients receiving Tracleer(r) experienced abnormal but reversible liver enzyme elevations. It is therefore important that patients undergo monthly liver monitoring. Due to the risk of birth defects, women who are pregnant, or of childbearing age who do not use a reliable method of contraception, must not take Tracleer(r).
About Pulmonary Arterial Hypertension (PAH)
Pulmonary arterial hypertension (PAH) is a chronic, life-threatening disorder characterized by abnormally high blood pressure in the arteries between the heart and lungs of an affected individual. The function of the heart and lungs is severely compromised, manifested by a limited exercise capacity, and, ultimately, a reduced life expectancy. Approximately 100,000 people in Europe and the United States are afflicted with either primary or secondary forms of the disease related to conditions or tissue disorders that affect the lungs, such as scleroderma, lupus, HIV/AIDS or congenital heart disease.
Actelion Ltd.
Actelion Ltd. is a biopharmaceutical company with its corporate headquarters in Allschwil/Basel, Switzerland. Actelion's first drug, Tracleer(r), an orally available dual endothelin receptor antagonist, has been approved as a therapy for pulmonary arterial hypertension. Actelion markets Tracleer(r) through its own subsidiaries in key markets worldwide, including the United States (based in south San Francisco), the European Union, and Japan, as well as Canada, Australia and Switzerland. Actelion, founded in late 1997, is a leading player in innovative science related to the endothelium -- the single layer of cells separating every blood vessel from the blood stream. Actelion focuses on the discovery, development and marketing of innovative drugs for significant unmet medical needs. Actelion shares are traded on the SWX Swiss Exchange (ticker symbol: ATLN).
For further information please contact:
Actelion Ltd. Gewerbestrasse 16 CH-4123 Allschwil