Long term follow up of long QT syndrome treated by overdrive pacing

Introduction: Sudden death and aborted sudden death have been observed in patients with biallelic variants in TECRL . Phenotypes have only begun to be described and no data are available on medical therapy after long-term follow-up. Methods: An international, multicenter retrospective review was conducted. We report new cases associated with TECRL variants and long-term follow-up from previously published cases. Results: We present 10 cases and 37 asymptomatic heterozygous carriers. Median age at onset of cardiac symptoms was 8 years (range 1-22 years) and cases were followed for an average of 10.3 years (SD 8.3), right censored by death in 3 cases. All patients on metoprolol, bisoprolol or atenolol were transitioned to nadolol or propranolol due to failure of therapy. Phenotypes typical of both long QT syndrome and catecholaminergic polymorphic ventricular tachycardia were observed. We also observed divergent phenotypes in some cases despite identical homozygous variants. None of 37 heterozygous family members had a cardiac phenotype. Conclusions: Patients with biallelic pathogenic TECRL variants present with variable cardiac arrhythmia phenotypes, including those typical of long QT syndrome and catecholaminergic polymorphic ventricular tachycardia. Nadolol and propranolol may be superior beta-blockers in this setting. No cardiac disease or sudden death was present in patients with a heterozygous genotype.

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Life-threatening arrhythmias with autosomal recessive TECRL variants

EP Europace ◽

10.1093/europace/euaa376 ◽

2020 ◽

Author(s):

Gregory Webster ◽

Elhadi H Aburawi ◽

Marie A Chaix ◽

Stephanie Chandler ◽

Roger Foo ◽

...

Keyword(s):

Sudden Death ◽

Long Qt Syndrome ◽

Polymorphic Ventricular Tachycardia ◽

Long Qt ◽

Cardiac Phenotype ◽

Cardiac Symptoms ◽

Long Term Follow Up ◽

Qt Syndrome

Abstract Aims Sudden death and aborted sudden death have been observed in patients with biallelic variants in TECRL. However, phenotypes have only begun to be described and no data are available on medical therapy after long-term follow-up. Methods and results An international, multi-centre retrospective review was conducted. We report new cases associated with TECRL variants and long-term follow-up from previously published cases. We present 10 cases and 37 asymptomatic heterozygous carriers. Median age at onset of cardiac symptoms was 8 years (range 1–22 years) and cases were followed for an average of 10.3 years (standard deviation 8.3), right censored by death in three cases. All patients on metoprolol, bisoprolol, or atenolol were transitioned to nadolol or propranolol due to failure of therapy. Phenotypes typical of both long QT syndrome and catecholaminergic polymorphic ventricular tachycardia (CPVT) were observed. We also observed divergent phenotypes in some cases despite identical homozygous variants. None of 37 heterozygous family members had a cardiac phenotype. Conclusion Patients with biallelic pathogenic TECRL variants present with variable cardiac arrhythmia phenotypes, including those typical of long QT syndrome and CPVT. Nadolol and propranolol may be superior beta-blockers in this setting. No cardiac disease or sudden death was present in patients with a heterozygous genotype.

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Role of implantable cardioverter defibrillator therapy in patients with acquired long QT syndrome: A long-term follow-up

EP Europace ◽

10.1093/europace/eur316 ◽

2011 ◽

Vol 14 (3) ◽

pp. 396-401 ◽

Cited By ~ 19

Author(s):

G. Monnig ◽

J. Kobe ◽

A. Loher ◽

K. Wasmer ◽

P. Milberg ◽

...

Keyword(s):

Long Qt Syndrome ◽

Implantable Cardioverter Defibrillator Therapy ◽

Long Qt ◽

Cardioverter Defibrillator ◽

Acquired Long Qt Syndrome ◽

Long Term Follow Up ◽

Qt Syndrome

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Parents' long-term adjustment to the genetic test results of minors at risk for Long QT Syndrome

PsycEXTRA Dataset ◽

10.1037/e524332011-015 ◽

2004 ◽

Author(s):

K. S. W. H. Hendriks ◽

F. J. M. Grosfeld ◽

A. A. M. Wilde ◽

J. van den Bout ◽

I. M. van Langen ◽

...

Keyword(s):

At Risk ◽

Long Qt Syndrome ◽

Genetic Test ◽

Test Results ◽

Long Qt ◽

Genetic Test Results ◽

Qt Syndrome

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The Efficacy of Beta-Blockers in Patients With Long QT Syndrome 1–3 According to Individuals’ Gender, Age, and QTc Intervals: A Network Meta-analysis

Frontiers in Pharmacology ◽

10.3389/fphar.2020.579525 ◽

2020 ◽

Vol 11 ◽

Author(s):

Lu Han ◽

Fuxiang Liu ◽

Qing Li ◽

Tao Qing ◽

Zhenyu Zhai ◽

...

Keyword(s):

Long Qt Syndrome ◽

Clinical Evidence ◽

Beta Blockers ◽

Meta Analysis ◽

Cardiac Events ◽

Long Qt ◽

Confounding Variables ◽

Qt Syndrome ◽

The Relationship

Long QT syndrome (LQTS) is an arrhythmic heart disease caused by congenital genetic mutations, and results in increased occurrence rates of polymorphic ventricular tachyarrhythmias and sudden cardiac death (SCD). Clinical evidence from numerous previous studies suggested that beta blockers (BBs), including atenolol, propranolol, metoprolol, and nadolol, exhibit different efficacies for reducing the risk of cardiac events (CEs), such as syncope, arrest cardiac arrest (ACA), and SCD, in patients with LQTS. In this study, we identified relevant studies in MEDLINE, PubMed, embase, and Cochrane databases and performed a meta-analysis to assess the relationship between the rate of CEs and LQTS individuals with confounding variables, including different gender, age, and QTc intervals. Moreover, a network meta-analysis was not only established to evaluate the effectiveness of different BBs, but also to provide the ranked efficacies of BBs treatment for preventing the recurrence of CEs in LQT1 and LQT2 patients. In conclusion, nadolol was recommended as a relatively effective strategy for LQT2 in order to improve the prognosis of patients during a long follow-up period.

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Universal Electrocardiographic Screening for Long QT Syndrome in Hospitalized Neonates

American Journal of Perinatology ◽

10.1055/s-0039-1678605 ◽

2019 ◽

Vol 37 (03) ◽

pp. 322-325

Author(s):

Bethany D. Kaemingk ◽

Timothy J. Ulrich ◽

Man Li ◽

William A. Carey ◽

Marc A. Ellsworth

Keyword(s):

Long Qt Syndrome ◽

Neonatal Intensive Care ◽

Electronic Monitoring ◽

Screening Method ◽

Unexpected Death ◽

Long Qt ◽

Level Ii ◽

Qt Syndrome ◽

Unnecessary Testing

Objective Long QT syndrome (LQTS) is a known cause of unexpected death, leading some to recommend routine neonatal electrocardiographic (ECG) screening. We used continuous electronic heart rate corrected QT interval (QTc) monitoring to screen for interval prolongation in a cohort of hospitalized neonates to identify those at a risk of having LQTS. We hypothesized that this screening method would yield an acceptable positive predictive value (PPV). Study Design A cohort of 589 infants hospitalized in a level II neonatal intensive care unit were screened through continuous electronic QTc monitoring linked to an investigator-designed, computerized data sniffer. Screening was conducted from days-of-life 3 through 7 or until hospital discharge. The data sniffer alerted investigators for a 24-hour average QTc of ≥475 ms. Positively screened patients were further evaluated with 12-lead ECG. Results Positive screens were obtained in 5.6% of patients, all of whom had negative follow-up ECG testing (PPV = 0%). Furthermore, one-quarter of positively screened neonates underwent echocardiography based on ECG findings, none of which identified clinically relevant pathology. Conclusion Electronic monitoring of QTc in hospitalized neonates during the first week of life was not an efficient way to identify those at a risk of having LQTS. Conversely, screening triggered unnecessary testing.

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