Procainamide infusion in the evaluation of unexplained cardiac arrest: From the Cardiac Arrest Survivors with Preserved Ejection Fraction Registry (CASPER)

Somani, Riyaz; Krahn, Andrew D.; Healey, Jeffrey S.; Chauhan, Vijay S.; Birnie, David H.; Champagne, Jean; Sanatani, Shubhayan; Angaran, Paul; Gow, Robert M.; Chakrabarti, Santabhanu; Gerull, Brenda; Yee, Raymond; Skanes, Allan C.; Gula, Lorne J.; Leong‐Sit, Peter; Klein, George J.; Gollob, Michael H.; Talajic, Mario; Gardner, Martin J.; Simpson, Christopher S.

doi:10.1016/j.hrthm.2014.03.022

Cited by 47 publications

(23 citation statements)

References 32 publications

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“…The diagnostic utility of provocative pharmacological tests in patients with UCA is well established. 9,11,20 The results in first-degree relatives were comparable to the diagnostic yield of pharmacological drug challenge in CASPER probands suggesting a higher proportion of false-positive test in our study population.…”

Section: Discussionsupporting

confidence: 75%

“…15 Drug challenge with procainamide and epinephrine was performed as previously described. 11,16 Cardiac abnormalities were defined as new diagnosis of a previously unknown primary electric disorder (eg, LQT syndrome or Brugada syndrome), a new diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) or as new diagnosis of another previously unknown structural heart disease (eg, hypertrophic cardiomyopathy, myocarditis, cardiac sarcoidosis, etc.). Diagnostic criteria for LQT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia (CPVT), ARVC, and ERS were based on current guidelines.…”

Section: Cardiac Investigationsmentioning

confidence: 99%

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Cardiac Abnormalities in First-Degree Relatives of Unexplained Cardiac Arrest Victims

Steinberg¹,

Padfield²,

Champagne³

et al. 2016

Circ: Arrhythmia and Electrophysiology

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Background— Unexplained cardiac arrest (UCA) may be explained by inherited arrhythmia syndromes. The Cardiac Arrest Survivors With Preserved Ejection Fraction Registry prospectively assessed first-degree relatives of UCA or sudden unexplained death victims to screen for cardiac abnormalities. Methods and Results— Around 398 first-degree family members (186 UCA, 212 sudden unexplained death victims’ relatives; mean age, 44±17 years) underwent extensive cardiac workup, including ECG, signal averaged ECG, exercise testing, cardiac imaging, Holter-monitoring, and selective provocative drug testing with epinephrine or procainamide. Genetic testing was performed when a mutation was identified in the UCA survivor or when the diagnostic workup revealed a phenotype suggestive of a specific inherited arrhythmia syndrome. The diagnostic strength was classified as definite, probable, or possible based on previously published definitions. Cardiac abnormalities were detected in 120 of 398 patients (30.2%) with 67 of 398 having a definite or probable diagnosis (17%), including Long-QT syndrome (13%), catecholaminergic polymorphic ventricular tachycardia (4%), arrhythmogenic right ventricular cardiomyopathy (4%), and Brugada syndrome (3%). The detection yield was similar for family members of UCA and sudden unexplained death victims (31% versus 27%; P =0.59). Genetic testing was performed more often in family members of UCA patients (29% versus 20%; P =0.03). Disease-causing mutations were identified in 20 of 398 relatives (5%). The most common pathogenic mutations were RyR2 (2%), SCN5A (1%), and KNCQ1 (0.8%). Conclusions— Cardiac screening revealed abnormalities in 30% of first-degree relatives of UCA or sudden unexplained death victims, with a clear working diagnosis in 17%. Long-QT, arrhythmogenic right ventricular cardiomyopathy, and catecholaminergic polymorphic ventricular tachycardia were the most common diagnoses. Systematic cascade screening and genetic testing in asymptomatic individuals will lead to preventive lifestyle and medical interventions with potential to prevent sudden cardiac death. Clinical Trial Registration— URL: http://www.clinicaltrials.gov . Unique identifier: NCT00292032.

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Section: Discussionsupporting

confidence: 75%

Section: Cardiac Investigationsmentioning

confidence: 99%

Cardiac Abnormalities in First-Degree Relatives of Unexplained Cardiac Arrest Victims

Steinberg¹,

Padfield²,

Champagne³

et al. 2016

Circ: Arrhythmia and Electrophysiology

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“…Procainamide testing may provoke a Brugada pattern irrespective of the baseline ECG and should be considered in the workup of SCD. 66 • Cardiac MRI (ARVC, sarcoidosis, myocarditis, myocardial injury from coronary spasm).…”

Section: Investigations In Survivorsmentioning

confidence: 99%

A Clinical Perspective on Sudden Cardiac Death

2016

Arrhythmia &Amp; Electrophysiology Review

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This article presents the epidemiology, aetiology and pathophysiology of sudden cardiac death. The modern management of survivors as well as of family members of victims is discussed, as are the relevant recommendations of guidelines prepared by learned societies. KeywordsOut-of-hospital cardiac arrest, sports-related sudden death, sudden cardiac death, sudden unexplained death syndrome Disclosure: The authors have no conflicts of interest to declare.

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“…Ajmaline is the sodium channel blocker most frequently used in Europe and, while considered sensitive, concerns have been raised regarding its specificity. Systematic use of ajmaline in survivors of unexplained arrest and family members of SCD identified a yield of 14% for diagnosing BrS, which contrasts relative to a 6.9% yield observed with procainamide, the agent used most often in North America . Observations highlighting a potential lack of specificity for ajmaline have included positive tests in family members that were genotype negative for the familial pathogenic SCN5A mutation and a study that identified positive ajmaline tests in an astounding 27% of individuals with atrioventricular nodal reentrant tachycardia and 5% of control patients .…”

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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Procainamide infusion in the evaluation of unexplained cardiac arrest: From the Cardiac Arrest Survivors with Preserved Ejection Fraction Registry (CASPER)

Cited by 47 publications

References 32 publications

Cardiac Abnormalities in First-Degree Relatives of Unexplained Cardiac Arrest Victims

Cardiac Abnormalities in First-Degree Relatives of Unexplained Cardiac Arrest Victims

A Clinical Perspective on Sudden Cardiac Death

Diagnostic evaluation and arrhythmia mechanisms in survivors of unexplained cardiac arrest

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