STOCKHOLM — Survival of patients with systemic sclerosis-associated pulmonary arterial hypertension has improved markedly since the introduction of bosentan, Dr. Mark H. Williams said at the annual congress of the European Society of Cardiology.
Prognosis of scleroderma-associated pulmonary arterial hypertension (PAH) has traditionally been considered “very poor,” with death often resulting from right heart failure, noted Dr. Williams of Royal Free Hospital, London.
He presented an observational study involving 92 patients with scleroderma-associated PAH diagnosed by cardiac catheterization. Of those, 45 who were diagnosed with World Health Organization functional class III PAH since bosentan (Tracleer) was approved for European marketing in 2002 received the dual endothelin receptor antagonist as first-line therapy.
The remaining 47 WHO class III patients, who were hemodynamically matched to the bosentan group but were treated during 1998–2002, served as controls. Of these, 27 received an intravenous prostanoid as first-line therapy; the other 20 were unable to obtain funding for this extremely costly form of therapy.
One-year survival in the bosentan group was 81%, compared with 68% in the historic controls. Two-year survival was 71% with bosentan, compared with 47% in controls.
Bosentan, a nonselective dual endothelin receptor antagonist, stabilized cardiac hemodynamics, Dr. Williams said. Pulmonary vascular resistance, assessed in the majority of patients by repeat cardiac catheterization 9–12 months into treatment, climbed by 38% in the control patients but decreased by 1% in the bosentan group, compared with baseline.
Right artery and mean arterial pressures did not differ significantly between the two groups.
Dr. Williams has no conflict of interest regarding Tracleer or its manufacturer, Actelion.
