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

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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.

Section snippets

Population

We prospectively studied 23 consecutive ES patients who were followed in an adult congenital heart disease clinic and received transthoracic echocardiography in 2005–2008. Enrolled patients were in WHO functional class III and IV and free from decompensated heart failure, severe arrhythmias causing hemodynamic compromise, and hemoptysis for ≥3 months before study entry. ES was defined as all of the following: 1) known intracardiac or great artery shunt; 2) comparable pulmonary arterial systolic

Demographics

A consecutive sample of 26 patients with ES were identified during the study period. Two patients with more-than-mild valvular heart diseases and 1 patient with significant hepatic and renal dysfunction were excluded from the study. The remaining 23 (13 female) patients, with a mean age of 31 ± 12 years, were included. Their underlying diagnoses were: unrestrictive ventricular septal defect (n = 15), large atrial septal defect (n = 6), and patent ductus arteriosus (n = 2). The mean follow-up

Main Findings

To our knowledge, this is the first study that examined in depth the effect of bosentan therapy on long-term change in cardiac function and its relation to symptomatic benefits in ES. We demonstrated a sustained clinical benefit (WHO functional class, SaO2, and 6MWD) of bosentan therapy without serious adverse events during long-term follow-up. Such clinical benefit was accompanied by a drop of PASP and an improvement in RV MPI and biventricular long-axis function. Furthermore, the extent of RV

Conclusion

Sustained improvement of pulmonary arterial hypertension and RV function in ES patients was evident 2 years after starting bosentan therapy. These hemodynamic improvements should provide insights on the symptomatic benefits gained in these patients.

Disclosures

None.

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