Key result
Bosentan is linked to a ~32m 6MWD increase in complex but not simple Eisenmenger syndrome.
Why the study?
Does bosentan improve functional capacity and biomarkers in adults with simple and complex Eisenmenger's syndrome?
Cohort (n=24)
Does bosentan improve functional capacity and biomarkers in adults with simple and complex Eisenmenger's syndrome?
p-value: p=.028
Bosentan therapy appears safe and improves functional capacity, particularly 6-minute walk distance, in patients with complex Eisenmenger's syndrome.
May benefit complex but not simple Eisenmenger syndrome; hypothesis-generating and requires randomized confirmation before practice change.
BACKGROUND: Eisenmenger's syndrome (ES) is associated with decreased longevity and reduced functional capacity. Targeted pharmacologic therapies improve functional capacity and survival in these patients. We sought to compare the response of patients with simple vs. complex ES following initiation of bosentan. METHODS: ES patients with a history of bosentan use were identified by chart review. Simple ES was defined as ES associated with atrial septal defect, ventricular septal defect, or patent ductus arteriosus. Complex ES consisted of patients with truncus arteriosus and single ventricle congenital heart disease. Six-minute walking distance (6MWD), maximal oxygen consumption (VO(2) max), brain natriuretic peptide (BNP), and resting oxygen saturation were compared between simple and complex ES patients before and after bosentan treatment. RESULTS: Twenty-four patients were included (11 simple, 13 complex). Resting oxygen saturation, 6MWD, VO(2) max, and BNP were not significantly different between the two groups prior to bosentan initiation. Ten patients received bosentan monotherapy, and bosentan was used in combination with sildenafil in 13 (five simple, eight complex). One patient received bosentan with iloprost. Mean duration of therapy was 38 ± 14 months in the simple group and 40 ± 8.1 months in the complex group (P= NS). Posttreatment, 6MWD increased from 274 ± 135 m to 326 ± 106 m in simple ES patients (P= .32). 6MWD in patients with complex ES increased from 332 ± 51 m to 364 ± 109 (P= .028). VO(2) max improved from 13.4 ± 3.8 to 17 ± 6 (P= .54) in the simple group, while VO(2) max in the complex group improved from 12.7 ± 2.3 to 15.5 ± 2.2 (P= .17). There was minimal change in BNP or resting oxygen saturation between the groups. CONCLUSIONS: Treatment with bosentan is both safe and effective in patients with both simple and complex forms of ES.
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Williams et al. (2011) conducted a cohort in Eisenmenger's syndrome (n=24). Bosentan vs. Pre-treatment baseline was evaluated on 6-minute walking distance (6MWD) (p=.028). Bosentan treatment in adults with Eisenmenger's syndrome increased 6-minute walking distance in complex ES (332 to 364 m, P=.028) but not significantly in simple ES (274 to 326 m, P=.32).
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