Pathology > Cardiovascular > Flashcards

Cardiovascular Flashcards

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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
What is endomyocardial fibrosis? In what population is it most prominant?
* Fibrosis of endocardium * leads to restrictive Cardiomyopathy * May involve mitral or tricuspid valves * Population * children/young adults in **Africa** and **tropical countries**
26
What is Loeffler's endomyocarditis? * Histology * Genes involved * Complications * Treatment
* Histology * Endomyocardial fibrosis * Eosinophils * Toxic agent: major basic protein * Genes involved * PDGFR tyrosine kinase * Complications * Restrictive Cardiomyopathy * Treatment * Imatinib (PDGFR tyrosine kinase inhibitor)
27
What is the identifying feature of myocarditis caused by Chagas disease?
Parasitization of myocytes by trypanosomes
28
What is the identifying features of Hypersensitivity myocarditis? What are some causes?
* ID: * Eosinophils * Causes: * Methyldopa * Sulfonamides * (Drugs)
29
What is the most common cause of myocarditis?
Viral * Coxsackie A or B (most common) * Poliovirus * Influenza A and B * CMV * HIV
30
What are infectious/parasitic causes of myocarditis?
1. Chagas (S. america) 2. Trichinosis (rare) 3. Corynebacterium diphtheriae * Patchy myocyte necrosis * Sparse lymphocytic infiltrate (normally huge!) 4. Lyme disease
31
What is the histologic pattern of myocarditis?
* Focal myocyte necrossis * Predominantly lymphocytic inflammatory infiltrate
32
What is a late phase complication of myocarditis?
DCM
33
Which drug can cause myocarditis after a single high dose?
Cyclophosphamide
34
Amyloidosis can produce which type of heart disease? This results from accumulation of what? How is it visualized?
* 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
35
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?
* Complications * Restrictive and Dilated cardiomyopathy * Deposition * More prominent in ventricles * Gross * Rust brown myocardium * Histo * Myocytes w/ hemosiderin * Stain * Prussian blue
36
How does Thyroid hormone affect the heart?
* Stimulatory * tachycardia * Palpitations * Cardiomegaly * Supraventricular arrythmias * Histo * hypertrophy Causes DCM
37
Whatis the histologic effect of hypothyroidism on the heart?
Myxedema heart * Myocytes swell * Basophilic degeneration * Mucopolysaccharide rich edematous fluid in interstitium
38
What can occur if fluid accumulates rapidly in the pericardium?
Cardiac tamponade
39
WHat is the most common cause of primary pericarditis?
Viral
40
A loud pericardial friction rub is characteristic of which cardiac pathology?
Fibrinous pericarditis
41
What is the most common cause of acute pericarditis with a hemorrhagic exudate?
Spread of malignant neoplasm | (also TB, cardiac surgery)
42
What are the causes of adhesive mediastinopericarditis?
1. infections 2. Cardiac surgery 3. Radiation therapy
43
What is the most common finding of Rheumatoid heart disease?
Fibrinous pericarditis | (thickening of pericardium)
44
What valve is affected by rhematoid valvulitis?
Aortic
45
What cardiac lesion is associated with SLE in the adult and consists of nonbacterial verrucous vegetations on both sides of the valve?
Libman Sacks endocarditis Characteristic: vegetations on both sides of valve
46
What is the most common primary tumor of the heart in adults?
Myxoma
47
What are the genetic mutations that predispose to myxoma?
1. GNAS1 activating mutation 2. PRKAR1A null mutations (carney complex)
48
Where are papillary fibroelastomas generally located?
On the valves
49
What are the effects of chemo on the heart?
Myocarditis
50
What are the effects of radiation on the heart?
1. Pericarditis 2. Pericardial effusions 3. Myocardial fibrosis 4. Accelerated coronary artery disease
51
What genes are responsible for the first heart field?
Hand1
52
What genes are responsible for the Second heart field?
Hand 2, FGF-10
53
What strutures arise from the first heart field?
* RA * LA * LV
54
What structures are formed by the second heart field?
* RV * aorta * pulm. artery
55
What is the conotruncal ridge responsible for?
It spirals and forms the outflow tract of the heart (aorta and pulm artery)
56
Which genes control atrial and ventricular septal formation?
NKX and TBX
57
Atrial Septal Defect is most likely due to a problem in which structure?
Septum secundum
58
Coarctation of the aorta is most commonly associated with what genetic disorder?
Turners syndrome
59
In the coronary artery occlusion sequence, what induces vasospasm?
Thromboxane A2
60
What are the causes of subendocardial infarction?
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
What is the most common cause of deaths in the hour following acute MI?
Arrhyhmia
62
Ischemic heart damage is most likely to occur where?
LV
63
What type of pericarditis is caused by MI?
Fibrinous or Fibrohemorrhaic
64
After an MI, when is myocardial rupture most common?
3-7 days post MI
65
What is the most common cause of rhythm disorders?
Ischemic injury
66
What is the ultimate mechanism in sudden cardiac death?
Trigger for lethal arrhythmia
67
What is Romaneo-Ward syndrome?
Autosomal dominant long QT syndrome (causes episodic ventricular arrhythmias)
68
What is the most common cause of aortic stenosis?
Calcification of a bicuspid valve
69
What is the most common cause of aortic insufficiency?
Dilation of the ascending aorta due to HTN or aging
70
What is the most common cause of Mitral stenosis?
Rheumatic heart disease
71
What is the most common cause of mitral insufficiency?
Myxomatous degeneration (mitral valve prolapse)
72
What is the most common valve abnormality?
Aortic Stenosis (primarily senile calcific aortic stenosis)
73
How is degenerative aortic stenosis different from rheumatic aortic stenosis?
**Commisural fusion** occurs only in Rheumatic stenosis Rheumatic may also have **mitral valve involvement**
74
In myxomatous mitral valves, which layer of thevalveis thickened?
Spongiosa layer (deposition of mucoid material)
75
When do patients present with symptoms in senile calcific aortic stenosis vs calcific stenosis of bicuspid aorta?
* Senile: 7th-9th decade * Bicuspid: 6th-7th
76
What complications are associated with a bicuspid aortic valve?
* Stenosis * Regurgitation * Infective endocarditis * Aortic dilation
77
What complications are associated with mitral annular calcification?
* Regurgitation * Interfering with mitral ring function * Stenosis * impaired opening * Sudden death * penetration of myocardium and disruption of conduction system
78
What complications are associated with a myxomatous mitral valve?
* Infective endocarditis \*\*\* * Mitral insufficiency (some chordal rupture with billowing) * Stroke / thrombus * Arrhythmia
79
Which bugs cause acute infective endocarditis?
* Staph aureus * Pseudomonas * streptococci * streptococcus intermedius group
80
What is Trousseau syndrome?
Migratory thrombophlebitis in visceral cancers Associated with NBTE
81
Which valves are affected by carcinoid heart disease?
Tricuspid and pulmonic
82
Secretion of what factors results in Carcinoid Syndrome? What symptoms are present?
* Factors secreted by tumor: * Serotonin * Kallikrein * Symptoms: * Flushing * Nausea * Vomiting * Diarrhea
83
What complications are associated with artificial heart valves?
* Thromboembolism (more common with mechanical) * IE (Staph. Epidermis) * Strutural degeneration (bioprosthesis) * Paravalvular leak * Intravascular hemolysis * Relative stenosis
84
Mutations in genes involving which structures only result in DCM?
1. Titan 2. Dystrophin 3. Desmin 4. Lamin 5. Sarcoglycan 6. Mitochondrial proteins
85
Mutations in genes involving which structures only cause Hypertrophic cardiomyopathy?
1. Myosin binding protein C 2. Myosin light chains 3. (beta-myosin heavy chain is most common but could cause DCM)
86
Mutations in genes involving which structures cause both DCM and HCM?
1. Beta myosin heavy chain 2. Troponin I/T 3. Alpha tropomyosin 4. Actin
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