Congenital heart diseaseEffect of Bosentan on Exercise Capacity and Quality of Life in Adults With Pulmonary Arterial Hypertension Associated With Congenital Heart Disease With and Without Down's Syndrome
Section snippets
Methods
The study population consisted of adult patients with PAH associated with CHD, including patients with Eisenmenger's syndrome. Patients were divided into the 2 groups of patients without Down's syndrome and patients with Down's syndrome. Enrolled patients had a diagnosis of any of the congenital heart defects of univentricular heart, patent ductus arteriosus, and such septal defects as ventricular septal defect, atrial septal defect, or atrioventricular septal defect. Patients with persistent
Results
From January 2005 to June 2008, a total of 58 adult patients with PAH associated with CHD started oral bosentan treatment. Median follow-up was 22 months (range 3 to 36). Forty-eight percent (n = 28) of enrolled patients had Down's syndrome. Table 1 lists patient baseline characteristics. Of the 6 patients without Down's syndrome without Eisenmenger's syndrome, 3 patients had persistent PAH after previous closure of their CHD defect, 2 patients had refused surgery, and for 1 patient, surgical
Discussion
This was the first study reporting on the effect of almost 2 years of bosentan treatment on exercise capacity and QoL in adult patients with PAH associated with CHD, including patients with Down's syndrome. In patients without Down's syndrome, we showed a significant increase in exercise capacity after the first 6 months of treatment and improvement in QoL. After the initial improvement, mean exercise tolerance decreased to baseline values and finally stabilized during longer term follow-up.
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