Inherited Cardiac Conditions Flashcards Preview

Cardiovascular RT > Inherited Cardiac Conditions > Flashcards

Flashcards in Inherited Cardiac Conditions Deck (22)
Loading flashcards...
1
Q

what is the name of a single gene mutation?

A

monogenic mutation

2
Q

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

A

polygenic inheritance

3
Q

what is hypertrophic cardiomyopathy?

A

increased thickness of cardiac wall causing cavity space to be lost

4
Q

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

A

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

5
Q

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

A

scarring leads to arrhythmias due to increased automacity of surrounding tissue

6
Q

when does a hypertrophic cardiomyopathy become apparent?

A

late teens/early twenties

7
Q

what is dilated cardiomyopathy?

A

heart cavity dilation and thinning of the ventricular walls

8
Q

what is the early form of dilated cardiomyopathy?

A

left ventricular enlargement

9
Q

what are the 4 major primary cardiomyopathies?

A
hypertrophic cardiomyopathy (HCM)
dilated cardiomyopathy (DCM)
arrhythmogenic right ventricular cardiomyopathy (ARVC)
restrictive cardiomyopathy (RCM)
10
Q

what is arrhythmogenic right ventricular cardiomyopathy?

A

heart tissue is progressively replaced by fibrous and fatty tissue

11
Q

what is restrictive cardiomyopathy?

A

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
Q

which of the 4 cardiomyopathies has the greatest genetic link?

A

hypertrophic cardiomyopathy

13
Q

what type of inheritance is hypertrophic cardiomyopathy?

A

autosomal dominant with variable penetrance

14
Q

what type of inheritance is dilated cardiacmyopathy?

A

commonly autosomal dominant trait

sometimes autosomal recessive

15
Q

what are the most common secondary syndromes of cardiomyopathies?

A

arrhythmias

heart failure

16
Q

what is long QT syndrome?

A

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

17
Q

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

A

torsades de pointes

18
Q

what occurs just before a torsades de pointes rhythm?

A

pause then an ectopic beat

19
Q

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

A

lifestyle + betablockers

20
Q

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

A

lifetsyle + ICD

+/- beta blockers

21
Q

what is the cause of brugada syndrome?

A

cardiac sodium channel mutation

22
Q

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

A

acquired heart disease