Congenital Long QT Syndrome

Krahn, Andrew D.; Laksman, Zachary; Sy, Raymond W.; Postema, Pieter G.; Ackerman, Michael J.; Wilde, Arthur A.M.; Han, Haobo

doi:10.1016/j.jacep.2022.02.017

Cited by 71 publications

(41 citation statements)

References 197 publications

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“…Conversely, Long QT syndrome (LQTS) is a heterogenous group of channelopathies that have genetic mutations associated with cardiac repolarization leading to prolongation of the QT on the surface electrogram that can lead to sudden cardiac death from Torsades de Pointes (TdP). Although there are many precipitants for TdP in patients with LQTS the patients at the greatest risk from risk of SCD with sexual activity are those with LQT1 and LQT2 which can be triggered by physical activity or emotional stress respectively ( 12 ).…”

Section: The Pathophysiology Of Arrhythmias and Sudden Death During Sexmentioning

confidence: 99%

Sex, Rhythm & Death: The effect of sexual activity on cardiac arrhythmias and sudden cardiac death

Dye

Engelstein

Swearingen

et al. 2022

Front. Cardiovasc. Med.

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Arrhythmias and sudden cardiac death with sexual activity are rare. However, the demographics are changing regarding the cardiovascular patients at risk for these events. Recent studies have highlighted that the individuals having cardiac events during sexual activity are becoming younger, with a higher proportion of female decedents than previously described. There needs to be an open dialog between the cardiovascular team and the cardiac patient to provide the education and reassurance necessary for cardiovascular patients to participate in sexual intercourse safely. This paper reviews how sexual activity can lead to an increase in cardiac arrhythmias and sudden cardiac arrest in patients that are not medically optimized or are unaware of their underlying cardiac condition. The most common cardiovascular diseases associated with sexually induced arrhythmias and arrest are discussed regarding their potential risk and the psychosocial impact of this risk on these patients. Finally, cardiovascular medications and implantable cardioverter-defibrillators (ICDs) are addressed by reviewing the literature on the safety profile of these cardiac interventions in this patient population. Overall, sexual activity is safe for most cardiac patients, and providing proper education to the patient and their partner can improve the safety profile for patients with higher risk cardiovascular conditions. To give the appropriate education and reassurance necessary, cardiovascular team members need an understanding of the pathophysiology of how sexual activity can provoke arrhythmias and sudden cardiac arrest. Healthcare providers also need to build comfort in speaking to all patients and ensure that sexual partners, female patients, and those in the LGBTQIA + community receive the same access to counseling but tailored to their individual needs.

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Section: The Pathophysiology Of Arrhythmias and Sudden Death During Sexmentioning

confidence: 99%

Sex, Rhythm & Death: The effect of sexual activity on cardiac arrhythmias and sudden cardiac death

Dye

Engelstein

Swearingen

et al. 2022

Front. Cardiovasc. Med.

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“…We conclude with a discussion summarizing the computational approaches and strategies that are being developed to navigate the increasingly complex genotype-phenotype landscape and understand variantspecific differences in channel structure and dysfunction. The goal of this review is to compliment several existing reviews that focus on the genetics or ventricular repolarization and use of novel in vitro strategies (e.g., human inducible pluripotent stem-cell-derived cardiomyocytes) to understand inherited arrhythmia syndromes [16][17][18].…”

Section: Introductionmentioning

confidence: 99%

Mutation-Specific Differences in Kv7.1 (KCNQ1) and Kv11.1 (KCNH2) Channel Dysfunction and Long QT Syndrome Phenotypes

Kekenes‐Huskey

Burgess

Sun

et al. 2022

IJMS

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The electrocardiogram (ECG) empowered clinician scientists to measure the electrical activity of the heart noninvasively to identify arrhythmias and heart disease. Shortly after the standardization of the 12-lead ECG for the diagnosis of heart disease, several families with autosomal recessive (Jervell and Lange-Nielsen Syndrome) and dominant (Romano–Ward Syndrome) forms of long QT syndrome (LQTS) were identified. An abnormally long heart rate-corrected QT-interval was established as a biomarker for the risk of sudden cardiac death. Since then, the International LQTS Registry was established; a phenotypic scoring system to identify LQTS patients was developed; the major genes that associate with typical forms of LQTS were identified; and guidelines for the successful management of patients advanced. In this review, we discuss the molecular and cellular mechanisms for LQTS associated with missense variants in KCNQ1 (LQT1) and KCNH2 (LQT2). We move beyond the “benign” to a “pathogenic” binary classification scheme for different KCNQ1 and KCNH2 missense variants and discuss gene- and mutation-specific differences in K+ channel dysfunction, which can predispose people to distinct clinical phenotypes (e.g., concealed, pleiotropic, severe, etc.). We conclude by discussing the emerging computational structural modeling strategies that will distinguish between dysfunctional subtypes of KCNQ1 and KCNH2 variants, with the goal of realizing a layered precision medicine approach focused on individuals.

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“…A prolonged QT interval on ECG should raise the suspicion of a long QT syndrome (LQTS). [1] LQTS may be inherited (gene variant) or acquired (drug-induced) and generally results from a cardiac ion channel dysfunction. Human ether-a-go-go related gene (hERG) is such a typically affected cardiac voltage-gated K + channel.…”

mentioning

confidence: 99%

“…Pharmacotherapy (mainly beta- and sodium channel blockers), device (implantable cardioverter defibrillator) and/or left cardiac sympathetic denervation therapy are the mainstay of treatment of LQTS. [1] Most vitally, all patients are advised lifestyle adjustments. [1] Considerations usually revolve around competitive sports activities, avoidance of QT-prolonging drugs and dehydration, whereas diet recommendations have been kept to a minimum.…”

mentioning

confidence: 99%

“… [1] Most vitally, all patients are advised lifestyle adjustments. [1] Considerations usually revolve around competitive sports activities, avoidance of QT-prolonging drugs and dehydration, whereas diet recommendations have been kept to a minimum. While the arrhythmogenic effect of citrus fruit (hERG-inhibiting properties of the flavonoid naringenin) and quinine-containing beverages (hERG-inhibiting properties of the optical isomer of quinidine) have been experimentally proven, energy drinks have raised concerns and public awareness of potentially life-threatening dietary triggers, especially for LQTS patients.…”

mentioning

confidence: 99%

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