M5. Effect of Macitentan on pulmonary vascular resistance index in patients with congenital heart disease-pulmonary arterial hypertension: a meta-analysis

Macitentan is a novel dual endothelin receptor antagonist which proven effective as treatment in adults with pulmonary arterial hypertension.

We performed a systematic search from inception until May 31, 2021, according to PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines comparing PVRi baseline and after Macitentan 10mg and selected studies according to our pre-defined inclusion and exclusion criteria. Two reviewers extracted data into the predefined tables in the Microsoft Excel (Microsoft Corp., New Mexico, US) sheet. We used RevMan software to perform a meta-analysis and draw Forest plots. We used a random effects model to estimate risk ratios. A two-sided p-value of ≤ 0.05 was considered statistically significant. A total of three randomized control trials (RCTs) with 325 patients were included in our analysis. Macitentan 10mg a day showed moderately associated with lower PVRi compare with baseline value (SMD: -0.72) and statistically significant (p = 0.002; 95% CI=-1.18-(-0.25)).

Macitentan was developed by modifying the structure of Bosentan in order to increase the efficacy and safety. Macitentan has slower receptor dissociation kinetics and longer duration of action which allows once-daily dosing. The safety profile of Macitentan appears to be superior with respect to hepatic safety and edema than other therapy such as Bosentan and Ambrisentan, respectively. Macitentan is a promising drug to treat pulmonary hypertension which is well tolerated and associated with improvements.