inherited cardiac conditions Flashcards
person presents with an ejection systolic murmur that increases with valvaslva manoeuvre and decreases on squatting
what is the diagnosis
Hypertrophic Cardiomyopathy
why are arrhythmias (particularly re-rentry) common in HOCM
scarring as not enough blood supply to provide for enlarged muscle thickness
scarring leads to increased automaticity of surrounding tissue
when does HOCM become apparent
late teens/early 20s
true / false
of the 4 main cardiomyopathies HOCM has the smallest genetic link
false
HOCM has the largest genetic link
what is seen in echo of HOCM
asymmetric hypertrophy
what is dilated cardiomyopathy
heart cavity dilates and thinning of ventricular walls
- early form is left ventricular enlargement
what is arrhythmogenic right ventricular cardiomyopathy
heart tissue progressively replaced by fibrous and fatty tissue
what is restrictive cardiomyopathy
walls of ventricles become stiff so resist normal filling with blood - reduced EDV
what kind of inheritance is HOCM
autosomal dominant
what kind of inheritance is DCM
autosomal dominant but can be recessive
what are the 2 most common complications of cardiomyopathies
arrhythmia
heart failure
asymptomatic
or
dyspnoea, angina, syncope, jerky pulse, large a waves, double apex beat
HOCM
what always occurs just before a TdP rhythm
pause then an ectopic beat
what is Long QT syndrome
inherited condition where patients have prolonged QT interval i.e. relaxation of ventricles
what does LQTS predispose to
TdP
what is given to LQTS patients that are low risk
BB + lifestyle
what is given to LQTS patients that are high risk
ICD +/- BB + lifestyle
what is a long term possible management of LQTS
oral LT potassium/high potassium diet - increases function of K+ channels and normalises QT
what kind of inheritance is brugada syndrome
autosomal dominant
true / false
Brugada syndrome is more common in asains
true
what is HOCM
increased thickness of cardiac wall causing cavity space to be lost
how causes Brugada syndrome
cardiac sodium channel mutation
what is seen in an ECG of Brugada syndrome
ST elevation
Partial RBBB
true / false
Brugada syndrome can cause AF, VT, VF and is associated with sudden cardiac death
true
