Cardiomyopathies and sutff Flashcards

1
Q

What is the ventricular morphology of dilated cardiomyopathy?

A

Dilated LV with little hypertrophy

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2
Q

What is the ventricular morphology of arrhythmogenic RVCM?

A

dilated RV with sections of missing muscle.

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3
Q

What is the ventricular morphology of HCM?

A

Marked hypertrophy.

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4
Q

What is the ventricular morphology of RCM?

A

fibrotic/infiltrated myocardium

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5
Q

What are the symptoms of DCM?

A

Fatigue, weakness, dyspnea, orthopnea, paroxysmal nocturnal dyspnea

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6
Q

What are the symptoms of ARVCM?

A

R heart failure
Palpatations
Syncope
Sudden death

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7
Q

What are the symptoms of HCM?

A
Exertional dyspnea
Angina
Syncope
A-Fib, V-Fib
CHF
Sudden Death (1.5%)
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8
Q

What are the symptoms of RCM?

A

Dyspnea

Fatigue

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9
Q

What presents in the physical exam for DCM?

A

pulmonary rales
s3
functional regurge
possible RV failure signs

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10
Q

What presents in the physical exam for ARVCM?

A

rhythm disturbances (ventricular arrhythmias)

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11
Q

What presents in the physical exam for HCM?

A

S4

Outflow obstruction: systolic murmur and mitral regurgitation

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12
Q

What presents in the physical exam for RCM?

A

Signs of RV failure
JVD
Hepatomegaly
Peripheral edema

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13
Q

What is the pathophysiology of DCM?

A

imparied systolic contraction

mural thrombi is common

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14
Q

What is the pathophysiology of ARVCM?

A

nothing.

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15
Q

What is the pathophysiology of HCM?

A

impaired diastolic relaxation

LV systolic function vigorous with dynamic obstruction of mitral valve during mid-to-late systole

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16
Q

What is the pathophysiology of RCM?

A

“stiff” LV
impaired diastolic relaxation
normal systolic function

17
Q

What is the cardiac size for DCM?

A

Dilated/enlarged

18
Q

What is the cardiac size for HCM?

A

normal or dilated

19
Q

What is the cardiac size for RCM?

A

usually normal

20
Q

What does the echo show for DCM?

A

dilated, poorly contractile LV

21
Q

What does the echo show for HCM?

A

LV hypertrophy more pronounced in septum (acquired) or concentric (genetic)

Systolic anterior movement of MV with mitral regurge

22
Q

What does the echo show for RCM?

A

Usually normal systolic contraction

Speckled appearance in infiltrative disorders

23
Q

What does the histology show for DCM?

A

Interstitial fibrosis

replacement fibrosis

24
Q

What does the histology show for ARVCM?

A

myocyte atrophy

fibrosis

fatty tissue

25
Q

What does the histology show for HCM?

A

increased diameter of myocytes

abnormal arteries

myofiber disarray

26
Q

What does the histology show for RCM?

A

interstitial fibrosis

27
Q

What is the pathogenesis for DCM?

A
  • often in 30s and 40s
  • genetic, usually autosomal dominant
  • in kids with DCM, often mitochondrial gene mutations
  • infectious: coxsackie, adenovirus, parvovirus, HIV
  • iron overload
  • child birth
28
Q

What is the pathogenesis for ARVCM?

A
  • autosomal dominant with variable penetrance
  • defective desmosomes > detachment of myocytes at intercalated discs > myocyte death and fibrosis
  • disease occurs in bursts
29
Q

What is the pathogenesis for HCM?

A
  • heart thickening usually happens in kids
  • autosomal dominant mutation of sarcomeric genes (B-myosin heavy chain, myosin binding protein C, Troponin T)
  • *Troponin T is important**
30
Q

What is the pathogenesis for RCM?

A
  • genetics, involving Troponin I and desmin genes
  • amyloidosis
  • endomyocardial fibrosis
  • Loeffler endomyocarditis: hypereosinophilia due to release of granulocytes
31
Q

What is the treatment for ARVCM?

A

none (80% dies of arrhythmias, 20% die of CHF)

32
Q

What is the treatment for HCM?

A
  • implanted automatic defibrillator
  • negative inotropic agents
  • myocardial reduction with surgery or alcohol
33
Q

What is the treatment for RCM?

A

Can be treated with platelet derived growth factor