Article
作者: Baban, Anwar ; Targetti, Mattia ; Lanzillo, Chiara ; Novelli, Giuseppe ; Narducci, Maria Lucia ; Graziosi, Maddalena ; Fanisio, Francesca ; Canestrelli, Stefano ; Gargaro, Alessio ; Pieroni, Maurizio ; Lopez, Carles Diez ; Gandjbakhch, Estelle ; Toso, Elisabetta ; Romeo, Fabiana ; Cappelletto, Chiara ; Casella, Michela ; Crescenzi, Cinzia ; Pelargonio, Gemma ; Re, Federica ; Fusco, Armando ; Autore, Camillo ; Drago, Fabrizio ; Olivotto, Iacopo ; Barile, Ludovica ; Sangiuolo, Federica Carla ; Catalano, Costantina ; Stefanini, Matteo ; Calò, Leonardo ; Tini, Giacomo ; Gaita, Fiorenzo ; Biagini, Elena ; Dello Russo, Antonio ; Sinagra, Gianfranco ; Merlo, Marco ; Musumeci, Maria Beatrice ; Toto, Federica ; Stolfo, Davide ; Radesich, Cinzia ; Cicenia, Marianna ; Ciabatti, Michele ; Paiotti, Elena ; Di Marco, Andrea ; Fedele, Elisa ; Laredo, Mikael ; Peretto, Giovanni ; Mango, Ruggiero ; Martino, Annamaria ; Gasperetti, Alessio ; Perotto, Maria
BACKGROUND:Electrocardiographic findings in arrhythmogenic left ventricular cardiomyopathy (ALVC) have been limited to small studies.
OBJECTIVES:The authors aimed to analyze the electrocardiogram (ECG) characteristics of ALVC, to correlate ECG with cardiac magnetic resonance and genetic data, and to evaluate its prognostic value.
METHODS:We reviewed data of 125 consecutive patients with ALVC (81.5% desmoplakin pathogenic/likely pathogenic variants). The composite endpoint of major arrhythmic events (MAEs) included sudden cardiac death, aborted sudden cardiac death, and appropriate implantable cardioverter-defibrillator shock. Predictors of MAE were evaluated with logistic regression.
RESULTS:ALVC showed distinct ECG signs, including left posterior fascicular block (LPFB) (13.6%), pathological Q waves (26.4%), R/S ratio in V1 ≥0.5 (26.4%), and SV1 + RV6 ≤12 mm and RI + RII ≤8 mm (44%). Fifteen (12%) patients had a normal ECG. MAE occurred in 35 patients (28%). In multivariable analysis, LPFB (OR: 4.7; 95% CI: 1.2-18.3), syncope (OR: 84.95; 95% CI: 14-496), transmural late gadolinium enhancement (OR: 9.95; 95% CI: 2.3-36), and right ventricular ejection fraction (OR: 0.92; 95% CI: 0.87-0.97) were the independent predictors of MAE. The model including these 4 variables achieved a remarkable predictive capability (area under the curve: 0.9). In the primary prevention scenario, with Cox regression, LPFB (HR: 3.98; 95% CI: 1.3-12.0), syncope (HR: 19.13; 95% CI: 5.8-63.0), and transmural late gadolinium enhancement (HR: 10.57; 95% CI: 2.9-38.0) were independent predictors of MAE.
CONCLUSIONS:In ALVC, ECG is a valuable diagnostic tool and may have a relevant prognostic role, since LFPB is a strong and independent predictor of MAE.