☰ 目次
2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension
European Society of Cardiology・2022・114 ページ · 弁膜症・大動脈・末梢・肺循環 · 予防・高血圧・脂質・糖尿病 · 呼吸器・睡眠
目次 閉じる
2022 ESC/ERS Guidelines for the diagnosis and1 treatment of pulmonary hypertension1 Table of contents2 List of tables5 List of figures5 Abbreviations and acronyms5 1 Preamble7 2 Introduction8 2.1 What is new8 2.2 Methods18 3 Definitions and classifications19 3.1 Definitions19 Class III Evidence or general agreement that the given treatment or procedure is not19 Class II19 Level of evidence A19 Level of evidence B19 Level of evidence C19 3.2 Classifications20 4 Epidemiology and risk factors21 4.1 Group 1, pulmonary arterial21 1.1 Idiopathic21 1.2 Heritablea21 1.3 Associated with drugs and toxinsa21 1.6 Persistent PH of the newborn21 2.2 Valvular heart disease21 2.3 Congenital/acquired cardiovascular conditions leading to21 3.1 Obstructive lung disease or emphysema21 3.2 Restrictive lung disease 3.3 Lung disease with mixed restrictive/obstructive pattern21 3.4 Hypoventilation syndromes21 3.5 Hypoxia without lung disease (e.g. high altitude) 3.6 Developmental lung disorders21 4.1 Chronic thrombo-embolic PH 4.2 Other pulmonary artery obstructionsc21 5.1 Haematological disordersd21 5.2 Systemic disorderse21 5.3 Metabolic disordersf21 5.6 Fibrosing mediastinitis21 2 10 20 30 40 50 6022 2 0 2 3 4 5 6 0 122 PULMONARY HYPERTENSION22 4.2 Group 2, pulmonary hypertension23 4.3 Group 3, pulmonary hypertension23 4.4 Group 4, pulmonary hypertension23 4.5 Group 5, pulmonary hypertension23 5 Pulmonary hypertension23 diagnosis23 5.1 Diagnosis23 Early24 5.2 Diagnostic algorithm31 2 saturation33 5.3 Screening and early detection35 6 Pulmonary arterial38 hypertension (group 1)38 6.1 Clinical characteristics38 6.2 Severity and risk assessment38 Pulmonary vasculopathy Right heart failure38 Current therapeutic targets38 NO-sGC-cGMP pathway Endothelin pathway Prostacyclin pathway38 6.3 Therapy43 5.0 μg 6–9 times per day47 1.25 ng/kg/47 7 Specific pulmonary arterial57 hypertension subsets57 7.1 Pulmonary arterial hypertension57 7.2 Pulmonary arterial hypertension58 7.3 Pulmonary arterial hypertension59 7.4 Pulmonary arterial hypertension60 7.5 Pulmonary arterial hypertension61 7.6 Pulmonary arterial hypertension63 7.7 Pulmonary arterial hypertension63 7.8 Paediatric pulmonary hypertension64 1 Congenital PVS Left heart failure Congenital MS Cardiomyopathy65 3 Congenital PS Congenital PS CTEPH65 8 Pulmonary hypertension68 associated with left heart disease (group 2)68 8.1 Definition, prognosis, and68 Variable degree of pulmonary congestion, vasoconstriction, vascular remodelling69 Left ventricular phenotype Right ventricular phenotype69 Pulmonary vascular disease69 Pulmonary congestion69 No PH IpcPH CpcPHa69 HFrEF Aortic valve HFmrEF69 HFpEF Mitral valve69 Heart failure/cardiomyopathy Valvular heart disease69 Controlled69 LHD69 RV–PA uncoupling69 20 1569 5 1069 20 40 60 80 100 12069 8.2 Diagnosis70 8.3 Therapy71 9 Pulmonary hypertension73 associated with lung diseases and/ or hypoxia (group 3)73 Remodelling of airways, lung parenchyma, and vessels74 Mostly ventilatory exercise limitation74 Mostly circulatory exercise limitation74 Remodelling of pulmonary vessels74 Remodelling of airways and parenchyma74 Hypoxaemia at rest and/or during exercise74 COPD ILD74 CPFE74 Emphysema Fibrosis Vascular pruning74 CPFE74 9.1 Diagnosis75 9.2 Therapy75 10 Chronic thrombo-embolic76 pulmonary hypertension (group 4)76 10.1 Diagnosis77 10.2 Therapy78 1 2 380 10.3 Chronic thrombo-embolic81 11 Pulmonary hypertension with82 unclear and/or multifactorial mechanisms (group 5)82 11.1 Haematological disorders82 11.2 Systemic disorders83 11.3 Metabolic disorders83 11.4 Chronic kidney failure83 11.5 Pulmonary tumour thrombotic83 11.6 Fibrosing mediastinitis83 12 Definition of a pulmonary84 hypertension centre84 Pulmonary hypertension centre84 12.1 Facilities and skills required for a85 12.2 European Reference Network85 12.3 Patient associations and patient85 13 Key messages86 14 Gaps in evidence86 14.1 Pulmonary arterial hypertension86 (group 1)86 14.2 Pulmonary hypertension associated86 with left heart disease (group 2)86 14.3 Pulmonary hypertension associated87 with lung diseases and/or hypoxia (group 3)87 14.4 Chronic thrombo-embolic87 pulmonary hypertension (group 4)87 14.5 Pulmonary hypertension with87 unclear and/or multifactorial mechanisms (group 5)87 15 ‘What to do’ and ‘What not to do’ messages from the Guidelines87 16 Quality indicators93 17 Supplementary data94 18 Data availability statement94 19 Author information94 20 Appendix94 21 References95
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