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CPR 2 Exam 1 > Cardiac Pathology 2 > Flashcards

Cardiac Pathology 2 Flashcards

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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
What is seen in a first degree heart block?
Prolonged PR interval
26
What is seen in second degree heart block?
Intermittent transmission
27
What is seen in third degree heart block?
Complete failure
28
What is long QT syndrome
Aberrant channel function causes an increased length of ventricular depolarization to repolarization
29
What does an increased QT interval lead to?
Torsades de Pointes
30
What happens in torsades de pointes?
Syncope | Sudden cardiac death (especially during exercise)
31
What channel dysfunction leads to long QT syndrome?
Sodium | Potassium
32
What is the classic scenario of long QT syndrome?
Stronger swimmer who drowns unexpectedly due to undiagnosed arrhythmia
33
What can trigger torsades de pointes?
Low blood calcium | Low blood potassium
34
What are hereditary chanellopathies?
Abnormal ion channels which cause arrhythmogenic disease
35
What is a sudden cardiac death?
Death without previous symptoms or death which occurs within 24 hours of symptom onset
36
What is the most common cause of sudden cardiac deaths?
Coronary artery disease
37
What causes sudden cardiac death in younger patients?
``` Drug abuse (cocaine, meth) Hereditary conduction abnormalities Hypertrophic or dilated cardiomyopathy Myocardial hypertrophy Myocarditis Mitral valve prolapse (w/chordae tendinae rupture) ```
38
What is the criteria for hypertensive heart disease?
1) Left ventricular hypertrophy | 2) Clinical history or pathologic evidence of hypertension in other organs
39
What pattern is usually seen with left ventricular hypertrophy?
Concentric
40
What is seen on autopsy with hypertensive heart disease?
Enlarged, heavy heart with increased concentric left ventricular wall thickness Granular kidneys with areas of hyaline arteriosclerosis
41
Diastolic dysfunction with hypertensive heart disease leads to what?
Atrial dilation -> afib -> congestive heart disease -> pulmonary edema Sudden cardiac death
42
What happens with cardiac myocyte hypertrophy?
Increase in cell size | Increase in nuclear size
43
What is cor pulmonale?
Right sided pulmonary hypertensive disease which leads to heart dysfunction and failure
44
What causes of cor pulmonale are due to pulmonary parenchyma?
``` COPD Diffuse pulmonary interstitial fibrosis Pneumonconioses Cystic fibrosis Bronchiectasis ```
45
What causes of cor pulmonale are due to pulmonary vessels?
``` Recurrent pulmonary thromboembolism Primary pulmonary hypertension Pulmonary arteritis (granulomatosis with polyangiitis) Vascular obstruction Extensive pulmonary tumor microembolism ```
46
What causes of cor pulmonale are due to chest movement?
Kyphoscoliosis Obesity (sleep apnea, pickwickian syndrome) Neuromuscular disease
47
What causes of cor pulmonale are due to pulmonary arterial constriction?
``` Metabolic acidosis Hypoxemia Chronic altitude sickness Obstruction of major airways Idiopathic alveolar hypoventilation ```
48
Acute cor pulmonale is due to what?
Thromboembolus to the lung which may have originated from DVT
49
What is initially seen with acute cor pulmonale?
Dilation of right ventricle
50
What are symptoms of acute cor pulmonale?
Fatigue Tachpnea Exertial dyspnea
51
What structural change occurs later with cor pulmonale?
Hypertrophy of the right ventricle
52
What is the most common valve abnormality?
Calcific aortic stenosis
53
What does calcific aortic stenosis cause?
Obstruction of blood flow across aortic valve
54
What causes the calcifications in calcific aortic stenosis?
Chronic progressive injury
55
How does calcific aortic stenosis manifest?
Elderly after years of wear and tear
56
What cells produce calcific aortic stenosis?
Osteoblast-like cells (deposit osteoid-like substance)
57
What diseases is calcific aortic stenosis associated with?
Chronic HTN Hyperlipidemia Inflammation
58
What causes a bicuspid aortic valve?
Congenital | Cusp fusion due to rheumatic heart disease
59
What valve abnormality shows accelerated course of calcific aortic stenosis?
Bicuspid aortic valve
60
What happens with bicuspid aortic valves become incompetent?
Aortic valve dilation or prolapse
61
Biscuspid aortic valves predispose patients to what?
Infective endocarditis
62
What does calcific aortic stenosis cause in the ventricle?
Increased pressure in the left ventricle which induced concentric left ventricular hypertrophy
63
What are the signs and symptoms of calcific aortic stenosis?
Angina (within 5 years) Syncope (within 3 years) CHF (within 2 years)
64
What is the treatment for calcific aortic stenosis?
Surgical replacement of diseased vessel
65
What is the function of the mitral annulus?
Insertion site for the leaflet tissue of mitral valves (fibrous rings that connects the leaflets)
66
Which population is mitral annular calcification more common in?
Elderly females
67
What can mitral annular calcification lead to?
``` Mitral regurgitation Stenosis Heart block Infective endocarditis Thrombus formation ```
68
What does deep calcification in mitral annular calcification lead to?
Heart block | Sudden death
69
What is mitral valve prolapse?
Mitral valve prolapses back into the left atrium during systole
70
Which population is mitral valve prolapse most common?
Women
71
Which diseases are associated with mitral valve prolapse?
Marfan syndrome Rheumatic fever Myocardial ischemia
72
What is seen grossly with mitral valve prolapse?
Thickened, rubbery valve with myxomatous degeneration Disruption of elastic fibers Interchordal ballooning (hooding) of mitral leaflets
73
What causes the myxomatous degeneration in mitral valve prolapse?
Increased proteoglycan deposits
74
What stain is used to visualize increased proteoglycan deposits in mitral valve prolapse?
Movat pentachrome stain
75
What murmur is heard with mitral valve prolapse?
Mid-systolic click with accompanying systolic murmur
76
What is the most common cause of mitral regurgitation in developed countries?
Mitral valve prolapse
77
What diagnostic test is done to confirm mitral valve prolapse?
Echo
78
What can chronic mitral valve prolapse lead to?
Progressive dyspnea
79
What are serious complications associated with mitral valve prolapse?
Infective endocarditis Chordal rupture with insufficiency Arrhythmias Thrombus formation
80
What does infective endocarditis present with?
Fever Chills Weakness
81
How does mitral insufficiency with chordal rupture present?
Sudden dyspnea as backflow of pressure causes pulmonary edema
82
What is the most common arrhythmia associated with mitral valve prolapse?
Afib
83
Thromboembolism in mitral valve prolapse is caused by what?
Leaflet thrombi | Afib
84
What causes rheumatic fever?
Immune reaction against the M streptococcal antigen after infection with group A strep
85
What characterizes the initial pharyngeal infection associated with rheumatic fever?
Sore throat Fever Punctuated by an erythematous blanching rash *Scarlet fever
86
What occurs 10 days to 6 weeks later after strep infection?
Immune reaction against group A strep by activated CD4 T-cells and antibody producing B-lymphcytes attack the body
87
What immune cells are involved in rheumatic fever?
CD4+ T-cells | Antibody producing B-lymphocytes
88
What are the symptoms of acute rheumatic fever?
``` Fever (101 F) Migratory polyarthritis Pancarditis Subcutaneous nodules Erythema marginatum Sydenham chorea (basal ganglia) ```
89
What test is used to confirm rheumatic fever?
``` Streptolysin O (streptococcal hemolytic exotoxin) DNase B (streptococcal enzyme) ```
90
Can streptococcus be cultured at the time of acute rheumatic fever?
No
91
What joints are typically affected by migratory polyarthritis?
Knees Ankles Elbows Writs
92
Are subcutaneous nodules in rheumatic fever painful?
No
93
Where is erythema marginatum found on the body?
Trunk | Proximal extremity
94
Facial grimacing is associated with which symptom of rheumatic fever?
Syndham chorea
95
Which parts of the heart are affected by acute rheumatic heart disease?
Pericardium Myocardium Endocardium
96
What is pancarditis?
Inflammation in all areas of the heart
97
What type of pericarditis occurs with acute rheumatic heart disease?
Fibrinous pericarditis
98
What valvular disease is common of acute rheumatic heart disease?
Valvulitis with vegetation (verrucae formation)
99
What valve does acute rheumatic heart disease affect first?
Mitral
100
What valve does acute rheumatic heart disease affect second?
Aortic
101
What valve does acute rheumatic heart disease affect third?
Tricuspid valve
102
What are the subendocardial lesions associated with acute rheumatic heart disease?
Maccallum plaques
103
What is a Maccallum plaque?
Irregular thickening typically in the left atrium due to regurgitant blood flow over the mitral valve
104
What is found on histology of acute rheumatic heart disease?
Aschoff bodies | Anitschkow cells
105
What are aschoff bodies?
Foci of T-lymphoctyes
106
What are Anitschkow cells
Activated macrophages
107
What causes chronic rheumatic heart disease?
Repetitive antigenic stimuli after the first immunologic reaction to a group A step infection
108
How does chronic rheumatic heart disease present?
Valvular leaflet thickening Short chordae tendinae Fusion Regurgitation
109
The repetitive inflammatory insults in chronic rheumatic heart disease results in what?
Fibrosis Valvular thickening Valvular fusion Valvular stenosis
110
Rheumatic heart disease is the principle cause of what?
Mitral valve stenosis
111
Whenever there is valvular structural abnormalities the patient is predisposed to what?
Infective endocarditis (especially bacterial endocarditis)
112
Mitral stenosis and mitral regurgitation both cause left atrial enlargement leading to what?
Atrial fibrillation (leading to stasis -> thrombus -> stroke)
113
Infective endocarditis is typically caused by what?
Bacteria
114
In infective endocarditis, organism deposition results in what?
Inflammatory response | Creation of fibrinous debris
115
What are risk factors for infective endocarditis?
``` IV drug use Male Old age Poor dentition Valvular disease Valve replacement ```
116
Why are old people at increased risk for infective endocarditis?
More likely to have valvular disease/valve replacement
117
Why are patients with poor dentition at increased risk for infective endocarditis?
Commensual bacteria in the mouth
118
What preexisting conditions put patients at risk for infective endocarditis?
``` Rheumatic heart disease Mitral valve prolapse Calcific stenosis Prosthetic heart valve Congenital heart disease ```
119
What is the most common cause of infective endocarditis in native valves that have a structural abnormality?
Streptococcus viridans
120
What is the most common cause of infective endocarditis in patients with poor dentition and invasive dental procedures?
HACEK organisms (Hemophilus, actinobacillus, cardiobacterium, eikenella, kingella)
121
What is the most common cause of infective endocarditis in patients with prosthetic valves?
Staphylococcus epidermidis
122
What is the most common cause of infective endocarditis in IV drug abuse?
Staphylococcus aureus
123
IV drug abuse causes what side heart endocarditis?
Right side (due to venous blood return)
124
What is the most common side that infective endocarditis presents on?
Left side
125
Staph aureus in IV drug users has what type of infective endocarditis vegetation?
Necrotic vegetation
126
How does acute endocarditis present?
Rapid development of fever, chills, and weakness
127
How does subacute endocarditis present?
Low grade fever | Fatigue
128
How is endocarditis diagnosed?
Clinically (Duke Criteria)
129
What are the clinical findings associated with endocarditis?
``` Subungual splinter hemorrhage Janeway lesions Osler nodes Roth spots Mycotic aneurysm formation ```
130
What is a subungal splinter hemorrhage?
Thin red to brown hemorrhagic lines under the fingernail that represent tiny clots that damage the capillaries under the nails
131
What are janeway lesions?
Small non-tender, erythematous macular or papular lesions on palms or soles representing septic microemboli with associated bacteria
132
What are roth spots?
Retinal hemorrhages caused by embolic processes
133
What is a mycotic aneurysm?
Dilation of vessel due to infection
134
What is nonbacterial thrombotic endocarditis?
Valvular thrombi form which consist of fibrin clots
135
When do the thrombi in nonbacterial thrombotic endocarditis come to attention?
When they embolize | Can cause downstream arterial occlusion (stroke)
136
What are the causes of nonbacterial thrombotic endocarditis?
Sepsis (proinflammatory, procoagulant cytokines) Cancer (procoagulant release from tumors) Antiphospholipid antibody syndrome SLE (Libman-Sacks)
137
What is decreased in sepsis?
Tissue factor pathway inhibitor | Protein C
138
What cancer is especially prone to nonbacterial thrombotic endocarditis?
Mucinous adenocarcinomas
139
What happens in antiphospholipid antibody syndrome?
Antiphospholipid antibodies form against phospholipids of the vascular endothelial membranes causing increased clotting
140
What are the antibodies in antiphospholipid antibody syndrome?
Anticardiolipin | Lupus anticoagulant
141
What valve is affected in Libman Sacks endocarditis?
Mitral valve
142
What causes carcinoid heart disease?
Serotonin
143
What are heart affects associated with carcinoid heart disease?
Plaque-like endocardial thickening | Valvular thickening
144
What are the systemic symptoms of carcinoid heart disease?
Flushing Diarrhea Dermatitis Bronchoconstriction
145
Symptoms of carcinoid tumors only occur when there is what?
Significant hepatic tumor burden
146
Which side of the heart are carcinoid tumor lesions found?
Right
147
Why is the left heart not affected by carcinoid tumors?
Pulmonary vascular degradation
148
Which drug causes a similar affect to carcinoid syndrome?
``` Fenfluramine (appetite suppresant) Ergot alkaloids (migraine) ```
149
Which stain is used to visualize the acellular mucopolysaccharide rich deposition caused by vasoactive compounds?
Movat stain
150
Which prosthetic valve lasts the longest?
Mechanical
151
Which prosthetic valve requires lifelong anticoagulant therapy?
Mechanical
152
Which prosthetic valve requires anticoagulation therapy for 3-6 months?
Bioprosthetic valve
153
What is the risk with mechanical valves?
Thromboembolism
154
What is the risk with bioprosthetic valves?
Mechanical failure (treating, incompetence)
155
What are risks with prosthetic valves?
Anticoagulant-related hemorrhage Dysfunction Hemolysis (hemolytic anemia)
156
What is heard with rheumatoid heart disease w/ mitral stenosis?
Diastolic rumbling murmur
157
What is heard with patent ductus arteriosus?
Continuous "machine-like" murmur
158
What is heard with an infantile coarctation of the aorta?
Machine-like murmur
159
What is heard with a calcific aortic stenosis?
Harsh systolic murmur (crescendo-decrescendo)
160
What is heard with mitral regurgitation?
Holosystolic murmur
161
What is heard with VSD?
Holosystolic murmur
162
What is heard with aortic regurgitation?
Diastolic decrescendo murmur
163
What is cardiomyopathy?
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
What are the three types of cardiomyopathy?
1) Dilated cardiomyopathy 2) Hypertrophic cardiomyopathy 3) Restrictive cardiomyopathy
165
What is dilated cardiomyopathy?
Enlargement of the cavities
166
What is hypertrophic cardiomyopathy?
Thickened ventricular wall
167
What is restrictive cardiomyopathy?
Motion of the heart is diminished
168
What is the most common type of cardiomyopathy?
Dilated
169
Which myocarditis has diastolic dysfunction?
Hypertrophic | Restrictive
170
Which myocarditis has systolic dysfunction?
Dilated
171
What causes the diastolic dysfunction of hypertrophic cardiomyopathy?
Thickened left ventricular wall
172
What causes the diastolic dysfunction with restrictive cardiomyopathy?
Rigid ventricular walls
173
What are the causes of dilated cardiomyopathy?
``` Familial Peripartum cardiomyopathy Alcohol Myocarditis Cardiotoxic drugs/substances Hemochromatosis ```
174
What is the definition of dilated cardiomyopathy?
Progressive cardiac dilation and systolic dysfunction, usually with dilated hypertrophy
175
What is the most common cause of dilated cardiomyopathy?
Familial
176
What is the gene that encodes the familial form of dilated cardiomyopathy?
TTN (Titin protein)
177
What is the inheritance pattern for TTN mutation?
Autosomal dominant
178
Titin is an important protein for what?
Sarcomeres
179
When and why does peripartum cardiomyopathy occur?
Late in pregnancy | Due to volume overload
180
How does alcohol cause dilated cardiomyopathy?
Alcohol = cytotoxic effects | Alcohol -> thiamine deficiency -> impaired cellular metabolism with decreased ATP production -> wet beri-beri
181
What are the symptoms of wet beri-beri?
Tachycardia Fatigue SOB Leg swelling
182
Which drug is cardiotoxic and causes dilated cardiomyopathy?
Doxorubicin
183
What stain is used to show iron?
Prussian blue stain
184
What is the cardiac morphology in dilated cardiomyopathy?
Dilation of all chambers Often hypertrophic Functional regurgitation of valves
185
What is the hypertrophy present in dilated cardiomyopathies?
Overall increase in myocyte mass which translates into an increased heart weight and size
186
Are the ventricular walls thickened in dilated cardiomyopathy?
No
187
What happens in dilated cardiomyopathy as the myocytes become stretched?
Decreased ability of the heart to pump out blood leading to systolic dysfunction and progressive congestive heart failure
188
What is the typical age of onset for dilated cardiomyopathy?
20-50 years old
189
What are the clinical features of dilated cardiomyopathy?
Progressive CHF -> dyspnea, exertional fatigue, decreased ejection fraction Systolic dysfunction Arrhythmias (sudden death) Thrombi formation with embolism
190
What type of cardiomyopathy is Takotsubo cardiomyopathy?
Dilated cardiomyopathy
191
What happens in Takosubu cardiomyopathy?
Severe emotional distress causes release of catecholamines
192
What does increased release of catecholamines cause?
Increased contractility | Constriction of coronary arteries
193
What is the end result of Takosubu cardiomyopathy?
Ischemic cardiomyopathy -> apical ballooning of left ventricle -> contractile dysfunction
194
Which gender is Takosubu cardiomyopathy most common in?
Females
195
What type of cardiomyopathy is arrhythmogenic right ventricular cardiomyopathy?
Dilated cardiomyopathy
196
What causes arrhythmogenic right ventricular cardiomyopathy?
Defect in one of the desmosal associated proteins which results in fibrofatty replacement of the right ventricular myocardium
197
What does the fibrofatty replacement in arrhythmogenic right ventricular cardiomyopathy lead to?
Region wall abnormalities Right ventricular dilation Ventricular fibrillation
198
What is the inheritance pattern with arrhythmogenic right ventricular cardiomyopathy?
Autosomal dominant (incomplete penetrance)
199
What is Naxos syndrome?
Variant of arrhythmogenic right ventricular cardiomyopathy that has an autosomal recessive pattern of inheritance
200
What is characteristic of Naxos syndrome?
Hyperkeratosis of the palmar and plantar surfaces of hte hands and feet Woolly hair
201
When does Naxos syndrome present?
12 years old or older
202
When does the fibrofatty replacement of the right ventricle occur in Naxos syndrome?
Adolescence
203
What is implanted in patients with Naxos syndrome?
Defibrillator
204
What is done to treat end stage Naxos syndrome?
Heart transplant
205
What gene is mutated in Naxos syndrome?
Plakoglobin (desmosome-associated protein)
206
What is hypertrophic cardomyopathy?
Genetic disorder involving mutations in sarcomeric proteins one of which is the beta-myosin heavy chain
207
What is characteristic of hypertrophic cardiomyopathy?
Myocyte hypertrophy | Myocyte disarray
208
What gender is most commonly affected by hypertrophic cardiomyopathy?
Male
209
What is myocyte hypertrophy in hypertrophic cardiomyopathy associated with?
Septal prominence (asymmetric septal hypertrophy)
210
What shape is the left ventricle in hypertrophic cardiomyopathy?
Banana
211
What is the microscopic finding with hypertrophic cardiomyopathy?
Myocyte disarray
212
What mutation is most common in hypertrophic cardiomyopathy?
Beta-myosin heavy chain
213
What is the classic presentation with hypertrophic cardiomyopathy?
Teenage athlete who dies from ventricular arrhythmia
214
What murmur can be heard with hypertrophy cardiomyopathy?
Systolic ejection murmur (mitral valve pushed toward septum)
215
What are symptoms of hypertrophy cardiomyopathy?
Exertional dyspnea Exertional chest pain Palpitations
216
What causes exertional dyspnea in hypertrophic cardiomyopathy?
Impaired diastolic filling-diminished cardiac output and pulmonary congestion
217
What causes exertional chest pain in hypertrophic cardiomyopathy?
Microvascular ischemia
218
What causes palpitations in hypertrophic cardiomyopathy?
Atrial fibrilation | Mural thrombus
219
What type of hypertrophy is seen with hypertrophic cardiomyopathy?
Asymmetric (septum and anterior mitral valve leaflet obstruct flow)
220
Which end points do both dilated and hypertrophic cardiomyopathy lead to?
Heart failure Sudden death Atrial fibrillation Stroke
221
Thrombus in the right atrium causes what?
PE
222
Thrombus in the left atrium causes what?
Stroke
223
What happens in restrictive cardiomyopathy?
Ventricular walls become too stiff due to a pathologic process
224
What dysfunction do the stiffened walls in restrictive cardiomyopathy lead to?
Diastolic
225
Is the ability of the heart to pump out blood diminished to a significant degree in restrictive cardiomyopathy?
No
226
What is the morphology of restrictive cardiomyopathy?
Ventricles are normal dimension | Atria dilated
227
What are the common causes of restrictive cardiomyopathy?
Amyloid | Fibrosis caused by irradiation
228
What happens eventually in restrictive cardiomyopathy?
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
What are the symptoms of restrictive cardiomyopathy?
``` Dyspnea Peripheral edema Palpitations Fatigue Weakness Exercise intolerance ```
230
What is amyloid?
Aggregate of insoluble proteins which collect in tissues causing dysfunction
231
What is the structure of amyloid?
Beta-pleated sheet
232
What causes amyloidosis?
Myeloma (plasma cell neoplasm) Chronic inflammatory states Mutated familial versions of transthyretin Senile amyloidosis
233
What does transthyretinin do?
Carries vitamin A and thyroxine throughout the body
234
What happens in transthyretinin is mutated?
Misfolds to create amyloid
235
What causes senile amyloidosis?
Normal transthyretin which misfolds into amyloid over many years
236
Which types of cardiomyopathy does amyloidosis cause?
Restrictive cardiomyopathy (diastolic dysfunction, decreased filling)
237
What is the staining pattern of amyloid?
Apple green birefringence pattern when stained by congo red
238
When is serum amyloid A produced?
Acute-phase reactant produced in times of inflammation
239
Where is transthyretin formed?
Liver
240
What condition is a mutated transthyretin formed?
Familial amyloid polyneuropathies
241
What are some obscure causes of restrictive cardiomyopathy?
Endomyocardial fibrosis Loeffler endocarditis Endocardial fibroelastosis
242
Where are endomyocardial fibrosis and Loeffler endocarditis found?
Tropical and sub-tropical regions
243
What is seen in endomyocardial fibrosis?
Fibrosis of endocardium and subendocardium
244
What is seen in Loeffler endocarditis?
Endomyocardial fibrosis Eosinophilic infiltration Myeloproliferative (leukemia/lymphoma)
245
What is seen in endocardial fibroelastosis?
Fibroelastic thickening of the left ventricular endocardium
246
What is endocardial fibroelastosis associated with?
Congenital heart defects
247
When is endocardial fibroelastosis diagnoses?
2 years
248
What might be the basis of developing endomyocardial fibrosis and loeffler endocarditis?
Malnutrition
249
What are the clinical signs with endocardial fibroelastosis?
Infant with feeding difficulty Breathlessness Failure to thrive Wheezing
250
What are the clinical signs with endomyocardial fibrosis?
Dyspnea (exertional) Fatigue Paroxysmal nocturnal dyspnea Orthopnea
251
What are the clinical signs with loeffler endocarditis?
Fever Cough Rash
252
Friederich ataxia causes what type of cardiomyopathy?
Hypertrophic
253
Storage diseases cause what type of cardiomyopathy?
Hypertrophic
254
What is myocarditis?
Inflammatory disease of the myocardium
255
What is myocarditis often associated with?
Fever
256
What is the most common cause of myocarditis?
Coxsackievirus B
257
How does coxsackievirus B present?
``` Fever Headache Sore throat GI distress Fatigue Chest pain Muscle pain ```
258
What are other causes of myocarditis?
``` HIV Lyme disease Chagas disease Trichinosis Rheumatic fever Lupus Hypersensitivity reactions ```
259
What cardiomyopathy is associated with myocarditis?
Dilated cardiomyopathy
260
What causes Chagas disease?
Triatomine bug or "kissing bug"
261
What draws the triatomine bug to humans?
Compounds released by the skin | Carbon dioxide exhaled
262
What form of trypanosoma cruzi is found in the myocardium?
Amastigote
263
What is the most common form of trypanosoma cruzi found on heart biopsy?
Amastigote of
264
What are the symptoms of trypanosoma cruzi?
``` Malaise Fever Anorexia Acute myocarditis Pericardial effusion Lymphadenopathy Muscle pains Hepatosplenomegaly Meningoencephalitis ```
265
With chronic trypansoma cruzi infection which cardiomyopathy can occur?
Dilated cardiomyopathy
266
How is diagnosis made in trypanosoma cruzi?
PCR | Peripheral blood smear
267
Where is trichinella spiralis found?
Muscle of pigs
268
What causes the myocarditis in trichinosis?
Parasite encysts causing myositis and myocarditis
269
What are the symptoms of the initial phase of trichinosis?
Nausea Vomiting Diarrhea Abdominal discomfort
270
What are the symptoms of trichinosis after it encysts?
``` Muscle weakness Muscle pain Chest pain Palpitations Cardiac dysfunction Light headedness SOB Fever ```
271
What causes Lyme disease?
Borrelia Burgdorferi carried by the ixodes tick
272
What are the symptoms of Lyme's disease?
Fever Chills Bullseye rash (erythema migrans)
273
What are the symptoms in the early disseminated phase of Lyme's disease?
Migratory polyarthritis Facial palsy Meningitis
274
What is the most common cardiac manifestation with Lyme's disease?
Carditis -> heart block (transient)
275
Erythema migrans is seen in what myocarditis?
Lyme's disease
276
What is the gold standard for diagnosing myocarditis?
Endomyocardial biopsy
277
What is the most common form of myocarditis?
Lymphocytic myocarditis
278
What is lymphocytic myocarditis seen in?
Viral infection | Autoimmune disease
279
What is eosinophilic myocarditis typically caused by?
Hypersensitivity caused by a drug
280
Which drugs cause eosinophilic myocarditis?
Methyldopa | Sulfonamides
281
Does Chagas disease present with increased eosinophils?
Yes
282
Can eosinophilic myocarditis be idiopathic?
Yes
283
What must you exclude in eosinophilic myocarditis?
Parasite infection
284
Which myocarditis is aggressive, rare, and has a fatal course?
Idiopathic giant cell myocarditis
285
What is seen on histology with idiopathic giant cell myocarditis?
Myocyte loss with scattered enlarged multinucleated cells (giant cells) Chronic inflammatory reaction
286
What is sarcoidosis?
Immune mediated granulomatous reaction which frequently involves the lungs
287
What is a classic finding in sarcoidosis?
Hilar lymphadenopathy
288
What is seen on histology with sarcoidosis?
Giant cells associated with non-necrotizing granulomatous inflammation
289
What are the two most important cardiotoxic drugs?
Doxorubicin | Daunorubicin
290
What type of drugs are doxorubicin and daunorubicin?
Antracycline drugs used in chemotherapy
291
What cancers does doxorubicin and daunorubicin treat?
Leukemias Lymphomas Kaposi sarcoma
292
What does doxorubicin and daunorubicin do to myocytes?
Damage myocyte membranes
293
What type of cardiomyopathy do doxorubicin and daunorubicin cause?
Dilated cardiomyopathy
294
What is the toxicity with doxorubicin and daunorubicin?
Dose dependent lifetime toxicity
295
What other drugs are cardiotoxic?
Lithium (mood stabilizer) Phenothiazines (anti-psychotic) Chloroquine (antimalarial) ^cause myocyte injury
296
What is the normal pericardium?
Fibroelastic sac with straw colored fluid less than 50mL
297
When does a pericardial effusion develop?
More than 50mL of fluid in pericardium
298
Are there symptoms in chronic or subacute pericardial effusion?
No (abnormal heart shadow on CXR)
299
Are there symptoms with acute pericardial effusion?
Yes cardiac tamponade
300
What happens in cardiac tamponade?
Increased intrapericardial pressure impeded cardiac diastolic filling
301
What are examples of hemopericardium?
Blood in pericardial space Trauma Ruptured MI Aortic dissection
302
What are examples of serous effusion?
Congestive heart failure
303
What are examples of purulent pericarditis?
Necroinflammatoy debris Acute inflammation (pus) *due to infective process
304
What is acute pericarditis?
Inflammation of the pericardial sac
305
Where is the inflammation in acute pericarditis?
Parietal pericardial layer | Visceral pericardial surface
306
What are the gross changes to the heart in pericarditis?
Roughened glandular appearance
307
What are the signs and symptoms associated with pericarditis?
``` Chest pain (sharp, pleuritic, position dependent) Pericardial friction rub Pericardial effusion ST elevation PR depression Fever ```
308
What is pleuritic chest pain?
Worsens during breathing
309
What are the most common types of acute pericarditis?
Fibrinous | Serofibrinous
310
If you have increased fibrin what is the type of pericarditis?
Fibrinous
311
If you have increased fibrin and fluid what is the type of pericarditis?
Serofibrinous
312
What types of pericarditis are common after MI?
Fibrinous | Serofibrinous
313
What type of pericarditis does uremia lead to?
Fibrinous pericarditis
314
What is uremia?
Increase in urea and other nitrogenous compounds in kidney disease
315
What is the clinical scenario with chronic kidney disease?
Elevated BUN w/ pericardial friction rub and chest pain
316
What is the gross appearance in fibrinous pericarditis?
Bread and butter
317
What is seen microscopically in fibrinous pericarditis?
Fibrin deposition | Inflammatory infiltrate
318
What causes purulent or suppurative pericarditis?
Active infection which involves the pericardium by direct extension Physical introduction or seeding from the blood
319
What is seen microscopically in suppurative pericarditis?
Neutrophils admixed with fibrin
320
What is caseous necrosis?
Dead tissue has a cheese-like consistency and appearance
321
What is caseous pericarditis associated with?
Tuberculosis | Fungal
322
What is seen on histology with caseous pericarditis?
Necrotizing granuloma with areas of central necrosis surrounded by giant cells and chronic inflammation
323
What is the most common cause of hemorrhagic pericarditis?
Breast, lung, esophageal carcinomas and melanomas
324
What happens in constrictive pericarditis?
Chronic pericarditis where inflammatory reaction has burned out to be replaced with dense fibrous ring
325
What cures constrictive pericarditis?
Resection of fibrous scar
326
What is limited in constrictive pericarditis?
Cardiac output -> diastolic heart failure
327
What happens in rare cases of constrictive pericarditis?
Fibrous ring calcifies like a plaster mold
328
What is the most common malignant primary tumor of hte heart?
Angiosarcoma
329
What are the benign tumors of the heart?
``` Myxoma Fibroma Lipoma Papillary fibroelastoma Rhabdomyoma ```
330
What is more common than primary malignant tumors?
Malignant metastatic tumors
331
What is the most common adult cardiac tumor?
Myxoma
332
What is the most common childhood cardiac tumor?
Rhabdomyoma
333
What are cardiac myxomas?
Stromal tumors of mesenchymal origin (gelatinous to solid)
334
What do cardiac myxomas arise from?
Intratrial septum | Fossa ovalis
335
Which atrium do cardiac myxomas most often arise from?
Left atrium
336
What type of valve obstruction is seen in cardiac myxomas?
Ball and valve type of obstruction
337
What are the symptoms of cardiac myxomas?
Dyspnea Orthopnea Pulmonary edema Syncope
338
What is the wrecking ball phenomenon?
Active beating of heart with peduculated mass can damage the nearby valve
339
What are complications of cardiac myxomas?
Embolization
340
What cytokine does myxomas secrete?
IL-6
341
What does symptoms does IL-6 cause with myxomas?
Fever | Malaise
342
What is heard on auscultation with myxoma?
Polp
343
What are two familial syndromes associated with myxomas?
McCune-Albright syndrome | Carney complex
344
What is McCune-Albright syndrome?
Fibrous dysplasia (normal bone is replaced with fibro-osseous tissue Cafe au lait spots Endocrine abnormalities Myxomas
345
What mutation is associated with McCune-Albright syndrome?
GNAS1
346
What is Carney's complex?
``` Skin changes (lentigines) Endocrine dysfunction (pituitary adenoma) Myxomas ```
347
What mutation is seen with Carney's complex?
PRKAR1A
348
What is the inheritance pattern seen with Carney's complex?
Autosomal dominant
349
What is seen on histology with myxomas?
Mucopolysaccharide materal | Embedded small nests of connective tissue derived from myxoma cells
350
What is a cardiac lipoma?
Localized mass composed of mature lobulated fat
351
Where are lipomas found in the heart?
Subendocardium Subepicardium Myocardium
352
What are papillary fibroelastomas?
"sea-anemone" like hair-like projections
353
Increased size of papillary fibroelastomas results in increased risk for what?
Embolism -> stroke
354
What do papilary fibroelastomas look like histologically?
Avascular core of connective tissue with an outside endothelial linging
355
What do papillary fibroelastomas resemble?
Lambl excrescences
356
How do rhabdomyomas present?
Cyanosis Heart failure Sudden cardiac death
357
How are rhabdomyomas classified?
Hamartomas of developing cardiac myocytes
358
What is a hamartoma?
Growth comprised of normal mature or developing cells which is made up of cell types found in the area where i grows
359
What are half of rhabdomyomas associated with?
Tuberous sclerosis
360
What is tuberous sclerosis?
AD disorder that causes multiple benign hamartomas throughout the body
361
Where are the hamartomas found in tuberous sclerosis?
``` Facial angiofibromas Shagreen patches (connective tissue nevus) Periungal fibromas Renal angiomyolipoma Cortical tubers Subependymal nodules ```
362
Which genetic mutations are associated with tuberous sclerosis?
TSC1 (hamrtin) | TSC2 (tuberin)
363
What do rhabdomyomas look like grossly?
Firm, gray-white, well circumscribed myocardial mass that protrude into ventricles
364
What do rhabdomyomas look like histologically?
Large vacuolated cells with abdundant glycogen which are called spider cells (enlarged centrally placed nucleus from which myofibrils radiate to the cell membrane)
365
Which population does angiosarcoma typically impact?
Older adults
366
Which types of cancer typically metastasize to the heart?
Melanoma Lung Breast
367
Which type of cancer metastasizes to the mediastinum?
Lymphoma
368
Which types of cancers metastasize to the heart through direct extension?
Breast Lung Esophageal
369
What are clinical findings in cardiac metastasis?
Mass effect (limiting cardiac filling) Decreased myocardial contractility Symptomatic pericardial effusion Superior vena cava syndrome
370
What circulating mediators are released during cardiac metastasis?
Procoagulant (nonbactieral thrombotic endocarditis mucinous tumors) Serotonin (carcinoid heart disease) Catecholamine (pheochromocytomas) Myeloma (amyloidosis)
371
What is an allograft heart transplant?
Replacing a patient's heart with a donor heart who is not genetically idential
372
Who is eligible for heart transplant?
Dilated cardiomyopathy | Severe ischemic heart disease
373
What drugs need to be started for a patient who had a recent heart transplants?
Immunosupressive
374
What are the three types of immune responses after transplants?
1) Cellular mediated rejection 2) Antibody mediated rejection 3) Allograft vasculopathy
375
Which two immune responses occur within the first month of transplantation to several years after?
Cellular mediated rejection | Antibody mediated rejection
376
What are cellular and antibody mediated rejection due to?
Foreign hearts HLA antigens
377
What cells are involved in cellular mediate rejection?
T-cells | Lymphocytes
378
What cells are involved in antibody mediated rejection?
Neutrophils Macrophages Antibodies
379
What type of response is seen with cellular mediated rejection?
Lymphocytic
380
What is the most significant long term limitation to survival in heart transplantation?
Allograft vasculopathy
381
What do almost all heart transplants patients have within 10 years?
Allograft vasculopathy
382
What happens in allograft vasculopathy?
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
What is characteristic of allograft vasculopathy?
Stenosing intimal proliferation
384
What is a complication of allograft vasculopathy?
Silent MI | Denervated transplanted heart (no angina)
385
Immunosuppression causes proliferation of what types of skin tumors?
Basal cell carcinoma Squamous cell carcinoma Melanoma
386
What is EBV lymphoproliferative disorder often due to?
Immunosuppression (T-cell)
387
What are common viral infections?
HSV CMV Varicella-zoster Papillomavirus
388
What are common nosocomial infection?
``` Legionella Pseudomonas aeruginosa Multidrug-resistnat organisms Vancomycin-resistant enterococci MRSA Aspergillus Candida Clostridioides ```
389
What are the common community acquired bacterial pathogens?
``` Streptococcus pneumoniae Mycoplasma Legionella Listeria monocytogenes Salmonella ```
390
What are the odds of heart transplant survival?
90% at 1 year | 70% at 7 years
391
What is seen on histology of EBV positive lymphoproliferative disorder?
Irregular atypical lymphocytes

CPR 2 Exam 1 (20 decks)

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