ERS 2022: Update on the Diagnosis and Treatment of Pulmonary Hypertension
This session highlighted the changes from the previous ERS/ESC guidelines on pulmonary hypertension, discussed the new guidelines in a pro/con debate, the clinical consequences of the new recommendations and how they will change diagnosis and treatment, specifically for Group 1 and Group 3 PH.
Estimates suggest that pulmonary hypertension (PH) affects 1% of the global population. After careful evaluation, the new definitions of PH have been endorsed and expanded in the recent 2022 ESC/ERS guidelines for diagnosing and treating pulmonary hypertension, including a revised cut-off level for pulmonary vascular resistance (PVR) and exercise PH definition. PH is defined by a mean pulmonary arterial pressure (mPAP) >20?mmHg at rest. The upper limit of normal PVR and the lowest prognostically significant threshold of PVR is ∼2 Wood units (WU). Despite the change in hemodynamic definition, the threshold for tricuspid regurgitation velocity (TRV) to estimate systolic PAP is maintained at >2.8 m/s. Ho JE et al.'s study showed that PAP/CO slope >3 mmHg/L/min is associated with a higher risk of cardiovascular events and a higher mortality risk.
The classification of PH has been updated, which includes repositioning of vasoreactive patients with idiopathic pulmonary arterial hypertension (IPAH) and a revision of group 5 PH, which includes repositioning of PH in lymphangioleiomyomatosis in group 3. Sleep-disordered breathing has been removed from the classification. The diagnostic strategy aims at early detection and fast-track referral for high-risk patients. Echocardiography is recommended as the first-line, non-invasive diagnostic investigation in suspected PH. Given the heterogeneous nature of PH and the unique geometry of the RV, no single echocardiographic parameter can accurately predict the status of PH and its underlying aetiology. As a result, a comprehensive echocardiographic evaluation for suspected PH involves estimating the systolic pulmonary arterial pressure (sPAP) and detecting additional signs suggestive of PH to assign an echocardiographic level of probability of PH.
Right heart catheterization (RHC) is recommended to confirm the diagnosis of PH and to support treatment decisions. A multi-step, pragmatic approach to diagnosis should be considered in patients with unexplained dyspnea or symptoms/signs suggestive of PH. The diagnostic algorithm does not address screening for specific populations at risk of PH.
Drugs for treating PAH should be prescribed in pulmonary arterial hypertension associated with connective tissue disease (PAH-CTD) according to the same treatment algorithm as in IPAH. In patients with IPAH/ heritable PAH (HPAH)/drug-associated PAH (DPAH) who present at high risk of death, initial combination therapy with a phosphodiesterase 5 inhibitor (PDE5i), an endothelin receptor antagonist (ERA), and i.v./s.c prostacyclin analogues should be considered. The guideline failed to give recommendations for or against the use of PDE5is in patients with heart failure with preserved ejection fraction (HFpEF) and combined post and pre-capillary PH since the literature was not convincing. PDE5is is not recommended in patients with HFpEF and isolated post-capillary PH. An individualized approach to treatment is recommended in patients with left heart disease (LHD) and combined post- and pre-capillary pulmonary hypertension (CpcPH) with a severe pre-capillary component (e.g., PVR >5 WU). Before the intervention, medical therapy should be considered in patients with chronic thrombo-embolic pulmonary hypertension (CTEPH) who are candidates for balloon pulmonary angioplasty (BPA). The historical term CTEPH is still used for patients with PH at rest.
The strengths and weaknesses of the guidelines are as follows:
Strengths of the guidelines-
- The new guidelines are the most detailed and comprehensive to date.
- Illustrations and algorithms have brought clarity to the guidelines.
- Novel mechanisms have allowed answering big questions where data is lacking (PICO).
- Patient involvement has been considered in guideline development.
Weakness of the guidelines-
- The new definition adds complexity to the diagnosis and requires careful implementation and review.
- Group one PAH classification could include the role of comorbidities in line with the diagnostic algorithm.
- Risk stratification could emphasize the urgency in treatment escalation in incident patients at the first follow-up assessment.
European Respiratory Society (ERS) International Congress 2022, 3rd-6th Sept. 2022, Barcelona
