Summary of 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

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

cover the entire spectrum of PH (Pulmonary Hypertension) with a focus on the diagnosis and treatment of Pulmonary Arterial Hypertension (PAH) and Chronic-Thromboembolic Pulmonary Hypertension (CTEPH).

This clinical practice guideline includes updates and revisions to many recommendations. One of the most significant changes is the hemodynamic redefinition of what constitutes pulmonary arterial hypertension (PAH). Below, we provide a selection of the most relevant updates.

The Guidelines update the PH threshold to a resting mPAP (Mean Pulmonary Arterial Pressure) of > 20 mmHg. The definition of PAH implies a Pulmonary Vascular Resistance (PVR) of > 2 WU (Wood Units) and a pulmonary wedge pressure (PEP) of ≤15 mmHg.

These values better reflect normal ranges, but they do not yet translate into new therapeutic recommendations, as the efficacy of PAH treatment in patients with Pulmonary Vascular Disease (PVD) and an mPAP of 21-24 mmHg and/or Pulmonary Vascular Resistance (PVR) of 2-3 Wood Units (WU) is still unknown.

The main diagnostic algorithm for pulmonary hypertension has been simplified following a three-step approach. It is worth noting that it does not address the screening of specific patient groups at risk of PH.

1. Starts when the primary care physician suspects this disease.

2. Continues with detection through echocardiography.

3. Culminates with confirmation through Right Heart Catheterization (RHC) in specialized pulmonary hypertension centers.

The 2022 ESC/ERS Guidelines for Pulmonary Hypertension mention that warning signs associated with worse outcomes have been identified, justifying immediate referral and management in specialized centers.

The Guidelines include four new recommendations:

• It is recommended to assign an echocardiographic probability of pulmonary hypertension, based on an abnormal Tricuspid Regurgitation Velocity (TRV) and the presence of other echocardiographic signs that suggest it.
  
• It is recommended to maintain the current threshold of TRV (> 2.8 m/s) to estimate the echocardiographic probability of PH, according to the updated hemodynamic definition (mPAP > 20mmHg at rest).
• According to the probability of PH indicated by echocardiography, it is suggested to consider additional tests depending on the clinical context (symptoms and risk factors or conditions associated with PAH/CTEPH - Chronic Thromboembolic Pulmonary Hypertension).
• In symptomatic patients with intermediate echocardiographic probability of PH, a Cardiopulmonary Exercise Test (CPET) can be considered to more accurately determine the likelihood of PH.

The 2022 ESC/ERS Guidelines for PAH suggest considering iron repletion for PAH patients with iron deficiency when anemia is absent.

Previous Guidelines recommend that women with PAH avoid pregnancy. And the 2022 edition adds new suggestions for them:

• Women of childbearing age with PAH are advised to receive counseling on contraception, with a clear understanding that the failure of the chosen method can have significant consequences for their condition.
  
• Women who are considering motherhood or become pregnant should receive timely counseling at an PAH center to help them make shared decisions. It is also recommended that both patients and their family members receive psychological support.
• Patients who choose to terminate their pregnancy should have the procedure performed at an PAH center, where psychological support is provided to both the patients and their families.
• Women with PAH who wish to become mothers should receive counseling on adoption and surrogacy, including genetic counseling before conception.
• The use of endothelin receptor antagonists and riociguat during pregnancy is not recommended, as preclinical models have reported potential teratogenic effects.

• For patients with PAH associated with HIV infection, it is recommended to follow antiretroviral treatment according to current Guideline recommendations.
• For these patients, initial monotherapy should be considered, followed by sequential combination therapy if necessary, taking into account comorbidities and drug interactions.

It is recommended to assess the risk for patients with persistent pulmonary arterial hypertension (PAH) after defect closure and for those with Eisenmenger syndrome. Furthermore, it is suggested that patients with this syndrome should avoid pregnancy.

• The 2022 ESC/ERS Guidelines for Pulmonary Hypertension recommend that the diagnosis in children includes right heart catheterization and acute vasoreactivity testing.
• Right heart catheterization should be performed to confirm the diagnosis of PH, preferably before initiating any treatment of PAH.
  
  
• For patients with IPAH/HPAH, it is advisable to perform an acute vasoreactivity test to identify those who would benefit from calcium channel blocker therapy.
  
  
• A positive acute vasoreactivity response in children should be defined in the same way as in adults: a reduction in mPAP ≥ 10 mmHg to achieve an absolute mPAP value ≤ 40 mmHg with unchanged or increased cardiac output (CO).
  
  
• Achieving and maintaining a low-risk profile is considered an appropriate treatment response.
  
  
• Screening for pulmonary hypertension in children with bronchopulmonary dysplasia is recommended.

REFERENCE

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