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

J Card Fail. 2012 May;18(5):379-84. doi: 10.1016/j.cardfail.2012.02.004. Epub 2012 Mar 10.

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 (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.

Publication types

  • Comparative Study
  • Randomized Controlled Trial

MeSH terms

  • Adult
  • Antihypertensive Agents / administration & dosage
  • Antihypertensive Agents / therapeutic use
  • Bosentan
  • Diastole
  • Dose-Response Relationship, Drug
  • Echocardiography, Doppler
  • Eisenmenger Complex / diagnosis
  • Eisenmenger Complex / drug therapy*
  • Eisenmenger Complex / physiopathology
  • Female
  • Follow-Up Studies
  • Heart Ventricles / drug effects
  • Heart Ventricles / physiopathology
  • Humans
  • Male
  • Prospective Studies
  • Pulmonary Wedge Pressure / drug effects
  • Sulfonamides / administration & dosage
  • Sulfonamides / therapeutic use*
  • Systole
  • Time Factors
  • Treatment Outcome
  • Ventricular Function, Right / drug effects*

Substances

  • Antihypertensive Agents
  • Sulfonamides
  • Bosentan