Paediatric/young versus adult patients with long QT syndrome,Open Heart

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Paediatric/young versus adult patients with long QT syndrome
Open Heart Pub Date : 2021-09-01 , DOI: 10.1136/openhrt-2021-001671
Sharen Lee ₁ , Jiandong Zhou ₂ , Kamalan Jeevaratnam ₃ , Wing Tak Wong ₄ , Ian Chi Kei Wong ₅ , Chloe Mak ₆ , Ngai Shing Mok ₇ , Tong Liu ₈ , Qingpeng Zhang ₂ , Gary Tse _{3,

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Affiliation

Cardiovascular Analytics Group, Hong Kong, China-UK Collaboration.
School of Data Science, City University of Hong Kong, Hong Kong, People's Republic of China.
Faculty of Health and Medical Sciences, University of Surrey, Guildford, Surrey, UK.
School of Life Sciences, Chinese University of Hong Kong, Hong Kong, People's Republic of China.
Research Department of Practice and Policy, University College London School of Pharmacy, London, UK.
Department of Pathology, Hong Kong Children's Hospital, Hong Kong, People's Republic of China.
Department of Medicine and Geriatrics, Princess Margaret Hospital, Hong Kong, People's Republic of China.
Tianjin Key Laboratory of Ionic-Molecular Function of Cardiovascular Disease, Department of Cardiology, Tianjin Institute of Cardiology, Second Hospital of Tianjin Medical University, Tianjin, People's Republic of China.
Cardiovascular Analytics Group, Hong Kong, China-UK Collaboration

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. Data are available in a public, open access repository. Data are available on reasonable request.

更新日期：2021-09-13

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