Paediatric/young versus adult patients with congenital long QT syndrome or catecholaminergic polymorphic ventricular tachycardia

Abstract Introduction Congenital long QT syndrome (LQTS) and catecholaminergic ventricular tachycardia (CPVT) are less prevalent cardiac ion channelopathies than Brugada syndrome in Asia. The present study compared paediatric/young and adult patients with these conditions. Method This was a territory-wide retrospective cohort study of consecutive patients diagnosed with congenital LQTS and CPVT attending public hospitals in Hong Kong. The primary outcome was spontaneous ventricular tachycardia/ventricular fibrillation (VT/VF). Results A total of 142 congenital LQTS (mean onset age= 27±23 years old) and 16 CPVT (mean presentation age=11±4 years old) patients were included. For congenital LQTS, arrhythmias other than VT/VF (HR=4.67, 95% confidence interval = [1.53–14.3], p=0.007), initial VT/VF (HR=3.25 [1.29–8.16], p=0.012) and Schwartz score (HR=1.90 [1.11–3.26], p=0.020) were predictive of the primary outcome for the overall cohort, whilst arrhythmias other than VT/VF (HR=5.41 [1.36–21.4], p=0.016) and Schwartz score (HR=4.67 [1.48–14.7], p=0.009) were predictive for the adult subgroup (>25 years old; n=58). All CPVT patients presented before the age of 25 but no significant predictors of VT/VF were identified. 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 in LQTS, and initial VT/VF/sudden cardiac death, palpitations, QTc, initially symptomatic and heart rate in CPVT. Conclusion Clinical and ECG presentation vary between the pediatric/young and adult congenital LQTS population. All CPVT patients presented before the age of 25. Machine learning models achieved more accurate VT/VF prediction. Funding Acknowledgement Type of funding sources: None. Kaplan-Meier survival curve for LQTSKaplan-Meier survival curve for CPVT