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Cardiovascular Flashcards

1
Q

What tool is used to diagnose cardiomyopathy?

A

Endomyocardial biopsy

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2
Q

What genetic mutation is linked to familial Dilated cardiomyopathy?

A

Titan mutations

In the sarcomere, stretches from one Z line to the next

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3
Q

The genetic cause of autosomal dominant dilated cardiomyopathy involves mutations in genes involving which cellular structure?

A

Cytoskeleton

(alters the contractile mechanism of the cell wall)

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4
Q

Which genetic mutation is most common in boys of teenage years with rapidly progressive cardiomyopathy?

A

Mutation in dystrophin gene

Boys with Duchenne and Beckers muscular dystrophy

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5
Q

Mutation of which genes can cause Dilated Cardiomyopathy?

A
  1. Titan (sarcomere)
  2. Cytoskeletal genes
  3. Mitochondrial genes
    • beta oxidation of fatty acids
    • oxidative phosphorylation
  4. Dystrophin
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6
Q

What are the causes of dilated cardiomyopathy?

A
  1. Genetic mutation
  2. Myocarditis (due to coxsackie B or other enteroviruses)
  3. Alcohol abuse
    • beriberi is similar
  4. Drugs
    • Doxorubicin
    • Cocaine
  5. Pregnancy
  6. Iron overload
    • Hemochromatosis
    • multiple blood transfusions
  7. Cobalt exposure (ingestion?)
  8. Supraphysiologic stress
    • tachycardia
    • hyperthyroidism
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7
Q

What are common problems caused by DCM?

A
  • Mural thrombus formation in atria and ventricles
  • Functional mitral or tricuspid regurgitation due to ventricular dilation
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8
Q

What is the primary cardiac abnormality in DCM?

A
  • Impairment of left ventricular function
  • Systolic dysfunction occurs
    • Ventricles cannot pump
    • Ultimately causes biventricular CHF
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9
Q

What is the ejection fraction of end stage DCM?

A

<25%

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10
Q

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) can be caused by defects in genes involving what cell structure?

A

Desmosomes (cell-cell adhesions)

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11
Q

What are the signs/symptoms of Naxos Syndrome? What is the cause?

A
  • Signs/Symptoms
    • Arrhythmogenic cardiomyopathy
    • plantar / palmar hyperkeratosis
  • Cause:
    • mutations in plakoglobin
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12
Q

What are the histological changes of ARVC?

A
  • RV wall is very thinned
    • causes failure and rhythm disturbances
  • Loss ofmyocytes
  • Fatty infiltration
  • Interstitial fibrosis
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13
Q

Hypertrophic Cardiomyopathy (HCM) is caused by mutations affecting what cellular structure? What is the most common mutation?

A
  • Mutations affecting sarcomeres
    • beta-myosin heavy chain is most common mutation
  • AD
  • 100% genetic
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14
Q

How does HCM cause heart failure?

A

Decrease in chamber size and compliance => Decreased SV => HF

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15
Q

What phase of the cardiac cycle is affected by hypertrophic cardiomyopathy? Dilated CM? Restrictive CM?

A
  • HCM: diastolic filling disorder, massive muscle mass prevents filling
  • DCM: Systolic dysfunction, thinned ventricles cannot pump
  • RCM: Diastolic, decreased compliance restricts ventricular filling
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16
Q

Where is hypertrophy usually located in HCM? What can result?

A
  • thickening occurs most often in the IV septum
  • Location is often subaortic
  • Result:
    • aortic outflow obstruction
    • Contact of anterior mitral leaflets with septum (thickening)
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17
Q

What is the histologic characteristic specific to HCM? What happens to individual myocytes?

A
  • Characteristic:
    • Haphazard disarray of myocyte
  • Individual myocytes
    • Hypertrophy
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18
Q

What is the most common cause of unexplained death in young athletes?

A

HCM

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19
Q

What murmur is found in HCM? What is the cause of the murmur?

A
  • Murmur:
    • harsh, systolic ejection murmur
  • Cause
    • anterior mitral leaflet moving towards septum
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20
Q

What are the symptoms of HCM?

A
  • Exertional dyspnea
    • LV outflow obstruction leads to increased pulm venous pressure
  • Angina
    • focal ischemia from hypertrophy (can’t supply whole wall) and abnormal intramural arteries
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21
Q

What major clinical problems are associated with HCM?

A
  • A fib w/ mural thrombus formation
  • Cardiac failure
  • Ventricular arrhythmias
  • Sudden cardiac death
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22
Q

What drug is used to treat HCM?

A

beta blockers (help ventricular relaxation)

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23
Q

Which part of the cardiac cycle is affected by restrictive cardiomyopathy?

A

Diastole

(Cant fill due to decreased compliance)

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24
Q

What causes Restrictive cardiomyopathy?

A
  • Fibrosis
    • sarcoidosis
    • Amyloidosis
  • Hemachromatosis
  • Leukemia or metastatic tumor
  • Storage diseases
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25
Q

What is endomyocardial fibrosis? In what population is it most prominant?

A
  • Fibrosis of endocardium
    • leads to restrictive Cardiomyopathy
    • May involve mitral or tricuspid valves
  • Population
    • children/young adults in Africa and tropical countries
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26
Q

What is Loeffler’s endomyocarditis?

  • Histology
  • Genes involved
  • Complications
  • Treatment
A
  • Histology
    • Endomyocardial fibrosis
    • Eosinophils
    • Toxic agent: major basic protein
  • Genes involved
    • PDGFR tyrosine kinase
  • Complications
    • Restrictive Cardiomyopathy
  • Treatment
    • Imatinib (PDGFR tyrosine kinase inhibitor)
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27
Q

What is the identifying feature of myocarditis caused by Chagas disease?

A

Parasitization of myocytes by trypanosomes

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28
Q

What is the identifying features of Hypersensitivity myocarditis? What are some causes?

A
  • ID:
    • Eosinophils
  • Causes:
    • Methyldopa
    • Sulfonamides
    • (Drugs)
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29
Q

What is the most common cause of myocarditis?

A

Viral

  • Coxsackie A or B (most common)
  • Poliovirus
  • Influenza A and B
  • CMV
  • HIV
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30
Q

What are infectious/parasitic causes of myocarditis?

A
  1. Chagas (S. america)
  2. Trichinosis (rare)
  3. Corynebacterium diphtheriae
    • Patchy myocyte necrosis
    • Sparse lymphocytic infiltrate (normally huge!)
  4. Lyme disease
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31
Q

What is the histologic pattern of myocarditis?

A
  • Focal myocyte necrossis
  • Predominantly lymphocytic inflammatory infiltrate
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32
Q

What is a late phase complication of myocarditis?

A

DCM

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33
Q

Which drug can cause myocarditis after a single high dose?

A

Cyclophosphamide

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34
Q

Amyloidosis can produce which type of heart disease? This results from accumulation of what? How is it visualized?

A
  • Heart disease:
    • Restrictive cardiomyopathy
  • Accumulation of:
    • Senile isolated cardiac amyloidosis: WT transthyretin (atria and ventricles)
    • Mutated transthyretin (atria only)
  • Visualized:
    • Polarized light: apple green birefringence
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35
Q

Iron overload

  • Causes what heart complications?
  • Where is the deposition located?
  • What is the gross appearance of the myocardium?
  • What is the histology?
  • How is it visualized?
A
  • Complications
    • Restrictive and Dilated cardiomyopathy
  • Deposition
    • More prominent in ventricles
  • Gross
    • Rust brown myocardium
  • Histo
    • Myocytes w/ hemosiderin
  • Stain
    • Prussian blue
36
Q

How does Thyroid hormone affect the heart?

A
  • Stimulatory
    • tachycardia
    • Palpitations
    • Cardiomegaly
    • Supraventricular arrythmias
  • Histo
    • hypertrophy

Causes DCM

37
Q

Whatis the histologic effect of hypothyroidism on the heart?

A

Myxedema heart

  • Myocytes swell
  • Basophilic degeneration
  • Mucopolysaccharide rich edematous fluid in interstitium
38
Q

What can occur if fluid accumulates rapidly in the pericardium?

A

Cardiac tamponade

39
Q

WHat is the most common cause of primary pericarditis?

40
Q

A loud pericardial friction rub is characteristic of which cardiac pathology?

A

Fibrinous pericarditis

41
Q

What is the most common cause of acute pericarditis with a hemorrhagic exudate?

A

Spread of malignant neoplasm

(also TB, cardiac surgery)

42
Q

What are the causes of adhesive mediastinopericarditis?

A
  1. infections
  2. Cardiac surgery
  3. Radiation therapy
43
Q

What is the most common finding of Rheumatoid heart disease?

A

Fibrinous pericarditis

(thickening of pericardium)

44
Q

What valve is affected by rhematoid valvulitis?

45
Q

What cardiac lesion is associated with SLE in the adult and consists of nonbacterial verrucous vegetations on both sides of the valve?

A

Libman Sacks endocarditis

Characteristic: vegetations on both sides of valve

46
Q

What is the most common primary tumor of the heart in adults?

47
Q

What are the genetic mutations that predispose to myxoma?

A
  1. GNAS1 activating mutation
  2. PRKAR1A null mutations (carney complex)
48
Q

Where are papillary fibroelastomas generally located?

A

On the valves

49
Q

What are the effects of chemo on the heart?

A

Myocarditis

50
Q

What are the effects of radiation on the heart?

A
  1. Pericarditis
  2. Pericardial effusions
  3. Myocardial fibrosis
  4. Accelerated coronary artery disease
51
Q

What genes are responsible for the first heart field?

52
Q

What genes are responsible for the Second heart field?

A

Hand 2, FGF-10

53
Q

What strutures arise from the first heart field?

54
Q

What structures are formed by the second heart field?

A
  • RV
  • aorta
  • pulm. artery
55
Q

What is the conotruncal ridge responsible for?

A

It spirals and forms the outflow tract of the heart (aorta and pulm artery)

56
Q

Which genes control atrial and ventricular septal formation?

A

NKX and TBX

57
Q

Atrial Septal Defect is most likely due to a problem in which structure?

A

Septum secundum

58
Q

Coarctation of the aorta is most commonly associated with what genetic disorder?

A

Turners syndrome

59
Q

In the coronary artery occlusion sequence, what induces vasospasm?

A

Thromboxane A2

60
Q

What are the causes of subendocardial infarction?

A
  1. Occlusion sequence with clot lysis before damage to full wall thickness
    • infarct in watershed
  2. Sufficiently prolonged severe reduction in BP (shock) and chronic stenosis
    • Circumferential
61
Q

What is the most common cause of deaths in the hour following acute MI?

62
Q

Ischemic heart damage is most likely to occur where?

63
Q

What type of pericarditis is caused by MI?

A

Fibrinous

or

Fibrohemorrhaic

64
Q

After an MI, when is myocardial rupture most common?

A

3-7 days post MI

65
Q

What is the most common cause of rhythm disorders?

A

Ischemic injury

66
Q

What is the ultimate mechanism in sudden cardiac death?

A

Trigger for lethal arrhythmia

67
Q

What is Romaneo-Ward syndrome?

A

Autosomal dominant long QT syndrome

(causes episodic ventricular arrhythmias)

68
Q

What is the most common cause of aortic stenosis?

A

Calcification of a bicuspid valve

69
Q

What is the most common cause of aortic insufficiency?

A

Dilation of the ascending aorta due to HTN or aging

70
Q

What is the most common cause of Mitral stenosis?

A

Rheumatic heart disease

71
Q

What is the most common cause of mitral insufficiency?

A

Myxomatous degeneration (mitral valve prolapse)

72
Q

What is the most common valve abnormality?

A

Aortic Stenosis

(primarily senile calcific aortic stenosis)

73
Q

How is degenerative aortic stenosis different from rheumatic aortic stenosis?

A

Commisural fusion occurs only in Rheumatic stenosis

Rheumatic may also have mitral valve involvement

74
Q

In myxomatous mitral valves, which layer of thevalveis thickened?

A

Spongiosa layer (deposition of mucoid material)

75
Q

When do patients present with symptoms in senile calcific aortic stenosis vs calcific stenosis of bicuspid aorta?

A
  • Senile: 7th-9th decade
  • Bicuspid: 6th-7th
76
Q

What complications are associated with a bicuspid aortic valve?

A
  • Stenosis
  • Regurgitation
  • Infective endocarditis
  • Aortic dilation
77
Q

What complications are associated with mitral annular calcification?

A
  • Regurgitation
    • Interfering with mitral ring function
  • Stenosis
    • impaired opening
  • Sudden death
    • penetration of myocardium and disruption of conduction system
78
Q

What complications are associated with a myxomatous mitral valve?

A
  • Infective endocarditis ***
  • Mitral insufficiency (some chordal rupture with billowing)
  • Stroke / thrombus
  • Arrhythmia
79
Q

Which bugs cause acute infective endocarditis?

A
  • Staph aureus
  • Pseudomonas
  • streptococci
  • streptococcus intermedius group
80
Q

What is Trousseau syndrome?

A

Migratory thrombophlebitis in visceral cancers

Associated with NBTE

81
Q

Which valves are affected by carcinoid heart disease?

A

Tricuspid and pulmonic

82
Q

Secretion of what factors results in Carcinoid Syndrome? What symptoms are present?

A
  • Factors secreted by tumor:
    • Serotonin
    • Kallikrein
  • Symptoms:
    • Flushing
    • Nausea
    • Vomiting
    • Diarrhea
83
Q

What complications are associated with artificial heart valves?

A
  • Thromboembolism (more common with mechanical)
  • IE (Staph. Epidermis)
  • Strutural degeneration (bioprosthesis)
  • Paravalvular leak
  • Intravascular hemolysis
  • Relative stenosis
84
Q

Mutations in genes involving which structures only result in DCM?

A
  1. Titan
  2. Dystrophin
  3. Desmin
  4. Lamin
  5. Sarcoglycan
  6. Mitochondrial proteins
85
Q

Mutations in genes involving which structures only cause Hypertrophic cardiomyopathy?

A
  1. Myosin binding protein C
  2. Myosin light chains
  3. (beta-myosin heavy chain is most common but could cause DCM)
86
Q

Mutations in genes involving which structures cause both DCM and HCM?

A
  1. Beta myosin heavy chain
  2. Troponin I/T
  3. Alpha tropomyosin
  4. Actin

Pathology (27 decks)

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