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.

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).

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.

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.

Type of funding sources: None.