cardiomyopathy

Question 1

types

Answer 1

• hypertrophic
• arrhythmogenic
• dilated

Question 2

what do they affect of the heart

Answer 2

mechanical or electrical function of the heart

Question 3

what are the secondary causes

Answer 3

• infiltrative
• storage related (haemochromatosis)
• drugs (alcohol)
• neuromuscular
• nutritional
• autoimmune (systemic lupus)
• cancer therapy

Question 4

what are some causes of hypertrophic cardiomyopathy

Answer 4

• aortic stenosis

- hypertension

Question 5

what is the most common cause of sudden death in young people

Answer 5

hypertrophic cardiomyopathy

Question 6

what kind of inheritance is hypertrophic cardiomyopathy

Answer 6

familial autosomal dominant

Question 7

what are some mutations that cause hypertrophic cardiomyopathy

Answer 7

• genes encoding sarcomeric proteins
• ß-myosin heavy chain MYH7
• myosin bingin protein C
• troponin T and I
• alpha myosin heavy chain

Question 8

where does hypertrophic cardiomyopathy involve

Answer 8

interventricular septum

Question 9

what can ventricular ejection cause

Answer 9

mitral regurgitation

Question 10

what are some characteristics of a mutation in troponin T

Answer 10

• mild hypertrophy

- high risk of sudden death

Question 11

when does hypertrophy due to myosin binding protein manifest

Answer 11

not until sixth decade or later

Question 12

symptoms of hypertrophic cardiomyopathy

Answer 12

• asymptotic
• chest pain
• SOB
• syncope
• arrhythmias
• sudden death

Question 13

signs of hypertrophic cardiomyopathy

Answer 13

• double apical pulsation
• jerky carotid pulse
• ejection systolic murmur
• pan systolic murmur
• fourth heart sound

Question 14

why is there a jerky carotid pulse in hypertrophic cardiomyopathy

Answer 14

rapid ejection and sudden obstruction to left ventricular outflow

Question 15

why is there ejection systolic murmur in hypertrophic cardiomyopathy

Answer 15

due to left ventricular outflow obstruction late in systole

Question 16

why is there pan systolic murmur in hypertrophic cardiomyopathy

Answer 16

due to mitral regurgitation

Question 17

what investigations are done for hypertrophic cardiomyopathy

Answer 17

• ECG
• echo
• CMR
• genetic analysis

Question 18

what is seen in ECG of hypertrophic cardiomyopathy

Answer 18

• left ventricular hypertrophy
• ST and T wave changes
• abnormal Q waves

Question 19

what is seen in echo of hypertrophic cardiomyopathy

Answer 19

• asymmetric left ventricular hypertrophy
• vigorously contracting ventricle
• patterns of hypertrophy

Question 20

what is diagnostic for hypertrophic cardiomyopathy

Answer 20

echo

Question 21

what can CMR detect in hypertrophic cardiomyopathy

Answer 21

• hypertrophy

- abnormal myocardial fibrosis

Question 22

treatment of hypertrophic cardiomyopathy

Answer 22

treatment of symptoms and prevent sudden death

Question 23

what are the risk factors of sudden death in hypertrophic cardiomyopathy

Answer 23

• massive left ventricular hypertrophy
• family history of sudden death
• non-sustained ventricular tachycardia
• prior unexplained syncope
• abnormal blood pressure on exercise

Question 24

what is given if low risk of sudden death in hypertrophic cardiomyopathy

Answer 24

amiodarone

Question 25

what is given if high risk of sudden death in hypertrophic cardiomyopathy

Answer 25

ICD insertion

Question 26

what is given for chest pain and dyspnoea in hypertrophic cardiomyopathy

Answer 26

beta blockers and verapamil alone or in combo

Question 27

what is given for left ventricular outflow tract obstruction in hypertrophic cardiomyopathy

Answer 27

disopyramide

Question 28

what drugs should be avoided in hypertrophic cardiomyopathy

Answer 28

vasodilators as they may aggravate left ventricular outflow or cause refractory hypotension

Question 29

where does the arrhythmogenic cardiomyopathy affect

Answer 29

right ventricle

Question 30

what are the myocytes replaced with in arrhythmogenic cardiomyopathy

Answer 30

fatty or fibro-fatty replacements

Question 31

what does fibrosis-fatty replacement lead to in arrhythmogenic cardiomyopathy

Answer 31

ventricular arrhythmia

Question 32

what mutations are there in arrhythmogenic cardiomyopathy

Answer 32

in genes coding for desmosomal proteins

Question 33

what is Naxos disease associated with

Answer 33

palmoplantar keratoderma and wooly hair

Question 34

what is Naxos disease due to

Answer 34

mutation in junctional plakoglobin

Question 35

what is Carvajal syndrome due to

Answer 35

mutation in desmoplakin

Question 36

what are the 2 recessive forms of arrhythmogenic cardiomyopathy

Answer 36

- Naxos disease | - Carvajal syndrome

Question 37

symptoms of arrhythmogenic cardiomyopathy

Answer 37

- asymptomatic - syncope - sudden death - signs of right heart failure

Question 38

what investigations are done for arrhythmogenic cardiomyopathy

Answer 38

- ECG - echo - CMR - genetic test

Question 39

what is seen on ECG of arrhythmogenic cardiomyopathy

Answer 39

- T wave inversion in precordial leads related to right ventricle - right uncle branch block - delayed depolarisations

Question 40

what is seen on echo of arrhythmogenic cardiomyopathy

Answer 40

right ventricular dilatation and aneurysm formation left ventricular dilatation

Question 41

what is seen on CMR of arrhythmogenic cardiomyopathy

Answer 41

can assess right ventricle more accurately can demonstrate fibro-fattty infiltration

Question 42

what can be the gold standard for arrhythmogenic cardiomyopathy

Answer 42

genetic testing

Question 43

what is first line treatment for arrhythmogenic cardiomyopathy

Answer 43

beta blockers

Question 44

what is treatment for symptomatic arrhythmias 1

Answer 44

amiodarone or sotalol

Question 45

what is done for life-threatening arrhythmogenic cardiomyopathy

Answer 45

ICD

Question 46

what is dilated cardiomyopathy characterised by

Answer 46

dilatation of ventricular chambers and systolic dysfunction with preserved wall thickness

Question 47

what kind of inheritance is dilated cardiomyopathy

Answer 47

autosomal dominant

Question 48

what are causes of sporadic dilated cardiomyopathy

Answer 48

- myocarditis - toxins - autoimmune - endocrine - neuromuscular

Question 49

what does dilated cardiomyopathy present with

Answer 49

- heart failure - cardiac arrrhythmias - condition defects - thromboembolism - sudden death

Question 50

what investigation are done for dilated cardiomyopathy

Answer 50

- CXR - ECG - echo - CMR - coronary angiography - biopsy

Question 51

what is seen on CXR for dilated cardiomyopathy

Answer 51

cardiac enlargement

Question 52

what is seen on ECG for dilated cardiomyopathy

Answer 52

- non specific ST and T wave changes - sinus tachycardia - arrhythmias

Question 53

what is seen on echo of dilated cardiomyopathy

Answer 53

- dilatation of left and/or right ventricle with poor contraction function

Question 54

what is seen on CMR of dilated cardiomyopathy

Answer 54

can show other causes of left ventricular dysfunction or abnormal myocardial fibrosis

Question 55

what are treatment options of dilated cardiomyopathy

Answer 55

- cardiac resynchronisation - ICDs - cardiac transplantation

Question 56

what is the appearance of left ventricular non-compaction

Answer 56

sponge like appearance of left ventricle

Question 57

where does left ventricular non-compaction affect

Answer 57

apical portion of the left ventricle

Question 58

how is left ventricular non-compaction diagnosed

Answer 58

- echo - CMR - left ventricular angiography

Question 59

what is Primary restrictive non-hypertrophic cardiomyopathy

Answer 59

rare condition in which there is normal or decreased volume of both ventricles with bi-atrial enlargement, normal wall thickness, normal cardiac valves and impaired ventricular function

Question 60

what condition are associated with primary restrictive non-hypertrophic cardiomyopathy

Answer 60

- amyloidosis - sarcoidosis - Loefflers endocarditis - end-myocardial fibrosis

Question 61

what investigations are done for primary restrictive non-hypertrophic cardiomyopathy

Answer 61

- CXR - ECG - echo - CMR - cardiac catheterisation - biopsy

Question 62

what is seen on echo of Primary restrictive non-hypertrophic cardiomyopathy

Answer 62

- symmetrical myocardial thickening - notmal systolic ejection fraction - imparted ventricular filling - myocardium is speckled - tramlines on M mode echo

Question 63

treatment for Primary restrictive non-hypertrophic cardiomyopathy

Answer 63

no specific treatment

Question 64

what treatment is sometimes done for Primary restrictive non-hypertrophic cardiomyopathy

Answer 64

cardiac transplantaiton

Question 65

what is effective for familial amyloidosis

Answer 65

liver transplantation