Gaps in evidence in the treatment of prevalent patients with pulmonary arterial hypertension at intermediate risk: An expert consensus

Giovanna Manzi, Department of Clinical, Internal, Anesthesiology and Cardiovascular Sciences, Policlinico Universitario Umberto I, Sapienza University of Rome, 00161 Rome, Italy.
Raymond L. Benza, Department of Cardiology, Mount Sinai Icahn School of Medicine, New York, NY, United States of America.
Paola Argiento, Department of Cardiology, Monaldi Hospital - University "L. Vanvitelli", Naples, Italy.
Gavino Casu, Clinical and Interventional Cardiology, Sassari University Hospital, Sassari, Italy.
Marco Corda, Cardiology Unit, Cardiovascular Department, ARNAS "G.Brotzu", Cagliari, Italy.
Michele Correale, Cardiothoracic Department, Policlinico Riuniti University Hospital, 71100 Foggia, Italy.
Michele D'Alto, Department of Cardiology, Monaldi Hospital - University "L. Vanvitelli", Naples, Italy.
Giuseppe Galgano, Department of Cardiology, F. Miulli Hospital, Acquaviva delle Fonti, Bari, Italy.
Andrea Garascia, De Gasperis Cardio Center, Niguarda Hospital, 20162 Milano, Italy.
Stefano Ghio, Fondazione IRCCS Policlinico S Matteo, Pavia, Italy.
Mardi Gomberg-Maitland, George Washington University School of Medicine and Health Sciences, Washington, DC, USA.
Massimiliano Mulé, Regional Referral Centre for Rare Lung Diseases, AOU Policlinico-San Marco, Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy.
Giuseppe Paciocco, Cardio-vascular and thoracic Department, Pneumology Unit, IRCCS-San Gerardo dei Tintori, University of Milano Bicocca, Monza, Italy.
Silvia Papa, Department of Clinical, Internal, Anesthesiology and Cardiovascular Sciences, Policlinico Universitario Umberto I, Sapienza University of Rome, 00161 Rome, Italy.
Daniele Prati, Section of Internal Medicine, Department of Clinical and Experimental Medicine, University of Verona, Verona, Italy.
Ioana R. Preston, Tufts University School of Medicine, Boston, MA, USA.
Claudia Raineri, Department of Cardiology, Citta` della salute e della Scienza - Ospedale Molinette, Turin, Italy.
Emanuele Romeo, Department of Cardiology, Monaldi Hospital - University "L. Vanvitelli", Naples, Italy.
Laura Scelsi, Fondazione IRCCS Policlinico S Matteo, Pavia, Italy.
Davide Stolfo, Cardiothoracovascular Department, Azienda Sanitaria Universitaria Giuliano Isontina (ASUGI), University Hospital of Trieste, Trieste, Italy; Division of Cardiology, Department of Mediupcocine, Karolinska Institutet, Stockholm, Sweden.
Patrizio Vitulo, Department of Pulmonary Medicine, IRCCS Mediterranean Institute for Transplantation and Advanced Specialized, Therapies Palermo Sicilia, Italy.
R James White, Department of Pulmonary and Critical Care Medicine, University of Rochester, Rochester, NY, United States of America.
Roberto Badagliacca, Department of Clinical, Internal, Anesthesiology and Cardiovascular Sciences, Policlinico Universitario Umberto I, Sapienza University of Rome, 00161 Rome, Italy. Electronic address: roberto.badagliacca@uniroma1.it.
Carmine Dario Vizza, Department of Clinical, Internal, Anesthesiology and Cardiovascular Sciences, Policlinico Universitario Umberto I, Sapienza University of Rome, 00161 Rome, Italy.

Document Type

Journal Article

Publication Date

9-10-2024

Journal

Vascular pharmacology

Volume

157

DOI

10.1016/j.vph.2024.107432

Keywords

Expert consensus; Intermediate risk; PAH therapeutic options; Pulmonary arterial hypertension

Abstract

Despite the innovations introduced in the 2022 European Society of Cardiology/European Respiratory Society Guidelines on Pulmonary Hypertension, risk discrimination and management of pulmonary arterial hypertension (PAH) patients at intermediate risk still represents a grey zone. Additionally, clinical evidence derived from currently available studies is limited. This expert panel survey intends to aid physicians in choosing the best therapeutic strategy for patients at intermediate risk despite ongoing oral therapy. An expert panel of 24 physicians, specialized in cardiology and/or pulmonology with expertise in handling all drugs available for the treatment of PAH participated in the survey. All potential therapeutic options for patients at intermediate risk were explored and analyzed to produce graded consensus statements regarding: the switch from endothelin receptor antagonist (ERA) or phosphodiesterase 5 inhibitor (PDE5i) to another oral drug of the same class; the addition of a drug targeting the prostacyclin pathway administered by different routes; the switch from PDE5i to riociguat.

Department

Medicine

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