Cardiomyopathy

Question 1

what is cardiomyopathy

Answer 1

cardiac muscle disease causing change in size of heart chambers and heart thickness

Question 2

what are the classifications of cardiomyopathy

Answer 2

dilated
hypertrophic
restrictive
arrhythmogenic right ventricular dysplasia

Question 3

features of dilated cardiomyopathy

Answer 3

enlarged heart

floppy heart

Question 4

causes or dilated cardiomyopathy

Answer 4

genetic (50%) (AD. AR, X-linked, mitrochondrial),
toxins (alcohol, doxorubicin)
cardiac infection
pregnancy
myocarditis
autoimmune

Question 5

what proteins are affected causing dilated cardiomyopathy

Answer 5

heart proteins: design, dystrophin, actin

Question 6

signs or dilated cardiomyopathy

Answer 6

SOB
poor exercise tolerance (low CO)
heart failure
arrhythmias

Question 7

how is dilated cardiomyopathy investigated

Answer 7

cardiac enlargement on chest x-ray
ECG (diffuse nonspecific ST segment + T wave
Echocardiogram
Cardiac MR 
Coronary angiography

Question 8

features of hypertrophic cardiomyopathy

Answer 8

big solid heart with strong contractions

diastolic dysfunction: contraction fine relaxation not

Question 9

what is the most common cardiac cause of sudden death in athletes

Answer 9

hypertrophic cardiomyopathy

Question 10

cause of hypertrophic cardiomyopathy

Answer 10

genetic

familial, autosomal dominant, beta myosin, myosin binding C protein, alpha tropmyosin

Question 11

features of hypertrophic cardiomyopathy

Answer 11

Bulging interventricular septum
Outflow tract obstruction
Heart cannot relax
LV luminal reduction
Disorganised myofibrates

Question 12

symptoms of cardiac myopathy

Answer 12

chest pain
dyspnoea
syncope
arrhythmia
AF (pulmonary oedema, tachycardia, increased left atrial pressure)

Question 13

signs of cardiac myopathy

Answer 13

4th heart sound (double apical pulsation)
Jerky carotid pulse (obstruction to ventricular outflow)
Ejection systolic murmur (aortic stenosis)
Pansystolic murmur (mitral regurgitation)

Question 14

how is hypertrophic cardiomyopathy diagnosed

Answer 14

ECG: LVH/abnormal Q waves in inferolateral leads
Echocardiography (diagnosed)
Cardiac MR

Question 15

features of restrictive cardiomyopathy

Answer 15

lack of compliance
stiff heart = diastolic dysfunction
biatrial dilatation due to back pressure
ventricle wall can appear normal

Question 16

causes of restrictive cardiomyopathy

Answer 16

deposition in myocardium
metabolic eg. iron
amyloid 
sarcoid
tumour 
fibrosis

Question 17

what is amyloid

Answer 17

abnormal protein deposition forms b sheets which body can’t get rid off

Question 18

classifications of amyloid

Answer 18

AA: chronic disease (rheumatoid)
AL: light chains, abnormal immunoglobin
AATR: senile cardiac amyloidosis
Haemodialysis: B2 microglobilin
Familial: Transthyretin
Diabetes
Alzhiemer’s

Question 19

features of amyloid

Answer 19

+ve congo red
Apple green birefringence
Affects conduction pathway

Question 20

what causes arrhythmogenic death

Answer 20

amyloid

Question 21

what is cause of arrhythmogenic right ventricular dysplasia

Answer 21

rare genetic disease - autosomal dominates + low penetrance (those with the gene don’t always have the disease)

Question 22

what is arrhythmogenic right ventricular dysplasia

Answer 22

muscle in right ventricle dies, replaced with scar and fat tissue causing re-entry = ventricular arrhythmias + enlarged heart

Question 23

symptoms and signs of arrhythmogenic right ventricular dysplasia

Answer 23

asymptomatic
ventricular arrhythmias
sudden death
syncope + funny turns

Question 24

how does an ECG app appear of arrhythmogenic right ventricular dysplasia

Answer 24

ECG:
T wave inversion in V1-V3
epsilon waves at end of QRS
also do echocardiography + cardiac MR

Question 25

treatment of arrhythmogenic right ventricular dysplasia

Answer 25

B-blockers 1st line if non-life threatening
amiodarone/sotalol for symptomatic arrhythmias
refractory/life threatening: ICD