scholarly journals P440Nonlinear dynamics of cardiac repolarization add unique prognostic value to cardiac MRI of myocardial scar for prediction of appropriate shock and cardiac death in primary prevention ICD recipients

EP Europace ◽  
2018 ◽  
Vol 20 (suppl_1) ◽  
pp. i84-i84
Author(s):  
J DeQuach ◽  
B O'Rourke ◽  
G F Tomaselli ◽  
S R Jones ◽  
K C Wu ◽  
...  
Author(s):  
Igor Klem ◽  
Michael Klein ◽  
Mohammad Khan ◽  
Eric Y. Yang ◽  
Faisal Nabi ◽  
...  

Background: Non-ischemic cardiomyopathy (NICM) is a leading cause of reduced left ventricular ejection fraction (LVEF) and is associated with high mortality risk from progressive heart failure and arrhythmias. Myocardial scar on cardiovascular magnetic resonance imaging (CMR) is increasingly recognized as risk marker for adverse outcomes, however LV dysfunction remains the basis for determining a patient's eligibility for primary prophylaxis implantable cardioverter-defibrillator (ICD). We wanted to investigate the relationship of LVEF and scar to long term mortality and mode of death in a large cohort of patients with NICM. Methods: This study is a prospective, longitudinal outcomes registry of 1020 consecutive patients with NICM who underwent clinical CMR for the assessment of LVEF and scar at three centers. Results: During a median follow-up of 5.2 (IQR 3.8, 6.6) years 277 (27%) patients died. On survival analysis LVEF≤35% and scar were strongly associated with all-cause (log-rank test p=0.002 and p<0.001, respectively) and cardiac death (p=0.001 and p<0.001, respectively). While scar was strongly related to sudden cardiac death (SCD) (p=0.001), there was no significant association between LVEF≤35% and SCD-risk (p=0.57). On multivariable analysis including established clinical factors, LVEF and scar are independent risk-markers of all-cause and cardiac death. The addition of LVEF provided incremental prognostic value albeit insignificant discrimination improvement by C-statistic for all-cause and cardiac death, however no incremental prognostic value for SCD. Conversely, scar extent demonstrated significant incremental prognostic value and discrimination improvement for all three endpoints. On net reclassification analysis, the addition of LVEF resulted in no significant improvement for all-cause death 11.0% (95% CI -6.2-25.9%), cardiac death 9.8% (95% CI -5.7-29.3%), and SCD 7.5% (95% CI -41.2-42.9%). Conversely, the addition of scar extent resulted in significant reclassification improvement of 25.5% (95% CI 11.7-41.0%) for all-cause death, 27.0% (95% CI 11.6-45.2%) for cardiac death, and 40.6% (95% CI 10.5-71.8%) for SCD. Conclusions: Myocardial scar and LVEF are both risk markers for all-cause and cardiac death in patients with NICM. However, while myocardial scar has strong and incremental prognostic value for SCD risk stratification, LVEF has no incremental prognostic value over clinical parameters. Scar assessment should be incorporated into patient selection criteria for primary prevention ICD placement.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
E Borisincova ◽  
P Votypka ◽  
K Rucklova ◽  
A Pilin ◽  
M Kulvajtova ◽  
...  

Abstract Introduction Hereditary cardiomyopathy is associated with an increased risk of ventricular arrhythmia and sudden cardiac death (SCD). Genetic stratification substantiates risk assessment and enables the primary prevention of SCD in relatives at risk. We have analyzed the genetic aetiology of SCD in a representative Czech cohort with post mortem diagnosis of various forms of cardiomyopathy and compared it to living cases with these cardiac disorders. Patients and methods Between 2018 and 2019, altogether 47 victims of SCD with post mortem diagnosis of hypertrophic- (HCM; 18/47), arrhythmogenic- (ACM; 19/47) and dilated cardiomyopathy (DCM; 10/47) were identified. Concurrently, genetic testing was performed in 114 living patients (HCM 54/114, ACM 22/114, DCM 38/114). Genetic counselling and cardiologic examination had been carried out in first-degree relatives in all patients/SCD victims. Massively parallel sequencing (MiSeq platform; Illumina.com) was utilized for a custom-made panel comprising 100 candidate genes (Sophia Genetics, Switzerland). The presence of pathogenic variants was validated by Sanger DNA sequencing and through family segregation analyses. Results The causative detection rate (according to ACMG.net classes 4 or 5) in SCD victims with DCM was 60% (6/10) and in living patients with DCM 47.4% (18/38). Variants in TTN, RBM20, DES and FLNC (mainly truncating variants) prevailed in both groups. The detection rate in ACM was 5% (1/19 in SCN5A gene) in SCD victims and 31.8% (7/22) in living patients. Interestingly, the most prevalent mutated gene PKP2 in living patients was not detected in SCD victims. The detection rate in SCD victims with post mortem diagnosis of HCM was 16% (3/18) and in living patients 35% (19/54). The most prevalent gene was MYBPC3 in both groups, while PRKAG2 was detected in one SCD victim and in one living case who survived cardiac arrest. Conclusion Post-mortem genetic analysis in DCM yields a high detection rate and allows potentially effective primary prevention of SCD in relatives at risk. In contrast, the molecular autopsy of HCM and ACM renders a much lower yield which is below the mutation detection rate in living phenotype positive individuals. The results help to improve the genetic counselling in affected families in Czech Republic. Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): Ministry of Health of the Czech Republic


2008 ◽  
Vol 14 (7) ◽  
pp. S140-S141
Author(s):  
Kenji Ando ◽  
Yoshimitsu Soga ◽  
Masahiko Goya ◽  
Shinichi Shirai ◽  
Shinya Nagayama ◽  
...  

2015 ◽  
Vol 104 (12) ◽  
pp. 1021-1032 ◽  
Author(s):  
Jörg Otto Schwab ◽  
Hendrik Bonnemeier ◽  
Thomas Kleemann ◽  
Johannes Brachmann ◽  
Sven Fischer ◽  
...  

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