Auditory stimuli as a trigger for arrhythmic events differentiate HERG-related (LQTS2) patients from KVLQT1-related patients (LQTS1)

Wilde, Arthur A.M.; Jongbloed, Roselie; Doevendans, Pieter A.; Düren, Donald R.; Hauer, Richard N.W.; Langen, Irene M. van; Tintelen, J Peter van; Smeets, Hubert J.M.; Meyer, H.; Geelen, J. L. M. C.

doi:10.1016/s0735-1097(98)00578-6

Cited by 281 publications

(168 citation statements)

References 28 publications

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“…Lifestyle adjustment, avoiding event related triggers (for example, swimming in LQT1 and loud acoustic stimuli in LQT2). 11,12 ) Avoiding stringent competition sports. Avoiding QT prolonging drugs (www.qtdrugs.org) and avoidance of fever.…”

Section: Will the Results Of A Genetic Test Influence Lifestyle And Prmentioning

confidence: 99%

“…Also, lifestyle advice is different for the different genotypes. 11,12 Prognosis (please describe) Regular preventive medical checkups to recognize disease progression earlier, and appropriate treatment improves prognosis. On appropriate treatment and with appropriate lifestyle adjustments prognosis is good and may not differ from normal.…”

Section: Therapy (Please Describe)mentioning

confidence: 99%

“…For every patient (clinically affected or not) there are known specific triggers for arrhythmias to be avoided (e.g., QT prolonging drugs, competitive sports, low potassium serum levels, swimming in LQT1, sudden loud noise in LQT2). 11,12 Thus, there should be thorough counselling concerning lifestyle modifications and choice of employment.…”

Section: If Applicable Further Consequences Of Testingmentioning

confidence: 99%

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Clinical utility gene card for: Long-QT Syndrome (types 1–13)

2013

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Section: Will the Results Of A Genetic Test Influence Lifestyle And Prmentioning

confidence: 99%

Section: Therapy (Please Describe)mentioning

confidence: 99%

Section: If Applicable Further Consequences Of Testingmentioning

confidence: 99%

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Clinical utility gene card for: Long-QT Syndrome (types 1–13)

2013

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“…Often, a sudden startle in the form of an auditory stimulus (a telephone, alarm clock, ambulance siren, etc.) is a specific trigger in LQT2 (Wilde et al, 1999). It has been noted that the risk of ventricular arrhythmia decreases during pregnancy, although a higher post-postpartum vulnerability to cardiac events is reported in LQTS, especially the LQT2 subtype (Khositseth et al, 2004).…”

Section: Genotype Specific Triggers Of Arrhythmic Events-thoughmentioning

confidence: 99%

“…TdP is the most common arrhythmia seen in all subtypes of LQTS, genotype-specific triggers and onset of TdP has been reported in patients with the LQT1, LQT2 and LQT3 (Ackerman et al, 1999;Moss et al, 1999;Wilde et al, 1999;Schwartz et al, 2001;Tan et al, 2006). Schwartz et al (2001) first reported genotype-specific triggers for cardiac events in patients with LQT1, LQT2 and LQT3.…”

Section: Genotype Specific Triggers Of Arrhythmic Events-thoughmentioning

confidence: 99%

Pharmacological approach to the treatment of long and short QT syndromes

Patel

Antzelevitch

2008

Pharmacology & Therapeutics

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Inherited channelopathies have received increasing attention in recent years. The past decade has witnessed impressive progress in our understanding of the molecular and cellular basis of arrhythmogenesis associated with inherited channelopathies. An imbalance in ionic forces induced by these channelopathies affects the duration of ventricular repolarization and amplifies the intrinsic electrical heterogeneity of the myocardium, creating an arrhythmogenic milieu. Today, many of the channelopathies have been linked to mutations in specific genes encoding either components of ion channels or membrane or regulatory proteins. Many of the channelopathies are genetically heterogeneous with a variable degree of expression of the disease. Defining the molecular basis of channelopathies can have a profound impact on patient management, particularly in cases in which genotype-specific pharmacotherapy is available.The long QT syndrome (LQTS) is one of the first identified and most studied channelopathies where abnormal prolongation of ventricular repolarization predisposes an individual to life threatening ventricular arrhythmia called Torsade de Pointes. On the other hand of the spectrum, molecular defects favoring premature repolarization lead to Short QT syndrome (SQTS), a recently described inherited channelopathy. Both of these channelopathies are associated with a high risk of sudden cardiac death due to malignant ventricular arrhythmia. Whereas pharmacological therapy is first line treatment for LQTS, defibrillators are considered as primary treatment for SQTS. This review provides a comprehensive review of the molecular genetics, clinical features, genotype-phenotype correlations and genotype-specific approach to pharmacotherapy of these two mirror-image channelopathies, SQTS and LQTS.

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