24 Exercise capacity improvement in adult congenital heart disease patients switching from bosentan to macitentan therapy

    loading  Checking for direct PDF access through Ovid



Therapeutic options for patients with congenital heart disease (PAH-CHD) are limited. Endothelin receptor antagonists do play a role,1 however the recent MAESTRO trial showed that Macitentan only improved six-minute walk distance (6 MWD) in the control group.2 We report real world use of patients who switch from Bosentan to Macitentan in a single centre PAH-CHD cohort.


Clinical data was collected retrospectively from September 2014 to April 2017, up to 12 months after Macitentan introduction. Non-parametric statistical tests were used.


16 PAH-CHD patients (13 female, age 52±10 years) were followed for 9.4 (0.7–25.9) months. All but 3 (19%) tolerated the change in medication, with no significant adverse events.


At three months, exercise capacity improved in the patients where paired 6 MW tests were available (n=6: mean 6 MW 261±159 m at baseline to 415±84 m at 3 months, p=0.05), but this was not maintained at later time points. There was no significant change in WHO FC. Oxygen saturations improved at 3 months (87±7 vs 91±8, p=0.3), but saturations post-exercise failed to improve. BNP fell at the 3 and 6 month assessment (from 223 to 163 ng/L, then to 181 ng/L at 6 months, p=0.4, p=0.7) but this was not sustained at 12 months (figure 1). Echocardiographic measurements including TAPSE showed a non-significant improvement from baseline to 6 to 9 months and a decline thereafter, in line with the BNP findings (figure 2).


Exercise capacity, BNP and TAPSE improved after 3 months in some patients with PAH-CHD, but this was not sustained beyond 6 months.

Related Topics

    loading  Loading Related Articles