Sudden Cardiac Arrest during Anesthesia in a 30-Month-Old Boy with Syndactyly: A Case of Genetically Proven Timothy Syndrome
Journal of Korean Medical Science
; : 788-791, 2013.
Article
ي En
| WPRIM
| ID: wpr-80565
المكتبة المسؤولة:
WPRO
ABSTRACT
Timothy syndrome, long QT syndrome type 8, is highly malignant with ventricular tachyarrhythmia. A 30-month-old boy had sudden cardiac arrest during anesthesia induction before plastic surgery for bilateral cutaneous syndactyly. After successful resuscitation, prolonged QT interval (QTc, 0.58-0.60 sec) and T-wave alternans were found in his electrocardiogram. Starting beta-blocker to prevent further tachycardia and collapse event, then there were no more arrhythmic events. The genes KCNQ1, KCNH2, KCNE1 and 2, and SCN5A were negative for long QT syndrome. The mutation p.Gly406Arg was confirmed in CACNA1C, which maintains L-type calcium channel depolarization in the heart and other systems.
Key words
النص الكامل:
1
الفهرس:
WPRIM
الموضوع الرئيسي:
Surgery, Plastic
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Long QT Syndrome
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Magnetic Resonance Imaging
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Death, Sudden, Cardiac
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Sequence Analysis, DNA
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Syndactyly
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Calcium Channels, L-Type
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Polymorphism, Single Nucleotide
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Electroencephalography
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Anesthesia
المحددات:
Humans
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Infant
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Male
اللغة:
En
مجلة:
Journal of Korean Medical Science
السنة:
2013
نوع:
Article