ISSN 2149-2263 | E-ISSN 2149-2271
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The Consistent Effectiveness and Safety of Macitentan Therapies Across Idiopathic and Congenital Heart Disease-Associated PulmonaryArterial Hypertension: A Single-Center Experience [Anatol J Cardiol]
Anatol J Cardiol. 2022; 26(10): 778-787 | DOI: 10.5152/AnatolJCardiol.2022.1889

The Consistent Effectiveness and Safety of Macitentan Therapies Across Idiopathic and Congenital Heart Disease-Associated PulmonaryArterial Hypertension: A Single-Center Experience

Cihangir Kaymaz1, Seda Tanyeri1, Hacer Ceren Tokgöz1, Özgür Yaşar Akbal1, Ali Karagöz1, Berhan Keskin1, Barkın Kültürsay1, Aykun Hakgör1, Şeyhmus Külahçıoğlu1, Zübeyde Bayram1, Süleyman Efe1, Ibrahim Halil Tanboğa2, Cem Doğan1, Mehmet Akbulut3, Nihal Özdemir1
1Department of Cardiology, University of Health Sciences Turkey, Hamidiye Faculty of Medicine, Koşuyolu Heart Training and Research Hospital, İstanbul, Turkey
2Department of Cardiology, Faculty of Medicine, Nişantaşı University, İstanbul, Turkey
3Department of Cardiology, Faculty of Medicine, Fırat University, Elazığ, Turkey

Background: In this single-center study, we evaluated efficacy and safety issues and predictors of survival in patients with idiopathic and congenital heart disease-associated pulmonary arterial hypertension who were under macitentan therapies.

Method: Our study retrospectively evaluated 221 patients with pulmonary arterial hypertension enrolled in our single-center study, and mono, dual, and triple macitentan therapies were noted in 30, 115, and 76 patients, respectively. The longitudinal changes in clinical, neurohumoral, and echocardiographic measures of pulmonary arterial hypertension were evaluated. The Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management, Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management 2.0, and Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management Lite 2 scores  at baseline, Swedish PAH Registry, Comparative Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension registry, and French Pulmonary Hypertension Network registry risk status both at baseline and first control were assessed.

Result: The median follow-up period was 1068 [415-2245] days. Macitentan was associated with significant improvements in functional class, 6-minute walk distance, N-terminal pro-brain natriuretic peptide (NT-proBNP), and echocardiographic measures without any deterioration of hemoglobin or hepatic enzymes. The low-risk scores with each model at baseline and/or first control are related to significantly better survival. Age, gender, and log-NT-proBNP in time-fixed and idiopathic pulmonary arterial hyper-tension, and log-NT-proBNP in time-dependent Cox proportional hazard regression analyses were independent predictors of mortality.

Conclusion: Mono- or sequential combination macitentan therapies were associated with sustained benefits in functional class, 6-minute walk distance, NT-proBNP, and echocardiographic measures in patients with idiopathic pulmonary arterial hypertension and con-genital heart disease-associated pulmonary arterial hypertension, and low-risk scores at baseline and/or first controls can be translated to better survival.

Keywords: Macitentan, pulmonary arterial hypertension, Eisenmenger syndrome, REVEAL-Lite 2, COMPERA, SPAHR

Cihangir Kaymaz, Seda Tanyeri, Hacer Ceren Tokgöz, Özgür Yaşar Akbal, Ali Karagöz, Berhan Keskin, Barkın Kültürsay, Aykun Hakgör, Şeyhmus Külahçıoğlu, Zübeyde Bayram, Süleyman Efe, Ibrahim Halil Tanboğa, Cem Doğan, Mehmet Akbulut, Nihal Özdemir. The Consistent Effectiveness and Safety of Macitentan Therapies Across Idiopathic and Congenital Heart Disease-Associated PulmonaryArterial Hypertension: A Single-Center Experience. Anatol J Cardiol. 2022; 26(10): 778-787

Corresponding Author: Cihangir Kaymaz
Manuscript Language: English


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