Cardiomyopathies current status & challenges
Cardiomyopathy and the history
Disease of the heart muscle that makes it harder for the heart to pump blood to the
rest of the body. The heart may relax or contract less well.
A distinction was made between:
Primary cardiomyopathy: cardiomyopathy with unknown etiology (oorzaak)
o Genetic cardiomyopathy
HCM; hypertrophic cardiomyopathy
ARVC/D; arrhythmogenic right ventricular cardiomyopathy
LVNC; left ventricular non-compaction cardiomyopathy
Glycogen storage
Conduction defects; how signals are transduced inside of the
heart muscle
Micothondiral myopathies
Ion channel disorders
LQTS
Brugada
o Acquired cardiomyopathy
Inflammatory (myocarditis)
Stress-provoked; tako tsubo cardiomyopathy (broken heart
syndrome)
Peripartum; problems with the heart around the pregnancy
Tachycardia-induced; when the heart is being stress because it
is it is beating too fast
Infants of insulin-dependent diabetic mothers
o Mixed cardiomyopathy: need genetic and acquired
DCM; dilated cardiomyopathy
Restrictive cardiomyopathy
Secondary cardiomyopathy: cardiomyopathy with known etiology
(oorzaak)/system disease
Remodelling of the heart
Remodelling of the heart takes place when there is a disease
Causes of remodelling of the heart:
Idiopathic cardiomyopathy; we don’t know what the cause is
Ischemic cardiomyopathy; heart muscle is diseased because of reduction of
proper blood flow into the heart
Diabetic cardiomyopathy; related to people with type I and type II diabetes
Familial (genetic) cardiomyopathy
2 forms of remodelling:
1. Eccentric remodelling (systolic heart failure):
heart dilates with large left ventricle – able to
fill well but not able to eject well (reduced
ejection fraction)
2. Concentric remodelling (diastolic heart
failure): heart muscle become enlarged so
less space for blood in the heart– not able to
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