First Report of Real World Utilization of an Activin Signaling Inhibitor in Patients With Severe Pulmonary Arterial Hypertension in the Middle East PH RISE-ME Study.
5 affiliations
- 1Department of Academics and Medical Education Mediclinic Airport Road Hospital Abu Dhabi United Arab Emirates.
- 2Department of Cardiology Mediclinic Airport Road Hospital Abu Dhabi United Arab Emirates.
- 3Respiratory Division, Integrated Hospital Institute Cleveland Clinic Abu Dhabi United Arab Emirates.
- 4Basic Medical Sciences, College of Medicine University of Sharjah Sharjah United Arab Emirates.
- 5Department of Pulmonary and Critical Care Medicine Lahey Hospital and Medical Center Burlington USA.
Abstract
Pulmonary arterial hypertension (PAH) remains associated with substantial morbidity and mortality despite advances in combination therapy. Sotatercept, an activin-signaling inhibitor, is a novel disease-modifying therapy targeting pulmonary vascular remodeling. Real-world data from Middle Eastern populations remain limited. We conducted a retrospective single-center cohort study evaluating the real-world effectiveness and safety of Sotatercept in adults with PAH treated at a tertiary pulmonary hypertension expert center in Abu Dhabi, United Arab Emirates, between January 2025 and September 2026. Clinical, echocardiographic, biochemical, and risk-stratification data were extracted from electronic medical records. Primary outcomes were changes in right atrial (RA) area and NT-proBNP. Secondary outcomes included systolic pulmonary arterial pressure (SPAP), TAPSE/SPAP ratio, 6-min walk distance (6MWD), risk profile, and safety. Thirty-six patients were prescribed Sotatercept, of whom 33 received at least one dose. Most patients were female (80.6%), with a mean age of 55 years. Significant reductions occurred in RA area (- 2.67 cm²; 95% CI, -4.16 to -1.18; p < 0.001), NT-proBNP (- 46.7 pmol/L; 95% CI, -87.19 to -20.39; p < 0.001), and SPAP (- 6.00 mmHg; 95% CI, -12.00 to -0.50; p = 0.031). TAPSE increased significantly (+ 0.25 cm; 95% CI, 0.17 to 0.33; p < 0.001), and TAPSE/SPAP improved significantly (+ 0.064; 95% CI, 0.030 to 0.108; p < 0.001). Patients improved by at least one risk category. The 6MWD did not change significantly. Treatment was well tolerated, with no adverse events leading to treatment discontinuation or death; two all-cause deaths occurred during follow-up. In this real-world Middle Eastern PAH cohort, Sotatercept was associated with improved right-heart remodeling, NT-proBNP, and risk profile, with acceptable short-term safety. These findings complement trial evidence and provide early regional support for activin pathway inhibition in routine practice.
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BibTeX
@article{jabi2026first,
title = {{First Report of Real World Utilization of an Activin Signaling Inhibitor in Patients With Severe Pulmonary Arterial Hypertension in the Middle East PH RISE-ME Study.}},
author = {Mutia Al Jabi and Hani Sabbour and Lama Mohamed and Sarah Alsultan and Maryam Almassabi and Moaz Osama and Amal Abudouleh and Malak Sondoqah and Mohamed Khalid Zaki and Ahmed Hesham and Khaled M Saleh and Mawhid U I Haque and Ioana Preston},
journal = {Pulmonary circulation},
year = {2026},
month = oct,
doi = {10.1002/pul2.70434},
url = {https://europepmc.org/article/MED/42924171},
note = {CC BY-NC},
}
RIS
TY - JOUR
TI - First Report of Real World Utilization of an Activin Signaling Inhibitor in Patients With Severe Pulmonary Arterial Hypertension in the Middle East PH RISE-ME Study.
AU - Mutia Al Jabi
AU - Hani Sabbour
AU - Lama Mohamed
AU - Sarah Alsultan
AU - Maryam Almassabi
AU - Moaz Osama
AU - Amal Abudouleh
AU - Malak Sondoqah
AU - Mohamed Khalid Zaki
AU - Ahmed Hesham
AU - Khaled M Saleh
AU - Mawhid U I Haque
AU - Ioana Preston
PY - 2026
DA - 2026/10/22
JO - Pulmonary circulation
DO - 10.1002/pul2.70434
UR - https://europepmc.org/article/MED/42924171
AB - Pulmonary arterial hypertension (PAH) remains associated with substantial morbidity and mortality despite advances in combination therapy. Sotatercept, an activin-signaling inhibitor, is a novel disease-modifying therapy targeting pulmonary vascular remodeling. Real-world data from Middle Eastern populations remain limited. We conducted a retrospective single-center cohort study evaluating the real-world effectiveness and safety of Sotatercept in adults with PAH treated at a tertiary pulmonary hypertension expert center in Abu Dhabi, United Arab Emirates, between January 2025 and September 2026. Clinical, echocardiographic, biochemical, and risk-stratification data were extracted from electronic medical records. Primary outcomes were changes in right atrial (RA) area and NT-proBNP. Secondary outcomes included systolic pulmonary arterial pressure (SPAP), TAPSE/SPAP ratio, 6-min walk distance (6MWD), risk profile, and safety. Thirty-six patients were prescribed Sotatercept, of whom 33 received at least one dose. Most patients were female (80.6%), with a mean age of 55 years. Significant reductions occurred in RA area (- 2.67 cm²; 95% CI, -4.16 to -1.18; p < 0.001), NT-proBNP (- 46.7 pmol/L; 95% CI, -87.19 to -20.39; p < 0.001), and SPAP (- 6.00 mmHg; 95% CI, -12.00 to -0.50; p = 0.031). TAPSE increased significantly (+ 0.25 cm; 95% CI, 0.17 to 0.33; p < 0.001), and TAPSE/SPAP improved significantly (+ 0.064; 95% CI, 0.030 to 0.108; p < 0.001). Patients improved by at least one risk category. The 6MWD did not change significantly. Treatment was well tolerated, with no adverse events leading to treatment discontinuation or death; two all-cause deaths occurred during follow-up. In this real-world Middle Eastern PAH cohort, Sotatercept was associated with improved right-heart remodeling, NT-proBNP, and risk profile, with acceptable short-term safety. These findings complement trial evidence and provide early regional support for activin pathway inhibition in routine practice.
SN - 2045-8932
SN - 2045-8940
ER -