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CPR 2 Exam 1 > Cardiac Pathology 2 > Flashcards

Cardiac Pathology 2 Flashcards

1
Q

Where is the SA node located?

A

Junction of right atrial appendage and superior vena cava

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2
Q

What is the primary pacemaker of the heart?

A

SA node

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3
Q

Where is the AV node located?

A

Right atrium along the interatrial septum

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4
Q

What delays the impulse from SA node to assure the atria contract before the ventricles?

A

AV node

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5
Q

How quickly does the SA node discharge?

A

70 bpm

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6
Q

How quickly does the AV node discharge

A

40-60 bpm

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7
Q

Where is the bundle of His located?

A

Upper part of the ventricular septum

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8
Q

Where does the bundle of his transmit electrical impulses to?

A

Perkinje fibers

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9
Q

What causes arrhythmias?

A

Abnormalities in gap junctions or spatial relationships between myocytes

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10
Q

What are examples of cardiomyopathies?

A

Hypertrophic cardiomyopathy
Dilated cardiomyopathy
Restrictive cardiomyopathy

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11
Q

What causes restrictive cardiomyopathies?

A

Amyloidosis

Sarcoidosis

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12
Q

What do cardiomyopathies cause in the heart?

A

Abnormal spacing and abnormal conduction pathways to form

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13
Q

How does myocarditis and valvular disease cause arrhythmias?

A

Enlargement of cavities and increased spacing between myocytes

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14
Q

How does congenital heart disease cause arrhythmia?

A

Structural abnormalities

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15
Q

What is the most common cause of arrhythmia?

A

Ischemic heart disease

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16
Q

How does MI lead to ventricular fibrillation?

A

Initial myocyte death

Fibrosis -> disturbs normal conduction pathways

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17
Q

What happens in sick sinus syndrome?

A

SA node damaged -> bradycardia

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18
Q

What node becomes the primary pacemaker when the SA node is damaged?

A

AV node

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19
Q

What are the clinical symptoms of bradycardia?

A

Lightheadedness
Palpitations
Fatigue

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20
Q

Sick sinus syndrome is sometimes associated with what?

A

Atrial fibrilation

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21
Q

What arrhythmia occurs with dilation of the atrial cavity causing the myocytes to become spaced out?

A

Atrial fibrilation

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22
Q

In what causes is left atrial dilation leading to a fib common?

A

Left ventricular hypertrophy

Mitral valve disease

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23
Q

What are complications of afib?

A

Thrombus formation

Thromboembolism (stroke)

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24
Q

Where does the rhythm of afib come from?

A

AV node -> irregular, irregular HR

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25
Q

What is seen in a first degree heart block?

A

Prolonged PR interval

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26
Q

What is seen in second degree heart block?

A

Intermittent transmission

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27
Q

What is seen in third degree heart block?

A

Complete failure

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28
Q

What is long QT syndrome

A

Aberrant channel function causes an increased length of ventricular depolarization to repolarization

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29
Q

What does an increased QT interval lead to?

A

Torsades de Pointes

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30
Q

What happens in torsades de pointes?

A

Syncope

Sudden cardiac death (especially during exercise)

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31
Q

What channel dysfunction leads to long QT syndrome?

A

Sodium

Potassium

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32
Q

What is the classic scenario of long QT syndrome?

A

Stronger swimmer who drowns unexpectedly due to undiagnosed arrhythmia

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33
Q

What can trigger torsades de pointes?

A

Low blood calcium

Low blood potassium

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34
Q

What are hereditary chanellopathies?

A

Abnormal ion channels which cause arrhythmogenic disease

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35
Q

What is a sudden cardiac death?

A

Death without previous symptoms or death which occurs within 24 hours of symptom onset

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36
Q

What is the most common cause of sudden cardiac deaths?

A

Coronary artery disease

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37
Q

What causes sudden cardiac death in younger patients?

A 
Drug abuse (cocaine, meth)
Hereditary conduction abnormalities
Hypertrophic or dilated cardiomyopathy
Myocardial hypertrophy
Myocarditis
Mitral valve prolapse (w/chordae tendinae rupture)
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38
Q

What is the criteria for hypertensive heart disease?

A

1) Left ventricular hypertrophy

2) Clinical history or pathologic evidence of hypertension in other organs

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39
Q

What pattern is usually seen with left ventricular hypertrophy?

A

Concentric

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40
Q

What is seen on autopsy with hypertensive heart disease?

A

Enlarged, heavy heart with increased concentric left ventricular wall thickness
Granular kidneys with areas of hyaline arteriosclerosis

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41
Q

Diastolic dysfunction with hypertensive heart disease leads to what?

A

Atrial dilation -> afib -> congestive heart disease -> pulmonary edema
Sudden cardiac death

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42
Q

What happens with cardiac myocyte hypertrophy?

A

Increase in cell size

Increase in nuclear size

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43
Q

What is cor pulmonale?

A

Right sided pulmonary hypertensive disease which leads to heart dysfunction and failure

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44
Q

What causes of cor pulmonale are due to pulmonary parenchyma?

A 
COPD
Diffuse pulmonary interstitial fibrosis
Pneumonconioses
Cystic fibrosis
Bronchiectasis
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45
Q

What causes of cor pulmonale are due to pulmonary vessels?

A 
Recurrent pulmonary thromboembolism
Primary pulmonary hypertension
Pulmonary arteritis (granulomatosis with polyangiitis)
Vascular obstruction
Extensive pulmonary tumor microembolism
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46
Q

What causes of cor pulmonale are due to chest movement?

A

Kyphoscoliosis
Obesity (sleep apnea, pickwickian syndrome)
Neuromuscular disease

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47
Q

What causes of cor pulmonale are due to pulmonary arterial constriction?

A 
Metabolic acidosis
Hypoxemia
Chronic altitude sickness
Obstruction of major airways
Idiopathic alveolar hypoventilation
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48
Q

Acute cor pulmonale is due to what?

A

Thromboembolus to the lung which may have originated from DVT

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49
Q

What is initially seen with acute cor pulmonale?

A

Dilation of right ventricle

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50
Q

What are symptoms of acute cor pulmonale?

A

Fatigue
Tachpnea
Exertial dyspnea

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51
Q

What structural change occurs later with cor pulmonale?

A

Hypertrophy of the right ventricle

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52
Q

What is the most common valve abnormality?

A

Calcific aortic stenosis

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53
Q

What does calcific aortic stenosis cause?

A

Obstruction of blood flow across aortic valve

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54
Q

What causes the calcifications in calcific aortic stenosis?

A

Chronic progressive injury

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55
Q

How does calcific aortic stenosis manifest?

A

Elderly after years of wear and tear

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56
Q

What cells produce calcific aortic stenosis?

A

Osteoblast-like cells (deposit osteoid-like substance)

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57
Q

What diseases is calcific aortic stenosis associated with?

A

Chronic HTN
Hyperlipidemia
Inflammation

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58
Q

What causes a bicuspid aortic valve?

A

Congenital

Cusp fusion due to rheumatic heart disease

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59
Q

What valve abnormality shows accelerated course of calcific aortic stenosis?

A

Bicuspid aortic valve

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60
Q

What happens with bicuspid aortic valves become incompetent?

A

Aortic valve dilation or prolapse

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61
Q

Biscuspid aortic valves predispose patients to what?

A

Infective endocarditis

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62
Q

What does calcific aortic stenosis cause in the ventricle?

A

Increased pressure in the left ventricle which induced concentric left ventricular hypertrophy

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63
Q

What are the signs and symptoms of calcific aortic stenosis?

A

Angina (within 5 years)
Syncope (within 3 years)
CHF (within 2 years)

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64
Q

What is the treatment for calcific aortic stenosis?

A

Surgical replacement of diseased vessel

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65
Q

What is the function of the mitral annulus?

A

Insertion site for the leaflet tissue of mitral valves (fibrous rings that connects the leaflets)

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66
Q

Which population is mitral annular calcification more common in?

A

Elderly females

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67
Q

What can mitral annular calcification lead to?

A 
Mitral regurgitation
Stenosis
Heart block
Infective endocarditis
Thrombus formation
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68
Q

What does deep calcification in mitral annular calcification lead to?

A

Heart block

Sudden death

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69
Q

What is mitral valve prolapse?

A

Mitral valve prolapses back into the left atrium during systole

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70
Q

Which population is mitral valve prolapse most common?

A

Women

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71
Q

Which diseases are associated with mitral valve prolapse?

A

Marfan syndrome
Rheumatic fever
Myocardial ischemia

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72
Q

What is seen grossly with mitral valve prolapse?

A

Thickened, rubbery valve with myxomatous degeneration
Disruption of elastic fibers
Interchordal ballooning (hooding) of mitral leaflets

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73
Q

What causes the myxomatous degeneration in mitral valve prolapse?

A

Increased proteoglycan deposits

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74
Q

What stain is used to visualize increased proteoglycan deposits in mitral valve prolapse?

A

Movat pentachrome stain

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75
Q

What murmur is heard with mitral valve prolapse?

A

Mid-systolic click with accompanying systolic murmur

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76
Q

What is the most common cause of mitral regurgitation in developed countries?

A

Mitral valve prolapse

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77
Q

What diagnostic test is done to confirm mitral valve prolapse?

A

Echo

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78
Q

What can chronic mitral valve prolapse lead to?

A

Progressive dyspnea

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79
Q

What are serious complications associated with mitral valve prolapse?

A

Infective endocarditis
Chordal rupture with insufficiency
Arrhythmias
Thrombus formation

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80
Q

What does infective endocarditis present with?

A

Fever
Chills
Weakness

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81
Q

How does mitral insufficiency with chordal rupture present?

A

Sudden dyspnea as backflow of pressure causes pulmonary edema

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82
Q

What is the most common arrhythmia associated with mitral valve prolapse?

A

Afib

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83
Q

Thromboembolism in mitral valve prolapse is caused by what?

A

Leaflet thrombi

Afib

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84
Q

What causes rheumatic fever?

A

Immune reaction against the M streptococcal antigen after infection with group A strep

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85
Q

What characterizes the initial pharyngeal infection associated with rheumatic fever?

A

Sore throat
Fever
Punctuated by an erythematous blanching rash
*Scarlet fever

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86
Q

What occurs 10 days to 6 weeks later after strep infection?

A

Immune reaction against group A strep by activated CD4 T-cells and antibody producing B-lymphcytes attack the body

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87
Q

What immune cells are involved in rheumatic fever?

A

CD4+ T-cells

Antibody producing B-lymphocytes

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88
Q

What are the symptoms of acute rheumatic fever?

A 
Fever (101 F)
Migratory polyarthritis
Pancarditis
Subcutaneous nodules
Erythema marginatum
Sydenham chorea (basal ganglia)
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89
Q

What test is used to confirm rheumatic fever?

A 
Streptolysin O (streptococcal hemolytic exotoxin)
DNase B (streptococcal enzyme)
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90
Q

Can streptococcus be cultured at the time of acute rheumatic fever?

A

No

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91
Q

What joints are typically affected by migratory polyarthritis?

A

Knees
Ankles
Elbows
Writs

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92
Q

Are subcutaneous nodules in rheumatic fever painful?

A

No

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93
Q

Where is erythema marginatum found on the body?

A

Trunk

Proximal extremity

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94
Q

Facial grimacing is associated with which symptom of rheumatic fever?

A

Syndham chorea

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95
Q

Which parts of the heart are affected by acute rheumatic heart disease?

A

Pericardium
Myocardium
Endocardium

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96
Q

What is pancarditis?

A

Inflammation in all areas of the heart

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97
Q

What type of pericarditis occurs with acute rheumatic heart disease?

A

Fibrinous pericarditis

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98
Q

What valvular disease is common of acute rheumatic heart disease?

A

Valvulitis with vegetation (verrucae formation)

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99
Q

What valve does acute rheumatic heart disease affect first?

A

Mitral

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100
Q

What valve does acute rheumatic heart disease affect second?

A

Aortic

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101
Q

What valve does acute rheumatic heart disease affect third?

A

Tricuspid valve

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102
Q

What are the subendocardial lesions associated with acute rheumatic heart disease?

A

Maccallum plaques

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103
Q

What is a Maccallum plaque?

A

Irregular thickening typically in the left atrium due to regurgitant blood flow over the mitral valve

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104
Q

What is found on histology of acute rheumatic heart disease?

A

Aschoff bodies

Anitschkow cells

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105
Q

What are aschoff bodies?

A

Foci of T-lymphoctyes

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106
Q

What are Anitschkow cells

A

Activated macrophages

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107
Q

What causes chronic rheumatic heart disease?

A

Repetitive antigenic stimuli after the first immunologic reaction to a group A step infection

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108
Q

How does chronic rheumatic heart disease present?

A

Valvular leaflet thickening
Short chordae tendinae
Fusion
Regurgitation

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109
Q

The repetitive inflammatory insults in chronic rheumatic heart disease results in what?

A

Fibrosis
Valvular thickening
Valvular fusion
Valvular stenosis

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110
Q

Rheumatic heart disease is the principle cause of what?

A

Mitral valve stenosis

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111
Q

Whenever there is valvular structural abnormalities the patient is predisposed to what?

A

Infective endocarditis (especially bacterial endocarditis)

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112
Q

Mitral stenosis and mitral regurgitation both cause left atrial enlargement leading to what?

A

Atrial fibrillation (leading to stasis -> thrombus -> stroke)

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113
Q

Infective endocarditis is typically caused by what?

A

Bacteria

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114
Q

In infective endocarditis, organism deposition results in what?

A

Inflammatory response

Creation of fibrinous debris

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115
Q

What are risk factors for infective endocarditis?

A 
IV drug use
Male
Old age
Poor dentition
Valvular disease
Valve replacement
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116
Q

Why are old people at increased risk for infective endocarditis?

A

More likely to have valvular disease/valve replacement

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117
Q

Why are patients with poor dentition at increased risk for infective endocarditis?

A

Commensual bacteria in the mouth

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118
Q

What preexisting conditions put patients at risk for infective endocarditis?

A 
Rheumatic heart disease
Mitral valve prolapse
Calcific stenosis
Prosthetic heart valve
Congenital heart disease
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119
Q

What is the most common cause of infective endocarditis in native valves that have a structural abnormality?

A

Streptococcus viridans

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120
Q

What is the most common cause of infective endocarditis in patients with poor dentition and invasive dental procedures?

A

HACEK organisms (Hemophilus, actinobacillus, cardiobacterium, eikenella, kingella)

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121
Q

What is the most common cause of infective endocarditis in patients with prosthetic valves?

A

Staphylococcus epidermidis

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122
Q

What is the most common cause of infective endocarditis in IV drug abuse?

A

Staphylococcus aureus

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123
Q

IV drug abuse causes what side heart endocarditis?

A

Right side (due to venous blood return)

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124
Q

What is the most common side that infective endocarditis presents on?

A

Left side

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125
Q

Staph aureus in IV drug users has what type of infective endocarditis vegetation?

A

Necrotic vegetation

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126
Q

How does acute endocarditis present?

A

Rapid development of fever, chills, and weakness

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127
Q

How does subacute endocarditis present?

A

Low grade fever

Fatigue

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128
Q

How is endocarditis diagnosed?

A

Clinically (Duke Criteria)

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129
Q

What are the clinical findings associated with endocarditis?

A 
Subungual splinter hemorrhage
Janeway lesions
Osler nodes
Roth spots
Mycotic aneurysm formation
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130
Q

What is a subungal splinter hemorrhage?

A

Thin red to brown hemorrhagic lines under the fingernail that represent tiny clots that damage the capillaries under the nails

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131
Q

What are janeway lesions?

A

Small non-tender, erythematous macular or papular lesions on palms or soles representing septic microemboli with associated bacteria

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132
Q

What are roth spots?

A

Retinal hemorrhages caused by embolic processes

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133
Q

What is a mycotic aneurysm?

A

Dilation of vessel due to infection

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134
Q

What is nonbacterial thrombotic endocarditis?

A

Valvular thrombi form which consist of fibrin clots

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135
Q

When do the thrombi in nonbacterial thrombotic endocarditis come to attention?

A

When they embolize

Can cause downstream arterial occlusion (stroke)

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136
Q

What are the causes of nonbacterial thrombotic endocarditis?

A

Sepsis (proinflammatory, procoagulant cytokines)
Cancer (procoagulant release from tumors)
Antiphospholipid antibody syndrome
SLE (Libman-Sacks)

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137
Q

What is decreased in sepsis?

A

Tissue factor pathway inhibitor

Protein C

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138
Q

What cancer is especially prone to nonbacterial thrombotic endocarditis?

A

Mucinous adenocarcinomas

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139
Q

What happens in antiphospholipid antibody syndrome?

A

Antiphospholipid antibodies form against phospholipids of the vascular endothelial membranes causing increased clotting

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140
Q

What are the antibodies in antiphospholipid antibody syndrome?

A

Anticardiolipin

Lupus anticoagulant

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141
Q

What valve is affected in Libman Sacks endocarditis?

A

Mitral valve

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142
Q

What causes carcinoid heart disease?

A

Serotonin

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143
Q

What are heart affects associated with carcinoid heart disease?

A

Plaque-like endocardial thickening

Valvular thickening

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144
Q

What are the systemic symptoms of carcinoid heart disease?

A

Flushing
Diarrhea
Dermatitis
Bronchoconstriction

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145
Q

Symptoms of carcinoid tumors only occur when there is what?

A

Significant hepatic tumor burden

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146
Q

Which side of the heart are carcinoid tumor lesions found?

A

Right

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147
Q

Why is the left heart not affected by carcinoid tumors?

A

Pulmonary vascular degradation

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148
Q

Which drug causes a similar affect to carcinoid syndrome?

A 
Fenfluramine (appetite suppresant)
Ergot alkaloids (migraine)
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149
Q

Which stain is used to visualize the acellular mucopolysaccharide rich deposition caused by vasoactive compounds?

A

Movat stain

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150
Q

Which prosthetic valve lasts the longest?

A

Mechanical

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151
Q

Which prosthetic valve requires lifelong anticoagulant therapy?

A

Mechanical

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152
Q

Which prosthetic valve requires anticoagulation therapy for 3-6 months?

A

Bioprosthetic valve

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153
Q

What is the risk with mechanical valves?

A

Thromboembolism

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154
Q

What is the risk with bioprosthetic valves?

A

Mechanical failure (treating, incompetence)

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155
Q

What are risks with prosthetic valves?

A

Anticoagulant-related hemorrhage
Dysfunction
Hemolysis (hemolytic anemia)

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156
Q

What is heard with rheumatoid heart disease w/ mitral stenosis?

A

Diastolic rumbling murmur

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157
Q

What is heard with patent ductus arteriosus?

A

Continuous “machine-like” murmur

158
Q

What is heard with an infantile coarctation of the aorta?

A

Machine-like murmur

159
Q

What is heard with a calcific aortic stenosis?

A

Harsh systolic murmur (crescendo-decrescendo)

160
Q

What is heard with mitral regurgitation?

A

Holosystolic murmur

161
Q

What is heard with VSD?

A

Holosystolic murmur

162
Q

What is heard with aortic regurgitation?

A

Diastolic decrescendo murmur

163
Q

What is cardiomyopathy?

A

Heart muscle disease where the heart is structurally and functionally abnormal characterized by mechanical and/or electrical dysfunction in the absence of coronary artery disease, hypertension, valvular disease, or congenital heart disease

164
Q

What are the three types of cardiomyopathy?

A

1) Dilated cardiomyopathy
2) Hypertrophic cardiomyopathy
3) Restrictive cardiomyopathy

165
Q

What is dilated cardiomyopathy?

A

Enlargement of the cavities

166
Q

What is hypertrophic cardiomyopathy?

A

Thickened ventricular wall

167
Q

What is restrictive cardiomyopathy?

A

Motion of the heart is diminished

168
Q

What is the most common type of cardiomyopathy?

A

Dilated

169
Q

Which myocarditis has diastolic dysfunction?

A

Hypertrophic

Restrictive

170
Q

Which myocarditis has systolic dysfunction?

A

Dilated

171
Q

What causes the diastolic dysfunction of hypertrophic cardiomyopathy?

A

Thickened left ventricular wall

172
Q

What causes the diastolic dysfunction with restrictive cardiomyopathy?

A

Rigid ventricular walls

173
Q

What are the causes of dilated cardiomyopathy?

A 
Familial
Peripartum cardiomyopathy
Alcohol
Myocarditis
Cardiotoxic drugs/substances
Hemochromatosis
174
Q

What is the definition of dilated cardiomyopathy?

A

Progressive cardiac dilation and systolic dysfunction, usually with dilated hypertrophy

175
Q

What is the most common cause of dilated cardiomyopathy?

A

Familial

176
Q

What is the gene that encodes the familial form of dilated cardiomyopathy?

A

TTN (Titin protein)

177
Q

What is the inheritance pattern for TTN mutation?

A

Autosomal dominant

178
Q

Titin is an important protein for what?

A

Sarcomeres

179
Q

When and why does peripartum cardiomyopathy occur?

A

Late in pregnancy

Due to volume overload

180
Q

How does alcohol cause dilated cardiomyopathy?

A

Alcohol = cytotoxic effects

Alcohol -> thiamine deficiency -> impaired cellular metabolism with decreased ATP production -> wet beri-beri

181
Q

What are the symptoms of wet beri-beri?

A

Tachycardia
Fatigue
SOB
Leg swelling

182
Q

Which drug is cardiotoxic and causes dilated cardiomyopathy?

A

Doxorubicin

183
Q

What stain is used to show iron?

A

Prussian blue stain

184
Q

What is the cardiac morphology in dilated cardiomyopathy?

A

Dilation of all chambers
Often hypertrophic
Functional regurgitation of valves

185
Q

What is the hypertrophy present in dilated cardiomyopathies?

A

Overall increase in myocyte mass which translates into an increased heart weight and size

186
Q

Are the ventricular walls thickened in dilated cardiomyopathy?

A

No

187
Q

What happens in dilated cardiomyopathy as the myocytes become stretched?

A

Decreased ability of the heart to pump out blood leading to systolic dysfunction and progressive congestive heart failure

188
Q

What is the typical age of onset for dilated cardiomyopathy?

A

20-50 years old

189
Q

What are the clinical features of dilated cardiomyopathy?

A

Progressive CHF -> dyspnea, exertional fatigue, decreased ejection fraction
Systolic dysfunction
Arrhythmias (sudden death)
Thrombi formation with embolism

190
Q

What type of cardiomyopathy is Takotsubo cardiomyopathy?

A

Dilated cardiomyopathy

191
Q

What happens in Takosubu cardiomyopathy?

A

Severe emotional distress causes release of catecholamines

192
Q

What does increased release of catecholamines cause?

A

Increased contractility

Constriction of coronary arteries

193
Q

What is the end result of Takosubu cardiomyopathy?

A

Ischemic cardiomyopathy -> apical ballooning of left ventricle -> contractile dysfunction

194
Q

Which gender is Takosubu cardiomyopathy most common in?

A

Females

195
Q

What type of cardiomyopathy is arrhythmogenic right ventricular cardiomyopathy?

A

Dilated cardiomyopathy

196
Q

What causes arrhythmogenic right ventricular cardiomyopathy?

A

Defect in one of the desmosal associated proteins which results in fibrofatty replacement of the right ventricular myocardium

197
Q

What does the fibrofatty replacement in arrhythmogenic right ventricular cardiomyopathy lead to?

A

Region wall abnormalities
Right ventricular dilation
Ventricular fibrillation

198
Q

What is the inheritance pattern with arrhythmogenic right ventricular cardiomyopathy?

A

Autosomal dominant (incomplete penetrance)

199
Q

What is Naxos syndrome?

A

Variant of arrhythmogenic right ventricular cardiomyopathy that has an autosomal recessive pattern of inheritance

200
Q

What is characteristic of Naxos syndrome?

A

Hyperkeratosis of the palmar and plantar surfaces of hte hands and feet
Woolly hair

201
Q

When does Naxos syndrome present?

A

12 years old or older

202
Q

When does the fibrofatty replacement of the right ventricle occur in Naxos syndrome?

A

Adolescence

203
Q

What is implanted in patients with Naxos syndrome?

A

Defibrillator

204
Q

What is done to treat end stage Naxos syndrome?

A

Heart transplant

205
Q

What gene is mutated in Naxos syndrome?

A

Plakoglobin (desmosome-associated protein)

206
Q

What is hypertrophic cardomyopathy?

A

Genetic disorder involving mutations in sarcomeric proteins one of which is the beta-myosin heavy chain

207
Q

What is characteristic of hypertrophic cardiomyopathy?

A

Myocyte hypertrophy

Myocyte disarray

208
Q

What gender is most commonly affected by hypertrophic cardiomyopathy?

A

Male

209
Q

What is myocyte hypertrophy in hypertrophic cardiomyopathy associated with?

A

Septal prominence (asymmetric septal hypertrophy)

210
Q

What shape is the left ventricle in hypertrophic cardiomyopathy?

A

Banana

211
Q

What is the microscopic finding with hypertrophic cardiomyopathy?

A

Myocyte disarray

212
Q

What mutation is most common in hypertrophic cardiomyopathy?

A

Beta-myosin heavy chain

213
Q

What is the classic presentation with hypertrophic cardiomyopathy?

A

Teenage athlete who dies from ventricular arrhythmia

214
Q

What murmur can be heard with hypertrophy cardiomyopathy?

A

Systolic ejection murmur (mitral valve pushed toward septum)

215
Q

What are symptoms of hypertrophy cardiomyopathy?

A

Exertional dyspnea
Exertional chest pain
Palpitations

216
Q

What causes exertional dyspnea in hypertrophic cardiomyopathy?

A

Impaired diastolic filling-diminished cardiac output and pulmonary congestion

217
Q

What causes exertional chest pain in hypertrophic cardiomyopathy?

A

Microvascular ischemia

218
Q

What causes palpitations in hypertrophic cardiomyopathy?

A

Atrial fibrilation

Mural thrombus

219
Q

What type of hypertrophy is seen with hypertrophic cardiomyopathy?

A

Asymmetric (septum and anterior mitral valve leaflet obstruct flow)

220
Q

Which end points do both dilated and hypertrophic cardiomyopathy lead to?

A

Heart failure
Sudden death
Atrial fibrillation
Stroke

221
Q

Thrombus in the right atrium causes what?

A

PE

222
Q

Thrombus in the left atrium causes what?

A

Stroke

223
Q

What happens in restrictive cardiomyopathy?

A

Ventricular walls become too stiff due to a pathologic process

224
Q

What dysfunction do the stiffened walls in restrictive cardiomyopathy lead to?

A

Diastolic

225
Q

Is the ability of the heart to pump out blood diminished to a significant degree in restrictive cardiomyopathy?

A

No

226
Q

What is the morphology of restrictive cardiomyopathy?

A

Ventricles are normal dimension

Atria dilated

227
Q

What are the common causes of restrictive cardiomyopathy?

A

Amyloid

Fibrosis caused by irradiation

228
Q

What happens eventually in restrictive cardiomyopathy?

A

Lack of compliance of the left ventricle leads to marked elevation of filling pressure, lack of cardiac output and left and right congestive heart failure

229
Q

What are the symptoms of restrictive cardiomyopathy?

A 
Dyspnea
Peripheral edema
Palpitations
Fatigue
Weakness
Exercise intolerance
230
Q

What is amyloid?

A

Aggregate of insoluble proteins which collect in tissues causing dysfunction

231
Q

What is the structure of amyloid?

A

Beta-pleated sheet

232
Q

What causes amyloidosis?

A

Myeloma (plasma cell neoplasm)
Chronic inflammatory states
Mutated familial versions of transthyretin
Senile amyloidosis

233
Q

What does transthyretinin do?

A

Carries vitamin A and thyroxine throughout the body

234
Q

What happens in transthyretinin is mutated?

A

Misfolds to create amyloid

235
Q

What causes senile amyloidosis?

A

Normal transthyretin which misfolds into amyloid over many years

236
Q

Which types of cardiomyopathy does amyloidosis cause?

A

Restrictive cardiomyopathy (diastolic dysfunction, decreased filling)

237
Q

What is the staining pattern of amyloid?

A

Apple green birefringence pattern when stained by congo red

238
Q

When is serum amyloid A produced?

A

Acute-phase reactant produced in times of inflammation

239
Q

Where is transthyretin formed?

A

Liver

240
Q

What condition is a mutated transthyretin formed?

A

Familial amyloid polyneuropathies

241
Q

What are some obscure causes of restrictive cardiomyopathy?

A

Endomyocardial fibrosis
Loeffler endocarditis
Endocardial fibroelastosis

242
Q

Where are endomyocardial fibrosis and Loeffler endocarditis found?

A

Tropical and sub-tropical regions

243
Q

What is seen in endomyocardial fibrosis?

A

Fibrosis of endocardium and subendocardium

244
Q

What is seen in Loeffler endocarditis?

A

Endomyocardial fibrosis
Eosinophilic infiltration
Myeloproliferative (leukemia/lymphoma)

245
Q

What is seen in endocardial fibroelastosis?

A

Fibroelastic thickening of the left ventricular endocardium

246
Q

What is endocardial fibroelastosis associated with?

A

Congenital heart defects

247
Q

When is endocardial fibroelastosis diagnoses?

A

2 years

248
Q

What might be the basis of developing endomyocardial fibrosis and loeffler endocarditis?

A

Malnutrition

249
Q

What are the clinical signs with endocardial fibroelastosis?

A

Infant with feeding difficulty
Breathlessness
Failure to thrive
Wheezing

250
Q

What are the clinical signs with endomyocardial fibrosis?

A

Dyspnea (exertional)
Fatigue
Paroxysmal nocturnal dyspnea
Orthopnea

251
Q

What are the clinical signs with loeffler endocarditis?

A

Fever
Cough
Rash

252
Q

Friederich ataxia causes what type of cardiomyopathy?

A

Hypertrophic

253
Q

Storage diseases cause what type of cardiomyopathy?

A

Hypertrophic

254
Q

What is myocarditis?

A

Inflammatory disease of the myocardium

255
Q

What is myocarditis often associated with?

A

Fever

256
Q

What is the most common cause of myocarditis?

A

Coxsackievirus B

257
Q

How does coxsackievirus B present?

A 
Fever
Headache
Sore throat
GI distress
Fatigue
Chest pain
Muscle pain
258
Q

What are other causes of myocarditis?

A 
HIV 
Lyme disease
Chagas disease
Trichinosis
Rheumatic fever
Lupus
Hypersensitivity reactions
259
Q

What cardiomyopathy is associated with myocarditis?

A

Dilated cardiomyopathy

260
Q

What causes Chagas disease?

A

Triatomine bug or “kissing bug”

261
Q

What draws the triatomine bug to humans?

A

Compounds released by the skin

Carbon dioxide exhaled

262
Q

What form of trypanosoma cruzi is found in the myocardium?

A

Amastigote

263
Q

What is the most common form of trypanosoma cruzi found on heart biopsy?

A

Amastigote of

264
Q

What are the symptoms of trypanosoma cruzi?

A 
Malaise
Fever
Anorexia
Acute myocarditis
Pericardial effusion
Lymphadenopathy
Muscle pains
Hepatosplenomegaly
Meningoencephalitis
265
Q

With chronic trypansoma cruzi infection which cardiomyopathy can occur?

A

Dilated cardiomyopathy

266
Q

How is diagnosis made in trypanosoma cruzi?

A

PCR

Peripheral blood smear

267
Q

Where is trichinella spiralis found?

A

Muscle of pigs

268
Q

What causes the myocarditis in trichinosis?

A

Parasite encysts causing myositis and myocarditis

269
Q

What are the symptoms of the initial phase of trichinosis?

A

Nausea
Vomiting
Diarrhea
Abdominal discomfort

270
Q

What are the symptoms of trichinosis after it encysts?

A 
Muscle weakness
Muscle pain
Chest pain
Palpitations
Cardiac dysfunction
Light headedness
SOB
Fever
271
Q

What causes Lyme disease?

A

Borrelia Burgdorferi carried by the ixodes tick

272
Q

What are the symptoms of Lyme’s disease?

A

Fever
Chills
Bullseye rash (erythema migrans)

273
Q

What are the symptoms in the early disseminated phase of Lyme’s disease?

A

Migratory polyarthritis
Facial palsy
Meningitis

274
Q

What is the most common cardiac manifestation with Lyme’s disease?

A

Carditis -> heart block (transient)

275
Q

Erythema migrans is seen in what myocarditis?

A

Lyme’s disease

276
Q

What is the gold standard for diagnosing myocarditis?

A

Endomyocardial biopsy

277
Q

What is the most common form of myocarditis?

A

Lymphocytic myocarditis

278
Q

What is lymphocytic myocarditis seen in?

A

Viral infection

Autoimmune disease

279
Q

What is eosinophilic myocarditis typically caused by?

A

Hypersensitivity caused by a drug

280
Q

Which drugs cause eosinophilic myocarditis?

A

Methyldopa

Sulfonamides

281
Q

Does Chagas disease present with increased eosinophils?

A

Yes

282
Q

Can eosinophilic myocarditis be idiopathic?

A

Yes

283
Q

What must you exclude in eosinophilic myocarditis?

A

Parasite infection

284
Q

Which myocarditis is aggressive, rare, and has a fatal course?

A

Idiopathic giant cell myocarditis

285
Q

What is seen on histology with idiopathic giant cell myocarditis?

A

Myocyte loss with scattered enlarged multinucleated cells (giant cells)
Chronic inflammatory reaction

286
Q

What is sarcoidosis?

A

Immune mediated granulomatous reaction which frequently involves the lungs

287
Q

What is a classic finding in sarcoidosis?

A

Hilar lymphadenopathy

288
Q

What is seen on histology with sarcoidosis?

A

Giant cells associated with non-necrotizing granulomatous inflammation

289
Q

What are the two most important cardiotoxic drugs?

A

Doxorubicin

Daunorubicin

290
Q

What type of drugs are doxorubicin and daunorubicin?

A

Antracycline drugs used in chemotherapy

291
Q

What cancers does doxorubicin and daunorubicin treat?

A

Leukemias
Lymphomas
Kaposi sarcoma

292
Q

What does doxorubicin and daunorubicin do to myocytes?

A

Damage myocyte membranes

293
Q

What type of cardiomyopathy do doxorubicin and daunorubicin cause?

A

Dilated cardiomyopathy

294
Q

What is the toxicity with doxorubicin and daunorubicin?

A

Dose dependent lifetime toxicity

295
Q

What other drugs are cardiotoxic?

A

Lithium (mood stabilizer)
Phenothiazines (anti-psychotic)
Chloroquine (antimalarial)
^cause myocyte injury

296
Q

What is the normal pericardium?

A

Fibroelastic sac with straw colored fluid less than 50mL

297
Q

When does a pericardial effusion develop?

A

More than 50mL of fluid in pericardium

298
Q

Are there symptoms in chronic or subacute pericardial effusion?

A

No (abnormal heart shadow on CXR)

299
Q

Are there symptoms with acute pericardial effusion?

A

Yes cardiac tamponade

300
Q

What happens in cardiac tamponade?

A

Increased intrapericardial pressure impeded cardiac diastolic filling

301
Q

What are examples of hemopericardium?

A

Blood in pericardial space
Trauma
Ruptured MI
Aortic dissection

302
Q

What are examples of serous effusion?

A

Congestive heart failure

303
Q

What are examples of purulent pericarditis?

A

Necroinflammatoy debris
Acute inflammation (pus)
*due to infective process

304
Q

What is acute pericarditis?

A

Inflammation of the pericardial sac

305
Q

Where is the inflammation in acute pericarditis?

A

Parietal pericardial layer

Visceral pericardial surface

306
Q

What are the gross changes to the heart in pericarditis?

A

Roughened glandular appearance

307
Q

What are the signs and symptoms associated with pericarditis?

A 
Chest pain (sharp, pleuritic, position dependent)
Pericardial friction rub
Pericardial effusion
ST elevation
PR depression
Fever
308
Q

What is pleuritic chest pain?

A

Worsens during breathing

309
Q

What are the most common types of acute pericarditis?

A

Fibrinous

Serofibrinous

310
Q

If you have increased fibrin what is the type of pericarditis?

A

Fibrinous

311
Q

If you have increased fibrin and fluid what is the type of pericarditis?

A

Serofibrinous

312
Q

What types of pericarditis are common after MI?

A

Fibrinous

Serofibrinous

313
Q

What type of pericarditis does uremia lead to?

A

Fibrinous pericarditis

314
Q

What is uremia?

A

Increase in urea and other nitrogenous compounds in kidney disease

315
Q

What is the clinical scenario with chronic kidney disease?

A

Elevated BUN w/ pericardial friction rub and chest pain

316
Q

What is the gross appearance in fibrinous pericarditis?

A

Bread and butter

317
Q

What is seen microscopically in fibrinous pericarditis?

A

Fibrin deposition

Inflammatory infiltrate

318
Q

What causes purulent or suppurative pericarditis?

A

Active infection which involves the pericardium by direct extension
Physical introduction or seeding from the blood

319
Q

What is seen microscopically in suppurative pericarditis?

A

Neutrophils admixed with fibrin

320
Q

What is caseous necrosis?

A

Dead tissue has a cheese-like consistency and appearance

321
Q

What is caseous pericarditis associated with?

A

Tuberculosis

Fungal

322
Q

What is seen on histology with caseous pericarditis?

A

Necrotizing granuloma with areas of central necrosis surrounded by giant cells and chronic inflammation

323
Q

What is the most common cause of hemorrhagic pericarditis?

A

Breast, lung, esophageal carcinomas and melanomas

324
Q

What happens in constrictive pericarditis?

A

Chronic pericarditis where inflammatory reaction has burned out to be replaced with dense fibrous ring

325
Q

What cures constrictive pericarditis?

A

Resection of fibrous scar

326
Q

What is limited in constrictive pericarditis?

A

Cardiac output -> diastolic heart failure

327
Q

What happens in rare cases of constrictive pericarditis?

A

Fibrous ring calcifies like a plaster mold

328
Q

What is the most common malignant primary tumor of hte heart?

A

Angiosarcoma

329
Q

What are the benign tumors of the heart?

A 
Myxoma
Fibroma
Lipoma
Papillary fibroelastoma
Rhabdomyoma
330
Q

What is more common than primary malignant tumors?

A

Malignant metastatic tumors

331
Q

What is the most common adult cardiac tumor?

A

Myxoma

332
Q

What is the most common childhood cardiac tumor?

A

Rhabdomyoma

333
Q

What are cardiac myxomas?

A

Stromal tumors of mesenchymal origin (gelatinous to solid)

334
Q

What do cardiac myxomas arise from?

A

Intratrial septum

Fossa ovalis

335
Q

Which atrium do cardiac myxomas most often arise from?

A

Left atrium

336
Q

What type of valve obstruction is seen in cardiac myxomas?

A

Ball and valve type of obstruction

337
Q

What are the symptoms of cardiac myxomas?

A

Dyspnea
Orthopnea
Pulmonary edema
Syncope

338
Q

What is the wrecking ball phenomenon?

A

Active beating of heart with peduculated mass can damage the nearby valve

339
Q

What are complications of cardiac myxomas?

A

Embolization

340
Q

What cytokine does myxomas secrete?

A

IL-6

341
Q

What does symptoms does IL-6 cause with myxomas?

A

Fever

Malaise

342
Q

What is heard on auscultation with myxoma?

A

Polp

343
Q

What are two familial syndromes associated with myxomas?

A

McCune-Albright syndrome

Carney complex

344
Q

What is McCune-Albright syndrome?

A

Fibrous dysplasia (normal bone is replaced with fibro-osseous tissue
Cafe au lait spots
Endocrine abnormalities
Myxomas

345
Q

What mutation is associated with McCune-Albright syndrome?

A

GNAS1

346
Q

What is Carney’s complex?

A 
Skin changes (lentigines)
Endocrine dysfunction  (pituitary adenoma)
Myxomas
347
Q

What mutation is seen with Carney’s complex?

A

PRKAR1A

348
Q

What is the inheritance pattern seen with Carney’s complex?

A

Autosomal dominant

349
Q

What is seen on histology with myxomas?

A

Mucopolysaccharide materal

Embedded small nests of connective tissue derived from myxoma cells

350
Q

What is a cardiac lipoma?

A

Localized mass composed of mature lobulated fat

351
Q

Where are lipomas found in the heart?

A

Subendocardium
Subepicardium
Myocardium

352
Q

What are papillary fibroelastomas?

A

“sea-anemone” like hair-like projections

353
Q

Increased size of papillary fibroelastomas results in increased risk for what?

A

Embolism -> stroke

354
Q

What do papilary fibroelastomas look like histologically?

A

Avascular core of connective tissue with an outside endothelial linging

355
Q

What do papillary fibroelastomas resemble?

A

Lambl excrescences

356
Q

How do rhabdomyomas present?

A

Cyanosis
Heart failure
Sudden cardiac death

357
Q

How are rhabdomyomas classified?

A

Hamartomas of developing cardiac myocytes

358
Q

What is a hamartoma?

A

Growth comprised of normal mature or developing cells which is made up of cell types found in the area where i grows

359
Q

What are half of rhabdomyomas associated with?

A

Tuberous sclerosis

360
Q

What is tuberous sclerosis?

A

AD disorder that causes multiple benign hamartomas throughout the body

361
Q

Where are the hamartomas found in tuberous sclerosis?

A 
Facial angiofibromas
Shagreen patches (connective tissue nevus)
Periungal fibromas
Renal angiomyolipoma
Cortical tubers
Subependymal nodules
362
Q

Which genetic mutations are associated with tuberous sclerosis?

A

TSC1 (hamrtin)

TSC2 (tuberin)

363
Q

What do rhabdomyomas look like grossly?

A

Firm, gray-white, well circumscribed myocardial mass that protrude into ventricles

364
Q

What do rhabdomyomas look like histologically?

A

Large vacuolated cells with abdundant glycogen which are called spider cells (enlarged centrally placed nucleus from which myofibrils radiate to the cell membrane)

365
Q

Which population does angiosarcoma typically impact?

A

Older adults

366
Q

Which types of cancer typically metastasize to the heart?

A

Melanoma
Lung
Breast

367
Q

Which type of cancer metastasizes to the mediastinum?

A

Lymphoma

368
Q

Which types of cancers metastasize to the heart through direct extension?

A

Breast
Lung
Esophageal

369
Q

What are clinical findings in cardiac metastasis?

A

Mass effect (limiting cardiac filling)
Decreased myocardial contractility
Symptomatic pericardial effusion
Superior vena cava syndrome

370
Q

What circulating mediators are released during cardiac metastasis?

A

Procoagulant (nonbactieral thrombotic endocarditis mucinous tumors)
Serotonin (carcinoid heart disease)
Catecholamine (pheochromocytomas)
Myeloma (amyloidosis)

371
Q

What is an allograft heart transplant?

A

Replacing a patient’s heart with a donor heart who is not genetically idential

372
Q

Who is eligible for heart transplant?

A

Dilated cardiomyopathy

Severe ischemic heart disease

373
Q

What drugs need to be started for a patient who had a recent heart transplants?

A

Immunosupressive

374
Q

What are the three types of immune responses after transplants?

A

1) Cellular mediated rejection
2) Antibody mediated rejection
3) Allograft vasculopathy

375
Q

Which two immune responses occur within the first month of transplantation to several years after?

A

Cellular mediated rejection

Antibody mediated rejection

376
Q

What are cellular and antibody mediated rejection due to?

A

Foreign hearts HLA antigens

377
Q

What cells are involved in cellular mediate rejection?

A

T-cells

Lymphocytes

378
Q

What cells are involved in antibody mediated rejection?

A

Neutrophils
Macrophages
Antibodies

379
Q

What type of response is seen with cellular mediated rejection?

A

Lymphocytic

380
Q

What is the most significant long term limitation to survival in heart transplantation?

A

Allograft vasculopathy

381
Q

What do almost all heart transplants patients have within 10 years?

A

Allograft vasculopathy

382
Q

What happens in allograft vasculopathy?

A

Immunologic response induces growth factors with intimal smooth muscle and extracellular matrix synthesis resulting in a progressive stenosing intimal proliferation of the coronary arteries which can lead to ischemic injury and heart attack

383
Q

What is characteristic of allograft vasculopathy?

A

Stenosing intimal proliferation

384
Q

What is a complication of allograft vasculopathy?

A

Silent MI

Denervated transplanted heart (no angina)

385
Q

Immunosuppression causes proliferation of what types of skin tumors?

A

Basal cell carcinoma
Squamous cell carcinoma
Melanoma

386
Q

What is EBV lymphoproliferative disorder often due to?

A

Immunosuppression (T-cell)

387
Q

What are common viral infections?

A

HSV
CMV
Varicella-zoster
Papillomavirus

388
Q

What are common nosocomial infection?

A 
Legionella
Pseudomonas aeruginosa
Multidrug-resistnat organisms
Vancomycin-resistant enterococci
MRSA
Aspergillus
Candida
Clostridioides
389
Q

What are the common community acquired bacterial pathogens?

A 
Streptococcus pneumoniae
Mycoplasma
Legionella
Listeria monocytogenes
Salmonella
390
Q

What are the odds of heart transplant survival?

A

90% at 1 year

70% at 7 years

391
Q

What is seen on histology of EBV positive lymphoproliferative disorder?

A

Irregular atypical lymphocytes

CPR 2 Exam 1 (20 decks)

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