Paediatric/young versus adult patients with long QT syndrome
Sharen Lee, Jiandong Zhou, Kamalan Jeevaratnam, Wing Tak Wong, Ian Chi Kei Wong, Chloe Mak, Ngai Shing Mok, Tong Liu, Qingpeng Zhang and Gary Tse 2021. Paediatric/young versus adult patients with long QT syndrome. Open Heart. 2021 (8), p. e001671. https://doi.org/10.1136/openhrt-2021-001671
|Authors||Sharen Lee, Jiandong Zhou, Kamalan Jeevaratnam, Wing Tak Wong, Ian Chi Kei Wong, Chloe Mak, Ngai Shing Mok, Tong Liu, Qingpeng Zhang and Gary Tse|
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.
|Keywords||Long QT syndrome; Children; Young patients; Adult patients|
|Journal citation||2021 (8), p. e001671|
|Publisher||BMJ Publishing Group|
|Digital Object Identifier (DOI)||https://doi.org/10.1136/openhrt-2021-001671|
|Online||12 Sep 2021|
|Publication process dates|
|Accepted||02 Aug 2021|
|Deposited||31 May 2023|
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