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Minerva Cardiology and Angiology 2021 Mar 11

DOI: 10.23736/S2724-5683.21.05575-7


language: English

Chronic thromboembolic pulmonary hypertension risk score evaluation and validation (CTEPH Solution): proposal of a study protocol aimed to realize a validated risk score for early diagnosis

Cristiano MIOTTI 1, Andrea M. D'ARMINI 2, Beatrice SCARDOVI 3, Stefano GHIO 4, Gianfranco SINAGRA 5, Walter SERRA 6, Antonella ROMANIELLO 7, Giuseppe GALGANO 8, Loris RONCON 9, Michele D'ALTO 10, Daniela GIANNAZZO 11, Patrizio VITULO 12, Amedeo BONGARZONI 13, Matteo RUZZOLINI 14, Carlo ALBERA 15, Gavino CASU 16, Martina PERAZZOLO MARRA 17, Sante D. PIERDOMENICO 18, Federico LUONGO 1, Giovanna MANZI 1, Silvia PAPA 1, Gianmarco SCOCCIA 1, Nadia CEDRONE 1, Roberto BADAGLIACCA 1, Carmine D. VIZZA 1

1 Department of Cardiovascular and Respiratory Sciences, Sapienza University of Rome, Rome, Italy; 2 Dipartimento di Scienze Clinico-Chirurgiche, Diagnostiche e Pediatriche, Sezione di Cardiochirurgia, Policlinico San Matteo Pavia, Pavia, Italy; 3 U.O.C Cardiologia, Ospedale Santo Spirito in Saxia, Roma, Italy; 4 Fondazione IRCCS Policlinico S. Matteo, Pavia, Italy; 5 Dipartimento Cardiovascolare Azienda Ospedaliero-Universitaria Ospedali Riuniti, Trieste, Italy; 6 UO Cardiologia, AOU di Parma, Ospedale Maggiore di Parma, Parma, Italy; 7 UOC Cardiologia, AO Sant’Andrea, Roma, Italy; 8 UOC Cardiologia, UTIC, Ospedale Generale Regionale Francesco Miulli, Acquaviva delle Fonti, Bari, Italy; 9 Divisione di Cardiologia, ULSS 18 Rovigo, Ospedale Santa Maria della Misericordia, Rovigo, Italy; 10 Department of Cardiology, Monaldi Hospital, University L. Vanvitelli, Naples, Italy; 11 AOU Policlinico Vittorio Emanuele, Divisione di Cardiologia, Ospedale Ferrarotto, Catania, Italy; 12 Dipartimento di Pneumologia, Istituto Mediterraneo Trapianti e Terapie Alta Specializzazione ISMETT, Palermo, Italy; 13 Dipartimento di Cardiologia, Ospedale San Carlo Borromeo, Milano, Italy; 14 Unità Operativa Complessa di Cardiologia e UTIC, Ospedale San Giovanni Calibita Fatebenefratelli, Roma, Italy; 15 SC Pneumologia U, Ospedale Molinette, Torino, Italy; 16 UOC Cardiologia, Ospedale San Francesco, Nuoro, Italy; 17 Dipartimento di Scienze Cardiologiche, Toraciche e Vascolari, Azienda Ospedaliera, Padova, Italy; 18 Unità di Malattie dell’apparato Cardiovascolare, Dipartimento di Scienze Mediche, Orali e Biotecnologiche, Università degli Studi G. d’Annunzio, Chieti-Pescara, Italy


BACKGROUND: Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is the most serious long-term complication of acute pulmonary embolism (PE) though it is the only potentially reversible form of Pulmonary Hypertension (PH). Its incidence is mainly limited to the first 2 years following the embolic event, however it is often underdiagnosed or misdiagnosed.
METHODS: This is a multicenter observational cross-sectional and prospective study. Patients with a prior diagnosis of PE will be enrolled and undergo baseline evaluation for prevalent PH detection through a clinical examination and an echocardiogram as first screening exam. All cases of intermediate-high echocardiographic probability of PH will be confirmed by right heart catheterization and then identified as CTEPH through appropriate imaging and functional examinations in order to exclude other causes of PH. A CTEPH Risk Score will be created using retrospective data from this prevalent cohort of patients and will be then validated on an incident cohort of patients with acute PE.
RESULTS: 1000 retrospective and 218 prospective patients are expected to be enrolled and the study is expected to be completed by the end of 2021. Up to now 841 patients (620 retrospective and 221 prospective) have been enrolled.
CONCLUSIONS: This study is the first large prospective study for the prediction of CTEPH development in patients with PE. It aims to create a comprehensive scoring tool that includes echocardiographic data which may allow early detection of CTEPH and the application of targeted follow up screening programs in patients with PE.

KEY WORDS: Chronic thromboembolic pulmonary hypertension; Risk score; Echocardiography; Incidence

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