| Autor: |
Nikoo, Mohammad Hossein, Hatamnejad, Mohammad Reza, Emkanjoo, Zahra, Arjangzadeh, Alireza, Moadab, Mehdi Motahari, Bazrafshan, Mehdi, drissi, Hamed Bazrafshan |
| Předmět: |
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| Zdroj: |
ARYA Atherosclerosis; Mar/Apr2024, Vol. 20 Issue 2, p1-7, 7p |
| Abstrakt: |
BACKGROUND: A structural heart disease or functional electrical abnormalities can cause an electrical storm. Case presentation: We present a young boy with an electrical storm who had no cardiac risk factors and a positive family history of sudden cardiac death. The stepwise diagnostic approach was ineffective in determining previously known causes as the origin of the electrical storm. However, whole-exome sequencing (with Next Generation Illumina Sequencing) revealed a mutation in the GJB2 (NM_004004:exon2:c.G71A:p.W24X) gene. CONCLUSION: A mutation in the GJB2 gene, which forms the connexin 26 protein, a crucial component of the myocytes’ intercalated disc of gap junction complex between the myocytes, results in an abnormal electrical cell-by-cell conductance, and, eventually, ventricular storm. General anesthesia was used to control the storm, and intracardiac pacing was fruitful in ceasing the subsequent VT storms. [ABSTRACT FROM AUTHOR] |
| Databáze: |
Complementary Index |
| Externí odkaz: |
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