Inherited Cardiomyopathies Flashcards

1
Q

What are signs of hypertrophy

A

jerky pulse
forceful or double tap apex
fourth heart sound
midsystolic murmur exacerbated by valsava maneuvre

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2
Q

what are ECG changes that might be seen

A

increase voltage= hypertophy

st depression indicated LV strain

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3
Q

What is the most common cause of SCD in young people

A

hypertrophic cardiomyopathy

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4
Q

what is the most common type of hypertrophy

A

septal

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5
Q

What murmur can arise from hypertrophy

A

mitral regurg

heart murmur similar to aortic stenosis may arise

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6
Q

what do symptoms of hypertrophy mimic

A

CHF but are made worse on diuretic

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7
Q

What are the risks in hypertrophy

A

progressive HF and AF

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8
Q

What type of inheritance is hypertrophic cardiomyopathy

A

autosommal dominant

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9
Q

Where are the mutated genes in hypertrophic cardiomyopathy

A

mutations in sarcometric genes and mitochondrial DNA

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10
Q

What is the treatment of hypertrophic cardiomyopathy

A

Ist =beta blocker

2nd -calcium channel blocker- verapamil

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11
Q

What happens in dilated cardiomyopathy

A

increased diastolic and sysolic volume but low ejection fraction

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12
Q

what type of inheritance is dilated cardiomyopthay

A

autosommal dominant

uncommonly recessive and x linked

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13
Q

where are the mutation is dilated cardiomyopathy

A

cytoskeletal proteins and nuclear transport proteins

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14
Q

What is the treatment for dilated

A

like heart failure- diuretci, ACE, betablocker, digoxin

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15
Q

how does arrhythmogenic right ventricular cardiomyopathy present

A

right sided heart problem eg RBBB
ECg changes
myocarditis

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16
Q

how is it ARVC caused

A

fatty or fibrofatty replacement of myocytes - progressive

17
Q

what ecg changes may be seen in ARVC

A

t wave inversion beyon V1
increased QRS duration
EPSILON waves

18
Q

How do you treat ARVC

A

beta blocker and class III anti arythmic eg SOTOLOL
Radiofrequency ablation
ICD if ventricular arrythmia or if SCD