Clinical Characteristics, Genetic Findings and Arrhythmic Outcomes of Patients with Catecholaminergic Polymorphic Ventricular Tachycardia from China: A Systematic Review
Justin Leung,
Sharen Lee,
Jiandong Zhou,
Kamalan Jeevaratnam,
Ishan Lakhani,
Danny Radford,
Emma Coakley-Youngs,
Levent Pay,
Göksel Çinier,
Meltem Altinsoy,
Amir Hossein Behnoush,
Elham Mahmoudi,
Paweł T. Matusik,
George Bazoukis,
Sebastian Garcia-Zamora,
Shaoying Zeng,
Ziliang Chen,
Yunlong Xia,
Tong Liu,
Gary Tse
Affiliations
Justin Leung
Cardiac Electrophysiology Unit, Cardiovascular Analytics Group, China-UK Collaboration, Hong Kong, China
Sharen Lee
Cardiac Electrophysiology Unit, Cardiovascular Analytics Group, China-UK Collaboration, Hong Kong, China
Jiandong Zhou
School of Data Science, City University of Hong Kong, Hong Kong, China
Kamalan Jeevaratnam
Faculty of Health and Medical Sciences, University of Surrey, Guildford GU2 7XH, UK
Ishan Lakhani
Cardiac Electrophysiology Unit, Cardiovascular Analytics Group, China-UK Collaboration, Hong Kong, China
Danny Radford
Kent and Medway Medical School, Canterbury CT2 7FS, UK
Emma Coakley-Youngs
Kent and Medway Medical School, Canterbury CT2 7FS, UK
Levent Pay
Department of Cardiology, Dr Siyami Ersek Thoracic and Cardiovascular Surgery Training and Research Hospital, Istanbul 34147, Turkey
Göksel Çinier
Department of Cardiology, Dr Siyami Ersek Thoracic and Cardiovascular Surgery Training and Research Hospital, Istanbul 34147, Turkey
Meltem Altinsoy
Department of Cardiology, University of Health Sciences, Diskapi Yildirim Beyazit Training and Research Hospital, Ankara 06145, Turkey
Amir Hossein Behnoush
Universal Scientific Education and Research Network (USERN), Tehran University of Medical Sciences, Tehran 1416643931, Iran
Elham Mahmoudi
Universal Scientific Education and Research Network (USERN), Tehran University of Medical Sciences, Tehran 1416643931, Iran
Paweł T. Matusik
Department of Electrocardiology, Institute of Cardiology, Jagiellonian University Medical College, John Paul II Hospital, 31-202 Kraków, Poland
George Bazoukis
Department of Cardiology, Larnaca General Hospital, Larnaca 6301, Cyprus
Sebastian Garcia-Zamora
Cardiac Intensive Care Unit, Department of Cardiology, Delta Clinic, Rosario S2000, Argentina
Shaoying Zeng
Department of Pediatric Cardiology, Guangdong Cardiovascular Institute, Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangdong Provincial People’s Hospital, Guangdong Academy of Medical Sciences, Guangzhou 510080, China
Ziliang Chen
Tianjin Key Laboratory of Ionic-Molecular Function of Cardiovascular Disease, Department of Cardiology, Tianjin Institute of Cardiology, Second Hospital of Tianjin Medical University, Tianjin 300211, China
Yunlong Xia
Department of Cardiology, First Affiliated Hospital of Dalian Medical University, Dalian 116014, China
Tong Liu
Tianjin Key Laboratory of Ionic-Molecular Function of Cardiovascular Disease, Department of Cardiology, Tianjin Institute of Cardiology, Second Hospital of Tianjin Medical University, Tianjin 300211, China
Gary Tse
Cardiac Electrophysiology Unit, Cardiovascular Analytics Group, China-UK Collaboration, Hong Kong, China
Introduction: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare inherited cardiac ion channelopathy. The present study aims to examine the clinical characteristics, genetic basis, and arrhythmic outcomes of CPVT patients from China to elucidate the difference between CPVT patients in Asia and Western countries. Methods: PubMed and Embase were systematically searched for case reports or series reporting on CPVT patients from China until 19 February 2022 using the keyword: “Catecholaminergic Polymorphic Ventricular Tachycardia” or “CPVT”, with the location limited to: “China” or “Hong Kong” or “Macau” in Embase, with no language or publication-type restriction. Articles that did not state a definite diagnosis of CPVT and articles with duplicate cases found in larger cohorts were excluded. All the included publications in this review were critically appraised based on the Joanna Briggs Institute Critical Appraisal Checklist. Clinical characteristics, genetic findings, and the primary outcome of spontaneous ventricular tachycardia/ventricular fibrillation (VT/VF) were analyzed. Results: A total of 58 unique cases from 15 studies (median presentation age: 8 (5.0–11.8) years old) were included. All patients, except one, presented at or before 19 years of age. There were 56 patients (96.6%) who were initially symptomatic. Premature ventricular complexes (PVCs) were present in 44 out of 51 patients (86.3%) and VT in 52 out of 58 patients (89.7%). Genetic tests were performed on 54 patients (93.1%) with a yield of 87%. RyR2, CASQ2, TERCL, and SCN10A mutations were found in 35 (71.4%), 12 (24.5%), 1 (0.02%) patient, and 1 patient (0.02%), respectively. There were 54 patients who were treated with beta-blockers, 8 received flecainide, 5 received amiodarone, 2 received verapamil and 2 received propafenone. Sympathectomy (n = 10), implantable cardioverter-defibrillator implantation (n = 8) and ablation (n = 1) were performed. On follow-up, 13 patients developed VT/VF. Conclusion: This was the first systematic review of CPVT patients from China. Most patients had symptoms on initial presentation, with syncope as the presenting complaint. RyR2 mutation accounts for more than half of the CPVT cases, followed by CASQ2, TERCL and SCN10A mutations.
