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Clinical and genetic analysis of long QT syndrome in two Malay children

Wong AR, Zilfalil BA, Bhuiyan ZA

Med J Malaysia, 2019 08;74(4):341-343.
PMID: 31424047

Long QT syndrome (LQTS) is predominantly a genetic cardiac arrhythmia disorder. We report here our study on long QT syndrome from two children from Kelantan, Malaysia. Clinical and genetic findings of these two unrelated Malay children with LQTS is discussed. We found a Long QT, type 1 causal mutation, p.Ile567Thr in the KCNQ1 gene in the first child. A pathogenic mutation could not be detected in the second child, explaining the heterogeneity of this disease.

Matched MeSH terms: Long QT Syndrome/diagnosis*
Fulltext The effects of ageing and adrenergic challenge on electrocardiographic phenotypes in a murine model of long QT syndrome type 3

Chadda KR, Ahmad S, Valli H, den Uijl I, Al-Hadithi AB, Salvage SC, et al.

Sci Rep, 2017 09 11;7(1):11070.
PMID: 28894151 DOI: 10.1038/s41598-017-11210-3

Long QT Syndrome 3 (LQTS3) arises from gain-of-function Nav1.5 mutations, prolonging action potential repolarisation and electrocardiographic (ECG) QT interval, associated with increased age-dependent risk for major arrhythmic events, and paradoxical responses to β-adrenergic agents. We investigated for independent and interacting effects of age and Scn5a+/ΔKPQ genotype in anaesthetised mice modelling LQTS3 on ECG phenotypes before and following β-agonist challenge, and upon fibrotic change. Prolonged ventricular recovery was independently associated with Scn5a+/ΔKPQ and age. Ventricular activation was prolonged in old Scn5a+/ΔKPQ mice (p = 0.03). We associated Scn5a+/ΔKPQ with increased atrial and ventricular fibrosis (both: p

Matched MeSH terms: Long QT Syndrome/diagnosis*
Fulltext Torsade de pointes and syncopal attacks in a 26-year old woman with congenital complete heart block and prolonged QT interval

Quek DK, Ong SB

Singapore Med J, 1990 Apr;31(2):185-8.
PMID: 2371586

A 26-year old woman with congenital complete heart block and prolonged QT interval presented for the first time with syncopal attacks associated with torsade de pointes in adulthood. Cardioversion followed by overdrive pacing was needed to finally control the unstable rhythm. During episodes of non-capture, paraoxysms of torsade de pointes leading to ventricular flutter were recorded by a 24-hour ambulatory electrocardiographic monitoring. Beta-blockade and permanent ventricular pacing finally abolished both the syncopal attacks and the torsade phenomena. The prognosis of congenital complete heart block associated with QT prolongation resembles that of the Romano-Ward syndrome. Recognition of this variant would facilitate earlier treatment of this rare but potentially lethal disorder.

Matched MeSH terms: Long QT Syndrome/diagnosis
Smartphone electrocardiogram for QT interval monitoring in Coronavirus Disease 2019 (COVID-19) patients treated with Hydroxychloroquine

Andy Ko TY, Chen LS, Pang IX, Ling HS, Wong TC, Sia Tonnii LL, et al.

Med J Malaysia, 2021 03;76(2):125-130.
PMID: 33742617

INTRODUCTION: The global pandemic of Corona Virus Disease 2019 (COVID-19) has led to the re-purposing of medications, such as hydroxychloroquine and lopinavir-ritonavir in the treatment of the earlier phase of COVID-19 before the recognized benefit of steroids and antiviral. We aim to explore the corrected QT (QTc) interval and 'torsadogenic' potential of hydroxychloroquine and lopinavir-ritonavir utilising a combination of smartphone electrocardiogram and 12-lead electrocardiogram monitoring.
MATERIALS AND METHODS: Between 16-April-2020 to 30-April- 2020, patients with suspected or confirmed for COVID-19 indicated for in-patient treatment with hydroxychloroquine with or without lopinavir-ritonavir to the Sarawak General Hospital were monitored with KardiaMobile smartphone electrocardiogram (AliveCor®, Mountain View, CA) or standard 12-lead electrocardiogram. The baseline and serial QTc intervals were monitored till the last dose of medications or until the normalization of the QTc interval.
RESULTS: Thirty patients were treated with hydroxychloroquine, and 20 (66.7%) patients received a combination of hydroxychloroquine and lopinavir-ritonavir therapy. The maximum QTc interval was significantly prolonged compared to baseline (434.6±28.2msec vs. 458.6±47.1msec, p=0.001). The maximum QTc interval (456.1±45.7msec vs. 464.6±45.2msec, p=0.635) and the delta QTc (32.6±38.5msec vs. 26.3±35.8msec, p=0.658) were not significantly different between patients on hydroxychloroquine or a combination of hydroxychloroquine and lopinavir-ritonavir. Five (16.7%) patients had QTc of 500msec or more. Four (13.3%) patients required discontinuation of hydroxychloroquine and 3 (10.0%) patients required discontinuation of lopinavirritonavir due to QTc prolongation. However, no torsade de pointes was observed.
CONCLUSIONS: QTc monitoring using smartphone electrocardiogram was feasible in COVID-19 patients treated with hydroxychloroquine with or without lopinavir-ritonavir. The usage of hydroxychloroquine and lopinavir-ritonavir resulted in QTc prolongation, but no torsade de pointes or arrhythmogenic death was observed.

Matched MeSH terms: Long QT Syndrome/diagnosis*