B-PO05-024 BIOLOGICAL LIFE-STAGE, MENARCHE ONSET, AND THE BURDEN OF CARDIAC EVENTS IN WOMEN WITH CONGENITAL LONG QT SYNDROME

Congenital long QT syndrome (LQTS) is a primary genetic and electrical disorder that increases risk for torsades de pointes, syncope, and sudden death. Post-pubertal women with LQTS require specialized multidisciplinary management before, during, and after pregnancy involving cardiology and obstetrics to reduce risk for cardiac events in themselves and their fetuses and babies. The risk of potentially life-threatening events is lower during pregnancy but increases significantly during the 9-month postpartum period. Treatment of women with LQTS with a preferred β-blocker at optimal doses along with close monitoring are indicated throughout pregnancy and during the high-risk postpartum period.

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Genotype-specific QT correction for heart rate and the risk of life-threatening cardiac events in adolescents with congenital long-QT syndrome

Heart Rhythm ◽

10.1016/j.hrthm.2011.03.009 ◽

2011 ◽

Vol 8 (8) ◽

pp. 1207-1213 ◽

Cited By ~ 13

Author(s):

Alon Barsheshet ◽

Derick R. Peterson ◽

Arthur J. Moss ◽

Peter J. Schwartz ◽

Elizabeth S. Kaufman ◽

...

Keyword(s):

Heart Rate ◽

Long Qt Syndrome ◽

Cardiac Events ◽

Long Qt ◽

Life Threatening ◽

Qt Syndrome ◽

Congenital Long Qt Syndrome

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Video-thoracoscopic left cardiac sympathetic denervation for long-QT syndrome

Asian Cardiovascular and Thoracic Annals ◽

10.1177/0218492320971492 ◽

2020 ◽

pp. 021849232097149

Author(s):

Savvas Lampridis ◽

Achilleas Antonopoulos ◽

Christos Kakos ◽

Sofoklis Mitsos ◽

Davide Patrini ◽

...

Keyword(s):

Long Qt Syndrome ◽

Sympathetic Denervation ◽

Cardiac Events ◽

Long Qt ◽

Video Assisted ◽

Left Cardiac Sympathetic Denervation ◽

The Mean ◽

Qt Syndrome ◽

Congenital Long Qt Syndrome ◽

Cardiac Sympathetic Denervation

Background Congenital long-QT syndrome represents the most common cardiac channelopathy and manifests as potentially lethal ventricular arrhythmias. Prevention strategies include beta-blockade pharmacotherapy, implantable cardioverter-defibrillators, and left cardiac sympathetic denervation, which can increase the threshold for ventricular fibrillation. Herein, we report our experience with video-assisted thoracoscopic left cardiac sympathetic denervation. Methods We performed a retrospective review of the electronic medical records of all patients with congenital long-QT syndrome who underwent video-assisted thoracoscopic left cardiac sympathetic denervation at our institution. Results From September 2009 to May 2016, 6 patients with a mean age of 30.5 years (range 20–47 years) underwent video-assisted thoracoscopic left cardiac sympathetic denervation for medically refractory long-QT syndrome. All patients had an uneventful recovery and were discharged 1–3 days after the operation. At a median follow-up of 14 months (range 12–60 months), 4 patients had no cardiac events while 2 experienced 1 episode of arrhythmic syncope and 1 episode of appropriate implantable cardioverter-defibrillator shock. Following surgery, the mean annual cardiac events in the study cohort decreased from 2.13 to 0.33 ( p = 0.004) and the mean corrected QT interval reduced from 560 ms to 491 ms ( p = 0.006). Conclusions Video-assisted thoracoscopic left cardiac sympathetic denervation is a safe and effective therapy in patients with congenital long-QT syndrome who continue to suffer from recurrent life-threatening arrhythmias or frequent implantable cardioverter-defibrillator discharges despite maximum tolerated doses of beta blockers.

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Identification of a common genetic substrate underlying postpartum cardiac events in congenital long QT syndrome

Heart Rhythm ◽

10.1016/j.hrthm.2004.01.006 ◽

2004 ◽

Vol 1 (1) ◽

pp. 60-64 ◽

Cited By ~ 81

Author(s):

Anant Khositseth ◽

David J Tester ◽

Melissa L Will ◽

Carla M Bell ◽

Michael J Ackerman

Keyword(s):

Long Qt Syndrome ◽

Cardiac Events ◽

Long Qt ◽

Qt Syndrome ◽

Congenital Long Qt Syndrome

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Congenital Long QT Syndrome in an Infant

Acta Chirurgica Latviensis ◽

10.2478/v10163-010-0023-4 ◽

2009 ◽

Vol 9 (1) ◽

pp. 111-112

Author(s):

Aris Lacis ◽

Inga Lace ◽

Elina Teivane ◽

Vita Knauere ◽

Inguna Lubaua ◽

...

Keyword(s):

Sudden Death ◽

Long Qt Syndrome ◽

Heart Rhythm ◽

Torsades De Pointes ◽

Cardiac Events ◽

Long Qt ◽

Heart Rhythm Disorders ◽

Qt Syndrome ◽

First Year Of Life ◽

Congenital Long Qt Syndrome

Congenital Long QT Syndrome in an InfantLong QT syndrome (LQTS) is a disorder of myocardial repolarization characterized by prolonged QT interval on ECG with prevalence close to 1/3000-1/5000. LQTS is characterized by the occurrence of syncopal episodes due to torsades de pointes ventricular tachycardia (VT) and by a high risk for sudden cardiac death among untreated patients (1, 2, 3). In 12% of patients with LQTS, sudden death is the first manifestation of the disease and only in 4% this happens in the first year of life (2). There is consensus that all symptomatic children with LQTS should be treated with β-blockers which are effective in preventing cardiac events and reducing mortality in 70%, but do not protect patients from sudden death completely (1,2,3,4). The prognosis is poor in untreated patients with annual mortality 20% and 10 year mortality up to 50% (1, 2). Here we present a case of relatively rare congenital heart rhythm disorders in an infant which required immediate treatment.

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