scholarly journals ACQUIRED BRUGADA SYNDROME PRESENTING AS VENTRICULAR TACHYCARDIA STORM

CHEST Journal ◽  
2007 ◽  
Vol 132 (4) ◽  
pp. 716A ◽  
Author(s):  
Jacob S. Koruth ◽  
Sunil Jagadesh ◽  
Karen S. Rovang ◽  
Aryan N. Mooss ◽  
Tom Hee ◽  
...  
Author(s):  
Javier Pinos ◽  
Tiago Luiz Luz Leiria ◽  
Bernardo Boccalon ◽  
Marcelo Lapa Kruse ◽  
Gustavo Glotz De Lima

2003 ◽  
Vol 67 (1) ◽  
pp. 93-95 ◽  
Author(s):  
Tsutomu Araki ◽  
Tetsuo Konno ◽  
Hideki Itoh ◽  
Hidekazu Ino ◽  
Masami Shimizu

Author(s):  
Gary Tse ◽  
Jiandong Zhou ◽  
Sharen Lee ◽  
Tong Liu ◽  
George Bazoukis ◽  
...  

Background A combination of clinical and electrocardiographic risk factors is used for risk stratification in Brugada syndrome. In this study, we tested the hypothesis that the incorporation of latent variables between variables using nonnegative matrix factorization can improve risk stratification compared with logistic regression. Methods and Results This was a retrospective cohort study of patients presented with Brugada electrocardiographic patterns between 2000 and 2016 from Hong Kong, China. The primary outcome was spontaneous ventricular tachycardia/ventricular fibrillation. The external validation cohort included patients from 3 countries. A total of 149 patients with Brugada syndrome (84% males, median age of presentation 50 [38–61] years) were included. Compared with the nonarrhythmic group (n=117, 79%), the spontaneous ventricular tachycardia/ ventricular fibrillation group (n=32, 21%) were more likely to suffer from syncope (69% versus 37%, P =0.001) and atrial fibrillation (16% versus 4%, P =0.023) as well as displayed longer QTc intervals (424 [399–449] versus 408 [386–425]; P =0.020). No difference in QRS interval was observed (108 [98–114] versus 102 [95–110], P =0.104). Logistic regression found that syncope (odds ratio, 3.79; 95% CI, 1.64–8.74; P =0.002), atrial fibrillation (odds ratio, 4.15; 95% CI, 1.12–15.36; P =0.033), QRS duration (odds ratio, 1.03; 95% CI, 1.002–1.06; P =0.037) and QTc interval (odds ratio, 1.02; 95% CI, 1.01–1.03; P =0.009) were significant predictors of spontaneous ventricular tachycardia/ventricular fibrillation. Increasing the number of latent variables of these electrocardiographic indices incorporated from n=0 (logistic regression) to n=6 by nonnegative matrix factorization improved the area under the curve of the receiving operating characteristics curve from 0.71 to 0.80. The model improves area under the curve of external validation cohort (n=227) from 0.64 to 0.71. Conclusions Nonnegative matrix factorization improves the predictive performance of arrhythmic outcomes by extracting latent features between different variables.


2004 ◽  
pp. 339-345
Author(s):  
G. Buja ◽  
L. Leoni ◽  
D. Corrado ◽  
C. Basso ◽  
B. Bauce ◽  
...  

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
H Ogita ◽  
D.P Zankov ◽  
A Shimizu

Abstract   Brugada syndrome (BrS) is diagnosed by a typical electrocardiography (ECG) with ST-segment elevation in precordial leads and tends to induce sudden cardiac death (SCD) due to ventricular tachycardia/fibrillation. About 20% of SCDs in non-structural cardiac diseases are considered to be caused by BrS. In patients with BrS, loss of function mutations in the Na+ channel is often observed, but the causative gene mutation is not detected for about 70% of BrS patients. Here, we investigated a family with clinically diagnosed BrS, in which no known gene mutations related to BrS had not been found, by whole exome sequencing. Novel heterozygous variant (c. 1616G>A, p. R539Q) in transmembrane protein 168 (TMEM168) was identified only in symptomatic family members. Similar to endogenous TMEM168, both wild-type and mutant TMEM168 localized at the nuclear membrane. Na+ current density in whole-cell patch-clamp recordings was significantly reduced in HL-1 cardiomyocytes transfected with TMEM168 R539Q mutant, compared with those with wild-type TMEM168. Next, heterozygous Tmem168 1616G>A knock-in mice were generated by the CRISPR/Cas9 genome editing technology. Although the knock-in mice had no abnormalities in ECG at the physiological state, the treatment with ajmaline caused various arrhythmias including ventricular tachycardia/fibrillation in the knock-in mice, but not in wild-type mice. Na+ current density and the parameters of action potentials were remarkably impaired in the cardiomyocytes of the knock-in mice. Optical mapping analysis in the whole heart showed the reduced left ventricular conduction velocity in the knock-in mice. The expression of Nav1.5, an α-subunit of the cardiac Na+ channel, was significantly decreased in the mutant TMEM168-transfected HL-1 cells and the knock-in hearts. We found that the decrease was caused by the enhanced ubiquitination of Nav1.5, which was mediated by increased binding of Nedd4–2 E3 ubiquitin ligase to Nav1.5 in the knock-in hearts. Co-immunoprecipitation experiments demonstrated that overactivity of Nedd4–2 is a result of Tmem168 mutant-mediated sequestration of a chaperon protein αB-crystallin, a Nav1.5-binding molecule that interferes with the interaction of Nedd4–2 with Nav1.5. These findings reveal the molecular mechanism of TMEM168 R539Q mutation-induced fatal ventricular arrhythmias in BrS. Funding Acknowledgement Type of funding source: Public Institution(s). Main funding source(s): JSPS Grants-in-aid for Scientific Research


2020 ◽  
Vol 59 (10) ◽  
pp. 1287-1290
Author(s):  
Shota Kakehashi ◽  
Tsukasa Kamakura ◽  
Takeshi Aiba ◽  
Kengo Kusano

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