RT Journal Article
SR Electronic
T1 Metformin added to bosentan therapy in patients with pulmonary arterial hypertension associated with congenital heart defects: a pilot study
JF ERJ Open Research
JO erjor
FD European Respiratory Society
SP 00060-2018
DO 10.1183/23120541.00060-2018
VO 4
IS 3
A1 Liao, Shutan
A1 Li, Dongsheng
A1 Hui, Zheng
A1 McLachlan, Craig S.
A1 Zhang, Yang
YR 2018
UL http://openres.ersjournals.com/content/4/3/00060-2018.abstract
AB Pulmonary arterial hypertension (PAH) is a common complication of a congenital heart defect (CHD). Recent studies suggest metformin may be a potential drug to improve cardiac function in PAH. A pilot study was conducted to investigate the efficacy of short-term treatment with a combination regimen consisting of bosentan and metformin in PAH-CHD patients as compared with bosentan monotherapy in a prospective, randomised study.Patients with PAH-CHD were randomised to receive bosentan (initially at 62.5 mg twice daily for 4 weeks and then 125 mg twice daily) for 3 months with or without the combination treatment of metformin (500 mg twice daily).93 patients were enrolled to bosentan monotherapy (n=48) or bosentan/metformin combination treatment (n=45). After 3 months, both treatments significantly improved World Health Organization functional class, 6-min walking distance (6MWD), N-terminal pro-brain natriuretic peptide and right heart haemodynamic parameters. The improvements in 6MWD and pulmonary vascular resistance index were significantly greater in patients treated with combination therapy than in those who received monotherapy (mean±sd 95±136 versus 48±119 m (p=0.017) and −1.8±1.2 versus −1.2±1.3 Wood units per m2 (p<0.001), respectively). Pulmonary endothelin (EDN)1 was significantly decreased after combination therapy (p=0.006). However, plasma EDN1 levels were not affected.Combination therapy with bosentan and metformin in PAH-CHD patients provides improvements in important outcomes such as exercise capacity and pulmonary haemodynamics, compared with bosentan alone.This study investigated, for the first time, the efficacy of a combination regimen consisting of bosentan and metformin in patients with pulmonary arterial hypertension associated with congenital heart defect as compared with bosentan monotherapy http://ow.ly/n4nJ30kT98d