Myocardial Disorders Flashcards
2 classes of primary myocardial disease
- cardiomyopathy (dilated, hypertrophic, restrictive)
2. myocarditis
Primary problem in dilated cardiomyopathy?
systolic failure –> volume overload to increase preload –> all 4 chambers are eccentrically hypertrophied and contraction is worse
Primary problem in hypertrophic cardiomyopathy?
hypertrophy resulting in hypercontractility during systole but poor diastolic LV compliance due to normal ventricular cavity w/tons of concentric hypertrophy and consequent atrial dilation
*normal LVEF
Primary problem in restrictive cardiomyopathy?
poor diastolic compliance due to infiltration of cells (phages/myocytes)/amyloid/collagenthat makes ventricle stiff and consequently lead to atrial dilation
What’s the difference between a primary and secondary cardiomyopathy?
primary is predominantly myocardial vs. secondary is associated w/systemic/multiorgan disease (e.g. amyloidosis, hemochromatosis)
What kinds of proteins are often implicated in primary genetic cardiomyopathies?
- cytoskeletal
- sarcomeric
- ion channels
T/F an individual w/hypertrophic cardiomyopathy may develop DCM late in disease
T
What is the pathogenesis associated with arrhythmogenic RV cardiomyopathy?
defect in desmosomal proteins –> breakdown of myocyte connections (adhesion and communication/gap jxn) in RV –> fat replacement –> RHF + vtach/vfib
What protein is typically responsible in arrhythmogenic RV cardiomyopathy?
plakoglobin
Features of DCM?
- progressive dilation of all four chambers + hypertrophy + increased heart weight
- flabby walls
- endocardial thrombi
- hypertrophied myocytes + stretched thin myocytes + interstitial fibrosis
- reduced LVEF
What are common causes of secondary DCM?
toxins like ethanol, adriamycin, prior myocarditis, pregnancy, hemochromatosis
Pathogenesis of DCM in hemochromatosis
excess iron absorption –> cardiac deposition –> interference w/ metal dependent enzymes for ox phos
- diabetes and cirrhosis
Complications of DCM
- heart failure
- mitral insufficiency
- dysrhythmia
- emboli
Hallmark of Takotsubo cardiomyopathy
catecholamine excess –> reversible systolic dysfunciton with apical ballooning –> ECG looks like an MI
Features of HCM?
- stiff LV w/ impaired filling
- hypercontractile LV
a. w/ LV outflow obstruction in 1/3 of cases–> IHSS/HOCM
b. w/ interventricular septum effects in 90% of cases–> ASH (no dilation but asymmetric hypertrophy) - myocyte hypertrophy and disarray w/ branching
- thickened intramyocardial arteries leading to angina, dysrhythmias
What is the marker of LVOT obstruction in someone with HCM?
septal scar from repeated battering from the anterior leaflet of MV –> bacterial endocarditis