How to Perform and Interpret Provocative Testing for the Diagnosis of Brugada Syndrome, Long-QT Syndrome, and Catecholaminergic Polymorphic Ventricular Tachycardia

Obeyesekere, Manoj; Klein, George J.; Modi, Simon; Leong‐Sit, Peter; Gula, Lorne J.; Yee, Raymond; Skanes, Allan C.; Krahn, Andrew D.

doi:10.1161/circep.111.965947

Cited by 76 publications

(49 citation statements)

References 52 publications

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“…Epinephrine infusion is performed to uncover latent LQTS and CPVT, and Brugada ECGs can be unmasked by a sodium channel blocker (flecainide, ajmaline, procainamide and pilsicainide). Techniques for drug provocation are summarized in a review by Obeyesekere et al (2011).…”

Section: Pharmacologic Provocationmentioning

confidence: 99%

Life threatening causes of syncope: Channelopathies and cardiomyopathies

Herman

Bennett

Chakrabarti

et al. 2014

Autonomic Neuroscience

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Section: Pharmacologic Provocationmentioning

confidence: 99%

Life threatening causes of syncope: Channelopathies and cardiomyopathies

Herman

Bennett

Chakrabarti

et al. 2014

Autonomic Neuroscience

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“…A full discussion of the diagnostic workup is beyond the scope of this article and may be found elsewhere. 68,69 It is important to remember that some patients with hereditary arrhythmogenic syndromes will have only positive family history or suspicious symptoms with a normal ECG (eg, CPVT or IVF). Others will have symptoms that are typically vasovagal but with an abnormal ECG.…”

Section: Discussionmentioning

confidence: 99%

Syncope in Hereditary Arrhythmogenic Syndromes

Adler

Viskin

2015

Cardiology Clinics

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“…Clinical recognition of each of these conditions relies heavily on electrocardiographic findings; however, their frequently concealed nature has necessitated the development of clinical tools capable of unmasking their presence. Different forms of provocative testing are available for LQTS, BrS, and CPVT, while clinicians are generally required to rely on 12‐lead surface ECGs in isolation for short QT syndrome and early repolarization syndrome . Although generally not considered a provocative test for short QT syndrome, treadmill testing may be helpful for confirming the diagnosis given that the QT interval often remains fixed regardless of heart rate.…”

Section: Provocative Testingmentioning

confidence: 99%

Diagnostic evaluation and arrhythmia mechanisms in survivors of unexplained cardiac arrest

Deif

Roberts

2019

Pacing Clinical Electrophis

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Identifying the cause of unexplained cardiac arrest is critical for appropriate management of both survivors and their family members. Aborted cardiac arrests whose cause remains unknown following investigation with a surface ECG, echocardiogram, and coronary angiogram are deemed unexplained. Many of these unexplained arrests are felt to be secondary to concealed forms of cardiac channelopathies and latent or subtle cardiomyopathies. This recognition has led to evaluating a diagnostic role for a series of additional investigations, including advanced imaging, genetic testing, and provocative forms of testing, including sodium channel blockade and treadmill testing. Despite evidence of an improved diagnostic yield through their systematic usage, clinical guidelines have yet to endorse a formal algorithm delineating investigations that must be performed before assigning a label of idiopathic ventricular fibrillation, which has resulted in markedly variables thresholds for concluding this diagnosis. Debate remains regarding the need for an invasive electrophysiology study among these patients, though identification of arrhythmic culprits requiring intracardiac electrograms for diagnostic confirmation have suggested a potential role when an initial comprehensive evaluation is unrevealing. Although progress is being made, the sizeable portion of arrests that remain unexplained despite completion of a comprehensive evaluation highlights an ongoing need for further research and additional tools to help unravel the ongoing mysteries of these near fatal events.

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How to Perform and Interpret Provocative Testing for the Diagnosis of Brugada Syndrome, Long-QT Syndrome, and Catecholaminergic Polymorphic Ventricular Tachycardia

Cited by 76 publications

References 52 publications

Life threatening causes of syncope: Channelopathies and cardiomyopathies

Life threatening causes of syncope: Channelopathies and cardiomyopathies

Syncope in Hereditary Arrhythmogenic Syndromes

Diagnostic evaluation and arrhythmia mechanisms in survivors of unexplained cardiac arrest

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