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