Cardiomyopathy, Myocarditis and pericarditis – pathophysiology, presentation, investigation and therapy Flashcards by Sarah Anderson

Define cardiomyopahty

What is the different classifications of cardiomyopathy

chronic disease of the heart muscle

hypertrophic,
dilated,
restrictive
myocarditis

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What happens in dilated cardiomyopathy

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

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What is the aetiology of dilated cardiomyopathy

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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What is the symptoms of dilated cardiomyopathy

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

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What is the onset of dilated cardiomyopathy

slow and progressive

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What is a common past medical history with dilated cardiomyopathy

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

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What would be found on examination of dilated cardiomyopathy

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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What is the investigations used for dilated cardiomyopathy

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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Why would you test for Brain Natriuretic Peptide

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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What drugs cause exasperation of dilated cardiomyopathy

NSAIDS

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What is the management of dilated cardiomyopathy

Correct any endocrine disturbance

advice on fluid and salt intake

advise on managing wight to identify fluid overload

Correct anemia

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What is the medical treatment options for dilated cardiomyopathy

ACEI,

ATII blockers,
diuretics

Beta blockers

Spironolactone - steroid drug promotes sodium excretion

Anticoagulants as required

Cardiac transplant

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Why is therapy difficult for dilated cardiomyopathy

As the cardiomyopathy can be irreversible

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What happens in restrictive cardiomyopathy

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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What is the aetiology of restrictive and infiltrative cardiomyopathy

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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What is infiltrative cardiomyopathy

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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Why is restrictive and infiltrative cardiomyopathy not passive

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

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What investigations are carried out for restrictive and infiltrative cardiomyopathy

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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What is the best imaging evaluation for cardiomyopathies

MRI

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What occurs in hypertrophic cardiomyopathy

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

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What is the outcome of hypertrophic cardiomyopathy

Relaxation can be impaired - restrictive manner

Systolic function usually adequate

some functional abnormality

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What is the cause of hypertrophic cardiomyopathy

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

The gene has variable expression and incomplete penetrance

50% chance of inheriting the gene

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What is the pathology of hypertrophic cardiomyopathy

Myocyte hypertrophy and disarray

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

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What are further problems caused by hyperopic cardiomyopathy

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

What is the symptoms of hypertrophic cardiomyopathy

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

What is the problems in diagnosing hypertrophic cardiomyopathy

Is mostly asymptomatic The gene is not know to be expressed until time passes signs are not always preset

What is the signs for hypertrophy cardiomyopathy

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

what investigations are there for hypertrophic cardiomyopathy

ECG Echo CMRI

What is the general measures that should be taken with cardiomyopathy

Avoid heavy exercise Avoid dehydration Explore FH and first degree relatives, ECGs and echoes may be required Consider genetic testing

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

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

What is the treatment for hypertrophic cardiomyopathy if in Af

anticoagulate

What is the treatment if hypertrophic cardiomyopathy becomes obstructive

surgical or alcohol septal ablation

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

Implantable cardioverter defibrillator

Define myocarditis

Acute or chronic inflammation of the myocardium

What is the outcome of myocarditis

Impairs myocardial function leading to heart failure impairs conduction and generates arrhythmia Eventually takes on the dilated cardiomyopathy appearance

What is the pathology of myocarditis

Infiltration of inflammatory cells into the myocardial layers, reduced function and heart failure,

How does heart block occur as a result of myocarditis

conduction system is involved and arrhythmias

What is the symptoms of myocarditis

Heart failure fatigue SOB Potential fever

What is the investigations carried out for myocarditis

ECG ECHO MRI Viral DNA PCR Biopsy Test for autoantibodies, strep antibodies, HIV, lymes disease

What is the management of myocarditis

Treat heart failure, Support brady and tachy arrhythmia Treat specific diagnosis (immunotherapy) stop possible drugs or toxic agent exposure

Define pericarditis

Inflammation of the pericardial layers with or without myocardial involvement

Causes of pericarditis

``` idiopathic viral bacterial, post MI, perforation, dissection of proximal aorta, neoplasia ```

What is the symptoms of pericarditis

chest pain with pleuritic features lying back makes it worse Fever

What is the signs for pericarditis

Temp up, pericardial rub LSE, Raised JVP low BP, muffled Heart Sounds

What does a raised JVP indicate

Effusion present

What signs for pericarditis would hint that its bacterial

High fever and very unwell despite no effusion

What is the investigations for pericarditis

ECG | echo, troponin may be raised if myocardial involvement too

What would be seen on an ECG of pericarditis

ST changes and PR depression of pericarditis

What drugs should be avoided with pericarditis

NSAIDS

When would you use a drain in pericarditis

If bacterial with small effusion Large effusion and some haemodynamic effects is present

What can pericardial effusion potentially result in

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

What is the symptoms of pericardial effusion resulting in tamponade

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

What is the signs of pericardial effusion resulting in tamponade

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

What is the investigations for pericardial effusion

ECHO | CXR

What is the best treatment for pericardial effusion

Drainage

Define constrictive pericarditis

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

What is the causes of constrictive pericarditis

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

What is the symptoms and signs of constrictive pericarditis

fatigue, SOB, cough ``` right heart failure with oedema, ascites, high JVP, jaundice, hepatomegally, Atrial fibrillation, Tricuspid regurgitation, pleural effusion, pericardial knock ```

What investigations are used in constrictive pericarditis

echo and right heart cauterisation

Why is constrictive pericarditis hard to differentiate from

restrictive cardiomyopathy

What is the treatment for constrictive pericarditis

Diuretics | pericardectomy

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

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