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2nd year > Cardiomyopathy > Flashcards

Cardiomyopathy Flashcards

1
Q

what is cardiomyopathy?

A

heart muscle disorder without coronary artery disease, hypertension, valvular or congenital heart diseases

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2
Q

prognosis

A

varying prognoses
can be asymptomatic to severe life-limiting and life threatening
some are associated with sudden cardiac death

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3
Q

types

A

dilated cardiomyopathy
hypertrophic cardiomyopathy
restrictive cardiomyopathy

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4
Q

epidemiology

A

can occur at younger age
suspect in any patient presenting with heart failure
hypertrophic cardiomyopathy is the most common cause of unexpected death in childhood
often genetic link

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5
Q

causes

A 
primary = idiopathic
secondary = has a cause
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6
Q

secondary causes

A 
connective tissue disorders
endocrine disorders 
drugs 
infection 
nutrition
genetics
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7
Q

connective tissue disorders that cause cardiomyopathy

A

sarcoidosis

SLE

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8
Q

endocrine disorders that cause cardiomyopathy

A

diabetes
thyroid disease
acromegaly

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9
Q

drugs that cause cardiomyopathy

A

chemotherapeutic agents
cocaine
alcohol

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10
Q

nutrition causes of cardiomyopathy

A

obesity
vitamin B1 deficiency
calcium deficiency
magnesium deficiency

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11
Q

genetic causes of cardiomyopathy

A

duchenne muscular dystrophy

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12
Q

clinical divisions of cardiomyopathy

A

dilated
hypertrophic
restrictive
arrhythmogenic right ventricular

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13
Q

what is dilated cardiomyopathy?

A 
most common type of cardiomyopathy 
enlarged ventricular 
normal wall thickness 
normal systolic function 
affects left or both ventricles 
most common indication for heart transplant
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14
Q

epidemiology of dilated cardiomyopathy

A

2/10,000
often in genetically susceptible individuals
typically presents in 20-60 year olds
more common in men and africans

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15
Q

causes of dilated cardiomyopathy

A 
ischaemic heart disease
alcohol 
cocaine use
thyroid disorder 
valvular disease 
genetic causes 
idiopathic
infection 
autoimmune disorders
connective tissue disorders
granulomatous disorders 
drugs
peripartum - poor prognosis
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16
Q

infective causes of cardiomyopathy

A

bacterial
HIV
viral - coxsackie or viral myocarditis

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17
Q

pathophysiology of dilated cardiomyopathy

A

damage to myocardium
some myocardial cells undergo necrosis
chronic fibrosis
remaining myocardial tissue dilates and hypertrophies to compensate for necrosis and fibrosis

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18
Q

what can occur as a result of hypertrophy?

A

mitral or tricuspid regurgitation
atrial fibrillation - and other arrhythmias
AV node block as a result of atrial dilatation
thrombus formation in large dilated chambers

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19
Q

onset

A

gradual except when acute myocarditis is the cause

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20
Q

presentation of dilated cardiomyopathy

A 
heart failure:
dyspnoea - exertional 
fatigue 
peripheral oedema 
raised JVP - if RV involved 
loud 3rd and 4th heart sounds 
arrhythmia 
thromboembolism - PE/stroke
acute myocarditis
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21
Q

what investigations need to be done for dilated cardiomyopathy?

A 
ECG
CXR
echocardiogram 
coronary angiogram 
cardiac muscle biopsy
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22
Q

ECG changes dilated cardiomyopathy

A

sinus tachycardia
T wave inversion and Q waves - even in absence of previous MI
ST depression
left BBB

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23
Q

CXR dilated cardiomyopathy

A

enlarged heart
heart failure
pleural effusion

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24
Q

Echo dilated cardiomyopathy

A

dilated chambers
hypokinetic chambers
used to rule out primary valve disorders
can detect a mural thrombus

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25
Q

coronary angiogram dilated cardiomyopathy

A

may be considered when there is doubt over diagnosis, potentially ischaemic heart disease or ischaemic heart disease is the cause

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26
Q

cardiac muscle biopsy dilated cardiomyopathy

A

rarely performed

considered if there a specific cause is suspected such as myocarditis, amyloidosis or sarcoidosis

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27
Q

management of dilated cardiomyopathy

A 
treat reversible causes 
prophylactic anticoagulation 
treat arrhythmias 
consider pacemaker for AVN block 
treat like heart failure 
consider implantable cardioverter defibrillator for patients at high risk of arrhythmia
consider heart transplant
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28
Q

treating potential reversible causes of dilated cardiomyopathy

A

toxoplasmosis
haemochromatosis
thyrotoxicosis

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29
Q

prophylactic anticoagulation for dilated cardiomyopathy

A

warfarin

DOACs

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30
Q

when to consider heart transplant for dilated cardiomyopathy?

A

younger patients - <60

70% 5-year survival

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31
Q

prognosis of dilated cardiomyopathy

A

poor
progression results in worsening heart failure
70% mortality in 5 years
sudden death due to thrombus formation or sudden arrhythmia

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32
Q

what worsens dilated cardiomyopathy prognosis?

A

ventricular wall is thin

ventricles are markedly dilated

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33
Q

what improves dilated cardiomyopathy prognosis?

A

ventricular wall maintains normal thickness

less dilated

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34
Q

what is hypertrophic cardiomyopathy?

A

autosomal dominant genetic disorder
causes diastolic dysfunction with or without outflow obstruction
common cause of sudden death in young athletes

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35
Q

what does hypertrophic cardiomyopathy cause?

A

hypertrophy of left ventricle causing left ventricular outflow obstruction, mitral valve problems, myocardial ischaemia and risk of tachyarrhythmias

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36
Q

epidemiology of hypertrophic cardiomyopathy

A 
most common genetic cardiac disease
1/500
more common in men and black people 
obstructive = 25% of cases 
most common cause of sudden cardiac death in young people
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37
Q

age of presentation of hypertrophic cardiomyopathy

A

between 20-40

38
Q

what causes hypertrophic cardiomyopathy?

A

mutation in any 1 or more of 12 genes that code for cardiac proteins
most significant = troponin T mutations, most likely to cause sudden cardiac death

39
Q

inheritance of hypertrophic cardiomyopathy

A

autosomal dominant inheritance

variable penetrance and expressivity

40
Q

pathophysiology of hypertrophic cardiomyopathy

A

genetic defects in genes coding for cardiac proteins causing disorganised cardiac matrix and left ventricular hypertrophy
hypertrophy can occur in any part of the left ventricle
most commonly affects anterior ventricular septum

41
Q

which proteins are affected in hypertrophic cardiomyopathy?

A

beta-myosin heavy chain
troponin
alpha-tropomyosin

42
Q

presentation of hypertrophic cardiomyopathy

A 
mostly asymptomatic 
symptoms:
dyspnoea - can be initiated by alcohol
chest pain 
syncope on exercise 
palpitations
sudden death 
family history
43
Q

syncope in hypertrophic cardiomyopathy

A

can be initiated by alcohol

can be seemingly unexplained

44
Q

sudden death from hypertrophic cardiomyopathy

A

can be due to arrhythmia or due to outflow tract obstruction
unexplained syncope is a risk factor for sudden death

45
Q

examination for hypertrophic cardiomyopathy

A

can be normal or abnormal

46
Q

abnormal examination findings for hypertrophic cardiomyopathy

A

forceful apex beat
late ejection systolic murmur exacerbated by valsalva manoeuvre and reduced by squatting
prominent JVP
abnormal blood pressure response to exercise - may fall or rise very little
atrial fibrillation - occurs in 20%

47
Q

what should happen to BP on exercise?

A

rise by 20-25mmHg

48
Q

investigations for hypertrophic cardiomyopathy

A 
ECG
Echo
CXR
Cardiac MRI
myocardial biopsy 
cardiac catheterisation
49
Q

ECG changes in hypertrophic cardiomyopathy

A 
non-specific 
ST segment changes
T wave inversion 
signs of left ventricular hypertrophy 
atrial fibrillation
50
Q

Echo in hypertrophic cardiomyopathy

A 
standard diagnostic test 
asymmetric septal hypertrophy >15mm
ratio of septal wall to posterior wall >1.4:1 
non-dilated left ventricular cavity 
normal systolic function 
absence of valvular disease
51
Q

CXR in hypertrophic cardiomyopathy

A

often normal
heart size may be normal or enlarged
left atrial enlargement seen in mitral regurgitation

52
Q

cardiac MRI in hypertrophic cardiomyopathy

A

assessing segmental hypertrophy

can assess systolic and diastolic dysfunction

53
Q

myocardial biopsy for hypertrophic cardiomyopathy

A

used to rule out other conditions

54
Q

cardiac catheterisation for hypertrophic cardiomyopathy

A

assesses the degree of outflow obstruction and other structural abnormalities

55
Q

aims of management for hypertrophic cardiomyopathy

A

reduce symptoms

reduce progression of disease

56
Q

management of hypertrophic cardiomyopathy

A

control arrhythmias
anticoagulation for AF
reduce outflow tract obstruction gradient and improve diastolic function
disopyramide used in severe cases
implantable cardioverter defibrillator for those at risk of sudden death
avoid competitive sports and strenuous exercise
surgical myectomy
heart transplatn for severe heart failure

57
Q

controlling arrhythmias in hypertrophic cardiomyopathy

A

anti-arrhythmic drugs

ablation therapy

58
Q

how to reduce outflow tract obstruction gradient and improve diastolic function

A

beta blockers
verapamil
reduce cardiac contractility and dilate hypertrophied left ventricle, reduce outflow tract obstruction and improve diastolic function
improve physical function of heart but do not treat arrhythmias

59
Q

disopyramide

A

negative inotrope

60
Q

what should we be wary of in hypertrophic cardiomyopathy management?

A 
drugs that reduce preload, as it decreases the chamber size and can exacerbate symptoms 
ACE inhibitors 
ARBs
nitrites 
diuretics
61
Q

surgical myectomy in hypertrophic cardiomyopathy

A

patients with symptoms resistant to medical management can be managed with resection of the hypertrophied muscle to reduce outflow tract obstruction

62
Q

mortality risk if hypertrophic cardiomyopathy

A

annual = 1-3%
higher risk if younger age at presentation
sudden death is most common cause of death
typically sudden death occurs in young patients with few or no symptoms

63
Q

risk factors for sudden death in hypertrophic cardiomyopathy

A 
unexplained syncopal events
episode of ventricular fibrillation or ventricular tachycardia 
abnormal BP response to exercise
family history of sudden cardiac death 
under 30
64
Q

how to prevent sudden death in hypertrophic cardiomyopathy

A

intra-cardiac devices

65
Q

when to use ICDs in hypertrophic cardiomyopathy?

A

secondary prevention in any patient who has had a cardiac arrest or previous episode of sustained VT

66
Q

good prognosis for hypertrophic cardiomyopathy

A

patients with LVH wall thickness of <20mm and no other risk factors/ low risk have a similar life expectancy to general population

67
Q

risk of infective endocarditis

A

low

no need for antibiotic prophylaxis

68
Q

what is restrictive cardiomyopathy?

A

reduced compliance of ventricular walls during diastolic filling
most commonly affects LV but can affect both

69
Q

how common is restrictive cardiomyopathy?

A

least common of the cardiomyopathies

70
Q

types of restrictive cardiomyopathy

A
  1. infiltration of myocardium by an invasive substance such as amyloid plaques, sarcoidosis or iron in haemochromatosis
  2. fibrotic myocardium - no external invasive substance
71
Q

what can restrictive cardiomyopathy cause?

A

high diastolic filling pressures
leads to pulmonary hypertension
associated with mural thrombi

72
Q

epidemiology of restrictive cardiomyopathy

A

most patients are elderly
equal affect on genders
not inherited
more prevalent in tropical regions

73
Q

causes of restrictive cardiomyopathy

A 
no underlying cause 
lofflers syndrome 
amyloidosis most common in western world
sarcoidosis
haemochromatosis
74
Q

lofflers syndrome

A

tropical disease
starts with arteritis which spreads to cause thrombus formation of the endocardium and valves
leads to fibrosis

75
Q

presentation of restrictive cardiomyopathy

A 
heart failure signs 
normal/enlarged heart 
features of right ventricular failure 
similar presentation to constrictive pericarditis 
AF in 75% of patients 
other arrhythmias - AVN block etc.
76
Q

signs of heart failure associated with restrictive cardiomyopathy

A 
dyspnoea - exertional
orthopnoea 
fatigue 
pulmonary oedema 
loud 3rd heart sound
77
Q

features of right ventricular failure associated with restrictive cardiomyopathy

A

raised JVP
hepatomegaly
oedema
ascites

78
Q

investigations for restrictive cardiomyopathy

A

ECG
CXR
echo

79
Q

ECG changes in restrictive cardiomyopathy

A

non-specific T wave and ST changes
pathological Q waves in absence of previous MI
Left ventricular hypertrophy

80
Q

CXR changes in restrictive cardiomyopathy

A

heart size is often normal or small

may be enlarged in late stage amyloidosis or haemochromatosis

81
Q

Echo changes in restrictive cardiomyopathy

A

normal systolic function
dilated atria
myocardial hypertrophy - increased wall thickness
can help differentiate from restrictive pericarditis

82
Q

restrictive pericarditis echo

A

thickened pericardium

83
Q

treatment aim for restrictive cardiomyopathy

A

treat underlying cause
early treatment for major underlying causes may reduce disease progression
no specific cardiac medical treatments
some cases suitable for endocardial resection
heart transplant

84
Q

prognosis of restrictive cardiomyopathy

A

poor
similar to dilated cardiomyopathy
30% 5 year survival
no effective treatments for most patients that alter the cause of disease

85
Q

cautions in restrictive cardiomyopathy

A

diuretics
digoxin
nitrites

86
Q

why is caution taken with diuretics in restrictive cardiomyopathy?

A

reduce preload

stiff ventricles rely on preload for filling

87
Q

why is caution taken with digoxin in restrictive cardiomyopathy?

A

amyloidosis patients have extreme digoxin sensitivity and can become easily toxic

88
Q

why is caution taken with nitrites in restrictive cardiomyopathy?

A

can reduce afterload and cause dangerous hypotension

89
Q

differential diagnoses for cardiomyopathy

A 
ischaemic heart disease
valvular heart disease - mitral and aortic valves 
athletes 
pericarditis 
pulmonary stenosis 
VSD
90
Q

type of dysfunction in dilated cardiomyopathy

A

systolic

91
Q

type of dysfunction in hypertrophic cardiomyopathy

A

diastolic potentially plus outflow obstruction

92
Q

type of dysfunction in restrictive cardiomyopathy

A

diastolic

2nd year (48 decks)

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