American Lung Association Pulmonary Hypertension Roundtable: Executive Summary

Abstract

The American Lung Association and Pulmonary Hypertension Association convened a scientific roundtable of pulmonary hypertension experts to discuss the latest recommendations from the European Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension (PH) and from the 7th World Symposium on Pulmonary Hypertension (WSPH). The aim of the roundtable was to discuss changes that were made compared to earlier recommendations and guidelines set out by the European Society of Cardiology and the European Respiratory Society in 2015, the 6th World Symposium on Pulmonary Hypertension in 2018, and the CHEST Guideline on Therapy for Pulmonary Arterial Hypertension in 2019. The overall objectives were to: 1) Create an educational resource for providers that summarizes currently available PAH guidelines, 2) Provide an expert critique of current guidelines, outlining strengths and weaknesses and resolving differences where guidelines do not agree. 3) Provide guidance for the incorporation of the recently approved drug, sotatercept into current guidelines. An executive summary was drafted following the roundtable meeting on April 8, 2024, and revised by the panel in September 2024, following publication of the proceedings from the 7th WSPH held in Barcelona June 29-July 1, 2024. The Executive Summary reviews changes to the hemodynamic criteria for defining pre- and post-capillary PH, exercise-induced PH, and PH associated with lung disease. Recommendations are given for proper diagnosis and clinical classifications of the various forms of PH. The role of screening for PH in high-risk populations and the use of risk scores for disease stratification are discussed. Finally, treatment algorithms for managing pulmonary arterial hypertension, PH associated with lung disease, and chronic thromboembolic pulmonary hypertension are presented.

Keywords: pharmacologic therapy; pulmonary arterial hypertension; treatment guidelines.

Figure 1

Clinical signs in patients with pulmonary hypertension. Figure source: Eur Heart J, Volume 43, Issue 38, 7 October 2022, Pages 3618–3731, https://doi.org/10.1093/eurheartj/ehac237 © the European Society of Cardiology and the European Respiratory Society 2022.

Figure 2

Transthoracic echocardiographic parameters in the assessment of pulmonary hypertension. ^aRefers to collapse on inspiration. Figure source: Eur Heart J, Volume 43, Issue 38, 7 October 2022, Pages 3618–3731, https://doi.org/10.1093/eurheartj/ehac237 © the European Society of Cardiology and the European Respiratory Society 2022.

Figure 3

Echocardiographic probability of PH and recommendations for further assessment. ^aOr unmeasurable. The TRV threshold of 2.8 m/s was not changed according to the updated hemodynamic definition of PH. ^bSigns from at least 2 categories must be present to alter the level of echocardiographic probability of PH. ^cFurther testing may be necessary (eg, imaging, CPET). ^dRHC should be performed if useful information/a therapeutic consequence is anticipated (eg, suspected PAH or CTEPH) and may not be indicated in patients without risk factors or associated conditions for PAH or CTEPH (eg, when mild PH and predominant LHD or lung disease are present). Figure source: Eur Heart J, Volume 43, Issue 38, 7 October 2022, Pages 3618–3731, https://doi.org/10.1093/eurheartj/ehac237 © the European Society of Cardiology and the European Respiratory Society 2022.

Figure 4

Diagnostic algorithm of patients with unexplained dyspnea and/or suspected pulmonary hypertension. ^aWarning signs include rapid progression of symptoms, severely reduced exercise capacity, pre‐syncope or syncope on mild exertion, and signs of right heart failure. ^bLung and heart assessment by specialist per local practice. ^cAs indicated; CT pulmonary angiography recommended if PH suspected. ^dIncludes connective tissue disease (especially systemic sclerosis), portal hypertension, HIV infection, and family history of PAH. ^eHistory of PE, permanent intravascular devices, inflammatory bowel diseases, essential thrombocythemia, splenectomy, high‐dose thyroid hormone replacement, and malignancy. Figure source: Eur Heart J, Volume 43, Issue 38, 7 October 2022, Pages 3618–3731, https://doi.org/10.1093/eurheartj/ehac237 © the European Society of Cardiology and the European Respiratory Society 2022.

Figure 5

Suggested diagnostic algorithm of patients with unexplained dyspnea: Expert Panel′s proposal.

Figure 6

Suggested management algorithm for patients with PH in parenchymal disease: Expert Panel′s proposal. ^aDry powder or nebulized; dry powder anecdotally associated with more cough.

Figure 7

Management strategy in CTEPH. ^aSelected symptomatic patients with CTEPD without PH can also be treated by PEA and BPA. ^bMDT meeting can be virtual. ^cTreatment assessment may differ, depending on the level of expertise in PEA and BPA. ^dFor inoperable patients with PVR > 4 WU, medical therapy should be considered before BPA; there are limited data on BPA as first‐line therapy. © the European Society of Cardiology and the European Respiratory Society 2022. This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/open_access/funder_policies/chorus/standard_publication_model).

Figure 8

Suggested management algorithm of patients with group 4 PH: Expert Panel′s proposal.

Figure 9

Suggested management algorithm of patients with confirmed PAH: Expert Panel′s proposal.