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Cardiomyopathies Flashcards

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1
Q

What is a cardiomyopathy?

A

Any disease of cardiac muscle; leads to changes in size of heart chambers and heart thickness

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2
Q

What are the classifications of cardiomyopathy?

A

Dilated
Restrictive
Hypertrophic

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3
Q

What are some of the causes of dilated cardiomyopathy?

A

genetics
alcohol - common in alcoholics
chemotherapy agents - doxorubicin

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4
Q

Clinical features of dilated cardiomyopathy?

A 
General picture of heart failure;
dyspnoea
pulmonary oedema
fatigue
RVF
emboli
AF 
VT
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5
Q

What are some of the signs of dilated cardiomyopathy?

A

raised pulse and JVP
decreased BP
low ejection fraction (low CO)
displaced apex

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6
Q

What is the effect of a hypertrophic heart on contraction?

A

strong contraction

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7
Q

Hypertrophic myopathy causes dysfunction of systole or diastole?

A

diastole (not systolic since contraction fine)

diastolic dysfunction - heart cannot relax

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8
Q

what are some of the genes involved in hypertrophic cardiomyopathy?

A

sarcomere-related genes:
beta-myosin heavy chain
myosin binding protein C

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9
Q

What would you see on biopsy of hypertrophic cardiomyopathy?

A

disorganised swirls of myofibrils

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10
Q

What is the type of inheritance of hypertrophic obstructive cardiomyopathy?

A

autosomal dominant

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11
Q

What might an ECG of hypertrophic cardiomyopathy show?

A 
LVH
progressive T wave inversion
deep Q waves (inferior & lateral leads)
AF
WPW syndrome
ventricular ectopics
VT
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12
Q

What would an ECHO show of hypertrophic cardiomyopathy?

A

asymmetrical septal hypertrophy

small LV cavity with hyper-contractile posterior wall

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13
Q

What medication would you give for symptomatic benefit of hypertrophic cardiomyopathy?

A

b-blocker or verapamil - aim is to reduce ventricular contractility

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14
Q

Which anti-arrhythmic drug might a person with a hypertrophic cardiomyopathy need, and why?

A

diastolic dysfunction - so heart can’t relax…therefore need something to either slow HR or increase time between APs…
Type 3 are rhythm control of atria or ventricles
So, give amiodarone to increase AP duration and effective refractory period

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15
Q

What would you have to consider when giving amiodarone as an anti-arryhthmic?

A

age of patient - amiodarone has adverse effects when used in the long run

if used along with a beta-blocker or verapamil then there is increased risk of bradycardia

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16
Q

Why would you give a person with hypertrophic cardiomyopathy an anti-coagulant?

A

for paroxysmal AF or systemic emboli

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17
Q

What are main features of restrictive cardiomyopathy?

A 
lack of compliance
stiff heart
diastolic dysfunction (doesn't fill well)
can look normal
bi-atrial dilatation
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18
Q

What causes atrial dilatation in restrictive cardiomyopathy?

A

back pressure from stiff ventricles

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19
Q

What are the main causes of restrictive cardiomyopathy?

A 
deposition of something in the myocardium:
amyloidosis
sarcoidosis
haemochromatosis
tumours
fibrosis
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20
Q

How would a restrictive cardiomyopathy present?

A 
feature of RVF
raised JVP
hepatomegaly
oedema
ascites
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21
Q

What is the sign for amyloidosis on biopsy?

A

stains positive for ‘congo red’
waxy pink material
exhibits apple green bienfringence

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22
Q

What would an ECG of restrictive cardiomyopathy look like?

A

low voltage conduction abnormalities, e.g. AV block or SA node dysfunction

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23
Q

Which cardiomyopathy involves largely the replacement of the right ventricle with fatty material?

A

Arrythmogenic Right Ventricular dysplasia

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24
Q

What does penetrance of genetic disease mean?

A

penetrance describes the proportion of individuals with a disease-causing mutation who exhibit clinical symptoms

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25
Why is arrhythmogenic right ventricular dysplasia difficult to diagnose?
the RV normally looks quite fatty range of non-specific symptoms (syncope & palpitations during exercise) sudden death can be first time it presents
26
What is myocarditis?
inflammation of the myocardium
27
What are the common viral causes of myocarditis?
``` Coxsackie A and B HIV Enterovirus Hepatitis Mumps ```
28
Which autoimmune conditions are associated with myocarditis?
SLE scleroderma sarcoid heart transplant rejection
29
What would the myocardium look like in infectious myocarditis?
thickened and 'beefy'
30
Which type of hypersensitivity reaction is non-infectious myocarditis?
type IV
31
Hypersensitivity to drugs causes which type of myocarditis?
eosinophilic myocarditis
32
Hypersensitivity to infection causes ?
rheumatic fever after strep sore throat
33
Which gram positive group of organisms causes the classical sore throat?
streptococcus - Group A Strep
34
What changes occur to the heart in rheumatic fever?
patchy inflammation of myocardium short, thick chordae tendinae mitral stenosis with thickening & fusion of valve leaflets
35
What does SLE stand for?
Systemic Lupus Erythematous
36
Which valves does SLE involve?
mitral or tricuspid
37
What are aschoff bodies?
nodules found in the heart in rheumatic fever; | contain fibrinoid collagen necrosis and abnormal macrophages
38
What is pericarditis?
inflammation of pericardial layers
39
Characteristic symptoms of pericarditis?
central chest pain | relieved sitting forwards
40
What would you typically see on ECG of pericarditis?
saddle-shaped ST elevation (V5&6)
41
What are the causes of pericarditis?
``` DR IS TRUMP Dressler's syndrome Radiotherapy Infection SLE TB Rheumatic fever (immune mediated) Uraemic (renal failure) Malignancy Post-MI (24-48hrs) ```
42
Which type of infections cause a purulent pericardial effusion?
bacterial (TB) and fungi
43
ECHO virus would produce what type of pericardial effusion?
serous
44
What causes Dressler's syndrome?
Assumed to be immune mediated; myocardial injury stimulus formation of autoantibodies against heart muscle
45
What are symptoms of Dressler's syndrome?
Recurrent fever & chest pain ± pleural or pericardial rub cardiac tamponade may occur
46
What is endocarditis?
inflammation of heart lining but generally refers to inflammation of heart valves
47
What are the risk factors of infectious endocarditis?
``` rheumatic heart disease prosthetic / bicuspid valves congenital defects calcific disease IV drug abuse septicaemia ```
48
What is the basic pathogenesis of endocarditis?
Damages heart valve causes turbulent blood flow over roughened endothelium. Platelets and fibrin deposited (thrombi), upon which bacteria settle and form a vegetation.
49
Which side of the heart is usually affected in infective endocarditis?
left side
50
Which heart valve is most likely infected in PWIDs?
tricuspid
51
Which organism will most likely infect a prosthetic valve?
staph epidermis
52
Which organism is most likely to cause infective endocarditis in PWIDs?
staph aureus | candida
53
Which organisms make up HACEK?
``` Haemophilus Aggregatibacter Cardiobacterium Eikenella Kingella ```
54
What gram stain are HACEK?
gram negative
55
Which antibiotics would you give for native valve endocarditis?
amoxicillin IV | gentamicin IV
56
Which antibiotic would you give for drug user endocarditis?
flucloxacillin IV
57
Which antibiotics would you give if you suspected MRSA?
vancomycin IV rifampicin PO gentamicin IV
58
Which antibiotics would you give for prosthetic valve infection?
vancomycin IV rifampicin PO gentamicin IV
59
What is the commonest coagulase -ve Staph organism?
staph epidermis
60
How long are antibiotics given for?
4-6 weeks
61
Which organism is most likely to cause native valve endocarditis?
staph aureus
62
What are Janeway lesions?
non-tender, small haemorrhage nodal lesions on palms or soles of feet
63
What are Roth spots?
retinal haemorrhages with white/pale centres
64
What are Osler's nodes?
painful, raised red lesions on hands and feet
65
What are signs on ECHO of endocardial involvement?
vegetation, abscess or new valvular regurgitation
66
What does staph epidermis usually contaminate?
skin | prosthetic material
67
Which organism is 2nd most common cause of native valve endocarditis?
strep viridians
68
What is NBTE?
Non-bacterial Thrombotic Endocarditis | non-infectious endocarditis
69
What are the risk factors for NBTE?
embolic disease | hyper-coagulable states
70
What predisposes you to hyper-coagulable states?
``` MI oral contraceptive pill post-surgery malignancy pregnancy SLE ```
71
What is the cause for Libman-Sacks endocarditis?
lupus
72
Which valve does SLE endocarditis affect?
mitral particularly
73
What is a carcinoid tumour?
neoplasms of neuroendocrine cells
74
What is carcinoid syndrome?
when a carcinoid tumour has spread to the liver
75
What does a neuroendocrine tumour produce in excess?
hormones
76
Which hormones do a carcinoid tumour produce?
histamine serotonin bradykinin
77
What is the effect of bradykinin on the vasculature?
inflammatory mediator - blood vessels dilate
78
Which anti-hypertensive drugs increase bradykinin effect?
ACE inhibitors block bradykinin degradation
79
What is the effect of serotonin on the vasculature?
vasoconstriction
80
What is the effect of histamine on the vasculature?
vasoconstriction -> bronchoconstriction
81
Why are primary tumours of the heart rare?
cardiac muscle cells are end-differentiated
82
What is the commonest tumour of the heart? Which chamber is it found?
atrial myxoma | left atrium
83
What are some of the complications of an atrial myxoma?
valve obstruction: tumour emboli and endocarditis
84
Which diseases might an atrial myxoma mimic?
infective endocarditis | mitral stenosis
85
What are some of the overlapping signs & symptoms of atrial myxoma and infective endocarditis?
clubbing fever weight loss systemic emboli
86
What are some of the overlapping signs & symptoms of atrial myxoma and mitral stenosis?
left atrial obstruction | AF

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