, Mehmet Akbulut 2
, Ali Akdoğan 3
, Halil Ataş 4
, Gülen Hatemi 5
, Ergün Barış Kaya 6
, M. Serdar Küçükoğlu 7
, Bülent Mutlu 4
, Gülfer Okumuş 8
, Zeynep Pınar Önen 9
, Bahri Akdeniz 10
, Reviewer1: Zeki Öngen 1
, Reviewer 2: Züleyha Bingöl 11
2Department of Cardiology, Faculty of Medicine, Firat University, Elazığ, Türkiye
3Department of Internal Medicine, Division of Rheumatology, Faculty of Medicine, Hacettepe University, Ankara, Türkiye
4Department of Cardiology, Pendik Training and Research Hospital, Marmara University, İstanbul, Türkiye
5Department of Internal Medicine, Division of Rheumatology, Cerrahpaşa Faculty of Medicine, İstanbul University-Cerrahpaşa, İstanbul, Türkiye
6Department of Cardiology, Faculty of Medicine, Hacettepe University, Ankara, Türkiye
7Department of Cardiology, Cardiology Institute, İstanbul University-Cerrahpaşa, İstanbul, Türkiye
8Department of Pulmonology, Faculty of Medicine, İstanbul University, İstanbul, Türkiye
9Department of Pulmonology, Faculty of Medicine, Ankara University, Ankara, Türkiye
10Department of Cardiology, Faculty of Medicine, Dokuz Eylül University, İzmir, Türkiye
11Department of Pulmonary Medicine, İstanbul Faculty of Medicine, İstanbul University, İstanbul, Türkiye
Abstract
Pulmonary arterial hypertension (PAH) is a progressive and life-threatening disease characterized by increased pulmonary vascular resistance and right ventricular (RV) dysfunction, requiring early diagnosis and timely management. This joint expert consensus report, developed in collaboration with the Turkish Society of Cardiology, Turkish Thoracic Society, and Pulmonary Vascular Disease Research Association (ADHAD), provides a practical, real-world framework for the diagnosis and management of PAH in Türkiye. The document presents updated, practice-oriented diagnostic algorithms designed to facilitate early recognition and accurate classification of PAH. A key objective is the standardization of data collection across centers; therefore, recommended echocardiographic and hemodynamic parameters, along with their normal and pathological ranges, are clearly defined to improve consistency in clinical evaluation and reporting. Risk assessment remains a cornerstone of patient evaluation; however, current guideline-based models have important limitations. In a disease with high mortality such as PAH, greater emphasis should be placed on dynamic risk evaluation at follow-up, incorporating detailed RV assessment through imaging and hemodynamic parameters, and enabling more timely treatment escalation when needed. Accordingly, treatment algorithms have been updated to reflect contemporary therapeutic strategies, emphasizing early combination therapy, individualized management, and prompt escalation in patients who fail to achieve or maintain a low-risk profile with particular attention to RV performance. Overall, this consensus aims to harmonize clinical practice, enhance data quality, and support more proactive and standardized management of PAH across Türkiye.