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ALAT Alanine aminotransferase ARB Angiotensin receptor blocker ARNI Angiotensin receptor–neprilysin inhibitor ASAT Aspartate aminotransferase ASIG Australian Scleroderma Interest Group BNP Brain natriuretic peptide BPA Balloon pulmonary angioplasty BPD Bronchopulmonary dysplasia CAMPHOR Cambridge Pulmonary Hypertension Outcome Review CCB Calcium channel blocker CDH Congenital diaphragmatic hernia cGMP Cyclic guanosine monophosphate CHD Congenital heart disease CI Cardiac index; Confidence interval cMRI Cardiac magnetic resonance imaging CO Cardiac output COMPERA Comparative, Prospective Registry of Newly Initiated Therapies for PH COPD Chronic obstructive pulmonary disease CpcPH Combined post- and pre-capillary pulmonary hypertension CPET Cardiopulmonary exercise testing CPFE Combined pulmonary fibrosis and emphysema CT Computed tomography CTD Connective tissue disease CTEPD Chronic thrombo-embolic pulmonary disease CTEPH Chronic thrombo-embolic pulmonary hypertension CTPA Computed tomography pulmonary angiography DECT Dual-energy computed tomography DLCO Lung diffusion capacity for carbon monoxide DPAH Drug- or toxin-associated pulmonary arterial hypertension dPAP Diastolic pulmonary arterial pressure DPG Diastolic pressure gradient DSA Digital subtraction angiography ECG Electrocardiogram ECMO Extracorporeal membrane oxygenation EHJ European Heart Journal EMA European Medicines Agency EOV Exercise oscillatory ventilation ERA Endothelin receptor antagonist ERJ European Respiratory Journal ERN European Reference Network ERN-LUNG European Reference Network on rare respiratory diseases ERS European Respiratory Society ESC European Society of Cardiology EtD Evidence to Decision FPHR French Pulmonary Hypertension Registry FVC Forced vital capacity GRADE Grading of Recommendations, Assessment, Development, and Evaluations HAART Highly active antiretroviral therapy Hb Haemoglobin HF Heart failure HFpEF Heart failure with preserved ejection fraction HIV Human immunodeficiency virus HPAH Heritable pulmonary arterial hypertension HPS Hepatopulmonary syndrome HR Hazard ratio HR-QoL Health-related quality of life ICU Intensive care unit IgG4 Immunogolobulin G4 ILD Interstitial lung disease IPAH Idiopathic pulmonary arterial hypertension IpcPH Isolated post-capillary pulmonary hypertension IP receptor Prostacyclin I2 receptor ISWT Incremental shuttle walking test i.v. Intravenous LA Left atrium/left atrial LAS Lung allocation score LHD Left heart disease LTx Lung transplantation LV Left ventricle/left ventricular LVAD Left ventricular assist device mPAP Mean pulmonary arterial pressure MR Magnetic resonance MRI Magnetic resonance imaging NOAC Novel oral anticoagulant NT-proBNP N-terminal pro-brain natriuretic peptide OR Odds ratio PA Pulmonary artery PAC Pulmonary arterial compliance PaCO2 Partial pressure of arterial carbon dioxide PADN Pulmonary artery denervation PAH Pulmonary arterial hypertension PAH-CTD Pulmonary arterial hypertension associated with connective tissue disease PAH-SSc Pulmonary arterial hypertension associated with systemic sclerosis PAH-SYMPACT Pulmonary Arterial Hypertension-Symptoms and Impact PaO2 Partial pressure of arterial oxygen PAP Pulmonary arterial pressure PAVM Pulmonary arteriovenous malformation PAWP Pulmonary arterial wedge pressure PCH Pulmonary capillary haemangiomatosis PDE5i Phosphodiesterase 5 inhibitor PE Pulmonary embolism PEA Pulmonary endarterectomy PET Positron emission tomography PETCO2 End-tidal partial pressure of carbon dioxide PFT Pulmonary function test PH Pulmonary hypertension PH-LHD Pulmonary hypertension associated with left heart disease PICO Population, Intervention, Comparator, Outcome PoPH Porto-pulmonary hypertension PPHN Persistent pulmonary hypertension of the newborn PROM Patient-reported outcome measure PVD Pulmonary vascular disease Downloaded from academic.oup.com/eurheartj/article/43/38/3618/6673929 by guest on 07 October 2026