Cardiomyopathy Flashcards

1
Q

What is cardiomyopathy?

A

Heart muscle disease

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2
Q

What are the main types of cardiomyopathy?

A

Hypertrophic
Dilated
Restrictive
Myocarditis

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3
Q

What is dilated cardiomyopathy?

A

When all four chambers of the heart enlarge

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4
Q

What are the causes of dilated cardiomyopathy?

A
Primary:
Idiopathic 
Genetic mutation 
Infection 
Inflammatory
Alcohol 
Drugs 
Post partum
Tropical disease
Haemaochromatosis
Sarcoid 

Secondary to pathological insult to myocardium

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5
Q

How does dilation occur in DCM?

A

New sarcormere are added in series and chamber grow larger leaving the walls thin compared to large chamber size - therefore less muscle used for contraction

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6
Q

How does DCM lead to congestive heart failure?

A

Less contraction -> less SV -> biventricular congestive heart failure as heart cant pump blood out to lungs and body efficiently -> systolic HF

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7
Q

What are the symptoms of DCM?

A
Progressive dyspnoea 
Fatigue 
Orthopnoea 
Paroxysmal nocturnal dyspnoea (PND)
Ankle swelling 
Weight gain (fluid overload)
Cough
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8
Q

What should be looked for in the clinical history for DCM?

A

PMH:
Illness, HPT, vascular disease, thyroid, neuromuscular disease

SH:
Travel, alcohol, job

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9
Q

What are clinical signs of DCM?

A
Poor perfusion 
Thready pulse 
Elevated JVP 
Displaced apex 
S3 and S3
MR murmur 
Pulmonary, sacral and ankle oedema
Pleural effusion 
Thready pulse 
Acites 
Hepatomegally
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10
Q

What are the conditions that DCM commonly presents with?

A
Heart failure
Cardiac arrhythmias
Conduction defects
Thromboembolism
Sudden death
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11
Q

How can DCM cause regurgitation of AV valves?

A

Dilation of chambers, stretches valve so that they cant close all of the way during systole

MR can produce holosystolic pressure and S3 sound due to blood slamming against ventricle wall during diastole

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12
Q

How can DCM cause arrhythmia?

A

Stretching of muscle wall can irritate myocytes important for conduction

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13
Q

What investigations should be carried out for DCM?

A
ECG 
CXR
BNP level 
FBC 
U+E
Echo 
CMRI 
Coronary angiogram
Biopsy depending on type of type of cardiomyopathy
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14
Q

What are general measures to treat DCM?

A

Correct anaemia
Remove exacerbating factors i.e. drugs
Reduce Na intake

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15
Q

What are specific measures to treat DCM?

A

ACEi
ATII blockers
Diuretics

B blockers
Spionolactone
Anticoagulants as required (thrombus formation common in DCM)

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16
Q

What are surgical measures to treat DCM?

A

Cardiac transplant

Sudden cardiac death risk with defibrillator implant

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17
Q

What is restrictive cardiac myopathy?

A

Myocardium become stiffer and less compliant - which means less blood enters the ventricle during diastole as they ventricles do not stretch to allow more blood in

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18
Q

How does RCM cause heart failure?

A

Stiffer myocardium means ventricles don’t stretch during diastole -> less blood pumped out -> diastolic heart failure

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19
Q

What are the causes of RCM?

A
Amyloidosis, sarcoid 
Familial, forms of HCM, diabetic
Haemachromatosis 
Fabry disease 
Fibrosis, carcinoid, radiation, drug effects
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20
Q

How do amyloid cause RCM?

A

Misfolding of proteins which make it insoluble and can deposit in tissue

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21
Q

How does sarcoids cause RCM?

A

Forms granulomas in myocardium

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22
Q

Why does bi-atrial dilation occur in RCM?

A

Less filling of ventricles means extra blood in atrium

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23
Q

How does haemachromatosis cause RCM?

A

Too much iron from the diet is absorbed and it builds up in the myocardium

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24
Q

What investigation should be carried out for RCM?

A
ECG 
CXR
BNP 
FBC 
U+Es - sarcoid and haemachormatosis 
Auto-Antibodies for sclerotic (hardening) connect tissue diseases
Cardiac biopsy - amyloid
Fabry - low plasma galactosidase A activity 
Echo 
CMRI
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25
Q

How does radiation cause RCM?

A

radiation generate reactive oxygen species in the myocardium which causes inflammation -> myocardial fibrosis

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26
Q

What can an ECG show in RCM?

A

Smaller QRS complexes due to restricted ventricular contraction

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27
Q

What is fabry disease?

A

Genetic disease a deficiency of the enzyme alpha-galactosidase A (a-Gal A) that causes a buildup of a type of fat in tissue

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28
Q

What are general measure for RCM?

A
Limited diuretic use 
B blockers 
Anticoagulats 
SCD risk assessment with IC implant 
Cardiac transplant 
Treat iron overload if due to amyloid or sarcoid
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29
Q

What is hypertrophic cardiomyopathy?

A

When walls are thick, heavy and hypercontractile - new sarcomeres are added in parallel

Usually affect Left ventricle

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30
Q

How does HCM lead to heart failure?

A

Larger muscles take up more room so less blood fills into the ventricle

Myocardium stiff and less compliant so less blood fills ventricle due to reduced stretch

Less EDV -> decreased SV -> diastolic heart failure

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31
Q

What genetic inheritance is HCM?

A

Autosomal dominant

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32
Q

Describe the pathology of HCM?

A

Myocyte hypertrophy and disarray
Wall thickness > 14mm
Can be apical, septal or generalised
Septal hypertrophy can cause mitral valve effect and lead to LVOT obstruction

33
Q

What is the Venturi effect?

A

Septal muscle growth can obstruct the LV outflow tract during systole

This increases blood velocity through the smaller opening which pulls the anterior leaflet of the mitral valve toward the septum - venturi effect

This further obstructs LVOT

34
Q

What are the symptoms of HCM?

A
Asymptomatic 
Fatigue 
Dysnoea 
Chest pain - like angina 
Exertional pre-syncope 
Syncope related to arrhythmia or LVOT 
Palpitations 
SCD
35
Q

What are the clinical signs of HCM?

A

Can be none
Notched pulse pattern
Irregular pulse if in AF or ectopy
Double impulse over apex, thrills and murmurs
LVOT murmur - will increase which Valsalva and decrease with squatting
JVP elevated in very restrictive filling

36
Q

What investigations are used for HCM?

A

ECG
Echo
CMRI
Risk stratification for SCD, may need ICD

37
Q

Why does squatting decrease LVOT murmur in HCM?

A

Systemic vascular resistance increases making it harder to eject blood and increases afterload

This increases ESV which stretches valves from septum so it is less obstructed, decreasing to LVOT murmur

38
Q

Why does the valsvalva manouver increase the LVOT in HCM?

A

Decreases venous return -> decrease preload, so less blood to stretch out ventricle so obstruction is larger and murmur intensity increases

39
Q

Why does the apex produces a double impulse?

A

Due to the mitral valve moving to the outflow tract causing increase in obstruction mid-systole

40
Q

What are general measures for treatment of HCM?

A
No heavy exercise
No dehydration 
FH and first degree relatives 
ECG
Echo 
Genetic testing
41
Q

Why does HCM lead to formation of arrhythmia?

A

Myocardium becomes ischaemic as cant pump effieicintly

Also why most common cause of sudden cardiac death

42
Q

What is myocyte disarray?

A

Lack of striations in myocardium

43
Q

What are specific measure to treat HCM?

A

Enhance relaxtion: B blocker, verapamil (CCB), disopyrimide
AF: anticoagulant
Surgical or alcohol septal ablation
ICD implant based on risk assessment

44
Q

What is myocarditis?

A

Acute or chronic inflammation of the myocardium

This can impair myocardial function, conduction and generate arrhythmia

45
Q

How does myocarditis lead to heart failure?

A

Myocardial inflammation causes swelling which damages myocyte needed to contract

This means less blood is pumped is pumped out of the heart during systole

46
Q

What are two possible outcomes of myocarditis?

A

Inflammation resolves and heart contractility returns to normal

If severe, can causes fibrosis and scar tissue of myocardium -> long term problems with contraction

47
Q

What are causes of myocarditis?

A

Viral infections - can trigger lymphocytic myocarditis where lymphocytes and water enter ISF space of myocytes : adenovirus

Bacterial: strep., mycobacterial
Lymes disease

48
Q

What is the pathology of myocarditis?

A

Infiltration or inflammatory cells into the myocardial layers, reduced function and heart faikur, heat block as conduction system involve and arrythmias

49
Q

What are the symptoms of myocarditis?

A
Arrhythmias
Positional chest pain
Fatigue 
Fever
sob
HF sign: peripheral oedema
50
Q

What investigations should be carried out for myocarditis?

A
ECG 
Biomarkers elevated (troponin, creatine kinase)
Echo 
CMRI
Viral DNA PCR 
Auto antibodies 
Strep antibodies 
Lyme B burgdoferi 
HIV 
Biopsy can diagnose but it is risky
51
Q

What can echo show in myocarditis?

A

Regional wall motion abnormalities (RWMA)

Inflamed heart muscle walls

52
Q

What can CMRI show in myocarditis?

A

Oedema

Cardiomegaly

53
Q

What can ECG show in myocarditis?

A

Sinus tachycardia

T wave inversion

54
Q

What are general measure in treatment of myocarditis?

A

Virus is self limiting
Other infections - antibiotics
HF: meds and fluid balance
Arrhythmias: resolves as inflammation does
Immunotherapy if biopsy point to specific outcome
Cardiac transplant if severe

55
Q

What is pericarditis?

A

Inflammation of the perIcardium

56
Q

How does pericarditis lead to pericardial effusion?

A

Inflammation causes fluid to build up the the pericardial cavity, as the serous pericardium cannot remove the fluid as quickly as it comes in

57
Q

What are the two layers to the pericardium?

A

Fibrous outer layer

Serous inner later (visceral, pericardial cavity, parietal)

58
Q

What are the causes of pericarditis?

A

Idiopathic
Viral

Bacterial 
Post MI (dressler's syndrome)
Perforation 
Dissection of aorta 
Neoplasia 
Autoimmne diseases: systemic lupus erythematosus
Cancer and chest radiation
59
Q

How does post MI lead to pericarditis?

A

Necrosis leads to inflammation that also involves serous pericardia

60
Q

What is the pathology of pericarditis?

A

Pericardial inflammation means that fluid and immune cells move from the cavity through BV to the fibrous and serous pericardium

This enlarges the layers

61
Q

What is the effect of a pericardial effusion?

A

Puts pressure on the heart, preventing it from fully stretching out or relaxing which can lead to tamponade

62
Q

What is a tamponade?

A

Perical effusion putting pressure on heart so it doesn’t fill properly, causing decrease in CO which can be medical emergency

63
Q

What is the pathophysiology of chronic pericarditis?

A

Immune cells initiate fibrosis of the serous pericardium, forming an inelastic shell around the heart, decreasing ventricular compliance -> decreases SV but HR increases to compensate

64
Q

What are the symptoms of pericarditis?

A

Fever

Chest pain - pleuritic and postual features (sitting forward improves)

65
Q

What are the signs of pericardial disease?

A

Pericardial rub
Raised JVP
Low BP
Muffled HS

66
Q

What investigations should be carried out for pericarditis?

A

ECG
Echo
CXR
Troponin may be raised

67
Q

What can an ECG show in pericarditis?

A
  1. ST elevation
    PR depression
  2. After few weeks - T wave inverts
  3. Returns to normal

Pericardial effusion:
Small QRS complex

68
Q

What can a CXR show in pericarditis?

A

Large pericardial effusion - large shadow

69
Q

What can an echo show in pericarditis?

A

Pleural cardial effusion - dancing heart

Chronic pericarditis - stiff pericardium restricting movement

70
Q

What are measures to treat pericarditis?

A

Relieve pain
Treat cause
If severe effusion: pericardiocentesis (drains fluid)

71
Q

What are symptoms of pericardial effusion?

A
Fatigue 
SOB 
Dizziness 
Low BP 
Occasionally chest pain
72
Q

What are signs of pericardial effusion?

A
Pulsus paradoxus 
JVP raised 
Low BP 
Pericardial rub 
Muffled HS
73
Q

What are key investigation for pericardial effusion?

A

Echo
CXR
ECG

74
Q

What can an ECG show for pericardial effusion?

A

Electrical alternans:

QRS complexes have different heights due to the heart swinging back and forth in a pool of pericardial fluid

75
Q

What are the causes for constrictive pericarditis?

A
Idiopathic 
Radiation 
Post surgery 
Autoimmune 
Renal failure 
Sarcoid
76
Q

What are symptoms of constrictive pericarditis?

A

Fatigue
SOB
Cough

77
Q

What are the signs of constrictive pericarditis?

A
RHF:
Oedema
Ascites
High JVP 
Jaundice 
Hepatomegally 
AF
Pleural effusion
78
Q

What investigations should be carried out for constrictive pericarditis?

A

Echo

R heart catheter to differentiate from restrictive cardiomyopathy

79
Q

What is the treatment for constrictive cardiomyopathy?

A

Limited diuretics

Pericardectomy