p.4 抽出テキスト

7.7. Pulmonary arterial hypertension with signs of venous/ capillary involvement .................................................................................... 3680 7.7.1. Diagnosis ........................................................................................... 3681 7.7.2. Therapy .............................................................................................. 3681 7.8. Paediatric pulmonary hypertension ............................................... 3681 7.8.1. Epidemiology and classification ................................................ 3681 7.8.2. Diagnosis and risk assessment ................................................. 3683 7.8.3. Therapy .............................................................................................. 3683 8. Pulmonary hypertension associated with left heart disease (group 2) .................................................................................................................. 3685 8.1. Definition, prognosis, and pathophysiology ............................... 3685 8.2. Diagnosis ................................................................................................... 3687 8.2.1. Diagnosis and control of the underlying left heart disease ................................................................................................ 3687 8.2.2. Evaluation of pulmonary hypertension and patient phenotyping .................................................................................................. 3687 8.2.3. Invasive assessment of haemodynamics ............................... 3687 8.3. Therapy ..................................................................................................... 3688 8.3.1. Pulmonary hypertension associated with left-sided heart failure ............................................................................................................... 3688

8.3.1.1. Heart failure with reduced ejection fraction ............. 3688 8.3.1.2. Heart failure with preserved ejection fraction ......... 3688 8.3.1.3. Interatrial shunt devices ..................................................... 3689 8.3.1.4. Remote pulmonary arterial pressure monitoring in heart failure ............................................................................................. 3689 8.3.2. Pulmonary hypertension associated with valvular heart disease ............................................................................................................. 3689 8.3.2.1. Mitral valve disease ............................................................... 3689 8.3.2.2. Aortic stenosis ....................................................................... 3689 8.3.2.3. Tricuspid regurgitation ........................................................ 3689 8.3.3. Recommendations on the use of drugs approved for PAH in PH-LHD ......................................................................................... 3689 9. Pulmonary hypertension associated with lung diseases and/or hypoxia (group 3) ................................................................................................ 3690 9.1. Diagnosis ................................................................................................... 3692 9.2. Therapy ..................................................................................................... 3692 9.2.1. Pulmonary hypertension associated with chronic obstructive pulmonary disease or emphysema ............................ 3692 9.2.2. Pulmonary hypertension associated with interstitial lung disease ............................................................................................................. 3692 9.2.3. Recommendations on the use of drugs approved for PAH in PH associated with lung disease .......................................... 3693 10. Chronic thrombo-embolic pulmonary hypertension (group 4) 3693 10.1. Diagnosis ................................................................................................ 3694 10.2. Therapy ................................................................................................... 3695 10.2.1. Surgical treatment ....................................................................... 3695 10.2.2. Medical therapy ............................................................................ 3695 10.2.3. Interventional treatment .......................................................... 3696 10.2.4. Multimodal treatment ............................................................... 3697 10.2.5. Follow-up ........................................................................................ 3698 10.3. Chronic thrombo-embolic pulmonary hypertension team and experience criteria ................................................................................ 3698 11. Pulmonary hypertension with unclear and/or multifactorial mechanisms (group 5) ....................................................................................... 3699 11.1. Haematological disorders ................................................................ 3699 11.2. Systemic disorders ............................................................................. 3700 11.3. Metabolic disorders ........................................................................... 3700 11.4. Chronic kidney failure ...................................................................... 3700 11.5. Pulmonary tumour thrombotic microangiopathy ................ 3700 11.6. Fibrosing mediastinitis ....................................................................... 3700 12. Definition of a pulmonary hypertension centre ............................. 3701 12.1. Facilities and skills required for a pulmonary hypertension centre .................................................................................................................. 3702 12.2. European Reference Network ..................................................... 3702 12.3. Patient associations and patient empowerment ................... 3702 13. Key messages ................................................................................................. 3703 14. Gaps in evidence ........................................................................................... 3703 14.1. Pulmonary arterial hypertension (group 1) ............................. 3703 14.2. Pulmonary hypertension associated with left heart disease (group 2) ............................................................................................................ 3703 14.3. Pulmonary hypertension associated with lung diseases and/ or hypoxia (group 3) .................................................................................... 3704 14.4. Chronic thrombo-embolic pulmonary hypertension (group 4) ............................................................................................................ 3704 14.5. Pulmonary hypertension with unclear and/or multifactorial mechanisms (group 5) ................................................................................. 3704 15. ‘What to do’ and ‘What not to do’ messages from the Guidelines ....................................................................................................... 3704 16. Quality indicators ......................................................................................... 3710 17. Supplementary data ..................................................................................... 3711 18. Data availability statement ....................................................................... 3711 19. Author information ..................................................................................... 3711 20. Appendix .......................................................................................................... 3711 21. References ....................................................................................................... 3712

Tables of Recommendations Recommendation Table 1 — Recommendations for right heart catheterization and vasoreactivity testing ............................................ 3648 Recommendation Table 2 — Recommendations for diagnostic strategy ................................................................................................................ 3652 Recommendation Table 3 — Recommendations for screening and improved detection of pulmonary arterial hypertension and chronic thrombo-embolic pulmonary hypertension ....................... 3654 Recommendation Table 4 — Recommendations for evaluating the disease severity and risk of death in patients with pulmonary arterial hypertension ..................................................................................... 3660 Recommendation Table 5 — Recommendations for general measures and special circumstances ....................................................... 3662 Recommendation Table 6 — Recommendations for women of childbearing potential .................................................................................... 3663 Recommendation Table 7 — Recommendations for the treatment of vasoreactive patients with idiopathic, heritable, or drug-associated pulmonary arterial hypertension ............................ 3664 Recommendation Table 8 — Recommendations for the treatment of non-vasoreactive patients with idiopathic, heritable, or drug-associated pulmonary arterial hypertension who present without cardiopulmonary comorbiditiesa ................................................... 3668 Recommendation Table 9 — Recommendations for initial oral drug combination therapy for patients with idiopathic, heritable, or drug-associated pulmonary arterial hypertension without cardiopulmonary comorbidities ................................................................ 3669 Recommendation Table 10 — Recommendations for sequential drug combination therapy for patients with idiopathic, heritable, or drug-associated pulmonary arterial hypertension ...................... 3670 Recommendation Table 11 — Recommendations for the treatment of non-vasoreactive patients with idiopathic, heritable, or drug-associated pulmonary arterial hypertension who present with cardiopulmonary comorbiditiesa .................................................... 3671 Downloaded from academic.oup.com/eurheartj/article/43/38/3618/6673929 by guest on 07 October 2026