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    Long-term Effect of Bosentan Therapy on Cardiac Function and Symptomatic Benefits in Adult Patients with Eisenmenger Syndrome
    (Churchill Livingstone Inc Medical Publishers, 2012) Kaya, Mehmet G.; Lam, Y-Y.; Erer, Betül; Ayhan, Selim; Vatankulu, Mehmet A.; Nurkalem, Zekeriya; Meric, Murat; Eryol, Namık K.; Eren, Mehmet
    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. (J Cardiac Fail 2012;18:379-384)