Cardiomyopathy, Myocarditis and pericarditis – pathophysiology, presentation, investigation and therapy Flashcards
Define cardiomyopahty
What is the different classifications of cardiomyopathy
chronic disease of the heart muscle
hypertrophic,
dilated,
restrictive
myocarditis
What happens in dilated cardiomyopathy
Ventricular function is impairedCan be one but more often all chambers dilated and functionally impaired
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)
,
What is the symptoms of dilated cardiomyopathy
dyspnoea, fatigue, orthopnoea, PND, ankle swelling, weight gain of fluid overload, cough
What is the onset of dilated cardiomyopathy
slow and progressive
What is a common past medical history with dilated cardiomyopathy
systemic illness, travel (tropical diseases) Hyper tension vascular disease, thyroid (endocrine) neuromuscular disease
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)
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
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
What drugs cause exasperation of dilated cardiomyopathy
NSAIDS
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
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
Why is therapy difficult for dilated cardiomyopathy
As the cardiomyopathy can be irreversible
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
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
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
Why is restrictive and infiltrative cardiomyopathy not passive
Relaxation of the ventricular wall is an active process that needs functioning intact myocytes,
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)
What is the best imaging evaluation for cardiomyopathies
MRI
What occurs in hypertrophic cardiomyopathy
a disease in which a portion of the myocardium (heart muscle) is hypertrophic (enlarged) without any obvious cause
What is the outcome of hypertrophic cardiomyopathy
Relaxation can be impaired - restrictive manner
Systolic function usually adequate
some functional abnormality
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
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
What is the pathology of hypertrophic cardiomyopathy
Myocyte hypertrophy and disarray
Can be generalised or segmental wall thickness >14mm or >12mm in primary relative
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
