biostudies-literatureUnknown139(1)Gatzoulis MA<h4>Background</h4>Eisenmenger syndrome describes congenital heart disease-associated severe pulmonary hypertension accompanied by right-to-left shunting. The multicenter, double-blind, randomized, placebo-controlled, 16-week, phase III MAESTRO study (Macitentan in Eisenmenger Syndrome to Restore Exercise Capacity) evaluated the efficacy and safety of the endothelin receptor antagonist macitentan in patients with Eisenmenger syndrome.<h4>Methods</h4>Patients with Eisenmenger syndrome aged ≥12 years and in World Health Organization functional class II-III were randomized 1:1 to placebo or macitentan 10 mg once daily for 16 weeks. Patients with complex cardiac defects, Down syndrome and background PAH therapy were eligible. The primary end point was change from baseline to week 16 in 6-minutCirculation51-63https://www.ebi.ac.uk/biostudies/studies/S-EPMC6314514biostudies-literatureEvaluation of Macitentan in Patients With Eisenmenger Syndrome.PMC6314514Gatzoulis MAHe JBeghetti MGesang SPulido TLandzberg MPapadakis KEfficace MMAESTRO Study InvestigatorsBerger RMGalie NfalseEvaluation of Macitentan in Patients With Eisenmenger Syndrome.<h4>Background</h4>Eisenmenger syndrome describes congenital heart disease-associated severe pulmonary hypertension accompanied by right-to-left shunting. The multicenter, double-blind, randomized, placebo-controlled, 16-week, phase III MAESTRO study (Macitentan in Eisenmenger Syndrome to Restore Exercise Capacity) evaluated the efficacy and safety of the endothelin receptor antagonist macitentan in patients with Eisenmenger syndrome.<h4>Methods</h4>Patients with Eisenmenger syndrome aged ≥12 years and in World Health Organization functional class II-III were randomized 1:1 to placebo or macitentan 10 mg once daily for 16 weeks. Patients with complex cardiac defects, Down syndrome and background PAH therapy were eligible. The primary end point was change from baseline to week 16 in 6-minut2019-01-01T00:00:00Z2019 Jan2026-06-20T04:48:09.959Z2026-06-20T03:10:27.28ZS-EPMC63145143058669410.1161/CIRCULATIONAHA.118.033575