Cardiomyopathy

Question 1

What are the 3 cardiomyopathy’s?

Answer 1

Hypertrophic, dilated, restrictive
Diseases of myocardium (muscular/conduction defects)

Question 2

What is the MC cause of death in young people?

Answer 2

Hypertrophic cardiomyopathy

Question 3

Cause of hypertrophic cardiomyopathy?

Answer 3

Familial (inherited, auto dom mutation of sarcomere proteins - troponin T + myosin B)
Exercise
Aortic stenosis

Question 4

Pathology of hypertrophic?

Answer 4

Abnormal thickening of cardiac muscle - thick non compliant heart = impaired diastolic filling therefore low CO

Question 5

Hypertrophic
Sx?

Answer 5

May present with sudden death
Others: chest pain, palpitations, SOB, syncope

Question 6

Hypertrophic
Dx?

Answer 6

Confirmed with abnormal ECG
Definitive = ECHO
genetic testing

Question 7

Hypertrophic
Tx?

Answer 7

Beta blocker (low contractility and increase filling time)
CCB
Amiodarone (anti arrhythmic)

Question 8

What is the MC cardiomyopathy generally?

Answer 8

dilated

Question 9

Dilated
Cause?

Answer 9

Auto dom familial (cytoskeleton gene mutation)
IHD
Alcohol

Question 10

Dilated
Pathology?

Answer 10

chambers of heart dilate (get bigger) and thin cardiac walls poorly contract therefore low CO

Question 11

Dilated
Sx?
Dx?
Tx?

Answer 11

SOB, heart failure, AF, thromboembolism

ECG, ECHO

Underlying conditions eg. AF, HF

Question 12

Restrictive
How common?
Cause?

Answer 12

Rare
granulomatous disease (sarcoidosis, AMYLOIDOSIS), idiopathic, post MI fibrotic

Question 13

Pathology of restrictive

Answer 13

Rigid fibrotic myocardium (stiffer = less compliant), can’t stretch = less filling + contracts poly therefore lower CO

Question 14

Restrictive
Sx?

Answer 14

severe, dyspnoea, S3+4 heart sounds, oedema, congestive HF, narrow pulse pressure (normally 120/80 but DCM 105/95 - therefore blood stasis as low gradient)

Question 15

Restrictive
Dx?
Tx?

Answer 15

ECG, ECHO, cardiac catheterisation (definitive)

None, consider transplant (Px typically die within a year)