Clinical characteristics, outcomes, and genetic findings of patients with catecholaminergic polymorphic ventricular tachycardia in Hong Kong: A systematic review

Jeremy Man Ho Hui; Yan Hiu Athena Lee; Kyle Hui; Jiandong Zhou; Danish Iltaf Satti; Cheuk To Chung; Danny Radford; Ishan Lakhani; Amir Hossein Behnoush; Leonardo Roever; Khalid Bin Waleed; Sharen Lee; Gary Tse

doi:10.4103/ACCJ.ACCJ_2_22

Print ISSN : 2666-6979
Online ISSN : 2666-6987

Users Online: 299

Submit article

REVIEW ARTICLE

Year : 2022 \| Volume : 4 \| Issue : 1 \| Page : 3-8

Clinical characteristics, outcomes, and genetic findings of patients with catecholaminergic polymorphic ventricular tachycardia in Hong Kong: A systematic review Jeremy Man Ho Hui¹, Yan Hiu Athena Lee¹, Kyle Hui¹, Jiandong Zhou², Danish Iltaf Satti¹, Cheuk To Chung¹, Danny Radford³, Ishan Lakhani¹, Amir Hossein Behnoush⁴, Leonardo Roever⁵, Khalid Bin Waleed⁶, Sharen Lee¹, Gary Tse⁷ ¹ International Health Informatics Study Network, Cardiovascular Analytics Group, Hong Kong, China-UK Collaboration, China ² Nuffield Department of Medicine, University of Oxford, Oxford, England, United Kingdom ³ International Health Informatics Study Network, Cardiovascular Analytics Group, Hong Kong, China-UK Collaboration; Kent and Medway Medical School, Canterbury, United Kingdom ⁴ International Health Informatics Study Network, Cardiovascular Analytics Group, Hong Kong, China-UK Collaboration; School of Medicine, Tehran University of Medical Sciences, Tehran, Iran ⁵ Department of Clinical Research, Federal University of Uberlândia, Uberlândia, Brazil ⁶ International Health Informatics Study Network, Cardiovascular Analytics Group, Hong Kong, China-UK Collaboration; Department of Cardiology, St George's Hospital University of London, London, United Kingdom ⁷ Kent and Medway Medical School, Canterbury, United Kingdom; Department of Cardiology, Tianjin Key Laboratory of Ionic-Molecular Function of Cardiovascular Disease, Tianjin Institute of Cardiology, Second Hospital of Tianjin Medical University, Tianjin, China Correspondence Address: Dr. Sharen Lee International Health Informatics Study Network, Cardiovascular Analytics Group, Hong Kong China Source of Support: None, Conflict of Interest: None DOI: 10.4103/ACCJ.ACCJ_2_22

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare cardiac ion channelopathy. This was the first systematic review of published works on the clinical characteristics, outcomes, and genetic findings of patients with CPVT from Hong Kong. PubMed and Embase were searched electronically from their inception until February 2022. The Joanna Briggs Institute Critical Appraisal Checklist was used to critically appraise included studies. Studies written in English describing at least one patient with CPVT were included. Studies describing overlapping CPVT patients from previous reports were excluded. Two studies describing 17 patients with CPVT were included. All included studies were rated of acceptable quality. The largest case series evaluated 16 CPVT patients with a mean presentation age of 11 ± 4 years. Of these, 15 patients (93.8%) were symptomatic at initial presentation. Ten patients presented with both premature ventricular complexes (PVCs) and ventricular tachycardia/ventricular fibrillation (VT/VF), whereas one had PVCs without VT/VF. Among the 14 patients (87.5%) who underwent genetic testing, eight (57.1%) tested positive for the ryanodine receptor 2 (RyR2) gene. Over a mean follow-up duration of 116 ± 36 months, six patients (37.5%) had incident VT/VF. In a 5-year review of autopsy data retrieved from public mortuaries for 289 sudden cardiac death patients, one CPVT patient (0.03%) was identified. All patients with CPVT in Hong Kong presented at or below 19 years old, of whom a majority were initially symptomatic or had incident VT/VF events. Novel genetic variants in the RyR2 gene not reported beyond our locality were identified.



[FULL TEXT] [PDF]*