Author + information
- Received January 28, 2020
- Revision received April 3, 2020
- Accepted April 6, 2020
- Published online June 1, 2020.
- Giovanni Donato Aquaro, MDa,∗ (, )@gdaqua,
- Antonio De Luca, MDb,
- Chiara Cappelletto, MDb,
- Francesca Raimondi, MDc,
- Francesco Bianco, MDd,
- Nicoletta Botto, BSca,
- Pierluigi Lesizza, MDb,
- Crysanthos Grigoratos, MD, PhDa,
- Monia Minati, MDe,
- Matteo Dell’Omodarme, PhDf,
- Alessandro Pingitore, MD, PhDg,
- Davide Stolfo, MDb,
- Matteo Dal Ferro, MDb,
- Marco Merlo, MDb,
- Gianluca Di Bella, MD, PhDh and
- Gianfranco Sinagra, MDb
- aFondazione Toscana G. Monasterio, Pisa, Italy
- bCardio-thoraco-vascular Department, University of Trieste, Trieste, Italy
- cPediatric Cardiology, Hôpital Necker-Enfants Malades, Paris, France
- dInstitute of Cardiology, “G. d'Annunzio” University, Chieti, Italy
- eCardiology Department, Policlinico Casilino, Rome, Italy
- fDipartimento di Fisica, “Enrico Fermi,” University of Pisa, Pisa, Italy
- gInstitute of Clinical Physiology, National Council of Research, Pisa, Italy
- hUniversity of Messina, Messina, Italy
- ↵∗Address for correspondence:
Dr. Giovanni Donato Aquaro, Fondazione Toscana G. Monasterio, Via Giuseppe Moruzzi, 1, 56124 Pisa, Italy.
Background Cardiac magnetic resonance (CMR) is widely used to assess tissue and functional abnormalities in arrhythmogenic right ventricular cardiomyopathy (ARVC). Recently, a ARVC risk score was proposed to predict the 5-year risk of malignant ventricular arrhythmias in patients with ARVC. However, CMR features such as fibrosis, fat infiltration, and left ventricular (LV) involvement were not considered.
Objectives The authors sought to evaluate the prognostic role of CMR phenotype in patients with definite ARVC and to evaluate the effectiveness of the novel 5-year ARVC risk score to predict cardiac events in different CMR presentations.
Methods A total of 140 patients with definite ARVC were enrolled (mean age 42 ± 17 years, 97 males) in this multicenter prospective registry. As per study design, CMR was performed in all the patients at enrollment. The novel 5-year ARVC risk score was retrospectively calculated using the patient’s characteristics at the time of enrollment. During a median follow-up of 5 years (2 to 8 years), the combined endpoint of sudden cardiac death, appropriate implantable cardioverter-defibrillator intervention, and aborted cardiac arrest was considered.
Results CMR was completely negative in 14 patients (10%), isolated right ventricular (RV) involvement was found in 58 (41%), biventricular in 52 (37%), and LV dominant in 16 (12%). During the follow-up, 48 patients (34%) had major events, but none occurred in patients with negative CMR. At Kaplan-Meier analysis, patients with LV involvement (LV dominant and biventricular) had a worse prognosis than those with lone RV (p < 0.0001). At multivariate analysis, the LV involvement, a LV-dominant phenotype, and the 5-year ARVC risk score were independent predictors of major events. The estimated 5-year risk was able to predict the observed risk in patients with lone RV but underestimated the risk in those with LV involvement.
Conclusions Different CMR presentations of ARVC are associated with different prognoses. The 5-year ARVC risk score is valid for the estimation of risk in patients with lone-RV presentation but underestimated the risk when LV is involved.
- arrhythmogenic right ventricular cardiomyopathy
- ARVC risk score
- cardiac magnetic resonance
- left dominant
The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the JACC author instructions page.
- Received January 28, 2020.
- Revision received April 3, 2020.
- Accepted April 6, 2020.
- 2020 American College of Cardiology Foundation
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