Background:Few meta-analyses evaluated the efficacy and safety of the pulmonary arterial hypertension-specific drug therapy(PAH-SDT)in patients with Eisenmenger syndrome(ES).Recently,some studies have reported conflicting results regarding improvements in exercise capacity.This study evaluated the efficacy and safety of PAH-SDT in patients with ES.Methods:Relevant studies were identified by searching major databases.Pooled outcomes were analyzed by using Review Manager 5.3.Results:In total,5 studies with 508 patients were included.Meta-analysis indicated that PAH-SDT reduced the mortality(OR=0.35;95%CI,0.13 to 0.95;P=0.04),slashed the mean pulmonary artery pressure(MD=-4.35 mmHg;95%CI,-7.19 to-1.50;P=0.003),decreased pulmonary vascular resistance index(MD=-480.08 dyn·s·cm-5·m2;95%CI,-753.51 to-206.64;P=0.0006),increased the 6-minute walk distance(MD=28.38 m;95%CI,2.99 to 53.77;P=0.03),and elevated the systemic oxygen saturation at rest(MD=1.00%;95%CI,0.12 to 1.88;P=0.03).PAH-SDT was generally well tolerated.Conclusions:PAH-SDT decreases mortality and improves hemodynamics and exercise capacity in patients with ES.Overall,PAH-SDT is well tolerated. |