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

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

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

View larger version (24K): [in a new window]   Figure 1 Pediatric Pulmonary Arterial Hypertension (PAH) Treatment Guidelines. *"Acute responder" was defined as: 1) 20% decrease in mean pulmonary artery pressure; 2) no change or an increase in cardiac index; and 3) no change or a decrease in the ratio of pulmonary vascular resistance to systemic vascular resistance in response to vasodilator testing. When bosentan became available (April 2001). CCB = calcium channel blockade; RHF = right heart failure.

View larger version (30K): [in a new window]   Figure 2 World Health Organization functional class at bosentan initiation and after at least eight weeks of treatment with bosentan. (Patients who died were assigned functional class IV.)

View larger version (24K): [in a new window]   Figure 3 Patient survival and treatment status at one year of treatment with bosentan (i.e., these patients were started before August 2002; >1 year before the data cutoff date, August 2003).

View larger version (26K): [in a new window]   Figure 4 Kaplan-Meier estimates of survival. Median survival follow-up was 15 months (range 3 to 28 months) in the entire bosentan-treated group, 15 months (range 3 to 28 months) in the bosentan subgroup without concomitant prostanoid therapy, and 16 months in the bosentan subgroup with concomitant prostanoid therapy (range 5 to 27 months).