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
How does radiation cause RCM?
radiation generate reactive oxygen species in the myocardium which causes inflammation -> myocardial fibrosis
26
What can an ECG show in RCM?
Smaller QRS complexes due to restricted ventricular contraction
27
What is fabry disease?
Genetic disease a deficiency of the enzyme alpha-galactosidase A (a-Gal A) that causes a buildup of a type of fat in tissue
28
What are general measure for RCM?
``` Limited diuretic use B blockers Anticoagulats SCD risk assessment with IC implant Cardiac transplant Treat iron overload if due to amyloid or sarcoid ```
29
What is hypertrophic cardiomyopathy?
When walls are thick, heavy and hypercontractile - new sarcomeres are added in parallel Usually affect Left ventricle
30
How does HCM lead to heart failure?
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
31
What genetic inheritance is HCM?
Autosomal dominant
32
Describe the pathology of HCM?
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
What is the Venturi effect?
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
What are the symptoms of HCM?
``` Asymptomatic Fatigue Dysnoea Chest pain - like angina Exertional pre-syncope Syncope related to arrhythmia or LVOT Palpitations SCD ```
35
What are the clinical signs of HCM?
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
What investigations are used for HCM?
ECG Echo CMRI Risk stratification for SCD, may need ICD
37
Why does squatting decrease LVOT murmur in HCM?
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
Why does the valsvalva manouver increase the LVOT in HCM?
Decreases venous return -> decrease preload, so less blood to stretch out ventricle so obstruction is larger and murmur intensity increases
39
Why does the apex produces a double impulse?
Due to the mitral valve moving to the outflow tract causing increase in obstruction mid-systole
40
What are general measures for treatment of HCM?
``` No heavy exercise No dehydration FH and first degree relatives ECG Echo Genetic testing ```
41
Why does HCM lead to formation of arrhythmia?
Myocardium becomes ischaemic as cant pump effieicintly Also why most common cause of sudden cardiac death
42
What is myocyte disarray?
Lack of striations in myocardium
43
What are specific measure to treat HCM?
Enhance relaxtion: B blocker, verapamil (CCB), disopyrimide AF: anticoagulant Surgical or alcohol septal ablation ICD implant based on risk assessment
44
What is myocarditis?
Acute or chronic inflammation of the myocardium This can impair myocardial function, conduction and generate arrhythmia
45
How does myocarditis lead to heart failure?
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
What are two possible outcomes of myocarditis?
Inflammation resolves and heart contractility returns to normal If severe, can causes fibrosis and scar tissue of myocardium -> long term problems with contraction
47
What are causes of myocarditis?
Viral infections - can trigger lymphocytic myocarditis where lymphocytes and water enter ISF space of myocytes : adenovirus Bacterial: strep., mycobacterial Lymes disease
48
What is the pathology of myocarditis?
Infiltration or inflammatory cells into the myocardial layers, reduced function and heart faikur, heat block as conduction system involve and arrythmias
49
What are the symptoms of myocarditis?
``` Arrhythmias Positional chest pain Fatigue Fever sob HF sign: peripheral oedema ```
50
What investigations should be carried out for myocarditis?
``` 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
What can echo show in myocarditis?
Regional wall motion abnormalities (RWMA) | Inflamed heart muscle walls
52
What can CMRI show in myocarditis?
Oedema | Cardiomegaly
53
What can ECG show in myocarditis?
Sinus tachycardia | T wave inversion
54
What are general measure in treatment of myocarditis?
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
What is pericarditis?
Inflammation of the perIcardium
56
How does pericarditis lead to pericardial effusion?
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
What are the two layers to the pericardium?
Fibrous outer layer | Serous inner later (visceral, pericardial cavity, parietal)
58
What are the causes of pericarditis?
Idiopathic Viral ``` Bacterial Post MI (dressler's syndrome) Perforation Dissection of aorta Neoplasia Autoimmne diseases: systemic lupus erythematosus Cancer and chest radiation ```
59
How does post MI lead to pericarditis?
Necrosis leads to inflammation that also involves serous pericardia
60
What is the pathology of pericarditis?
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
What is the effect of a pericardial effusion?
Puts pressure on the heart, preventing it from fully stretching out or relaxing which can lead to tamponade
62
What is a tamponade?
Perical effusion putting pressure on heart so it doesn't fill properly, causing decrease in CO which can be medical emergency
63
What is the pathophysiology of chronic pericarditis?
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
What are the symptoms of pericarditis?
Fever | Chest pain - pleuritic and postual features (sitting forward improves)
65
What are the signs of pericardial disease?
Pericardial rub Raised JVP Low BP Muffled HS
66
What investigations should be carried out for pericarditis?
ECG Echo CXR Troponin may be raised
67
What can an ECG show in pericarditis?
1. ST elevation PR depression 2. After few weeks - T wave inverts 3. Returns to normal Pericardial effusion: Small QRS complex
68
What can a CXR show in pericarditis?
Large pericardial effusion - large shadow
69
What can an echo show in pericarditis?
Pleural cardial effusion - dancing heart | Chronic pericarditis - stiff pericardium restricting movement
70
What are measures to treat pericarditis?
Relieve pain Treat cause If severe effusion: pericardiocentesis (drains fluid)
71
What are symptoms of pericardial effusion?
``` Fatigue SOB Dizziness Low BP Occasionally chest pain ```
72
What are signs of pericardial effusion?
``` Pulsus paradoxus JVP raised Low BP Pericardial rub Muffled HS ```
73
What are key investigation for pericardial effusion?
Echo CXR ECG
74
What can an ECG show for pericardial effusion?
Electrical alternans: | QRS complexes have different heights due to the heart swinging back and forth in a pool of pericardial fluid
75
What are the causes for constrictive pericarditis?
``` Idiopathic Radiation Post surgery Autoimmune Renal failure Sarcoid ```
76
What are symptoms of constrictive pericarditis?
Fatigue SOB Cough
77
What are the signs of constrictive pericarditis?
``` RHF: Oedema Ascites High JVP Jaundice Hepatomegally AF Pleural effusion ```
78
What investigations should be carried out for constrictive pericarditis?
Echo | R heart catheter to differentiate from restrictive cardiomyopathy
79
What is the treatment for constrictive cardiomyopathy?
Limited diuretics | Pericardectomy