The 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension

The 2022 guideline on pulmonary hypertension (PH) was a joint project of the European Society of Cardiology and the European Respiratory Society (ERS).

The new guideline was developed by the task force for the diagnosis and treatment of pulmonary hypertension of the ESC and the ERS [1–3]. Prof. Stephan Rosenkranz (Heart Center at the University Cologne, Germany) pointed out that patient representatives were actively involved for the first time.

PH is not a rare condition as it affects 1% of the global and 10% of the elderly population. The clinical classification of PH in 5 groups was maintained in the novel guideline, with only minor changes. “When assessing patients, it is of utmost importance to find the right diagnosis, because therapy is different,” Prof. Rosenkranz stressed. In the new guideline, there are minor changes in the haemodynamic definition of PH, for example:

• The threshold of PH was lowered to a mean pulmonary arterial pressure (mPAP) >20 mmHg
• The threshold of pre-capillary PH was lowered to a pulmonary vascular resistance (PVR) >2 Woods units (WU)
• The threshold of isolated post-capillary PH was lowered to a PVR ≤2 WU
• The threshold of combined post- and pre-capillary PH was lowered to a PVR >2 WU.

New diagnostic algorithm aims at earlier detection

The statement contains a new definition of exercise PH, in case the mPAP/cardiac output slope between rest and exercise is >3 mmHg/L/min.

“In the new guideline, the diagnostic algorithm has been re-structured. Importantly, a fast-track referral in case of warning signs is included,” Prof. Rosenkranz said.

The general practitioner plays a key role in patients with unexplained dyspnoea and/or suspicion of PH. They can perform a general assessment (see Figure) and according to the suspected cause (lung disease, PH, or cardiac disease) the patient is referred to a cardiologist or a pulmonologist.

Figure: Step 1 of the novel diagnostic algorithm of patients with unexplained exertional dyspnoea and/or suspected PH [2]



In step 2 of the diagnostic algorithm a more comprehensive assessment of the heart and lung is performed. In step 3, in case of a high PH probability, referral to a PH centre for invasive assessment and comprehensive PH work-up is suggested. At any time, a fast-track referral to a PH centre in case of warning signs or when pulmonary arterial hypertension (PAH) and chronic thrombo-embolic pulmonary hypertension (CTEPH) are suspected is recommended.

The new guideline presents far more details in transthoracic echocardiographic parameters in the assessment of PH. The presence of risk factors for PAH moves the patient up 1 category in the PH probability. “We added the ratio of tricuspid annular plane systolic excursion and systolic pulmonary arterial pressure (TAPSE /sPAP) as a sensitive marker to diagnose PH”; Prof. Rosenkranz explained. A TAPSE/sPAP ratio of <0.55 mm/mmHg in the echocardiography is suggestive of pulmonary hypertension. Right heart catheterisation remains gold standard to diagnose PH.

Modified PAH treatment algorithm with a focus on cardiopulmonary comorbidity

Prof. Marion Delcroix (University Hospitals Leuven, Belgium) presented the part of group 1 of the clinical classification, patients with PAH. The haemodynamic definition of PAH implies a PVR >2 WU and pulmonary arterial wedge pressure (PAWP) ≤15 mm Hg.

Once the diagnosis is confirmed, a comprehensive risk assessment in PAH in a 3-strata model is recommended in the new guideline. “The single components of low risk, intermediate risk, and high risk did not change, but the NT-proBNP threshold was adjusted,” Prof. Delcroix said.

The PAH treatment algorithm has been modified, highlighting the importance of cardiopulmonary comorbidities. In case of a confirmed diagnosis at a PH centre initial monotherapy with PDE5 inhibitors or endothelin receptor antagonists is recommended in patients with cardiopulmonary comorbidities, whereas all other patients benefit from a combination of these agents.

At follow up, a novel simplified 4-strata risk-assessment tool is recommended as a basic risk-stratification tool.

PH associated with left heart disease (LHD): the most frequent subgroup.

Prof. Rosenkranz also explained novel recommendations in group 2 of the clinical classification, PH associated with left heart disease (LHD), the most frequent subgroup. There are new recommendations for right heart catheterisation in patients with suspected PH and LHD – if it aids in the management decision – and in patients with severe tricuspid regurgitation (± LHD) prior to surgical or interventional valve repair.

Regarding therapy, there are 2 new class I recommendations:

• In patients with LHD and combined post- and pre-capillary PH with a severe pre-capillary component, an individualised approach to treatment is recommended
• When patients with PH and multiple risk factors for LHD, who have a normal PAWP at rest but an abnormal response to exercise or fluid challenge, are treated with PAH drugs, close monitoring is recommended

Prof. Delcroix then presented the part of PH associated with lung disease, group 3 of the clinical classification and a common cause of PH. For the first time, there is a recommendation for PH medical therapy in group 3, based on a single positive study: inhaled treprostinil may be considered in PH associated with interstitial lung disease (ILD).

PDE5 inhibitors may be considered in patients with severe PH associated with ILD (individual decision-making in PH centres), but is not recommended in patients with ILD and non-severe PH.

Multi-modality approach warranted in chronic thromboembolic PH

For chronic thromboembolic PH (CTEPH; group 5) there is an overlap in surgical/invasive procedures and medical therapy.

In a new diagnostic algorithm for symptomatic CTEPH patients, perfusion imaging and echocardiography is recommended. In case there are signs of PH, referral to a CTEPH/PH centre is recommended.

“In a patient with confirmed CTEPH, lifelong therapeutic anticoagulation is recommended,” Prof. Delcroix explained.

In case patients are operable, pulmonary endarterectomy is the treatment of choice. If this is not possible, patients will receive medical therapy. The same is true for patients with persistent/recurrent PH after pulmonary endarterectomy. Riociguat is recommended for symptomatic patient with inoperable CTEPH or with persistent/recurrent PH after pulmonary endarterectomy (class I).

Finally, medical therapy should be considered prior to the intervention in patients with CTEPH who are candidates for balloon pulmonary angioplasty. The last part of the guidelines outlines requirements for a PH centre.

1. Presentations in Session: “2022 ESC Guidelines Overview”, ESC Congress 2022, Barcelona, Spain, 26–29 August.
2. Presentations in Session: “2022 ESC-ERS Guidelines for the diagnosis and treatment of pulmonary hypertension”, ESC Congress 2022, Barcelona, Spain, 26–29 August.
3. Humbert M, et al. Eur Heart J. 2022 Aug 26;ehac237. doi: 10.1093/eurheartj/ehac237. Online ahead of print.

 

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Posted on 27 October, 20221 November, 2022