Life threatening causes of syncope: Channelopathies and cardiomyopathies

Herman, Adam; Bennett, Matthew T.; Chakrabarti, S.; Krahn, Andrew D.

doi:10.1016/j.autneu.2014.04.003

Cited by 5 publications

(3 citation statements)

References 85 publications

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“…Patients with certain genetic diseases that causes abnormal function of the cardiac ion channels such as Brugada, prolonged QT, short QT or catecholaminergic polymorphic ventricular tachycardia are more susceptible to sudden cardiac death [ 23 ]. During asymptomatic periods, patients with QT changes or catecholaminergic polymorphic ventricular tachycardia will have normal ECGs and no underlying structural heart disease identified on echocardiography, which can make diagnosis difficult.…”

Section: Investigationsmentioning

confidence: 99%

Syncope: a review of emergency department management and disposition

Patel

Quinn

2015

Clin Exp Emerg Med

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Syncope is defined as a transient loss of consciousness due to cerebral hypoperfusion with spontaneous return to baseline function without intervention. It is a common chief complaint of patients presenting to the emergency department. The differential diagnosis for syncope is broad and the management varies significantly depending on the underlying etiology. In the emergency department, determining the cause of a syncopal episode can be difficult. However, a thorough history and certain physical exam findings can assist in evaluating for life-threatening diagnoses. Risk-stratifying patients into low, moderate and high-risk groups can assist in medical decision making and help determine the patient’s disposition. Advancements in ambulatory monitoring have made it possible to obtain prolonged cardiac evaluations of patients in the outpatient setting. This review will focus on the diagnosis and management of the various types of syncope.

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Section: Investigationsmentioning

confidence: 99%

Syncope: a review of emergency department management and disposition

Patel

Quinn

2015

Clin Exp Emerg Med

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“…ARVC (3) CPVT (1) five patients (16%) experienced ATP (median time 1.04 years) and 15 patients (10%) experienced both a shock and ATP. The overall rate of experiencing any appropriate therapy (ATP or shock) in ICD recipients was 0.16 per year (SEM=0.14).…”

Section: Genetic Testing N=4mentioning

confidence: 99%

Etiology of Cardiac Arrest in the Absence of Manifest Structural Heart Disease - Update From the Casper Prospective Cohort

Herman¹,

Bennett²,

Chakrabarti³

et al. 2014

Canadian Journal of Cardiology

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Background: The Cardiac Arrest Survivors with Preserved Ejection Fraction Registry (CASPER) enrolls patients with apparently unexplained cardiac arrest and no evident cardiac disease, and aims to identify the underlying conditions responsible for the cardiac arrest through systematic clinical testing. A combination of exercise testing, drug provocation, electrophysiological testing, advanced cardiac imaging, and targeted genetic testing may unmask the cause of cardiac arrest when a cause is not apparent. Methods and Results: The first 200 survivors (48.6 ± 14.7 years, 41% female) of unexplained cardiac arrest from CASPER, from 14 centers across Canada were evaluated to determine the results of investigation and follow-up risk of recurrence. Patients were free of evidence of coronary artery disease, left ventricular dysfunction or evident repolarization syndromes. Advanced testing determined a probable or definite diagnosis in 41% of patients. During a median follow-up of 3.15±2.34 years, 23% of patients had either a shock from their ICD or appropriate anti-tachycardia pacing or both. The annual ICD appropriate intervention rate was 0.16 per year (SEM=0.14), with no clear difference in intervention rate between diagnosed and undiagnosed subjects, or between those diagnosed with a primary electrical versus structural etiology. Conclusions: Obtaining a diagnosis in previously unexplained cardiac arrest patients requires systematic clinical testing and regular follow-up to unmask the cause of cardiac arrest. Nearly half of apparent unexplained cardiac arrest patients ultimately received a diagnosis, allowing for improved treatment and family screening. A substantial proportion of patients received appropriate ICD therapy during follow-up.

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“…Genetic causes are found in various potentially lethal channelopathies and cardiomyopathies including long and short QT syndrome (LQTS and SQTS), Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia (CPVT), hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), arrhythmogenic right ventricular dysplasia/ cardiomyopathy (ARVD/C), Barth syndrome, and left ventricular non-compaction. 1 Knowledge of genetics deepens the understanding of pathophysiology and remarkably changes the diagnosis, treatment, and genetic counselling for recurrence risk and family planning. This group is highly genetically heterogeneous ( Table 1 2 ).…”

Section: Introductionmentioning

confidence: 99%

Genetic basis of channelopathies and cardiomyopathies in Hong Kong Chinese patients: a 10-year regional laboratory experience

Sp²,

et al. 2018

Hong Kong Med J

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We have characterised the genetic heterogeneity in channelopathies and cardiomyopathies among Hong Kong Chinese patients in a 10-year case series. Correct interpretation of genetic findings is difficult and requires expertise and experience. Caution regarding issues of non-penetrance, variable expressivity, phenotype-genotype correlation, susceptibility risk, and digenic inheritance is necessary for genetic counselling and cascade screening.

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Life threatening causes of syncope: Channelopathies and cardiomyopathies

Cited by 5 publications

References 85 publications

Syncope: a review of emergency department management and disposition

Syncope: a review of emergency department management and disposition

Etiology of Cardiac Arrest in the Absence of Manifest Structural Heart Disease - Update From the Casper Prospective Cohort

Genetic basis of channelopathies and cardiomyopathies in Hong Kong Chinese patients: a 10-year regional laboratory experience

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