Cardiomyopathy

Question 1

Inherited cardiomyopathies tend to be inherited in which pattern?

Answer 1

Autosomal Dominant

Question 2

What are the two inherited forms of cardiomyopathy?

Answer 2

Hypertrophic obstructive cardiomyopathy

Arrhythmogenic right ventricular dysplasia

Question 3

How can you prevent sudden cardiac death in primary cardiomyopathies?

Answer 3

implantable cardioverter-defibrillator

Question 4

What condition is a common cause of sudden death?

Answer 4

Hypertrophic obstructive cardiomyopathy (HOCM)

Question 5

What causes the pathology in HOCM?

Answer 5

Usually due to a mutation in the gene encoding β-myosin heavy chain protein

Question 6

HOCM usually causes sudden death in which group?

Answer 6

young athletes

Question 7

Echo findings in HOCM?

Answer 7

Mitral regurgitation

Systolic Anterior Motion (SAM) of the anterior mitral valve and asymmetric septal hypertrophy

Question 8

What is the pathophysiology Arrhythmogenic right ventricular dysplasia?

Answer 8

Right ventricular myocardium is replaced by fatty and fibrofatty tissue

Around 50% of patients have a mutation of one of the several genes which encode components of desmosome

Question 9

What ECG abnormalities would you see in Arrhythmogenic right ventricular dysplasia?

Answer 9

ECG abnormalities in V1-3, typically T wave inversion.

An epsilon wave is found in about 50% of those with ARV - this is best described as a terminal notch in the QRS complex

Question 10

Can cardiomyopathies be acquired?

Answer 10

yes

Question 11

What is the mixed category of cardiomyopathies?

Answer 11

patients will have a genetic predisposition to cardiomyopathy which is then triggered by the secondary proces

Question 12

Name 4 causes of dilated cardiomyopathy?

Answer 12

alcohol
Coxsackie B virus
wet beri beri
doxorubicin

Question 13

Name 3 causes of Restrictive cardiomyopathy?

Answer 13

amyloidosis
post-radiotherapy
Loeffler’s endocarditis

Question 14

When does peripartum cardiomyopathy typically develop?

Answer 14

Typical develops between last month of pregnancy and 5 months post-partum

Question 15

Periartum cardiomyopathy is common in who?

Answer 15

older women
greater parity
multiple gestations

Question 16

What is Takotsubo cardiomyopathy?

Answer 16

‘Stress’-induced cardiomyopathy e.g. patient just found out family member dies then develops chest pain and features of heart failure

Question 17

How do you treat Takotsubo cardiomyopathy?

Answer 17

Treatment is supportive

Question 18

What happens to the myocardium in Takotsubo cardiomyopathy?

Answer 18

Transient, apical ballooning of the myocardium

Question 19

What are secondary cardiomyopathies?

Answer 19

pathological myocardial involvement as part of a generalized systemic disorder

Question 20

What are Infective secondary cardiomyopathies typicall caused by?

Answer 20

Coxsackie B virus

Chagas disease

Question 21

What are Infiltrative secondary cardiomyopathies typicall caused by?

Answer 21

Amyloidosis

Question 22

What are Storage secondary cardiomyopathies typicall caused by?

Answer 22

Haemochromatosis

Question 23

What are Toxicity secondary cardiomyopathies typicall caused by?

Answer 23

Doxorubicin

Alcoholic cardiomyopathy

Question 24

What are Inflammatory (granulomatous) secondary cardiomyopathies typicall caused by?

Answer 24

Sarcoidosis

Question 25

What are Endocrine secondary cardiomyopathies typicall caused by?

Answer 25

Diabetes mellitus
Thyrotoxicosis
Acromegaly

Question 26

What are Neuromuscular secondary cardiomyopathies typicall caused by?

Answer 26

Friedreich’s ataxia
Duchenne-Becker muscular dystrophy
Myotonic dystrophy

Question 27

What are Nutritional deficiencies secondary cardiomyopathies typicall caused by?

Answer 27

Beriberi (thiamine)

Question 28

What are Autoimmune secondary cardiomyopathies typicall caused by?

Answer 28

Systemic lupus erythematosis

Question 29

What is the most common form of cardiomyopathy?

Answer 29

Dilated cardiomyopathy (DCM) is the most common form of cardiomyopathy, accounting for 90% of cases.

Question 30

What is the most common cause of dilated cardiomyopathy?

Answer 30

idiopathic

Question 31

What are some cardiovascular causes of dilated cardiomyopathy?

Answer 31

myocarditis: e.g. Coxsackie B, HIV, diphtheria, Chagas disease
ischaemic heart disease
hypertension

Question 32

What drugs can cause dilated cardiomyopathy?

Answer 32

iatrogenic: e.g. doxorubicin

substance abuse: e.g. alcohol, cocaine

Question 33

What nutrional cause of dilated cardiomyopathy?

Answer 33

thiamine deficiency, wet beriberi

dry is peripheral neuropahty

Question 34

What role do genetics play indilated cardiomyopathy?

Answer 34

either a familial genetic predisposition to DCM or a specific syndrome e.g. Duchenne muscular dystrophy

Question 35

What are some infiltrative causes of dilated cardiomyopathy?

Answer 35

haemochromatosis, sarcoidosis

Question 36

What are the three key points in the pathophysiology of dilated cardiomyopathy?

Answer 36

1. dilated heart leading to predominately systolic dysfunction
2. all 4 chambers are dilated, but the left ventricle more so than right ventricle
3. eccentric hypertrophy (sarcomeres added in series) is seen

Question 37

What type of murmur would you hear in dilated cardiomyopathy?

Answer 37

systolic murmur

This is because stretching of the valves may result in mitral and tricuspid regurgitation

Question 38

What would you see on CXR for dilated cardiomyopathy?

Answer 38

‘balloon’ appearance of the heart

Question 39

Would you hear an S3 in dilated cardiomyopathy?

Answer 39

yes

Question 40

dilated cardiomyopathy presents with what?

Answer 40

classic findings of heart failure

Question 41

What would an echo show in dilated cardiomyopathies?

Answer 41

reduction in the LVEF (normal range is 55-70%)

dilated left ventricle and no regional wall motion abnormalities