Importance of cardiac magnetic resonance findings in the diagnosis of left dominant arrythmogenic cardiomyopathy

被引:1
|
作者
Feliu, Eloisa [1 ]
Moscicki, Rafal [2 ]
Carrillo, Luna [3 ]
Garcia-Fernandez, Amaya [4 ]
Martinez Martinez, Juan Gabriel [4 ]
Miguel Ruiz-Nodar, Juan [4 ]
机构
[1] Hosp Gen Univ Alicante, Inscanner, Unidad Resonancia Magnet, Alicante, Spain
[2] Hosp Gen Elda, Serv Cardiol, Alicante, Spain
[3] Hosp Torrevieja, Serv Cardiol, Alicante, Spain
[4] Hosp Gen Univ Alicante, Serv Cardiol, Maestro Alonso S-N, Alicante 03010, Spain
来源
REVISTA ESPANOLA DE CARDIOLOGIA | 2020年 / 73卷 / 11期
关键词
Left dominant arrhythmogenic cardiomyopathy; Cardiomyopathy; Cardiac magnetic resonance; Sudden cardiac death; Late gadolinium enhancement; Fibrosis; Imaging; Prognosis; VENTRICULAR CARDIOMYOPATHY; ARRHYTHMOGENIC CARDIOMYOPATHY; DYSPLASIA;
D O I
10.1016/j.recesp.2019.12.007
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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. (C) 2020 Sociedad Espanola de Cardiologia. Published by Elsevier Espana, S.L.U. All rights reserved.
引用
收藏
页码:885 / 892
页数:8
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