Congenital heart disease
Effect 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

https://doi.org/10.1016/j.amjcard.2009.01.021Get rights and content

Pulmonary arterial hypertension associated with congenital heart disease caused by systemic-to-pulmonary shunting was associated with a high risk of morbidity and mortality. In this retrospective study, the longer term treatment effect of bosentan on exercise capacity and quality of life (QoL) were evaluated in 58 adult patients (>18 years) with pulmonary arterial hypertension associated with congenital heart disease, including patients with Down's syndrome. All patients were evaluated at baseline and during follow-up using laboratory tests, 6-minute walk test, QoL questionnaires, and Doppler echocardiography. Treatment efficacy was analyzed separately for patients without (n = 30) and with Down's syndrome (n = 28). Median follow-up of all patients treated with bosentan was 22 months (range 3 to 36). In patients without Down's syndrome, mean 6-minute walk distance increased from 427 ± 97 to 461 ± 104 m (p <0.01) after 6 months of treatment, followed by a gradual return to baseline and disease stabilization. QoL improved significantly during treatment and was maintained during 18 months of follow-up (p <0.05). In patients with Down's syndrome, 6-minute walk distance and QoL were stable during treatment. In conclusion, findings suggested that in patients without Down's syndrome, longer term bosentan treatment resulted in a persistent improvement in QoL and stabilization of exercise capacity.

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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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