Eloísa Feliu1, Rafal Moscicki2, Luna Carrillo3, Amaya García-Fernández4, Juan Gabriel Martínez Martínez4, Juan Miguel Ruiz-Nodar5. 1. Unidad de Resonancia Magnética, Inscanner, Hospital General Universitario de Alicante, Alicante, Spain. 2. Servicio de Cardiología, Hospital General de Elda, Elda, Alicante, Spain. 3. Servicio de Cardiología, Hospital de Torrevieja, Torrevieja, Alicante, Spain. 4. Servicio de Cardiología, Hospital General Universitario de Alicante, Alicante, Spain. 5. Servicio de Cardiología, Hospital General Universitario de Alicante, Alicante, Spain. Electronic address: ruiz_jmi@gva.es.
Abstract
INTRODUCTION AND OBJECTIVES: Left dominant arrhythmogenic cardiomyopathy (LDAC) has recently been recognized as falling on the spectrum of arrhythmogenic cardiomyopathy. It is characterized by fibroadipose replacement of the left ventricle. The aim of this study was to describe the most frequent forms of clinical presentation of LDAC, imaging findings, and events at follow-up, highlighting the importance of cardiac magnetic resonance (CMR). METHODS: Prospective registry of patients with findings compatible with LDAC. CMR image analysis and clinical follow-up was performed. The primary endpoint was the appearance of major adverse cardiovascular events (MACE) during follow-up, defined as sudden cardiac death, sustained ventricular arrhythmias, and heart transplant. RESULTS: We included 74 consecutive patients (mean age, 48.6 years; 50 men [67.6%]). The most frequent CMR indications were chest pain with normal coronary angiography, ventricular arrhythmias, and suspicion of cardiomyopathies. The main CMR findings were midwall and/or subepicardial pattern of late gadolinium enhancement (91.9%), fatty epicardial infiltration (83.8%), and left ventricle segmental contractility abnormalities (47.9%). At a mean follow-up of 3.74 years, 24 patients (32.4%) had a MACE (sudden cardiac death 8.1%, sustained ventricular arrhythmias 21.6%, and heart transplant 4.1%). Independent predictors for the appearance for MACE were a CMR study showing severe late gadolinium enhancement, male sex, and practicing sports. CONCLUSIONS: CMR is a key tool for diagnosing LDAC. Characteristic findings are subepicardial fatty infiltration and midwall-subepicardial late gadolinium enhancement. The prognosis of this population is poor with a high incidence of sudden cardiac death and ventricular arrhythmias.
INTRODUCTION AND OBJECTIVES: Left dominant arrhythmogenic cardiomyopathy (LDAC) has recently been recognized as falling on the spectrum of arrhythmogenic cardiomyopathy. It is characterized by fibroadipose replacement of the left ventricle. The aim of this study was to describe the most frequent forms of clinical presentation of LDAC, imaging findings, and events at follow-up, highlighting the importance of cardiac magnetic resonance (CMR). METHODS: Prospective registry of patients with findings compatible with LDAC. CMR image analysis and clinical follow-up was performed. The primary endpoint was the appearance of major adverse cardiovascular events (MACE) during follow-up, defined as sudden cardiac death, sustained ventricular arrhythmias, and heart transplant. RESULTS: We included 74 consecutive patients (mean age, 48.6 years; 50 men [67.6%]). The most frequent CMR indications were chest pain with normal coronary angiography, ventricular arrhythmias, and suspicion of cardiomyopathies. The main CMR findings were midwall and/or subepicardial pattern of late gadolinium enhancement (91.9%), fatty epicardial infiltration (83.8%), and left ventricle segmental contractility abnormalities (47.9%). At a mean follow-up of 3.74 years, 24 patients (32.4%) had a MACE (sudden cardiac death 8.1%, sustained ventricular arrhythmias 21.6%, and heart transplant 4.1%). Independent predictors for the appearance for MACE were a CMR study showing severe late gadolinium enhancement, male sex, and practicing sports. CONCLUSIONS: CMR is a key tool for diagnosing LDAC. Characteristic findings are subepicardial fatty infiltration and midwall-subepicardial late gadolinium enhancement. The prognosis of this population is poor with a high incidence of sudden cardiac death and ventricular arrhythmias.
Authors: Fernando de la Guía-Galipienso; Eloísa Feliu-Rey; Rafael Raso-Raso; Aurelio Quesada-Dorador; Christoph Meyer-Josten; Carl J Lavie; Daniel P Morin; Fabian Sanchis-Gomar Journal: HeartRhythm Case Rep Date: 2021-04-09
Authors: Anna Palmisano; Fatemeh Darvizeh; Giulia Cundari; Giuseppe Rovere; Giovanni Ferrandino; Valeria Nicoletti; Francesco Cilia; Silvia De Vizio; Roberto Palumbo; Antonio Esposito; Marco Francone Journal: Radiol Med Date: 2021-08-22 Impact factor: 3.469