Long-Term Effect of Bosentan Therapy on Cardiac Function and Symptomatic Benefits in Adult Patients With Eisenmenger Syndrome

• Published in: Journal of cardiac failure 2012-05, Vol.18 (5), p.379-384
• Main Authors: Kaya, Mehmet G., MD, Lam, Yat-Yin, MD, FESC, Erer, Betul, MD, Ayhan, Selim, MD, Vatankulu, Mehmet A., MD, Nurkalem, Zekeriya, MD, Meric, Murat, MD, Eren, Mehmet, MD, Eryol, Namik K., MD
• Format: Article
• Language: English
• Subjects: Adult; Antihypertensive Agents - administration & dosage; Antihypertensive Agents - therapeutic use; Bosentan; Cardiovascular; Diastole; Dose-Response Relationship, Drug; Echocardiography, Doppler; Eisenmenger Complex - diagnosis; Eisenmenger Complex - drug therapy; Eisenmenger Complex - physiopathology; Eisenmenger syndrome; Female; Follow-Up Studies; Heart Ventricles - drug effects; Heart Ventricles - physiopathology; Humans; long-axis function; Male; myocardial performance index; Prospective Studies; Pulmonary Wedge Pressure - drug effects; Sulfonamides - administration & dosage; Sulfonamides - therapeutic use; Systole; Time Factors; Treatment Outcome; Ventricular Function, Right - drug effects
• ISSN: 1071-9164; 1532-8414
• DOI: 10.1016/j.cardfail.2012.02.004

Abstract 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 (SaO2 ) were also recorded. The PASP, WHO functional class, 6MWD, and SaO2 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.