Diagnostic Dilemmas: Overlapping Features of Brugada Syndrome and Arrhythmogenic Right Ventricular Cardiomyopathy

Hoogendijk, Mark G.

doi:10.3389/fphys.2012.00144

Cited by 22 publications

(15 citation statements)

References 95 publications

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“…Of note, there are several rhythm abnormalities reported in recent years such as Brugada syndrome or arrhythmogenic right ventricular cardiomyopathy, which have RBBB‐like morphology that portend adverse outcomes. It is possible that such an underlying rhythm abnormality, without overt disease manifestation or unmasked electrocardiographic abnormality with prominent ST segment changes, may be responsible for the higher mortality in patients who presented with RBBB prior to cooling.…”

Section: Discussionmentioning

confidence: 99%

Therapeutic Hypothermia‐Induced Electrocardiographic Changes and Relations to In‐hospital Mortality

Lam

Dhingra

Conley

et al. 2013

Clinical Cardiology

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Background: Therapeutic hypothermia improves survival for selected patients who remain comatose after cardiac arrest. Hypothermia triggers changes in electrocardiographic (ECG) parameters; however, the association of these changes to in-hospital mortality remains unclear. Hypothesis: QT interval changes induced by therapeutic hypothermia are not associated with in-hospital mortality. Methods:We retrospectively compared precooling ECG parameters to ECG parameters during hypothermia on all consecutive patients with available information who received hypothermia at our academic medical center between December 2006 and July 2012 (N = 101; 24% women). Paired 2-sample t test was used to compare precooling vs cooling ECG parameters. In-hospital mortality related to ECG parameter changes was compared using the Pearson χ 2 test. Results: Therapeutic hypothermia resulted in increases in PR and QTc intervals and decreases in heart rate and QRS intervals (P for all <0.02). During hospitalization, 45 of the 101 patients died. Survivors vs nonsurvivors did not differ in heart rate change (P = 0.74), PR change (P = 0.57), QRS change (P = 0.09), or QTc change (P = 0.67). Comparing patients who had reduced QTc intervals with hypothermia to those who had prolonged QTc with hypothermia, 14 out of 30 died in the former group, whereas 31 out of 71 died in the latter group (46.7% vs 43.7%, odds ratio [OR]: 1.13, 95% CI: 0.48-2.66). Patients presenting with right bundle branch block (RBBB) had a higher risk of in-hospital death compared to those without RBBB (72.2% vs 38.6%, OR: 4.14, 95% CI: 1.35-12.73). Conclusions: Therapeutic hypothermia prolonged QTc interval with no association to in-hospital mortality. Presence of RBBB on initial presentation was related to increased mortality.

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Section: Discussionmentioning

confidence: 99%

Therapeutic Hypothermia‐Induced Electrocardiographic Changes and Relations to In‐hospital Mortality

Lam

Dhingra

Conley

et al. 2013

Clinical Cardiology

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“…Spontaneous fluctuations between diagnostic and nondiagnostic ECG abnormalities have been described in inherited ion channel disease, such as Brugada syndrome . ARVC and Brugada syndrome could be overlapped and conduction abnormalities, such as TAD of QRS ≥55 ms and LP, appear to be common in Brugada syndrome …”

Section: Discussionmentioning

confidence: 99%

Changes in Follow‐Up ECG and Signal‐Averaged ECG in Patients with Arrhythmogenic Right Ventricular Cardiomyopathy

Bae

Kim

Jang

et al. 2013

Pacing Clinical Electrophis

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“…BrS is an inherited arrhythmogenic disorder that exhibits ECG ST-segment elevation with a negative T-wave in the right precordial leads (V 1 –V 3 ), predisposing to VF and SCD in otherwise healthy individuals 52. BrS is recognized as an important cause of SCD in young men, especially in Southeast Asia 53.…”

Section: Catheter Ablation Of the Right Ventricular Outflow Tract Maymentioning

confidence: 99%

“…Other mutations are located in CACNA1C , CACNB2b 58 and CACNA2D1 ,59 which encode α1-, β 2b -, and α2δ1-subunits of Ca v 1.2, respectively, with resultant reduction of the L-type calcium current ( I Ca,L ) and in KCNE3 , encoding MiRP2, α-, and β-subunits of several potassium channels,60 and in KCNJ8 , encoding the ATP-sensitive potassium channel 61. Overall, mutations in SCN5A account for approximately 20% and those in other genes 10%, leaving no definitive genetic defects in 70% of BrS patients 52. Although the heart appears grossly normal, right ventricular structural abnormalities, such as fibrofatty degenerative changes, lymphocytic myocarditis, and atypical cardiomyopathic alterations in the ventricular outflow tract (RVOT) are not infrequently observed.…”

Section: Catheter Ablation Of the Right Ventricular Outflow Tract Maymentioning

confidence: 99%

“…There has been much debate about whether it is a depolarization disorder (ie, conduction delay or failure) or repolarization disorder (ie, transmural repolarization gradient through the right ventricular wall) 52. More significantly, the theory of loss of function of Na v 1.5 or other ion channels as the basis has been questioned.…”

Section: Catheter Ablation Of the Right Ventricular Outflow Tract Maymentioning

confidence: 99%

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Gene mutations in cardiac arrhythmias: a review of recent evidence in ion channelopathies

Hsiao

Tien²,

Lo³

et al. 2013

TACG

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Over the past 15 years, molecular genetic studies have linked gene mutations to many inherited arrhythmogenic disorders, in particular, “ion channelopathies”, in which mutations in genes encode functional units of ion channels and/or their transporter-associated proteins in patients without primary cardiac structural abnormalities. These disorders are exemplified by congenital long QT syndrome (LQTS), short QT syndrome, Brugada syndrome (BrS) and catecholaminergic polymorphic ventricular tachycardia (CPVT). Functional and pathophysiological studies have led to better understanding of the clinical spectrum, ion channel structures and cellular electrophysiology involving dynamics of intracellular calcium cycling in many subtypes of these disorders and more importantly, development of potentially more effective pharmacological agents and even curative gene therapy. In this review, we have summarized (1) the significance of unveiling mutations in genes encoding transporter-associated proteins as the cause of congenital LQTS, (2) the technique of catheter ablation applied at the right ventricular outflow tract may be curative for severely symptomatic BrS, (3) mutations with channel function modulated by protein Kinase A-dependent phosphorylation can be the culprit of CPVT mimicry in Andersen-Tawil syndrome (LQT7), (4) ablation of the ion channel anchoring protein may prevent arrhythmogenesis in Timothy syndrome (LQT8), (5) altered intracellular Ca2+ cycling can be the basis of effective targeted pharmacotherapy in CPVT, and (6) the technology of induced pluripotent stem cells is a promising diagnostic and research tool as it has become a new paradigm for pathophysiological study of patient- and disease-specific cells aimed at screening new drugs and eventual clinical application of gene therapy. Lastly, we have discussed (7) genotype-phenotype correlation in relation to risk stratification of patients with congenital LQTS in clinical practice.

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Diagnostic Dilemmas: Overlapping Features of Brugada Syndrome and Arrhythmogenic Right Ventricular Cardiomyopathy

Cited by 22 publications

References 95 publications

Therapeutic Hypothermia‐Induced Electrocardiographic Changes and Relations to In‐hospital Mortality

Therapeutic Hypothermia‐Induced Electrocardiographic Changes and Relations to In‐hospital Mortality

Changes in Follow‐Up ECG and Signal‐Averaged ECG in Patients with Arrhythmogenic Right Ventricular Cardiomyopathy

Gene mutations in cardiac arrhythmias: a review of recent evidence in ion channelopathies

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