Cardiovascular Pathology Flashcards
What is cardiomyopathy?
Any disease of the cardiac muscle
-Often results in changes in the size of the heart chambers and thickness of the heart
Classification of myopathy?
Dilated
Hypertrophic
Restrictive
Arrhythmogenic right ventricular dysplasia
What is a simple description of hypertrophic cardiomyopathy?
Muscle bound
What is a simple description of restrictive cardiomyopathy?
Stiff
What is a simple description of dilated cardiomyopathy?
Weak/flabby
Features of dilated cardiomyopathy?
Flabby/floppy heart
Big heart= 2-3 times normal size
Histological features are non-specific
Main cause of DCM (dilated cardiomyopathy)?
Genetics- AD,AR, X-linked and mitochondrial
Genes that encode heart muscle proteins eg
Desmin and dystrophin
Causes of DCM?
Genetics
Alcohol
Drugs- chemotherapy drugs
Rare causes of DCM?
Pregnancy
Cardiac Infection
Clinical features of DCM?
General picture of heart failure
Shortness of breath, poor exercise tolerance
Low ejection fraction (low cardiac output)
Features of hypertrophic cardiomyopathy?
Big Solid hearts
- Hypertrophic and strong contraction (differes from DCM)
- Diastolic dysfunction- (not systolic as contraction is fine)
- Heart can’t relax
- Eventual outflow obstruction
(causes sudden death in athletes)
Causes of hypertrophic cardiomyopathy?
Genetics - lots of different genes
-Beta myosin heavy chain, Myosin binding protein C, alpha tropomyosin
Examples of genes that cause hypertrophic cardiomyopathy?
- Beta myosin heavy chain
- Myosin binding protein C
- Alpha tropomyosin
Pathophysiology of hypertrophic cardiomyopathy?
Bulging interventricular septum
Outflow tract obstruction
LV luminal reduction
Features of restrictive cardiomyopathy?
Lack of heart compliance
Stiff heart
Doesn’t fill well so diastolic dysfunction
Can look normal
Biatrial dilatation as a result of back pressure
What happens to the walls of the ventricles in restrictive cardiomyopathy?
Walls become stiff but not necessarily thickened
Heart may appear grossly normal
Causes of restrictive cardiomyopathy?
Deposition of something in myocardium Metabolic byproducts - Iron Amyloid Sarcoid- multi system granulomatous disorder Tumours Fibrosis - following radiation
What is amyloid?
Abnormal deposition of an abnormal protein
Lots of different types as lots of abnormal proteins
Tendency to form beta pleated sheets
Body can’t get rid of them
2 Main types of amyloids?
AA- most main- related to chronic diseases like rheumatoid AL Haemodialysis associated Familial forms Diabetes Alzheimers
Features of amyloid?
Generally resembles restrictive cardiomyopathy
Arrhythmogenic death