title: Long-term effect of bosentan therapy on cardiac function and symptomatic benefits in adult patients with Eisenmenger syndrome.
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.
  
  
  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.
authors.affiliation: Department of Cardiology, Erciyes University Medicine School, Kayseri, Turkey.
authors.forename: Mehmet G
authors.initials: MG
authors.lastname: Kaya
authors.forename: Yat-Yin
authors.initials: YY
authors.lastname: Lam
authors.forename: Betul
authors.initials: B
authors.lastname: Erer
authors.forename: Selim
authors.initials: S
authors.lastname: Ayhan
authors.forename: Mehmet A
authors.initials: MA
authors.lastname: Vatankulu
authors.forename: Zekeriya
authors.initials: Z
authors.lastname: Nurkalem
authors.forename: Murat
authors.initials: M
authors.lastname: Meric
authors.forename: Mehmet
authors.initials: M
authors.lastname: Eren
authors.forename: Namik K
authors.initials: NK
authors.lastname: Eryol
mesh_terms: D000328:Adult; D000959:Antihypertensive Agents / Q000008:administration & dosage / Q000627:therapeutic use; D000077300:Bosentan; D003971:Diastole; D004305:Dose-Response Relationship, Drug; D015150:Echocardiography, Doppler; D004541:Eisenmenger Complex / Q000175:diagnosis / Q000188:drug therapy* / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D006352:Heart Ventricles / Q000187:drug effects / Q000503:physiopathology; D006801:Humans; D008297:Male; D011446:Prospective Studies; D011669:Pulmonary Wedge Pressure / Q000187:drug effects; D013449:Sulfonamides / Q000008:administration & dosage / Q000627:therapeutic use*; D013599:Systole; D013997:Time Factors; D016896:Treatment Outcome; D016278:Ventricular Function, Right / Q000187:drug effects*
chemical_list: D000959:Antihypertensive Agents; D013449:Sulfonamides; D000077300:Bosentan