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Localized Structural Alterations Underlying a Subset of Unexplained Sudden Cardiac Death.

ABSTRACT: BACKGROUND:Sudden cardiac death because of ventricular fibrillation (VF) is commonly unexplained in younger victims. Detailed electrophysiological mapping in such patients has not been reported. METHODS:We evaluated 24 patients (29±13 years) who survived idiopathic VF. First, we used multielectrode body surface recordings to identify the drivers maintaining VF. Then, we analyzed electrograms in the driver regions using endocardial and epicardial catheter mapping during sinus rhythm. Established electrogram criteria were used to identify the presence of structural alterations. RESULTS:VF occurred spontaneously in 3 patients and was induced in 16, whereas VF was noninducible in 5. VF mapping demonstrated reentrant and focal activities (87% versus 13%, respectively) in all. The activities were dominant in one ventricle in 9 patients, whereas they had biventricular distribution in others. During sinus rhythm areas of abnormal electrograms were identified in 15/24 patients (62.5%) revealing localized structural alterations: in the right ventricle in 11, the left ventricle in 1, and both in 3. They covered a limited surface (13±6 cm2) representing 5±3% of the total surface and were recorded predominantly on the epicardium. Seventy-six percent of these areas were colocated with VF drivers (P<0.001). In the 9 patients without structural alteration, we observed a high incidence of Purkinje triggers (7/9 versus 4/15, P=0.033). Catheter ablation resulted in arrhythmia-free outcome in 15/18 patients at 17±11 months follow-up. CONCLUSIONS:This study shows that localized structural alterations underlie a significant subset of previously unexplained sudden cardiac death. In the other subset, Purkinje electrical pathology seems as a dominant mechanism.

SUBMITTER: Haissaguerre M

PROVIDER: S-EPMC7661047 | biostudies-literature | 2018 Jul

REPOSITORIES: biostudies-literature

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Localized Structural Alterations Underlying a Subset of Unexplained Sudden Cardiac Death.

Haïssaguerre Michel M Hocini Mélèze M Cheniti Ghassen G Duchateau Josselin J Sacher Frédéric F Puyo Stéphane S Cochet Hubert H Takigawa Masateru M Denis Arnaud A Martin Ruairidh R Derval Nicolas N Bordachar Pierre P Ritter Philippe P Ploux Sylvain S Pambrun Thomas T Klotz Nicolas N Massoullié Gregoire G Pillois Xavier X Dallet Corentin C Schott Jean-Jacques JJ Scouarnec Solena S Ackerman Michael J MJ Tester David D Piot Olivier O Pasquié Jean-Luc JL Leclerc Christophe C Hermida Jean-Sylvain JS Gandjbakhch Estelle E Maury Philippe P Labrousse Louis L Coronel Ruben R Jais Pierre P Benoist David D Vigmond Edward E Potse Mark M Walton Richard R Nademanee Koonlawee K Bernus Olivier O Dubois Remi R

Circulation. Arrhythmia and electrophysiology 20180701 7

<h4>Background</h4>Sudden cardiac death because of ventricular fibrillation (VF) is commonly unexplained in younger victims. Detailed electrophysiological mapping in such patients has not been reported.<h4>Methods</h4>We evaluated 24 patients (29±13 years) who survived idiopathic VF. First, we used multielectrode body surface recordings to identify the drivers maintaining VF. Then, we analyzed electrograms in the driver regions using endocardial and epicardial catheter mapping during sinus rhyth ...[more]

PMID: 30002064

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Project description:ImportanceUnexplained sudden cardiac death (SCD) describes SCD with no cause identified. Genetic testing helps to diagnose inherited cardiac diseases in unexplained SCD; however, the associations between pathogenic or likely pathogenic (P/LP) variants of inherited cardiomyopathies (CMs) and arrhythmia syndromes and the risk of unexplained SCD in both White and African American adults living the United States has never been systematically examined.ObjectiveTo investigate cases of unexplained SCD to determine the frequency of P/LP genetic variants of inherited CMs and arrhythmia syndromes.Design, setting, and participantsThis genetic association study included 683 African American and White adults who died of unexplained SCD and were included in an autopsy registry. Overall, 413 individuals had DNA of acceptable quality for genetic sequencing. Data were collected from January 1995 to December 2015. A total of 30 CM genes and 38 arrhythmia genes were sequenced, and variants in these genes, curated as P/LP, were examined to study their frequency. Data analysis was performed from June 2018 to March 2021.Main outcomes and measuresThe frequency of P/LP variants for CM or arrhythmia in individuals with unexplained SCD.ResultsThe median (interquartile range) age at death of the 413 included individuals was 41 (29-48) years, 259 (62.7%) were men, and 208 (50.4%) were African American adults. A total of 76 patients (18.4%) with unexplained SCD carried variants considered P/LP for CM and arrhythmia genes. In total, 52 patients (12.6%) had 49 P/LP variants for CM, 22 (5.3%) carried 23 P/LP variants for arrhythmia, and 2 (0.5%) had P/LP variants for both CM and arrhythmia. Overall, 41 P/LP variants for hypertrophic CM were found in 45 patients (10.9%), 9 P/LP variants for dilated CM were found in 11 patients (2.7%), and 10 P/LP variants for long QT syndrome were found in 11 patients (2.7%). No significant difference was found in clinical and heart characteristics between individuals with or without P/LP variants. African American and White patients were equally likely to harbor P/LP variants.Conclusions and relevanceIn this large genetic association study of community cases of unexplained SCD, nearly 20% of patients carried P/LP variants, suggesting that genetics may contribute to a significant number of cases of unexplained SCD. Our findings regarding both the association of unexplained SCD with CM genes and race-specific genetic variants suggest new avenues of study for this poorly understood entity.

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Localized Structural Alterations Underlying a Subset of Unexplained Sudden Cardiac Death.

Publications

Localized Structural Alterations Underlying a Subset of Unexplained Sudden Cardiac Death.

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