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empower patients and improve the patient experience, addressing issues such as health literacy, digital skills, healthy lifestyles, mental health, and self-management. Health care can be delivered more effectively and efficiently if patients are full partners in the process.

13 Key messages

(1) The haemodynamic definition of PH has been updated as mPAP .20 mmHg. The definition of PAH also implies a PVR .2 WU and PAWP ≤15 mmHg. These cut-off values better reflect the limits of normal ranges, but do not yet translate into new therapeutic recommendations, since the efficacy of PAH therapy in patients with PVD and an mPAP 21– 24 mmHg and/or PVR 2–3 WU is still unknown. (2) The main diagnostic algorithm for PH has been simplified following a three-step approach, from suspicion by first-line physicians, detection by echocardiography, and confirmation with RHC in PH centres. Warning signs associated with worse outcomes have been identified, which justify immediate referral and management in PH centres. (3) Screening strategies for PAH in patients with SSc and in those at risk of HPAH are proposed based on the results of published cohort studies. Their implementation may shorten the time from symptom onset to diagnosis of PAH. (4) An improved recognition of CT and echocardiographic signs of CTEPH at the time of an acute PE event, together with a systematic follow-up of patients with acute PE, as indicated in the 2019 ESC/ERS Guidelines for the diagnosis and management of acute pulmonary embolism, should help to remediate the underdiagnosis of CTEPH. (5) The three-strata risk-stratification assessment in PAH has been refined after being validated in multiple registries. The MRI and echocardiographic criteria have been added to the ESC/ERS table, refining non-invasive evaluation at diagnosis. (6) A four-strata risk stratification, dividing the large, intermediate-risk group into intermediate–low and intermediate–high risk, is proposed at follow-up. (7) The treatment algorithm for PAH has been simplified, with a clear focus on risk assessment, cardiopulmonary comorbidities, and treatment goals. Initial combination therapy and treatment escalation at follow-up when appropriate are current standards. (8) The Task Force has attempted to close the gap between paediatric and adult PAH care, with therapeutic and follow-up strategies based on risk stratification and treatment response, extrapolated from that in adults but adapted for age. (9) The recommendations on sex-related issues in patients with PAH, including pregnancy, have been updated, with information and shared decision-making as key points. (10) The recommendations for rehabilitation and exercise programmes in PH have been updated following the release of additional supportive evidence. (11) For the first time, there is a recommendation for PH medical therapy in group 3 PH, based on a single positive RCT in patients with ILD. (12) The concept of CTEPD with or without PH has been intro-

duced, enabling further research on the natural history and management in the absence of PH. (13) The treatment algorithm for CTEPH has been modified, includ-

ing multimodal therapy with surgery, PH drugs, and BPA.

14 Gaps in evidence

14.1 Pulmonary arterial hypertension

(group 1)

• The efficacy and safety of PAH drugs in group 1 patients with an mPAP 21–24 mmHg, PVR 2–3 WU, and exercise PH has to be established. • The role of PAH drugs in different PAH subgroups, including schistosomiasis-associated PAH, needs to be explored. • Risk-stratification assessment in PAH needs to be further prospectively validated through goal-orientated outcome studies, and optimized for patients with PAH and comorbidities. • New PAH phenotypes observed in patients with significant cardiopulmonary comorbidities are common and should be the focus of more research. • The importance of PAH patient phenotypes and the relevance of comorbidities on treatment goals and outcomes must be further evaluated. • The impact of PAH therapies and treatment strategies on survival needs to be further assessed. • Pulmonary arterial hypertension drugs targeting novel pathways are emerging and the impact of add-on use of this medication on outcomes has to be evaluated in RCTs. • The role of RV imaging techniques (echocardiography, cMRI) in diagnosing and stratifying risk in PAH needs to be further studied. The proposed cut-off values for risk stratification need to be properly validated in multicentre studies. • The role of CPET in the early diagnosis of PAH in populations at risk of developing PAH, and in assessing prognosis in PAH on top of clinical and haemodynamic data, needs further investigation. • The role of exercise echocardiography and exercise RHC in patients at risk of developing PAH, with abnormal CPET but normal rest echocardiogram, also needs further evaluation. • The use of mechanical circulatory support, particularly in reversible PH or in patients with advanced right HF with an exit strategy (such as LTx), has to be further studied. • Differences in natural history and treatment response between adults and children should be further investigated. • Further studies are needed on the effects of PADN in PAH and in other PH groups. • The impact of centre volume, organization, and expertise on treatment outcome needs further investigation.

14.2 Pulmonary hypertension associated

with left heart disease (group 2)

• The management of patients with group 2 PH needs further study with RCTs. • Additional research is needed to facilitate non-invasive diagnosis of HFpEF-associated PH and distinguishing it from PAH. • The role of fluid challenge and exercise testing to reveal left HF needs further validation. Downloaded from academic.oup.com/eurheartj/article/43/38/3618/6673929 by guest on 07 October 2026