Cardiomyopathies Flashcards

1
Q

Chagas’ disease -D (4)

A

– Protozoan infection Trypanosoma cruzi
– affects, heart, esophagus, colon.
- acute phase with fever, myalgias, hepatosplenomegaly, myocarditis.
- Apical aneurysm. 

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Duchenne’s myotonic dystrophy - D (4)

A
  • 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. 
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Fabry’s disease - H (4)

A

– 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. 

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Amyloid -R (8)

A

– 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. 

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Sarcoidosis – R (5)

A

– 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. 

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

LV compaction (5)

A

– 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.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Takotsubo

A

> 80% of cases are women, typically 50–75 years.
– apical dilatation, systolic dysfunction = significant reduction EF

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

ARVD/ARVCM (5)

A

– 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. 

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Restrictive cardiomyopathy

A

– diastolic>systolic dysfunction.
– later systolic dysfunction.
– pulmonary systolic pressure usually moderate/severe elevation.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Hemachromatosis

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Fabry dz imaging/thx

A

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.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Endomyocardial fibrosis

A

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.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Radiation cardiotoxicity

A

Can present as either restriction CM or constrictive pericarditis
My cardio fibrosis >right than left

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Idiopathic restrictive CM

A

Biopsy necessary to rule out others.
Medical treatment.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Cardiac sarcoidosis

A

Wall motion abnormalities not in coronary anatomy distribution.
Arrhythmias.
AV block.
Check echo and Holter then CMR if first two are abnormal.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Hypeosinophilic syndrome

A

Eosinophilia >1500 on at least two separate occasions.
No parasites or allergies
Direct damage to the heart muscle from eosinophils.
Can see fibrosis of both RV/LV apices, thickening of posterior mitral valve leaflet/posterior wall.
Specific, chromosomal alteration can respond to Imatinib