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Flashcards in Inherited Cardiac Conditions Deck (22):
1

what is the name of a single gene mutation?

monogenic mutation

2

what type of inheritence are genetic predispositions usually the result of?

polygenic inheritance

3

what is hypertrophic cardiomyopathy?

increased thickness of cardiac wall causing cavity space to be lost

4

why is there a lot of scarring within a heart with hypertrophic cardiomyopathy?

because there is not enough blood supply to provide for such a big muscle- ischaemia

5

why are arrhythmias (particularly re-entry) common in a heart with hypertrophic cardiomyopathy?

scarring leads to arrhythmias due to increased automacity of surrounding tissue

6

when does a hypertrophic cardiomyopathy become apparent?

late teens/early twenties

7

what is dilated cardiomyopathy?

heart cavity dilation and thinning of the ventricular walls

8

what is the early form of dilated cardiomyopathy?

left ventricular enlargement

9

what are the 4 major primary cardiomyopathies?

hypertrophic cardiomyopathy (HCM)
dilated cardiomyopathy (DCM)
arrhythmogenic right ventricular cardiomyopathy (ARVC)
restrictive cardiomyopathy (RCM)

10

what is arrhythmogenic right ventricular cardiomyopathy?

heart tissue is progressively replaced by fibrous and fatty tissue

11

what is restrictive cardiomyopathy?

a disorder of the heart muscle in which walls of the ventricles become stiff but not necessarily thickened so resist normal filling with blood, causes reduced EDV

12

which of the 4 cardiomyopathies has the greatest genetic link?

hypertrophic cardiomyopathy

13

what type of inheritance is hypertrophic cardiomyopathy?

autosomal dominant with variable penetrance

14

what type of inheritance is dilated cardiacmyopathy?

commonly autosomal dominant trait
sometimes autosomal recessive

15

what are the most common secondary syndromes of cardiomyopathies?

arrhythmias
heart failure

16

what is long QT syndrome?

a inherited condition where patients have a prolonged QT interval (ie relaxation of the ventricles)

17

what very lethal type of VT do patients with long QT syndrome have a predisposition to?

torsades de pointes

18

what occurs just before a torsades de pointes rhythm?

pause then an ectopic beat

19

what is the treatment for low risk patients with inherited long QT syndrome?

lifestyle + betablockers

20

what is the treatment for high risk patients with inherited long QT syndrome?

lifetsyle + ICD
+/- beta blockers

21

what is the cause of brugada syndrome?

cardiac sodium channel mutation

22

what is the main cause of sudden cardiac death in the elderly?

acquired heart disease