Yesterday, the 2022 ESC and European Respiratory Society (ERS) Guidelines for the diagnosis and treatment of pulmonary hypertension (PH) were presented.1 The Task Force Chairs were Professor Stephan Rosenkranz (University Hospital Cologne - Cologne, Germany; ESC Task Force Chair) and Professor Marion Delcroix (University Hospitals of Leuven - Leuven, Belgium; ERS Task Force Chair).
“In recent years, substantial progress has been made in detecting and managing PH,” says Prof. Rosenkranz. “All the new evidence has been integrated into this fourth edition of the ESC/ERS Guidelines, with multidisciplinary input from the Task Force, which included cardiologists and pneumologists, a thoracic surgeon, methodologists and patients among its members.” The 2022 guidelines cover the whole spectrum of PH, with an emphasis on diagnosing and treating pulmonary arterial hypertension (PAH) and chronic thrombo-embolic pulmonary hypertension (CTEPH).
So what’s new? The 6th World Symposium on Pulmonary Hypertension in 2019 reconsidered the haemodynamic definition of PH2 and amended PH definitions have been endorsed and expanded in the 2022 ESC/ERS Guidelines. The haemodynamic definition of PH in general has been updated to mean pulmonary arterial pressure (mPAP) >20 mmHg. The definition of PAH also implies a pulmonary vascular resistance (PVR) >2 WU and a pulmonary arterial wedge pressure ≤15 mmHg. “These cut-off values better reflect the limits of normal ranges and are associated with mortality,” notes Prof. Delcroix, “but it should be pointed out that they do not yet translate into new therapeutic recommendations in PAH since the efficacy of therapies in patients with pulmonary vascular disease and an mPAP 21–24 mmHg and/or PVR 2–3 WU remain unknown.”
The main diagnostic algorithm for PH has been simplified aiming at earlier detection in the community and now follows a three-step approach, from suspicion by first-line physicians, to detection by echocardiography and confirmation with right heart catheterisation in PH centres.
In addition, expedited referral is recommended for high-risk or complex patients. To shorten the time from symptom onset to diagnosis of PAH, screening strategies for PAH in patients with scleroderma and in patients at risk of heritable PAH are proposed based on recent results from cohort studies. To improve underdiagnosis of CTEPH, the new guidelines advocate enhanced recognition of computed tomography and echocardiographic signs at the time of acute pulmonary embolism (PE), together with a systematic follow-up of patients with acute PE, as indicated in the 2019 ESC Guidelines for the diagnosis and management of acute pulmonary embolism.3
Echocardiographic and cardiac magnetic resonance imaging criteria have been added to the risk-stratification table, refining non-invasive evaluation at diagnosis. Furthermore, a four-strata risk stratification, dividing the large, intermediate-risk group into intermediate-low and intermediate-high risk, is proposed at follow-up.
The PAH treatment algorithm has been simplified, highlighting the importance of cardiopulmonary comorbidities, risk assessment both at diagnosis and at follow-up, and the importance of combination therapies. Treatment strategies during follow-up are based on the four-strata risk model and are intended to facilitate clinical decision-making.
The guidelines emphasise the importance of group 2 PH, i.e. in patients with left heart disease. In group 3 PH, for the first time there is a recommendation for PH medical therapy, based on a single positive randomised trial in patients with interstitial lung disease. In addition, the treatment algorithm for CTEPH has been modified, including multi-modal therapy with surgery, drugs and balloon pulmonary angioplasty. Supervised exercise training is now recommended in patients with PAH under medical therapy.
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, which have been extrapolated from those in adults and adapted for age. There are also updates to the recommendations on sex-related issues in patients with PAH, including pregnancy. The guidelines end with the definition of a PH centre, with new recommendations that PH centres maintain a PH registry and collaborate with patient associations.
Read all the latest recommendations in full in the European Heart Journal.
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