Cardiac pathologic findings reveal a high rate of sudden cardiac death of undetermined etiology in younger women

Chugh, Sumeet S.; Chung, Kiyon; Zheng, Zhi‐Jie; John, Benjamin T.; Titus, Jack L.

doi:10.1016/s0002-8703(03)00323-5

Cited by 42 publications

(26 citation statements)

References 25 publications

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“…30 Among sudden death autopsy series, ventricular interstitial fibrosis in the absence of other structural abnormalities is a consistent albeit uncommon finding, affecting an estimated 1 to 3% of cases. [31][32][33] Murine models show a clear relationship between inducibility of ventricular arrhythmias and degree of interstitial fibrosis. 34,35 Increased fibrosis alters tissue electrophysiology through partial decoupling of muscle fibres, conduction slowing, zigzag courses of conduction propagation, localized source-sink mismatches, and conduction blocks.…”

Section: Discussionmentioning

confidence: 99%

Diffuse Ventricular Fibrosis Is a Late Outcome of Tachycardia-Mediated Cardiomyopathy After Successful Ablation

Ling

Kalman

Ellims

et al. 2013

Circ: Arrhythmia and Electrophysiology

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Background-Successful arrhythmia ablation normalizes ejection fraction (EF) in tachycardia-mediated cardiomyopathy, but recurrent heart failure and late sudden death have been reported. The aim of this study was to characterize the left ventricle (LV) of tachycardia-mediated cardiomyopathy patients long after definitive arrhythmia cure. Methods and Results-Thirty-three patients with a history of successfully ablated incessant focal atrial tachycardia 64±36 months prior, and 20 healthy controls were recruited. At ablation, 18 patients had EF<50% (AT-low EF) that recovered within 3 months from 37±12 to 56±4% (P<0.001), whereas 15 patients had EF>55% (AT-normal EF). No subjects had EF of 50% to 55%. Subjects underwent echocardiography with speckle tracking and contrast-enhanced cardiac MRI with ventricular T 1 mapping as an index of diffuse fibrosis. Contrast-enhanced cardiac MRI was performed using a clinical 1.5-T scanner and 0.2 mmol/kg gadolinium-diethylene triamine penta-acetic acid for contrast.

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Section: Discussionmentioning

confidence: 99%

Diffuse Ventricular Fibrosis Is a Late Outcome of Tachycardia-Mediated Cardiomyopathy After Successful Ablation

Ling

Kalman

Ellims

et al. 2013

Circ: Arrhythmia and Electrophysiology

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“…It is possible that some proportion of these patients had heritable monogenic disorders such as the long QT syndrome, Brugada syndrome and catecholaminergic polymorphic ventricular tachycardia (6,(20)(21)(22)(23)(24). Defects in the SCN5A gene, also causing long QT syndrome 3 and the Brugada syndrome, have been reported in some cases of the sudden infant death syndrome (25,26). A retrospective postmortem molecular analysis of sudden cardiac death with structurally normal heart was conducted from a sudden cardiac death autopsy series based at the Edwards Registry of Cardiovascular Disease in St Paul, Minnesota.…”

Section: Spectrum Of Etiologies Of Sudden Cardiac Deathmentioning

confidence: 99%

“…However, given the lower overall sudden cardiac death rates in females, SUD cases are likely to comprise a higher proportion of sudden cardiac death cases in women compared to men, particularly among younger adults. From the Edwards Registry 270-patient autopsy series of sudden cardiac death, the age-group 35-44 years contained 72 patients, of which 27 were women (32% of total women) and 45 men (24% of total men) (25). Detailed cardiac pathologic examinations revealed significant gender-related differences in prevalence of SUD.…”

Section: Spectrum Of Etiologies Of Sudden Cardiac Deathmentioning

confidence: 99%

Epidemiology of Sudden Cardiac Death: Clinical and Research Implications

Chugh

Reinier

Teodorescu

et al. 2008

Progress in Cardiovascular Diseases

597

458

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The current annual incidence of sudden cardiac death in the US is likely to be in the range of 180-250,000 per year. Coinciding with the decreased mortality from coronary artery disease, there is evidence pointing toward a significant decrease in rates of sudden cardiac death in the US during the second half of the twentieth century. However the alarming rise in prevalence of obesity and diabetes in the first decade of the new millennium both in the US and worldwide, would indicate that this favorable trend is unlikely to persist. We are likely to witness a resurgence of coronary artery disease and heart failure, as a result of which sudden cardiac death will have to be confronted as a shared and indiscriminate, worldwide public health problem. There is also increasing recognition of the fact that discovery of meaningful and relevant risk stratification and prevention methodologies will require careful prospective community-wide analyses, with access to large archives of DNA, serum and tissue that link with well-phenotyped databases. The purpose of this review is to summarize current knowledge of sudden cardiac death epidemiology. We will discuss the significance and strengths of community-wide evaluations of sudden cardiac death, summarize recent observations from such studies, and finally highlight specific potential predictors that warrant further evaluation as determinants of sudden cardiac death in the general population.

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“…Such familial proarrhythmic risk may differ among women, in whom fewer SCDs are associated with coronary disease, 7 and structurally normal hearts are more commonly encountered at autopsy. 8 …”

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confidence: 99%

Cardiac Sodium Channel Gene Variants and Sudden Cardiac Death in Women

et al. 2008

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Background-Several cardiac ion channel genes have been implicated in monogenic traits with a high risk of sudden cardiac death (SCD). Mutations or rare variants in these genes have been proposed as potential contributors to more common forms of SCD, but this hypothesis has not been assessed systematically. Methods and Results-We directly sequenced the entire coding region and splice junctions of 5 cardiac ion channel genes, SCN5A, KCNQ1, KCNH2, KCNE1, and KCNE2, in 113 SCD cases from 2 large prospective cohorts of women (Nurses' Health Study) and men (Health Professional Follow-Up Study). Controls from the same population were then screened for the presence of mutations or rare variants identified in cases, and sequence variants without prior functional data were expressed in Xenopus oocytes to assess their biophysical consequences. No mutations or rare variants were identified in any of the 53 subjects who were men. In contrast, in 6 of 60 women (10%), we identified 5 rare missense variants in SCN5A that either had been associated previously with long-QT syndrome (A572D and G615E), had been reported to alter sodium channel function (F2004L), or had not been reported previously in control populations (A572F and W1205C). Of the 4 variants without prior functional data, 3 variants were located in the I-II linker (A572D, A572F, and G615E), and all resulted in significantly shorter recovery times from inactivation. When compared with 733 control samples from the same population, the overall frequency of these rare variants in SCN5A was significantly higher in the SCD cases (6/60, 10.0%) than in controls (12/733, 1.6%; Pϭ0.001). Conclusion-Functionally significant mutations and rare variants in SCN5A may contribute to SCD risk among women.(Circulation. 2008;117:16-23.)

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Cardiac pathologic findings reveal a high rate of sudden cardiac death of undetermined etiology in younger women

Cited by 42 publications

References 25 publications

Diffuse Ventricular Fibrosis Is a Late Outcome of Tachycardia-Mediated Cardiomyopathy After Successful Ablation

Diffuse Ventricular Fibrosis Is a Late Outcome of Tachycardia-Mediated Cardiomyopathy After Successful Ablation

Epidemiology of Sudden Cardiac Death: Clinical and Research Implications

Cardiac Sodium Channel Gene Variants and Sudden Cardiac Death in Women

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