Clinical practice guidelines on the diagnosis and treatment of pulmonary hypertension from the European Society of Cardiology (ESC) and the European Respiratory Society (ERS) were published in August 2022 in The European Respiratory Journal.
The updated definition of pulmonary hypertension is a mean pulmonary arterial pressure of >20 mm Hg at rest. This definition includes a pulmonary vascular resistance of >2 Wood units and a pulmonary arterial wedge pressure of ≤15 mm Hg.
For patients with suspected pulmonary hypertension, echocardiography is recommended as the initial noninvasive diagnostic study. For those who have unexplained pulmonary hypertension, the preferred imaging study is ventilation/perfusion or perfusion lung scanning to detect chronic thromboembolic pulmonary hypertension (CTEPH).
Right heart catheterization is recommended to confirm the diagnosis of pulmonary hypertension.
Annual screening for pulmonary arterial hypertension (PAH) is recommended for patients with systemic sclerosis. Annual screening, as well as counseling about the risk of PAH, is also recommended for first-degree relatives of patients with heritable pulmonary arterial hypertension and for persons in whom testing revealed mutations that can cause PAH.
For patients who have new-onset or persistent dyspnea or exercise limitations after pulmonary embolism (PE), additional workup to detect CTEPH and/or chronic thromboembolic pulmonary disease is advised. For those who remain symptomatic and have mismatched perfusion lung defects after 3 months of anticoagulation therapy for acute PE, referral for further evaluation at a pulmonary hypertension/CTEPH center should be considered.
Clinical evaluation, exercise tests, biochemical markers, echocardiography, and hemodynamic testing are recommended to assess disease severity in patients with PAH. The results are used to categorize patient as being at low, intermediate, or high risk.
The management of PAH focuses on risk assessment, cardiopulmonary comorbidities, and treatment goals. Initial combination therapy and treatment escalation at follow-up are recommended.
For patients with PAH who show a response to acute vasoreactivity tests, high-dose calcium channel blocker therapy is recommended. After 3-4 months of closely monitored therapy, patients should undergo a complete reevaluation that includes right heart catheterization.
For all patients with CTEPH, lifelong anticoagulation therapy is recommended. For those who also have antiphospholipid syndrome, anticoagulation with a vitamin K antagonist is preferred.
Patients with pulmonary hypertension who have an inadequate response to oral combination therapy should be considered for lung transplantation.
For more information, please go to Pulmonary Arterial Hypertension.
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Cite this: Pulmonary Hypertension Clinical Practice Guidelines (ESC/ERS, 2022) - Medscape - Sep 06, 2022.