Long-term effect of bosentan therapy on cardiac function and symptomatic benefits in adult patients with Eisenmenger syndrome.

BACKGROUND Bosentan improves symptoms in patients with Eisenmenger syndrome (ES). This study evaluated the effect of long-term bosentan therapy on cardiac function and its relation to symptomatic benefits in ES patients. METHODS AND RESULTS Twenty-three consecutive adult ES patients (15 with ventricular septal defect, 6 with atrial septal defect, and 2 with patent ductus arteriosus) underwent standard and tissue Doppler echocardiography before and 24 ± 9 months after bosentan therapy. Echocardiographic measurements included pulmonary arterial systolic pressure (PASP), myocardial performance index (MPI), tricuspid and lateral mitral annular pulsed-wave tissue Doppler systolic (Sa) and early diastolic (Ea) long-axis motions. Patients' World Health Organization (WHO) functional class, 6-minute walk distance (6MWD), and systemic arterial oxygen saturations (SaO(2)) were also recorded. The PASP, WHO functional class, 6MWD, and SaO(2) all improved (118 ± 22 to 111 ± 19 mm Hg, 3.2 ± 0.4 to 2.4 ± 0.5, 286 ± 129 m to 395 ± 120 m, and 84.6 ± 6.5% to 88.8 ± 3.9%, respectively; all P < .01) after therapy. There was also significant improvement in right ventricular (RV) MPI (by 23.9%: 0.46 ± 0.15 to 0.35 ± 0.09) and biventricular long-axis function (tricuspid Sa and Ea: 6.7 ± 1.5 to 8.8 ± 1.7 cm/s and 5.7 ± 1.3 to 7.0 ± 1.2 cm/s, respectively; lateral Sa and Ea: 6.8 ± 1.3 to 8.4 ± 1.5 cm/s and 7.6 ± 2.0 to 8.5 ± 2.1 cm/s, respectively; all P < .05). Posttherapy RV MPI was moderately correlated with PASP and 6MWD. CONCLUSIONS Sustained improvement of pulmonary arterial hypertension and RV function in ES patients was evident 2 years after bosentan therapy, and this may provide insights on the symptomatic benefits gained in these patients.