Cardiomyopathies Flashcards
Chagas’ disease -D (4)
– Protozoan infection Trypanosoma cruzi
– affects, heart, esophagus, colon.
- acute phase with fever, myalgias, hepatosplenomegaly, myocarditis.
- Apical aneurysm. 
Duchenne’s myotonic dystrophy - D (4)
- inherited myopathic disorder affecting both skeletal/cardiac muscle.
– can have asymptomatic LV dysfunction b/c limited physical activity. - HF and arrhythmias seen
– looks like dilated cardiomyopathy. 
Fabry’s disease - H (4)
– Inherited, X-linked glycolipid storage disease.
– presents in women later with unexplained LV hypertrophy
– dx by plasma alpha – galactosidase A activity.
– conduction system abnormalities and arrhythmias common. 
Amyloid -R (8)
– Extracellular, tissue deposition of serum protein, subunit, fibrils
– 50% cardiac involvement in primary AL amyloidosis/monoclonal light chains
– 5% with secondary a a amyloid
– conduction system disease.
– myocardial involvement.
– progressive diastolic dysfunction.
– valve thickening.
– strain pattern with preserved apical function. 
Sarcoidosis – R (5)
– Systemic disease + pulmonary involvement.
– subclinical cardiac involvement up to 20%
– conduction system, abnormalities, ventricular arrhythmia‘s, HF
– non-coronary disease pattern with regional WMA.
– both systolic/diastolic dysfunction. 
LV compaction (5)
– Rare, primary genetic, CM
– HF, angina, arrhythmias, thromboembolic events.
- deep ventricular trabeculations/hypokinesis, particularly inferior/lateral walls.
– EF may be reduced.
- Non-compacted: compacted myocardium, > 2:1 at end systole in short axis view.
Takotsubo
> 80% of cases are women, typically 50–75 years.
– apical dilatation, systolic dysfunction = significant reduction EF
ARVD/ARVCM (5)
– Familial inheritance at least 30%
– fibrofatty replacement of RV, most often AD pattern.
– presents with SCD or ventricular arrhythmia’s
– Echo findings nonspecific
– needs MRI and EP evaluation. 
Restrictive cardiomyopathy
– diastolic>systolic dysfunction.
– later systolic dysfunction.
– pulmonary systolic pressure usually moderate/severe elevation.
Hemachromatosis
One of the few restrictive cardiomyopathy is reversible with treatment.
Single mutation in the HFE gene located on chromosome 6 at position 282 of the HFE protein.
Normal LV wall thickness, + LV dilatation
Fabry dz imaging/thx
2-Layered or binary appearance in the endocardium of the LV.
(Likely deposits of glycosphingolipid)
CMR with late gadolinium enhancement, basilar inferio-lateral wall.
Enzyme replacement therapy early in disease, can reduce LV, hypertrophy and improvement in function.
Endomyocardial fibrosis
Endocardium fibrosis, especially at the apices of the LV/RV-distinctive 2D appearance
Tropical
Can have exudative ascites without peripheral edema.
Exudative pericardial, effusion common.
Radiation cardiotoxicity
Can present as either restriction CM or constrictive pericarditis
My cardio fibrosis >right than left
Idiopathic restrictive CM
Biopsy necessary to rule out others.
Medical treatment.
Cardiac sarcoidosis
Wall motion abnormalities not in coronary anatomy distribution.
Arrhythmias.
AV block.
Check echo and Holter then CMR if first two are abnormal.