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

Shadi Shafaghi; Zargham Hossein Ahmadi; Mohammad Sadegh  Keshmiri; Farah  Naghashzadeh; Arezoo Mohamadifar; Majid Malek Mohammad; Neda Behzadnia; Leila Saliminejad; Sharare Shadanfar; Masoud Shafaghi; Sina Aghdasi; Sima Noorali; Babak  Sharif-Kashani

doi:10.18502/jimc.v5i4.11345

Shadi Shafaghi Lung Transplantation Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran
Zargham Hossein Ahmadi Lung Transplantation Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran
Mohammad Sadegh Keshmiri Lung Transplantation Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran
Farah Naghashzadeh Lung Transplantation Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran
Arezoo Mohamadifar Chronic Respiratory Disease Research Center, Masih Daneshvari Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Majid Malek Mohammad Tracheal Diseases Research Center, National Research Institute of Tuberculosis and Lung Disease (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran
Neda Behzadnia Lung Transplantation Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran
Leila Saliminejad Lung Transplantation Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran
Sharare Shadanfar Lung Transplantation Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran
Masoud Shafaghi Strategic Planning and Executive Office Manager of International Federation of Inventors’ Associations-IFIA, Geneva, Switzerland
Sina Aghdasi Lung Transplantation Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran
Sima Noorali Lung Transplantation Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran
Babak Sharif-Kashani Tobacco Prevention and Control Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran

DOI: https://doi.org/10.18502/jimc.v5i4.11345

Keywords: Emphasis, Macitentan, Pulmonary hypertension, Quality of life

Abstract

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.