B-PO04-153 SUBTLE REPOLARIZATION ABNORMALITIES IN IDIOPATHIC VENTRICULAR FIBRILLATION ARE UNCOVERED BY NONINVASIVE ELECTROCARDIOGRAPHIC IMAGING, BUT NOT THE 12-LEAD ELECTROCARDIOGRAM

Abstract Funding Acknowledgements Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Ministry of Health of the Czech Republic Introduction The complex diagnostic work up in SCA survivors often does not yield a concrete cardiological diagnosis. Moreover, there is conflicting evidence whether genetic testing could support or guide clinical diagnostics. Purpose To assess the molecular architecture of idiopathic ventricular fibrillation in cases without apparent evidence of specific structural or arrhythmic cardiac disease at initial diagnostic work up in a representative Czech cohort. Patients and Methods Between 2013 - 2020 we have ascertained 100 SCA survivors (54 M / 46 F; age range at cardiac arrest 5-69 years). Genetic counselling was followed by massively parallel DNA sequencing using custom-made panels comprising 100 cardiac conditions-related genes. Subsequently, thorough cardiological screening examinations in first degree relatives were carried out. Presence of pathogenic variants was validated by Sanger DNA sequencing and through family segregation analyses. Results Highly likely or certain molecular aetiology (i.e. based on the presence of Class 4 or 5 variants) was disclosed in 20/100 (20%) in PKP2 (3x), SCN5A (4x), RYR2 (3x), TTN (2x), PLN, FLNC, PRKAG2, KCNH2, KCNQ1, SLC4A, TNNT2, and DSP. Interestingly, the KCNE1 p.Asp85Asn (LQT 5 lite) variant, was detected in further 3/100 cases, representing a recognized risk factor for ventricular arrhythmias. Conclusions Genetic testing facilitates stratification of the cause of arrhythmia in a substantial portion of SCA survivors. The utility of positive outcomes of genetic testing was substantiated in 10/20 gene positive patients, where the genetic stratification led to diagnosis of concealed arrhythmogenic cardiomyopathy, whose extent of morphological changes was under the diagnostic sensibility of imaging modalities or ECG. Our results enable individualized care in SCA survivors and assure targeted preventive approaches in their relatives.

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Abstract 15210: Do Idiopathic Ventricular Fibrillation and Brugada Syndrome Patients Have Favor Outcomes in the Targeted Temperature Management Era?

Circulation ◽

10.1161/circ.142.suppl_3.15210 ◽

2020 ◽

Vol 142 (Suppl_3) ◽

Author(s):

Eisuke Kagawa ◽

Masaya Kato ◽

Noboru Oda ◽

Eiji Kunita ◽

Michiaki NAGAI ◽

...

Keyword(s):

Cardiac Arrest ◽

Survival Rate ◽

Ventricular Fibrillation ◽

Brugada Syndrome ◽

Cardiac Dysfunction ◽

Targeted Temperature Management ◽

Temperature Management ◽

Circulatory Support ◽

Idiopathic Ventricular Fibrillation ◽

Coronary Syndrome

Introduction: Idiopathic ventricular fibrillation (IVF) including Brugada syndrome (BS) is one of causes of cardiac arrest without prior overt cardiac dysfunction. Hypothesis: We assessed the hypothesis that patents of IVF had favor outcomes than those of non-IVF after cardiac arrest treated with targeted temperature management (TTM). Methods: Patients who were treated with TTM after cardiac arrest between 2000 and 2019 were enrolled in the study. Patients were divided into 2 groups according to whether the patients were diagnosed as IVF or not. The patients treated with TTM were routinely performed coronary angiography. Results: Among the study patients (N = 306), 35 (11%) patients were IVF and 7 were BS. The patients of the IVF group were significantly younger (median 53 y vs. 64 y) than those of the non-IVF group. The prevalence of initial rhythm was shockable (69% vs. 47%, P = 0.02) was significantly higher in the patients of the IVF group than those of the non-IVF group. Among the patients in the non-IVF group, 114 patients (42%) were diagnosed as acute coronary syndrome and 93 patients (35%) were treated with coronary revascularization. The prevalence of male sex (77% vs 74%, P = 0.70) and witnessed to arrest (80% vs. 81%, P = 0.87), and low-flow time (29 min vs. 38 min [20 - 43 min vs. 21 - 52 min, P = 0.15]) were similar between the 2 groups. The prevalence of performing extracorporeal resuscitation (9% s 43%, P < 0.001) were lower in the patients of the IVF group. The 8-y survival rate were shown in the figure. All of the BS patients were witnessed arrest and were discharged without severe neurological deficit. The IVF as the cause of arrest was independently associated with 8-y survival. Conclusions: The patients of IVF had favor outcomes than those of non-VF. One of causes may be the lower prevalence of requiring extracorporeal circulatory support due to less cardiac dysfunction. The patients of BS had the tendency toward higher survival rate than those of non-BS IVF patients.

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Abstract 2769: Bradycardia-Dependent Augmentation of J-wave Elevation in Patients with Idiopathic Ventricular Fibrillation

Circulation ◽

10.1161/circ.118.suppl_18.s_774-d ◽

2008 ◽

Vol 118 (suppl_18) ◽

Author(s):

Koichi Mizumaki ◽

Akira Fujiki ◽

Masao Sakabe ◽

Tamotsu Sakamoto ◽

Yosuke Nakatani ◽

...

Keyword(s):

Ventricular Fibrillation ◽

Linear Regression ◽

Wave Amplitude ◽

Idiopathic Ventricular Fibrillation ◽

Cardioverter Defibrillator ◽

Rate Dependent ◽

Wave Elevation ◽

Male Patients ◽

And Control ◽

J Wave

Although J-wave elevation (JWE) in the inferior or lateral leads is characteristic of subtype of idiopathic ventricular fibrillation (IVF), difference between malignant and benign JWE remains to be elucidated. So, we assessed the hypothesis that the rate dependent changes in JWE could be different between patients with IVF and control subjects. Six male patients with IVF and 18 age matched male controls with JWE (>2 mm) in the lateral leads were studied. CM-5 lead digital Holter ECG was recorded and J wave amplitude was automatically measured. J/RR relationships were evaluated by means of J/RR linear regression lines for 24 hours, and according to four 6-hour periods. From J-RR linear regression lines, J-RR slope (mm/sec), J wave amplitude (mm) at RR intervals of both 0.6 sec and 1.2 sec (J (0.6) and J (1.2) ) were determined. In all 6 patients with IVF, the time of spontaneous episodes of VF were investigated from the implantable cardioverter defibrillator (ICD) stored data. For 24 hours, J-RR slope was greater in IVF patients than in controls (3.5±0.7 vs 2.4±0.8, p<0.01). (J (0.6) ) wasn’t different between 2 groups; however, J (1.2) was greater in IVF patients than in controls (2.8±0.9 vs 2.0±0.6, p<0.05). In IVF patients, ST/RR slope was highest during 18:00–24:00; however, these circadian changes were blunted in controls. J(1.2) was greater during 18:00 – 6:00 in IVF patients than in controls. Seven (59%) of total 9 episodes of spontaneous VF of IVF patients occurred during 18:00 – 6:00. In patients with subtype of IVF, JWE augmented during bradycardia especially at night. This could relate to nighttime occurrence of IVF.

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