Key result
Bosentan treatment in patients with PAH and complex CHD increased six-minute walking distance from 371.9 m at baseline to 428.4 m at six months (p=0.005), with sustained improvement up to four years.
Why the study?
Does bosentan improve exercise capacity in patients with pulmonary arterial hypertension associated with complex congenital heart disease?
Cohort (n=14)
Does bosentan improve exercise capacity in patients with pulmonary arterial hypertension associated with complex congenital heart disease?
Absolute Event Rate: 428.4% vs 371.9%
p-value: p=0.005
Bosentan therapy is safe and provides sustained improvement in exercise capacity for patients with PAH associated with complex congenital heart disease.
May support bosentan for exercise capacity gains in PAH-complex CHD; leaves open randomized confirmation before practice change.
BACKGROUND: Bosentan is recommended for symptomatic patients with Eisenmenger syndrome due to simple congenital lesions such as atrial and ventricular septal defects (VSD). However, its long-term efficacy and safety in patients with pulmonary arterial hypertension (PAH) associated with complex congenital heart disease (CHD) is unknown. OBJECTIVES: We examined the short- and long-term effects and safety profile of bosentan in patients with PAH and complex CHD. METHODS: We followed 14 patients with PAH and complex CHD for a mean of four years. Demographic parameters, exercise capacity assessed by the six-minute walking test (6MWT) and oxygen saturation were assessed at baseline, six months and at follow-up. RESULTS: Mean age was 37.1 ± 11.7 years; 90% were in WHO class III or IV. The most common diagnosis was pulmonary atresia with VSD (35.7%), followed by truncus arteriosus (28.6%), patent ductus arteriosus (21.4%) and transposition of the great arteries (14.3%). After six months of treatment, six-minute walking distance (6MWD) increased from 371.9 to 428.4 m (p=0.005) and functional class was improved (p=0.005). After four years, one patient discontinued bosentan due to side effects and four patients were started on sildenafil, after a mean 38 months of bosentan treatment. Mean 6MWD for patients on bosentan monotherapy (n=8) was 440.1 ± 103.8 m, whereas for patients on bosentan-sildenafil combination therapy (n=4) it was 428.8 ± 96.9 m, after four years of therapy. Two patients died during follow-up. CONCLUSIONS: Bosentan was safe and was associated with improved exercise capacity in patients with PAH and complex CHD. This improvement was sustained for up to four years and the safety profile was similar to simple CHD patients.
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Baptista et al. (2013) conducted a cohort in Pulmonary arterial hypertension (PAH) associated with complex congenital heart disease (CHD) (n=14). Bosentan vs. Baseline was evaluated on Six-minute walking distance (6MWD) at six months (p=0.005). Bosentan treatment in patients with PAH and complex CHD increased six-minute walking distance from 371.9 m at baseline to 428.4 m at six months (p=0.005), with sustained improvement up to four years.
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