Non-Ischemic Myocardial Disease Flashcards

1
Q

the most common cause of myocarditis in the US is _____ which is caused mainly by ______

A

the most common cause of myocarditis in the US is viral myocarditis which is caused mainly by Coxsackie A & B

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2
Q

describe the pathogenesis of viral myocarditis

A
  • direct viral cytotoxicity
  • cell-mediated immune rxns against infected myocytes
  • cytokines release can aggravate myocardial dysfunction
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3
Q
A
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4
Q

describe clinical features of viral myocarditis

A
  • clinical features:
    • varies from asymptomatic to sudden acute heart failure or arrhythmias
    • usually nonspecific symptoms
      • flu-like
      • fatigue, dyspnea, palpitations, chest pain, fever
      • may mimic acute MI
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5
Q

describe investigations of viral myocarditis

A
  • investigations:
    • PCR for viral nucleic acids
    • serologic studies
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6
Q

describe complications of viral myocarditis

A
  • acute heart failure
  • viral myocarditis may lead to dilated cardiomyopathy → chronic CHF
  • arrhythmias: ventricular arrhythmias are the most dangerous → SCD
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7
Q

describe myocarditis caused by Trichinella spiralis (Trichinosis)

A
  • most common helminth associated with myocarditis
  • histologically: eosinophils predominance
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8
Q

describe myocarditis caused by Trypanosoma cruzi (Chaga’s disease)

A
  • endemic in South America
  • myocardium is involved in majority of cases
  • parasitization of scattered myofibers by trypanosomes accompanied by a mixed inflammatory infiltrate
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9
Q

describe myocarditis caused by Toxoplasmosis (Toxoplasma gondii)

A
  • household cats are the most common vector
  • causes myocarditis in immunocompromised
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10
Q
A
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11
Q

fungal myocarditis occurs in ____ individuals

describe the cells seen

A

fungal myocarditis occurs in immunocompromised individuals

  • morphology is characterized by the presence of mixed inflammatory infiltrate composed of neutrophils, lymphocytes and plasma cells
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12
Q

describe immunologically mediated myocarditis

A
  • immunologically mediated
    • SLE, polymyositis
    • drug hypersensitivity:
      • methyldopa, sulfonamides
      • mild, self-limiting
      • composed of lymphocytes, macrophages and a high proportion of eosinophils
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13
Q

describe giant cell myocarditis

A
  • idiopathic
  • associated with SLE, thyrotoxicosis
  • fatal disease of adults in their 3rd or 5th decades
  • cause of death = CHF or arrhythmias
  • histologically:
    • granulomatous inflammation with many giant cells, lymphocytes, eosinophils and plasma cells
    • focal to extensive necrosis
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14
Q
A
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15
Q

describe dilated cardiomyopathy (DCM)

A
  • also called congestive cardiomyopathy
  • characterized by:
    • progressive cardiac dilation
    • eccentric hypertrophy
    • contractile (systolic) dysfunction
  • leading to progressive heart failure
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16
Q

name the etiopathogenesis of DCM

A
  • idiopathic: majority of cases
  • genetic mutations = 25-35% of cases
    • most common: AD inheritance of mutations affecting cytoskeletal proteins
    • less common: X-linked DCM due to mutations in the dystrophin gene
    • uncommon: mutation of mt proteins involved in oxidative phosphorylation
17
Q

____ deficiency and chronic ____ can lead to cardiomyopathy

A

thiamine deficiency and chronic anemia can lead to cardiomyopathy

18
Q

____, a chemotherapy drug, can cause cardiomyopathy

A

doxorubicin, a chemotherapy drug, can cause cardiomyopathy

19
Q

describe peripartum cardiomyopathy

A
  • occurs in the later period of gestation to first few weeks after delivery
  • due to pregnancy-associated
    • volume overload
    • HTN
    • nutritional disturbances
  • 50% recover spontaneously
20
Q
A
21
Q
A
22
Q

describe clinical features of DCM

A
  • affects mostly individuals between 25-50 years
  • fundamental defect is ineffective contraction
  • ejection fraction is <25% (normal = 50-65%)
  • progressive CHF refractory to therapy, usually end-stage by the time of clinical discovery
  • treatment = cardiac transplantation
23
Q

describe arrhythmogenic right ventricular cardiomyopathy (ARVC)

A
  • AD disorder
  • characterized by thin RV due to myocyte replacement by fatty infiltration → right-sided HF and arrhythmia → SCD
  • defect in desmosomal adhesion proteins
  • associated with Naxos Syndrome
24
Q

in ARVC, there is a defect in _____ and is associated with _____

A

in ARVC, there is a defect in desmosomal adhesion proteins and is associated with Naxos Syndrome

25
Q
A
26
Q

describe hypertrophic cardiomyopathy (HCM)

A
  • characterized by:
    • myocardial hypertrophy
    • abnormal diastolic filling
    • in 30% of cases there is intermittent left ventricular outflow obstruction (caused by anterior leaflet of MV)
  • primarily a problem with impaired diastolic filling rather than systolic dysfunction
  • overall size of LV chamber is small
  • presents as exertional dyspnea or sudden death in young adults
27
Q

describe the etiology of HCM

A
  • familial AD with variable expression in most cases
  • occurs due to missense point mutation in any of the following genes coding for sarcomeric proteins:
    • B-myosin heavy chain = most common
    • myosin-binding protein C
    • cardiac troponin T
28
Q

describe the pathogenesis of HCM

A
  • pathogenesis of HCM
    • gain of function mutation of the sarcomeric proteins → myocyte hypercontractility → increase energy use → intense compensatory hypertrophy (myofiber disarray) and fibroblast proliferation
29
Q

describe the image

A

hypertrophic cardiomyopathy

30
Q

describe the gross morphology of HCM

A
  • gross:
    • massive myocardial hypertrophy (thick walled) without ventricular dilation
    • asymmetric septal hypertrophy: most prominent in the subaortic region
    • on longitudinal section: banana-like configuration of ventricular cavity
    • endocardial plaque/sclerosis in left outflow tract
31
Q
A

HCM: disarray, extreme hypertrophy and characteristic branching of myocytes, as well as interstitial fibrosis

32
Q

describe the clinical features and treatment of HCM

A
  • clinical features
    • exertional dyspnea
    • angina or MI
    • harsh ejection systolic murmur
    • sudden death due to arrhythmias
    • CHF
  • treatment: therapy to relax the ventricles, partial septal muscle excision to reduce the outflow obstruction
33
Q

describe restrictive cardiomyopathy

A
  • least common type of cardiomyopathy
  • characterized by stiff walls → loss of ventricular compliance → impaired ventricular filling during diastole (like constrictive pericarditis, HCM) → diastolic dysfunction
  • systole is not forceful → systolic dysfunction
34
Q

____ is the most common cause of RCM

list other causes of RCM

A

endomyocardial fibrosis is the most common cause of RCM

  • Loffler’s syndrome
  • radiation fibrosis
  • amyloidosis
  • hemochromatosis (can cause DCM)
  • metastatic tumors
35
Q

list clinical features and complications of RCM

A
  • clinical features of RCM
    • exertional dyspnea
    • fatigue
    • chest pain
  • complications:
    • arrhythmias
    • CHF
36
Q

describe the morphology and diagnosis of RCM

A
  • morphology of RCM
    • ventricles normal in size or slightly enlarged
    • cavities are not dilated
    • myocardium is firm
    • both atria dilated
    • patchy variable interstitial fibrosis
  • diagnosis:
    • endomyocardial biopsy
37
Q

____ is the most common cause of RCM

describe this condition

A

endomyocardial fibrosis (EMF) is the most common cause of RCM

  • type of RCM
  • children and young adults
  • Africa and the tropics
  • dense fibrosis of endocardium and subendocardium from apex to AV valves
  • reduced volume and compliance of affected chambers
38
Q

____ is another form of RCM that leads to peripheral hypereosinophilia

explain this condition

A

Loefflers endomyocarditis is another form of RCM that leads to peripheral hypereosinophilia

  • clinically a RCM
  • endocardial fibrosis with large mural thrombi
  • peripheral hypereosinophilia
  • abnormal degranulated eosinophils
    • MBP from the granules damage the endocardium leading to necrosis and fibrosis