inherited cardiac conditions Flashcards
what does cardiomyopathy refer to?
primary heart muscle disease
all cardiomyopathies carry an arrhythmic risk
what is hypertrophic cardiomyopathy (HCM) caused by?
sarcomeric protein gene mutations
what may HCM cause?
angina, dyspnoea, palpitations, dizzy spells or syncope
what may be a feature of HCM?
Left ventricular outflow tract (LVOT) obstruction
what causes dilated cardiomyopathy (DCM)?
cytoskeletal gene mutations
What do people with DCM usually present with?
with heart failure symptoms
what does DCM cause?
LV/RV or 4 chamber dilatation and dysfunction
what causes Arrhythmia cardiomyopathy ( ARVC/ALVC) ?
by desmosome gene mutations
what does Arrhythmia cardiomyopathy cause?
May be a little structural change or may diffusely involve RV and LV
what causes channelopathy?
Inherited arrhythmia caused by ion channel protein gene mutations
what channels does channelopathy usually effect?
Usually relate to potassium, sodium or calcium channels
what do channelopathies include?
include long QT, short QT, and Brugada (abnormal electrical activity of the heart)
what may someone with channelopathy present with?
-a structurally normal heart
-with recurrent syncope
what do patients with channelopathy need to beware of?
Be aware of QT prolonging drugs – they can kill people with long QT syndrome
what inherited conditions are young people who die in cardiac death most likely to have?
-Most likely a cardiomyopathy or ion Channelopathy
-Sudden arrhythmic death syndrome (SADS) usually refers to normal heart/ arrhythmia
