Lec 9 Cardiomyopathy Pathology Flashcards

1
Q

What is a cardiomyopathy?

A

structural or functional abnormality of myocardium independent of other valvular/coronary/myocardial involvement in systemic disease

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2
Q

What are 3 types of cardiomyopathy?

A
  • dilated
  • hypertrophic
  • restrictive
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3
Q

What is most common type of cardiomyopathy?

A

dilated

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4
Q

Who gets dilated cardiomyopathy [DCM]?

A
  • may occur at any age
  • most common males 20-50
  • often idiopathic or congenital
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5
Q

What are findings of dilated cardiomyopathy?

A
  • left ventricular dilation
  • systolic dysfunction [EF < 50%]
  • S3
  • heart failure
  • dilated heart on echo
  • balloon appearance of heart on CXR
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6
Q

What are causes of dilated cardiomyopathy?

A
  • idiopathic or congenital
  • ABCCCD
  • – Alcohol abuse
  • – wet bebriberi [thiamine deficiency]
  • – coxsackie B virus myocarditis
  • – chronic cocaine use
  • – chagas disease
  • – doxorubicin toxicity
  • hemochromatosis
  • peripartum cardiomyopathy
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7
Q

What is major genetic cause of dilated cardiomyopathy?

A

mutation in TTN gene that encodes titin

autosomal dominant

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8
Q

What are pathological findings of dilated cardiomyopathy?

A
  • enlarged globular heart [2-3x normal weight]
  • 4 chamber dilation + hypertrophy
  • thin flabby walls
  • annular dilation of atrial-ventricular valves
  • eccentric hypertrophy
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9
Q

What are histo features of dilated cardiomyopathy?

A
  • hypertrophied myocytes
  • boxcar nuclei
  • some myocytes stretched and irregular
  • interstitial fibrosis
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10
Q

What are major infectious causes of dilated cardiomyopathy?

A

viral myocarditis: coxsackie B, adenovirus

parasite: T. cruzi = chagas disease

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11
Q

What are some toxins that can cause dilated cardiomyopathy?

A

alcohol or cocaine use

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12
Q

What are mechs of alcoholic dilated cardiomyopathy?

A
  • direct toxic affect of alcoholic
  • secondary nutritional thiamine deficiency
  • alcohol additives
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13
Q

Who gets dilated cardiomyopathy from alcohol?

A

men 30-55 with > 10 yrs of alcohol consumption

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14
Q

If sudden cardiac death in young athlete what should you think?

A

hypertrophic cardiomyopathy

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15
Q

Who gets hypertrophic cardiomyopathy?

A

100% familial/genetic
males > females
young atheletes

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16
Q

What happens in hypertrophic cardiomyopathy?

A

left ventricle hypertrophy

diastolic dysfunction = can’t fill the heart

17
Q

What is genetics of hypertrophic cardiomyopathy?

A

mutation in sarcomere protein for myosin heavy chain

autosomal dominant

18
Q

What are path findings of hypertrophic cardiomyopathy?

A

hypertrophy of myocardium

  • asymmetrical hypertrophy beflow aortic valve in most pts
  • greater hypertrophy of interventricular septum than free LV wall
  • aberrant myofibers in conduction system –> fatal arrhythmia + sudden death
19
Q

What is most common cause of restrictive cardiomyopathy?

A

amyloidosis

20
Q

What are path findings of restrictive cardiomyopathy?

A
  • ventricles normal or slightly enlarged
  • myocardium firm and normal thickness
  • bi-atrial dilation because poor ventricular filling and pressure overloads
  • patchy areas of fibrosis
21
Q

What is arythmogenic right ventricular cardiomyopathy [ARVC]?

A

inherited cardiomypathy

  • causes RV failure and rhythm disturbances [ventricular fibrillation or tachycardia]
  • fatty and fibrous tissue replace heart muscle
  • can lead to suddne death
  • usually in young people
  • autosomal dom mut in desmosomal protein
22
Q

What is isolated left ventricular noncompaction [LVNC]?

A

congentical myocardial disorder causes CM in children

persistence of noncompacted endocardial layer charactersisc of early fetal period

LV dilated or hypertrophied

sponge-like appearance of LV wall

23
Q

If you see eosinophils in cardiomyopathy what should you think?

A

hypersensitiviy

parasites [Chagas]

24
Q

If you see lymphocytes in cardiomyopathy what should you think?

A

viral

25
Q

If you see giant cells in cardiomyopathy what should you think?

A

sarcoidosis

giant cell myocarditis