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