Using bosentan to manage PAH in adults who have congenital heart disease

June 1, 2006

The endothelin receptor antagonist bosentan has been demonstrated to be effective in the management of pulmonary arterial hypertension (PAH). Now, Benza and associates report that bosentan is beneficial in patients who have PAH associated with congenital heart disease. Specifically, they found that treatment with bosentan significantly reduced right atrial pressure and mean pulmonary artery pressure. Moreover, they found no evidence of systemic desaturation or worsening volume overload associated with bosentan therapy.

The endothelin receptor antagonist bosentan has been demonstrated to be effective in the management of pulmonary arterial hypertension (PAH). Now, Benza and associates report that bosentan is beneficial in patients who have PAH associated with congenital heart disease. Specifically, they found that treatment with bosentan significantly reduced right atrial pressure and mean pulmonary artery pressure. Moreover, they found no evidence of systemic desaturation or worsening volume overload associated with bosentan therapy.

This study enrolled 19 women and 5 men with PAH associated with congenital heart disease (New York Heart Association [NYHA] functional class III in 79% of patients). The participants' hemodynamic values, exercise capacity, and Borg dyspnea scores were assessed at baseline and again at 3, 6, and 12 months after bosentan therapy was started.

After 12 months of therapy, there was significant improvement in hemodynamics and NYHA functional status (Table). There also was a trend for improvement in the mean 6-minute walk test (P = .05).

No patients died or required hospitalization during the study, and there were no significant changes in arterial oxygen saturation. Three of the study participants had to discontinue bosentan therapy because of elevated liver function test results.

The authors note that studies are needed to evaluate the long-term effects of concomitant bosentan and prostacyclin therapy in patients with PAH.