Paediatric/young versus adult patients with long QT syndrome
Tong Liu,
Gary Tse,
Ian Chi Kei Wong,
Qingpeng Zhang,
Jiandong Zhou,
Sharen Lee,
Ngai Shing Mok,
Chloe Mak,
Kamalan Jeevaratnam,
Wing Tak Wong
Affiliations
Tong Liu
1 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
Tianjin Key Laboratory of Ionic-Molecular Function of Cardiovascular Disease, Department of Cardiology, Tianjin Institute of Cardiology, The Second Hospital of Tianjin Medical University, Tianjin, China
Ian Chi Kei Wong
Research Department of Practice and Policy, University College London School of Pharmacy, London, UK
Qingpeng Zhang
2City University of Hong Kong
Jiandong Zhou
School of Data Science, City University of Hong Kong, Hong Kong, People`s Republic of China
Sharen Lee
6Chinese University of Hong Kong
Ngai Shing Mok
Department of Medicine and Geriatrics, Princess Margaret Hospital, Hong Kong, Hong Kong
Chloe Mak
Department of Pathology, Hong Kong Children`s Hospital, Hong Kong, Hong Kong
Kamalan Jeevaratnam
Faculty of Health and Medical Sciences, University of Surrey, Guildford, Surrey, UK
Wing Tak Wong
School of Life Sciences, Chinese University of Hong Kong, Hong Kong, People`s Republic of China
Introduction Long QT syndrome (LQTS) is a less prevalent cardiac ion channelopathy than Brugada syndrome in Asia. The present study compared the outcomes between paediatric/young and adult LQTS patients.Methods This was a population-based retrospective cohort study of consecutive patients diagnosed with LQTS attending public hospitals in Hong Kong. The primary outcome was spontaneous ventricular tachycardia/ventricular fibrillation (VT/VF).Results A total of 142 LQTS (mean onset age=27±23 years old) were included. Arrhythmias other than VT/VF (HR 4.67, 95% CI (1.53 to 14.3), p=0.007), initial VT/VF (HR=3.25 (95% CI 1.29 to 8.16), p=0.012) and Schwartz score (HR=1.90 (95% CI 1.11 to 3.26), p=0.020) were predictive of the primary outcome for the overall cohort, while arrhythmias other than VT/VF (HR=5.41 (95% CI 1.36 to 21.4), p=0.016) and Schwartz score (HR=4.67 (95% CI 1.48 to 14.7), p=0.009) were predictive for the adult subgroup (>25 years old; n=58). A random survival forest model identified initial VT/VF, Schwartz score, initial QTc interval, family history of LQTS, initially asymptomatic and arrhythmias other than VT/VF as the most important variables for risk prediction.Conclusion Clinical and ECG presentation varies between the paediatric/young and adult LQTS population. Machine learning models achieved more accurate VT/VF prediction.
