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and implement all ESC Guidelines. Pulmonary national societies are also encouraged to share these guidelines with their members and develop a summary or editorials in their own language, if appropriate. Implementation programmes are needed because it has been shown that the outcome of disease may be favourably influenced by the thorough application of clinical recommendations. Health professionals are encouraged to take the ESC/ERS Guidelines fully into account when exercising their clinical judgement, as well as in determining and implementing preventive, diagnostic, or therapeutic medical strategies. However, the ESC/ERS Guidelines do not override, in any way, the individual responsibility of health professionals to make appropriate and accurate decisions in considering each patient’s health condition and in consulting with that patient or the patient’s caregiver where appropriate and/or necessary. It is also the health professional’s responsibility to verify the rules and regulations applicable to drugs and devices at the time of prescription and, where appropriate, to respect the ethical rules of their profession in each country. Off-label use of medication may be presented in these guidelines if a sufficient level of evidence shows that it can be considered medically appropriate to a given condition and if patients could benefit from the recommended therapy. However, the final decisions concerning an individual patient must be made by the responsible health professional, giving special consideration to: • The specific situation of the patient. In this respect, it is specified that, unless otherwise provided for by national regulations, offlabel use of medication should be limited to situations where it is in the patient’s interest to do so, with regards to the quality, safety, and efficacy of care, and only after the patient has been fully informed and provided consent. • Country-specific health regulations, indications by governmental drug regulatory agencies, and the ethical rules to which health professionals are subject, where applicable.

2 Introduction

Pulmonary hypertension (PH) is a pathophysiological disorder that may involve multiple clinical conditions and may be associated with a variety of cardiovascular and respiratory diseases. The complexity of managing PH requires a multifaceted, holistic, and multidisciplinary approach, with active involvement of patients with PH in partnership with clinicians. Streamlining the care of patients with PH in daily clinical practice is a challenging but essential requirement for effectively managing PH. In recent years, substantial progress has been made in detecting and managing PH, and new evidence has been timeously integrated in this fourth edition of the ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Reflecting the multidisciplinary input into managing patients with PH and interpreting new evidence, the Task Force included cardiologists and pneumologists, a thoracic surgeon, methodologists, and patients. These comprehensive clinical practice guidelines cover the whole spectrum of PH, with an emphasis on diagnosing and treating pulmonary arterial hypertension (PAH) and chronic thrombo-embolic pulmonary hypertension (CTEPH).

2.1 What is new

One of the most important proposals from the 6th World Symposium on Pulmonary Hypertension (WSPH) was to reconsider the haemodynamic definition of PH.1 After careful evaluation, the new definitions of PH have been endorsed and expanded in these guidelines, including a revised cut-off level for pulmonary vascular resistance (PVR) and a definition of exercise PH.

The classification of PH has been updated, including repositioning of vasoreactive patients with idiopathic pulmonary arterial hypertension (IPAH) and a revision of group 5 PH, including repositioning of PH in lymphangioleiomyomatosis in group 3. Concerning the diagnosis of PH, a new algorithm has been developed aiming at earlier detection of PH in the community. In addition, expedited referral is recommended for high-risk or complex patients. Screening strategies are also proposed. The risk-stratification table has been expanded to include additional echocardiographic and cardiac magnetic resonance imaging (cMRI) prognostic indicators. The recommendations for initial drug therapies have been simplified, building on this revised, three-strata, multiparametric risk model to replace functional classification. At follow-up, a four-strata risk-assessment tool is now proposed based on refined cut-off levels for World Health Organization functional class (WHO-FC), 6-minute walking distance (6MWD), and N-terminal pro-brain natriuretic peptide (NT-proBNP), categorizing patients as low, intermediate–low, intermediate–high, or high risk. The PAH treatment algorithm has been modified, highlighting the importance of cardiopulmonary comorbidities, risk assessment both at diagnosis and follow-up, and the importance of combination therapies. Treatment strategies during follow-up have been based on the fourstrata model intended to facilitate more granular decision-making. The recommendations for managing PH associated with left heart disease (PH-LHD) and lung disease have been updated, including a new haemodynamic definition of severe PH in patients with lung disease. In group 4 PH, the term chronic thrombo-embolic pulmonary disease (CTEPD) with or without PH has been introduced, acknowledging the presence of similar symptoms, perfusion defects, and organized fibrotic obstructions in patients with or without PH at rest. Interventional treatment by balloon pulmonary angioplasty (BPA) in combination with medical therapy has been upgraded in the therapeutic algorithm of CTEPH. New standards for PH centres have been presented and, for the first time, patient representatives were actively involved in developing these guidelines. Questions with direct consequences for clinical practitioners regarding each PH classification subgroup were selected and addressed, namely guidance on: initial treatment strategy for group 1 PH (Population, Intervention, Control, Outcome [PICO] I); use of oral phosphodiesterase 5 inhibitors (PDE5is) for the treatment of group 2 PH (PICO II); use of oral PDE5is for the treatment of group 3 PH (PICO III); and use of PH drugs prior to BPA for the treatment of group 4 PH (PICO IV). These questions were considered to be important because: most contemporary PH registries describe variable use of initial oral monotherapy and combination therapy; large case series show widespread use of PDE5is in group 2 PH, despite a class III recommendation in the 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension; large case series show widespread use of PDE5is in group 3 PH, despite a class III recommendation in the 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension; and there is no clear guidance for therapy with PH drugs in patients with inoperable CTEPH prior to BPA. Downloaded from academic.oup.com/eurheartj/article/43/38/3618/6673929 by guest on 07 October 2026