Cardiomyopathy, Myocarditis and pericarditis – pathophysiology, presentation, investigation and therapy Flashcards

1
Q

Define cardiomyopahty

What is the different classifications of cardiomyopathy

A

chronic disease of the heart muscle

hypertrophic,
dilated,
restrictive
myocarditis

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2
Q

What happens in dilated cardiomyopathy

A

Ventricular function is impairedCan be one but more often all chambers dilated and functionally impaired

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3
Q

What is the aetiology of dilated cardiomyopathy

A

Ischaemia

Valvular disease

Genetics and familial DCM
muscular dystrophy

Inflammatory/infectious

toxic exposure (alcohol, drugs, endocrine)

Post child birth

tropical disease

Injury, cell loss, scar replacement (sarcoid)

,

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4
Q

What is the symptoms of dilated cardiomyopathy

A
dyspnoea, 
fatigue, 
orthopnoea, PND, ankle swelling, 
weight gain of fluid overload, 
cough
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5
Q

What is the onset of dilated cardiomyopathy

A

slow and progressive

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6
Q

What is a common past medical history with dilated cardiomyopathy

A
systemic illness, 
travel (tropical diseases)
Hyper tension 
vascular disease, thyroid (endocrine)
neuromuscular disease
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7
Q

What would be found on examination of dilated cardiomyopathy

A

Poor superficial perfusion,

pulse - irreg if in AF,

SOB at rest,

narrow pulse pressure,

JVP elevated+/- TR waves,

displaced apex,

S3 and S4,

MR murmur often,

pulmonary oedema,

pleural effusions, ankle oedema, sacral oedema,

acites, (the accumulation of fluid in the peritoneal cavity)

hepatomegally (liver enlargement)

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8
Q

What is the investigations used for dilated cardiomyopathy

A

Repeated ECG noting left bundle branch block if present

CXR

N termial pro Brain Natriuetic Peptide

Basic bloods Full; Blood Count, urea and electrolytes

Echo

Cardiovascular magnetic resonance imaging MRI

Coronary angiogram

Sometimes biopsy

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9
Q

Why would you test for Brain Natriuretic Peptide

A

as Brain Natriuretic Peptide polypeptide secreted by the ventricles of the heart in response to excessive stretching of heart muscle cells

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10
Q

What drugs cause exasperation of dilated cardiomyopathy

A

NSAIDS

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11
Q

What is the management of dilated cardiomyopathy

A

Correct any endocrine disturbance

advice on fluid and salt intake

advise on managing wight to identify fluid overload

Correct anemia

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12
Q

What is the medical treatment options for dilated cardiomyopathy

A

ACEI,

ATII blockers,
diuretics

Beta blockers

Spironolactone - steroid drug promotes sodium excretion

Anticoagulants as required

Cardiac transplant

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13
Q

Why is therapy difficult for dilated cardiomyopathy

A

As the cardiomyopathy can be irreversible

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14
Q

What happens in restrictive cardiomyopathy

A

walls are rigid, and the heart is restricted from stretching and filling with blood properly.

the systolic function may or not be impaired

there is a reduced compliance

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15
Q

What is the aetiology of restrictive and infiltrative cardiomyopathy

A

Clinical disorders;
Scleroderma - hardening and contraction of skin
Diabetic
Sarcoid/amyloid

Endomyocardial;
Fibrosis 
Radiation 
Drug effects 
Carcinoid - tumour in glands

Diseases;
Haemochromatosis

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16
Q

What is infiltrative cardiomyopathy

A

Diverse group of cardiac diseases, which are characterized by the deposition of abnormal substances within heart tissue that cause the ventricular walls to develop either diastolic dysfunction or, less commonly, systolic dysfunction

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17
Q

Why is restrictive and infiltrative cardiomyopathy not passive

A

Relaxation of the ventricular wall is an active process that needs functioning intact myocytes,

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18
Q

What investigations are carried out for restrictive and infiltrative cardiomyopathy

A

Repeated ECG

CXR

N termial pro Brain Natriuetic Peptide

Basic bloods FBC, U+E,

antibodies testing (for sclerotic CT diseases)

Test for Fabry
(low plasma alpha galactosidase A activity)

Echo

MRI,

Biopsy (amyloid non cardiac)

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19
Q

What is the best imaging evaluation for cardiomyopathies

A

MRI

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20
Q

What occurs in hypertrophic cardiomyopathy

A

a disease in which a portion of the myocardium (heart muscle) is hypertrophic (enlarged) without any obvious cause

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21
Q

What is the outcome of hypertrophic cardiomyopathy

A

Relaxation can be impaired - restrictive manner

Systolic function usually adequate

some functional abnormality

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22
Q

What is the cause of hypertrophic cardiomyopathy

A

Inherited Autosomal dominant sarcomere gene defect that changes the genes in the heart muscle protein

Thyroid problems and diabetes can also cause hypertrophic cardiomyopathy

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23
Q

What is the gene expression for hypertrophic cardiomyopathy and what are your chances of inheriting

A

The gene has variable expression and incomplete penetrance

50% chance of inheriting the gene

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24
Q

What is the pathology of hypertrophic cardiomyopathy

A

Myocyte hypertrophy and disarray

Can be generalised or segmental wall thickness >14mm or >12mm in primary relative

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25
Q

What are further problems caused by hyperopic cardiomyopathy

A

Coronary arteries also affected with small vessel narrowing and consequent ischaemia and fibrosis, arrhythmias can occur

can also cause left ventricle out flow obstruction if mitral valve defect also present

26
Q

What is the symptoms of hypertrophic cardiomyopathy

A
Asymptomatic for many, 
fatigue, 
dyspnoea, 
anginal like chest pain, 
exertional pre syncope, 
syncope related to arrhythmias or LVOT obstruction
27
Q

What is the problems in diagnosing hypertrophic cardiomyopathy

A

Is mostly asymptomatic

The gene is not know to be expressed until time passes

signs are not always preset

28
Q

What is the signs for hypertrophy cardiomyopathy

A

Notched pulse pattern

Irreg pulse if in AF or ectopy

Double impulse over apex, thrills and murmurs, often dynamic

LVOT murmur will increase with valsalve and decrease with squatting

JVP can be raised in very restrictive filling

29
Q

what investigations are there for hypertrophic cardiomyopathy

A

ECG
Echo
CMRI

30
Q

What is the general measures that should be taken with cardiomyopathy

A

Avoid heavy exercise

Avoid dehydration

Explore FH and first degree relatives,

ECGs and echoes may be required

Consider genetic testing

31
Q

What drugs are used to enhance relaxation for a symptomatic hypertrophic cardiomyopathy

A

Drugs to try and enhance relaxation:, beta blockers, verapamil, disopyrimide

32
Q

What is the treatment for hypertrophic cardiomyopathy if in Af

A

anticoagulate

33
Q

What is the treatment if hypertrophic cardiomyopathy becomes obstructive

A

surgical or alcohol septal ablation

34
Q

What is given to those with high risk hypertrophic cardiomyopathy and sudden cardiac death

A

Implantable cardioverter defibrillator

35
Q

Define myocarditis

A

Acute or chronic inflammation of the myocardium

36
Q

What is the outcome of myocarditis

A

Impairs myocardial function leading to heart failure
impairs conduction and generates arrhythmia

Eventually takes on the dilated cardiomyopathy appearance

37
Q

What is the pathology of myocarditis

A

Infiltration of inflammatory cells into the myocardial layers,

reduced function and heart failure,

38
Q

How does heart block occur as a result of myocarditis

A

conduction system is involved and arrhythmias

39
Q

What is the symptoms of myocarditis

A

Heart failure
fatigue
SOB
Potential fever

40
Q

What is the investigations carried out for myocarditis

A

ECG

ECHO

MRI

Viral DNA PCR

Biopsy

Test for autoantibodies, strep antibodies, HIV, lymes disease

41
Q

What is the management of myocarditis

A

Treat heart failure,
Support brady and tachy arrhythmia

Treat specific diagnosis (immunotherapy)

stop possible drugs or toxic agent exposure

42
Q

Define pericarditis

A

Inflammation of the pericardial layers with or without myocardial involvement

43
Q

Causes of pericarditis

A
idiopathic 
viral 
bacterial,
post MI, 
perforation, 
dissection of proximal aorta, 
neoplasia
44
Q

What is the symptoms of pericarditis

A

chest pain with pleuritic features

lying back makes it worse

Fever

45
Q

What is the signs for pericarditis

A

Temp up,
pericardial rub LSE,

Raised JVP
low BP,

muffled Heart Sounds

46
Q

What does a raised JVP indicate

A

Effusion present

47
Q

What signs for pericarditis would hint that its bacterial

A

High fever and very unwell despite no effusion

48
Q

What is the investigations for pericarditis

A

ECG

echo, troponin may be raised if myocardial involvement too

49
Q

What would be seen on an ECG of pericarditis

A

ST changes and PR depression of pericarditis

50
Q

What drugs should be avoided with pericarditis

A

NSAIDS

51
Q

When would you use a drain in pericarditis

A

If bacterial with small effusion

Large effusion and some haemodynamic effects is present

52
Q

What can pericardial effusion potentially result in

A

Tamponade

compression of the heart by an accumulation of fluid in the pericardial sac

53
Q

What is the symptoms of pericardial effusion resulting in tamponade

A

fatige,
SOB,
dizzy with low BP, occasionally chest pain.

54
Q

What is the signs of pericardial effusion resulting in tamponade

A
pulsus paradoxus, 
JVP raised, 
low BP, 
\+/- rub, +/- muffled Heart Sound
Pulmonary oedema (very rare)
55
Q

What is the investigations for pericardial effusion

A

ECHO

CXR

56
Q

What is the best treatment for pericardial effusion

A

Drainage

57
Q

Define constrictive pericarditis

A

is long-term (chronic) inflammation of the sac-like covering of the heart (the pericardium) with thickening and scarring.

58
Q

What is the causes of constrictive pericarditis

A

idopathic, radiation, post surgery, autoimmune, renal failure, sarcoid.

59
Q

What is the symptoms and signs of constrictive pericarditis

A

fatigue, SOB, cough

right heart failure with oedema, 
ascites, 
high JVP, 
jaundice, hepatomegally, 
Atrial fibrillation, 
Tricuspid regurgitation, 
pleural effusion, pericardial knock
60
Q

What investigations are used in constrictive pericarditis

A

echo and right heart cauterisation

61
Q

Why is constrictive pericarditis hard to differentiate from

A

restrictive cardiomyopathy

62
Q

What is the treatment for constrictive pericarditis

A

Diuretics

pericardectomy