Hey guys!
We'll be talking about congenital long QT syndrome today!
It is caused by inherited ion channel defects in the heart. Symptoms include syncope and sudden death from torsades de pointes. Includes -
Romano - Ward syndrome:
- Autosomal dominant
- Pure cardiac phenotype
- Autosomal dominant
- Pure cardiac phenotype
Jervell and Lange - Nielsen syndrome:
- Autosomal recessive
- Sensorineural deafness
- Autosomal recessive
- Sensorineural deafness
Here are a few mnemonics on the same (:
Romano - Ward syndrome mnemonic:
Romantic Romano: Romance has to do something with the heart.
War Ward: The heart is at war and that's why it has a long QT <3
The D of WarD can remind you of Dominant.
Romantic Romano: Romance has to do something with the heart.
War Ward: The heart is at war and that's why it has a long QT <3
The D of WarD can remind you of Dominant.
Jervell and Lange - Nielsen syndrome mnemonic:
JeRvell has an R for recessive.
Lange Long: Lange has a "long" QT.
NielSen No sound: Nielsen hears no sound (Sensorineural deafness).
JeRvell has an R for recessive.
Lange Long: Lange has a "long" QT.
NielSen No sound: Nielsen hears no sound (Sensorineural deafness).
Gold standard therapy is beta blockers. They reduce rate of sudden death. ICD is definitive treatment.
That's all!
Nothing cutie about QT. (T_T)
-IkaN
Nothing cutie about QT. (T_T)
-IkaN
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