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Recommendation Table 12 — Recommendations for intensive care management for pulmonary arterial hypertension ..................... 3672 Recommendation Table 13 — Recommendations for lung transplantation ................................................................................................. 3673 Recommendation Table 14 — Recommendations for pulmonary arterial hypertension associated with drugs or toxins ................... 3674 Recommendation Table 15 — Recommendations for pulmonary arterial hypertension associated with connective tissue disease 3675 Recommendation Table 16 — Recommendations for pulmonary arterial hypertension associated with human immunodeficiency virus infection ................................................................................................... 3676 Recommendation Table 17 — Recommendations for pulmonary arterial hypertension associated with portal hypertension .......... 3677 Recommendation Table 18 — Recommendations for shunt closure in patients with pulmonary–systemic flow ratio .1.5:1 based on calculated pulmonary vascular resistance ......................... 3680 Recommendation Table 19 — Recommendations for pulmonary arterial hypertension associated with adult congenital heart disease 3680 Recommendation Table 20 — Recommendations for pulmonary arterial hypertension with signs of venous/capillary involvement 3681 Recommendation Table 21 — Recommendations for paediatric pulmonary hypertension .............................................................................. 3685 Recommendation Table 22 — Recommendations for pulmonary hypertension associated with left heart disease ................................ 3690 Recommendation Table 23 — Recommendations for pulmonary hypertension associated with lung disease and/or hypoxia .......... 3693 Recommendation Table 24 — Recommendations for chronic thrombo-embolic pulmonary hypertension and chronic thromboembolic pulmonary disease without pulmonary hypertension ........ 3698 Recommendation Table 25 — Recommendations for pulmonary hypertension centres ..................................................................................... 3702

List of tables

Table 1 Strength of the recommendations according to GRADE 3635 Table 2 Quality of evidence grades and their definitions .............. 3635 Table 3 Classes of recommendations .................................................... 3636 Table 4 Levels of evidence .......................................................................... 3636 Table 5 Haemodynamic definitions of pulmonary hypertension 3637 Table 6 Clinical classification of pulmonary hypertension ............ 3638 Table 7 Drugs and toxins associated with pulmonary arterial hypertension ..................................................................................................... 3640 Table 8 Electrocardiogram abnormalities in patients with pulmonary hypertension .............................................................................. 3643 Table 9 Radiographic signs of pulmonary hypertension and concomitant abnormalities ......................................................................... 3643 Table 10 Additional echocardiographic signs suggestive of pulmonary hypertension .............................................................................. 3645 Table 11 Haemodynamic measures obtained during right heart catheterization .................................................................................................. 3646 Table 12 Route of administration, half-life, dosages, and duration of administration of the recommended test compounds for vasoreactivity testing in pulmonary arterial hypertension ............. 3647 Table 13 Phenotypic features associated with pulmonary arterial hypertension mutations ............................................................................... 3649 Table 14 Characteristic diagnostic features of patients with different forms of pulmonary hypertension ........................................ 3651 Table 15 World Health Organization classification of functional status of patients with pulmonary hypertension ............................... 3656 Table 16 Comprehensive risk assessment in pulmonary arterial hypertension (three-strata model) .......................................................... 3657 Table 17 Suggested assessment and timing for the follow-up of patients with pulmonary arterial hypertension .................................. 3658 Table 18 Variables used to calculate the simplified four-strata risk-assessment tool ....................................................................................... 3659 Table 19 Dosing of pulmonary arterial hypertension medication in adults ............................................................................................................... 3664 Table 20 Criteria for lung transplantation and listing in patients with pulmonary arterial hypertension ................................................... 3672 Table 21 Clinical classification of pulmonary arterial hypertension associated with congenital heart disease .............................................. 3678 Table 22 Use of pulmonary arterial hypertension therapies in children ................................................................................................................ 3684 Table 23 Patient phenotyping and likelihood for left heart disease as cause of pulmonary hypertension ...................................................... 3688 Table 24 Pulmonary hypertension with unclear and/or multifactorial mechanisms ........................................................................... 3699

List of figures

Figure 1 Central illustration ........................................................................ 3639 Figure 2 Symptoms in patients with pulmonary hypertension ... 3641 Figure 3 Clinical signs in patients with pulmonary hypertension 3642 Figure 4 Transthoracic echocardiographic parameters in the assessment of pulmonary hypertension ................................................ 3644 Figure 5 Echocardiographic probability of pulmonary hypertension and recommendations for further assessment ...... 3645 Figure 6 Diagnostic algorithm of patients with unexplained dyspnoea and/or suspected pulmonary hypertension .................... 3650 Figure 7 Pathophysiology and current therapeutic targets of pulmonary arterial hypertension (group 1) ......................................... 3655 Figure 8 Vasoreactivity testing algorithm of patients with presumed diagnosis of idiopathic, heritable, or drug-associated pulmonary arterial hypertension .............................................................. 3665 Figure 9 Evidence-based pulmonary arterial hypertension treatment algorithm for patients with idiopathic, heritable, drug-associated, and connective tissue disease-associated pulmonary arterial hypertension .............................................................. 3666 Figure 10 Neonatal and paediatric vs. adult pulmonary hypertension ..................................................................................................... 3682 Figure 11 Pathophysiology of pulmonary hypertension associated with left heart disease (group 2) .............................................................. 3686 Figure 12 Pathophysiology of pulmonary hypertension associated with lung disease (group 3) ........................................................................ 3691 Figure 13 Diagnostic strategy in chronic thrombo-embolic pulmonary hypertension .............................................................................. 3694 Figure 14 Management strategy in chronic thrombo-embolic pulmonary hypertension .............................................................................. 3696 Figure 15 Overlap in treatments/multimodality approaches in chronic thrombo-embolic pulmonary hypertension ....................... 3697 Figure 16 Pulmonary hypertension centre schematic ..................... 3701

Abbreviations and acronyms

6MWD 6-minute walking distance 6MWT 6-minute walking test ABG Arterial blood gas analysis ACEi Angiotensin-converting enzyme inhibitor Downloaded from academic.oup.com/eurheartj/article/43/38/3618/6673929 by guest on 07 October 2026