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Predictors of Adverse Outcomes in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy

A Meta-Analysis of Observational Studies

Bazoukis, George, MD, MSc*; Letsas, Konstantinos P., MD, PhD*; Thomopoulos, Costas, MD, PhD; Tse, Gary, MD, PhD; Korantzopoulos, Panagiotis, MD, PhD§; Naka, Katerina K., MD, PhD; Kalfakakou, Vassiliki, PhD; Vlachos, Konstantinos, MD*; Bazoukis, Xenophon, MD**; Papadatos, Stamatis S., MD††; Michelongona, Paschalia, MD‡‡; Saplaouras, Athanasios, MD*; Georgopoulos, Stamatis, MD*; Karamichalakis, Nikolaos, MD*; Asvestas, Dimitris, MD*; Liu, Tong, MD, PhD§§; Efremidis, Michael, MD*; Sideris, Antonios, MD*; Tsioufis, Costas, MD, PhD¶¶; Baranchuk, Adrian, MD, PhD‖‖; Stavrakis, Stavros, MD, PhD***

doi: 10.1097/CRD.0000000000000220
Review Articles

Arrhythmogenic cardiomyopathy (AC) is a hereditary disorder characterized by degeneration of cardiac myocytes and their subsequent replacement by fat and fibrous tissue primarily in the right ventricle. Our study aimed to systematically evaluate the impact of significant demographic, clinical, electrocardiographic, and echocardiographic factors in arrhythmic events in AC patients. MEDLINE and Cochrane library databases were manually searched without year or language restriction or any other limits until July 31, 2017. A pooled odds ratio with 95% confidence intervals was calculated for each of the risk factors. Our search retrieved 26 studies (n = 2680 patients, mean age: 37.9 years old, males: 51.9%) which were included in the quantitative synthesis. The most reliable predicting factors/parameters are the following: (1) male gender, (2) presyncope, (3) left ventricular dysfunction, (4) T-wave inversions in inferior leads, (5) proband status, (6) late potentials, (7) syncope, (8) inducibility at electrophysiological study, (9) right ventricular dysfunction, (10) epsilon waves, and (11) premature ventricular contractions greater than 1000/24 h. On the contrary, family history of sudden cardiac death, palpitations, premature ventricular contractions greater than 500/24 h, and T-wave inversions in right precordial leads fail to determine the outcome in this meta-analysis. In conclusion, multiple risk factors have been associated with arrhythmic events in AC patients. However, larger studies are needed to discriminate those patients who will benefit from implantable cardioverter defibrillators.

From the *Department of Cardiology, Cardiac Electrophysiology Laboratory, Evangelismos General Hospital of Athens, Athens, Greece

Department of Cardiology, Helena Venizelou Hospital, Athens, Greece

Department of Medicine and Therapeutics, Faculty of Medicine, Chinese University of Hong Kong, Hong Kong, P.R. China and Li Ka Shing Institute of Health Sciences, Faculty of Medicine, Chinese University of Hong Kong, Hong Kong, P.R. China

§First Department of Cardiology

Second Department of Cardiology, University of Ioannina, School of Medicine, Ioannina, Greece

Laboratory of Physiology, Faculty of Medicine, School of Health Sciences, University of Ioannina, Ioannina, Greece

**Department of Cardiology, ‘G. Hatzikosta’ General Hospital, Ioannina, Greece

††Department of Internal Medicine, National and Kapodistrian University of Athens Medical School, Sotiria General Hospital of Athens, Greece

‡‡NIMITS, Military Hospital, Athens, Greece

§§Department of Cardiology, Tianjin Institute of Cardiology, Second Hospital of Tianjin Medical University, People’s Republic of China

¶¶First Cardiology Clinic, Hippokration Hospital, University of Athens, Athens, Greece

‖‖Division of Cardiology, Electrophysiology and Pacing, Queen’s University, Kingston General Hospital, Kingston, Ontario, Canada

***Department of Medicine, Cardiovascular Section, Heart Rhythm Institute, University of Oklahoma Health Sciences Center, Oklahoma City, OK.

Disclosure: The authors declare no conflicts of interest regarding the overview and meta-analyses, but C.T. declares consultancy fees from Astra Zeneca and lecture honoraria from Sanofi.

Correspondence: George Bazoukis, MD, MSc, Department of Cardiology, General Hospital of Athens “Evangelismos”, Ipsilantou 47, Athens, Greece. E-mail:

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