Pulmonary hypertension (PH) is a serious condition characterized by elevated pressure in the pulmonary arteries. Accurate and timely diagnosis is crucial for effective management and improved patient outcomes. The 2022 European Society of Cardiology (ESC) and European Respiratory Society (ERS) guidelines have updated the diagnostic criteria and algorithms for pulmonary hypertension, aiming for earlier detection and more precise classification. This article highlights the key changes in Pulmonary Hypertension Diagnosis Criteria as outlined in these updated guidelines.
One of the fundamental shifts is the revised definition of pulmonary hypertension itself. According to the 2022 guidelines, pulmonary hypertension is now defined as a mean pulmonary arterial pressure (mPAP) greater than 20 mm Hg at rest, reduced from the previous threshold of 25 mm Hg. This change aims to improve the sensitivity of diagnostic criteria and identify patients with PH earlier in the disease course. Furthermore, the definition of pulmonary arterial hypertension (PAH) continues to require a pulmonary vascular resistance (PVR) greater than 2 Wood Units and a pulmonary arterial wedge pressure (PAWP) of ≤15 mm Hg, emphasizing the pre-capillary nature of PAH.
The diagnostic algorithm for pulmonary hypertension has been streamlined into a three-step approach. This simplified algorithm is designed to facilitate earlier suspicion and referral to specialized centers. The first step involves suspicion of PH by first-line physicians, based on clinical symptoms and risk factors. The second step is detection using echocardiography, a non-invasive and readily available tool to assess pulmonary artery pressure and right ventricular function. Echocardiography plays a vital role in identifying patients who require further investigation. Finally, confirmation of PH and hemodynamic assessment are achieved through right heart catheterization (RHC), performed in specialized PH centers. RHC remains the gold standard for confirming the diagnosis of PH and determining its severity and type.
The guidelines also emphasize improved recognition of chronic thromboembolic pulmonary hypertension (CTEPH). The updated criteria highlight the importance of considering CTEPH in patients, particularly after an acute pulmonary embolism (PE). Clinicians are encouraged to be vigilant for CT and echocardiographic signs suggestive of CTEPH during and after a PE event. Systematic follow-up of patients with acute PE is recommended to help reduce the underdiagnosis of CTEPH, a potentially treatable form of pulmonary hypertension. Early identification of CTEPH is critical as specific treatments, such as pulmonary endarterectomy or balloon pulmonary angioplasty, can significantly improve outcomes.
In the context of group 3 pulmonary hypertension, associated with lung diseases, the guidelines offer new insights into diagnosis and management. While the primary approach remains optimizing the underlying lung disease, including conditions like interstitial lung disease (ILD), the guidelines acknowledge the role of pulmonary hypertension in these patients. For individuals with severe PH associated with ILD, particularly in specialized PH centers, phosphodiesterase 5 (PDE5) inhibitors may be considered. This recommendation, based on emerging evidence, underscores the evolving understanding of PH in the context of lung disease and the potential for targeted therapies in select patients.
Finally, the concept of chronic thromboembolic pulmonary disease (CTEPD), both with and without pulmonary hypertension, has been introduced. CTEPD is defined by symptomatic patients exhibiting mismatched perfusion defects on V/Q scans and signs of chronic, organized thrombi on CT pulmonary angiography or digital subtraction angiography, even in the absence of elevated pulmonary artery pressure. This new classification aims to facilitate further research into the natural history and management of thromboembolic disease and its relationship to pulmonary hypertension.
In conclusion, the 2022 ESC/ERS guidelines provide updated and refined pulmonary hypertension diagnosis criteria aimed at earlier and more accurate identification of this complex condition. The revised definition of PH, the simplified diagnostic algorithm, and the enhanced focus on CTEPH and PH associated with lung disease represent significant advancements in the field, ultimately striving to improve the care and outcomes for patients with pulmonary hypertension.
