Cardiomyopathies Flashcards
What are the 4 main types of cardiomyopathy?
HOCM
ARVCM
Dilated cardiomyopathy
Restrictive cardiomyopathy
What form of mendelian inheritance is associated with HOCM?
Autosomal dominant
What occurs in HOCM?
LVH with impaired diastolic filling and mitral valve abnormalities.
It is the most common cause of sudden cardiac death in young people.
What tests are used to diagnose HOCM?
ECG - shows LVH, ST and T wave changes plus abnormal Q waves
Echocardiogram (this is diagnostic)
What pattern of inheritance is seen in ARVCM?
Both autosomal dominant and autosomal recessive.
Usually involves desmosomal genes - it is an uncommon condition.
What are typical symptoms of ARVCM?
Symptomatic ventricular arrhythmia
Syncope
Sudden death
What pathology is seen in ARVCM?
Fibro-fatty replacement of cardiomyocytes in the right ventricles.
How is ARVCM treated?
Beta-blockers (given first-line in all non-life-threatening arrhythmias)
Amiodarone/Sotalol is used for symptomatic arrhythmias
In those with refractory or life-threatening arrhythmia, an ICD is needed.
What is the most common form of cardiomyopathy?
Dilated cardiomyopathy
What occurs in dilated cardiomyopathy?
Enlargement of ventricles, however, wall size remains the same. There is systolic dysfunction as the heart becomes floppy and the ejection fraction is reduced.
How does dilated cardiomyopathy present?
Heart failure (e.g. dyspnoea, pulmonary oedema)
Cardiac arrhythmias
Conduction defects
Thromboembolism
Sudden death
What can cause dilated cardiomyopathy?
Can be genetic - predominately autosomal dominant
Other causes include:
Toxins (e.g. drugs, alcohol, chemotherapy)
Myocarditis
Autoimmune disorders
Endocrine disorders
Neuromuscular disorders
How is dilated cardiomyopathy managed?
Manage heart failure symptoms
Consider fitting an ICD
What occurs in restrictive cardiomyopathy?
Ventricles have normal/decreased volume with bi-atrial enlargement. Wall thickness remains the same, just stiff.
As a result, there is restricted filling although near-normal systolic function.
What causes restrictive cardiomyopathy??
Myocardial infiltration (e.g. amyloidosis, sarcoidosis or tumors)
Fibrotic damage to the myocardium (may result from radiation exposure)
