Pulmonary Vascular & Cardiovascular Network


Pulmonary Vascular Disease Section

Key messages from the 2022 ESC/ERS Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension

1. Per coverage by the American College of Cardiology, “Pulmonary hypertension (PH) is now defined by a mean pulmonary arterial pressure >20 mm Hg at rest. The definition of pulmonary arterial hypertension (PAH) also implies a pulmonary vascular resistance (PVR) >2 Wood units and pulmonary arterial wedge pressure ≤15 mm Hg.”1 These cut-off values do not translate into new therapeutic recommendations.

Dr. Mary Jo S. Farmer, directory of pulmonary hypertension services at Baystate Health in Springfield, Mass.

Dr. Mary Jo S. Farmer

2. The diagnostic algorithm for PH now follows a simplified three-step approach, involving first suspicion by first-line physicians, then detection by echocardiography, and confirmation with right heart catheterization, preferably in a PH center.

3. Pulmonary vasoreactivity testing is only recommended in patients with idiopathic PAH, heritable PAH, or drug/toxin associated PAH to identify potential candidates for calcium channel blocker therapy. Inhaled nitric oxide or inhaled iloprost are the recommended agents.

Dr. Vijay Balasubramanian

Dr. Vijay Balasubramanian

4. The role of cardiac MRI in prognostication of patients with PAH has been confirmed such that measures of right ventricular volume, right ventricular ejection fraction, and stroke volume are included as risk assessment variables.

5. The primary limitation of the 2015 ESC/ERS three-strata risk-assessment tool is that 60% to 70% of the patients are classified as intermediate risk (IR). A four-strata risk stratification, dividing the IR group into IR “low” and IR “high” risk, is proposed at follow up.

6. No general recommendation is made for or against the use of anticoagulation in PAH given the absence of robust data and increased risk of bleeding.

7. In patients with PH-ILD, inhaled treprostinil may be considered based on findings from the INCREASE trial, but further long-term outcome data are needed.

8. Improved recognition of the signs of chronic thromboembolic pulmonary hypertension (CTEPH) on CT and echocardiographic imagery at the time of an acute pulmonary embolism (PE) event, along with systematic follow-up of patients with acute PE, is recommended to help mitigate the underdiagnosis of CTEPH.

9. The treatment algorithm for PAH has been simplified, and now includes a focus on cardiopulmonary comorbidities, risk assessment, and treatment goals. Current standards include initial combination therapy and treatment escalation at follow-up, when appropriate.

10. Per coverage by the American College of Cardiology, “The recommendations on sex-related issues in patients with PAH, including pregnancy, have been updated, with information and shared decision making as key points.” Calcium channel blockers, inhaled/IV/subcutaneous prostacyclin analogues, and phosphodiesterase 5 inhibitors all and are considered safe during pregnancy, despite limited data on this use.

11. Per the guideline, “Patients with PAH should be treated with the best standard of pharmacological treatment and be in stable clinical condition before embarking on a supervised rehabilitation program.”2 Additional studies have shown that exercise training has a beneficial impact on 6-minute walk distance, quality of life, World Health Organization function classification, and peak VO2.

12. Immunization of PAH patients against SARS-CoV-2, influenza, and Streptococcus pneumoniae is recommended.

This edition of clinical practice guidelines focuses on early diagnosis of PAH and optimal treatments.

*Mary Jo S. Farmer, MD, PhD

Vijay Balasubramanian, MD, MRCP (UK)

* The authors for this article were listed in the incorrect order in the print edition of CHEST Physician. The order has been corrected here.


1. Mukherjee, D. 2022 ESC/ERS guidelines for pulmonary hypertension: key points. American College of Cardiology. August 30, 2022.

2. Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022;43(38):3618-3731.

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