Impact of Macitentan on the Health-Related Quality of Life in Pulmonary Arterial Hypertension in an Iranian Population

Background: Pulmonary arterial hypertension is a progressive disease of the lung vascular system which causes patients’ functional limitations and reduces their Health-Related Quality of Life (HRQoL). This study was aimed at determining the effectiveness of Macitentan on the HRQoL of Iranian PAH patients by the emphasis 10 questionnaire. 
Methods: 31 idiopathic class I pulmonary hypertension patients were enrolled in this cohort study. Patients completed the emphasis 10 questionnaire at baseline and 6 months after Macitentan consumption. The absolute changes in emphasis 10 scores were calculated. The effect of Macitentan on functional activity and paraclinical indexes was assessed. 
Results: The mean quality of life scores before and after the Macitentan, were 25.83 (±11.80 SD) and 16.93 (±10.36 SD), respectively (p-value=0.001). Besides, administration of Macitentan increased the mean distance in the 6-minute walk test, from 227.00±(87.275 SD) to 263.67 (±115.855 SD) (p-value=0.044), led to a reduction in the mean hospitalization days from 2.23 (±4.049 SD) to 0.33 (±1.322 SD), (p-value=0.015) and reduction in times of hospitalization from 0.57(0.93) to 0.10(0.30), (p-value=0.017). Although it had a positive effect on mean PAP (81 to 68), O2 saturation at the beginning of 6MW (90 to 91%), final O2 Saturation in 6MW (81 to 85%), and pro-BNP (907 to 412), this effect was not statistically significant. 
Conclusion: Treatment with Macitentan significantly improved patient functional activity and reduced the mean hospitalization days. Thus, due to the low side effects and high efficacy, it can be advised as the first line of treatment.