Heart PT 2 Flashcards

(179 cards)

1
Q

Rhythmic disorders are most caused by?

A

ischemia

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2
Q

What is sick sinus syndrome and what does it cause

A

SA node is damaged d/t ischemia => causing bradycardia

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3
Q

What is a-fib? What can it cause and describe the heartbeat?

A
  • Sporadically depolarizing atrial myocytes d/t atrial dilation, causing variable transmission through the AV node, which can cause clots and an irregular irregular heartbeat
    • Thus, give blood thinners
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4
Q

What is an AV block?

A

Fucked up AV node causes abnormal heart rhythm that causes the heart to beat too slowly (heart block)

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5
Q

What is the difference between first, second and third degree heart block?

A
  • First degree heart block has a prolonged PR interval
  • Second degree heart block has intermittent transmission
  • Third degree heart block has complete heart failure.
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6
Q

What causes arrhythmias?

A
  • Abnormalities in structure of gap junction/ spatial relationship,
  • Ischmia/hypertrophy/ inflammation,
  • Amyloid deposits or scarring/
  • Genetics (AD)
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7
Q

What is the most common inherited arrhythmogenic disease?

A
  • Long QT syndrome: arrythmias d/t with too much prolongation of cardiac repolarization d/t K+/Na+ channelopathy
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8
Q

Most common inherited arrhythmogenic disease have what pattern of genetic inheritance?

A

AD

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9
Q

What are 4 genes implicated in long QT syndrome?

A
    • KCNQ1
    • KCNH2
    • SCN5A
    • CAV3
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10
Q

Long QT syndrome is associated with ____ of K+ channels or _____ of Na+ channels

A

LOF of K+

GOF of Na+

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11
Q

arrhythmias associated with short repolarization intervals.

What is this and what can patients experience

A

Short QT syndrome

palpitations, syncome, sudden cardiac death

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12
Q

What syndrome has ECG abnormalities (ST-elevation; RBBB) without a structural defect in the heart?

What do they present with?

A

Brugada syndrome;

-syncope/SCD during rest, sleep or large meals

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13
Q

Pablo came into ER and was pronounced death after an unexpected cardiac cause within 24 hours of onset of symptoms. What can we diagnose this patient as? Are symptoms always required?

A
  • Sudden cardiac death (SCD)
  • No
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14
Q

Pablo was diagnosed with SCD (sudden cardiac death). SCD is due to?

A

fatal arrhythmia d/t damage of the myocardium d/t ischemia.

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15
Q

Pablo, who suffered from SCD, was able to be resuscitated. Based on statistics, what will his lab/ECG show?

A
  • 80-90% of resuscitated patients have NO lab or ECG changes because they have no long-term damage.
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16
Q

What is the leading cause of SCD?

A
  • CAD in 80-90% of patients: patients will usually have >75% stenosis of 1 or more of 3 main coronary arteries.
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17
Q

What is OFTEN first sign of IHD (ischemic heart disease)?

A

Sudden cardiac death :(

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18
Q

Hypertrophy of the heart is an adaptive response to chronically elevated pressures; with continued overload, the result can be

A

dysfunction, dilation, CHF or SCD.

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19
Q

What criteria are required to diagnose a patient with left-sided hypertensive heart diseases (systemic hypertensive heart disease)?

A
  • 1. HTN
    1. LV hypertrophy that is concentric w no other probs => decrease lumen size
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20
Q

In systemic hypertensive heart disease, how will the heart adaptively response?

A
  • Wall of LV thickens to more than 1.5 cm => weighs more than 500 grams.
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21
Q

In Systemic (left-sided) HHD as the LV wall continues to increase in thickness, what associated morphological changes occur?

A
  • ↑ interstitial CT –> stiffness = impaired diastolic filling; LV does not fill with blood –> LEFT ATRIAL ENLARGEMENT => a-fib
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22
Q

In many pt’s, systemic HHD comes to attention due to what signs/sx’s?

A
  • L atria enlarged => causes A-fib => leads to CHF, a risk factor for SCD.
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23
Q

Isolated pulmonary (right-sided) HTN heart disease (cor pulmonale) arises in the setting of what?

A
  • Pulmonary HTN
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24
Q

What is the most common cause of pulmonary HTN?

A
  • Left-sided heart disease.
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25
How can we distinguish whether **right-sided hypertensive disease (pulmonary hypertensive disease)** is **acute** or **chronic**?
* **Acute pulmonary HHD i**s caused by **a large PE** =\> pulmonary HTN =\> RV dilation w/t hypertrophy * Chronic pulmonary HHD is caused **primary pulmonary HTN** or **chronic dz that affects parynchma (COPD, CF, emphysema)** =\> pulmonary HTN =\> RV wall thickens
26
Aubrey comes in and we suspect **pulmonary HHD.** What morphological changes will tell us whether or not it is acute or chronic?
* **Acute**: RV dilation without hypertrophy * **Chronic**: RV wall thickens
27
What are **5 diseases** affecting the **lung parenchyma** =\> **cor pulmonale?**
**1. COPD** **2. Diffuse pulmonary intersitial fibrosis** **3. Pneuoconiosis** **4. CF** **5. Bronchostasis**
28
\_\_\_\_\_\_\_\_ can lead to **cor pulmonale** because of the physical pressure on the rib cage from being hunched over.
**Kyphoscoliosis**
29
Pathologic changes of **valves** include what 3 types?
1. **_Damage to collagen_** that weakens leaflets 1. Mitral valve prolapse 2. _**Nodular calcificatio**n_ that beings in interstitial cells 3. **_Fibrotic thickening_**
30
Why are valves so vulnerable to damage?
* Because they are so **thin** and receive nourishment via diffusion, they have **little BS.**
31
**When do we begin to clinically notice a valvular disease?** If **chronic**, what can these diseases cause
**_when they begin to cause clinical problems._** 1. **_Stenosis_** 1. Stenosis is stiffening/thickening of a valve, causing failure of a valve to **open** completely, which disrupts forward flow. 2. Chronic stenosis can cause what? **Pressure-overload** **hypertrophy** =\> **CHF** 2. **_Insufficiency/regurgitation/incompetence_** 1. Insufficiency: valve does NOT CLOSE completely, which reverses flow 2. Chronic regurgitation may cause **volume-overload hypertrophy** =\> CHF 3. **Both**
32
Other words for **regurg**
1. **incompetence** 2. **insuffiiency**
33
**_Chronic stenosis**_ may cause what type of overload hypertrophy vs. _**chronic insufficiency_**?
- Chronic **stenosis** = cause **pressure-overload** hypertrophy - C**hronic insufficienc**y= cause **volume**- **overload** hypertrophy
34
Incompetence of a valve due to a **abnormality in one of its support structure,** NOT the valve itself is called?
* **_Functional regurgitation_**
35
**Functional mitral valve regurg** is clinically important in what 2 conditions?
1. **IHD** 2. **Dilated cardiomyopathy**
36
1. What is the major etiology causing **mitral stenosis?**
1. **Rheumatic heart disease (post-inflammatory scarring)**
37
1. What is the major etiology causing **mitral regurgitation?**
* Problems in leaflets/commissures due to: post-inflammatory scarring and infective endocarditis * Mitral valve prolapse * Drugs
38
1. What is the major etiology causing **aortic stenosis?**
1. Abnormalities in leaflets/commissures due to **rheumatic heart disease (RHD)** 2. **Senile calcification aortic stenosis** 1. **Normal aortic valve that undergoes calcification.** Thus, not bicuspid 3. **Calcification of a congenitally deformed valve (bicuspid or unicuspid**)
39
1. What is the major etiology causing **aortic regurgitation?**
1. **RHD** that causes post-inflammatory scarring =\> damages leaflets and commissures 2. **Aortic insufficiency:** dilation of ascending aorta, often due to HTN or aging.
40
Abnormalities of the **tensor apparatus** that cause **aortic Regurgitation** may be caused by what 4 things?
**a. -Syphilitic aortitis \*** b. Ankylosing spondylitis c. Rheumatoid arthritis **d. Marfan Syndrome \***
41
What is **aortic insuffiency?**
**Dilation of ascending aorta**, often d/t a **HTN** or **aging =\> can cause _aortic regurg_**
42
**_Aortic stenosis_** is a stiff aortic valve, causing obstruction of \_\_\_\_\_\_\_\_\_outflow and decrease \_\_\_\_. Thus, it is a ____ problem.
* **LV outflow** * **CO** * **systemic =\> decrease blood going to body**
43
3 cardiac signs of **aortic stenosis.**
1. Exertional **dizziness or syncope** 2. Exertional **dyspnea** 3. Exertional **angina**
44
What is the most common valve abnormality?
* **_Calcific aortic stenosis_**; mounds of calcification prevent the cusps from completely opening.
45
When are you most likely to get **calcific aortic stenosis** and what is it usually due to?
* **60-80 yo** * "**Wear and tear"** due to. w/ **chronic HTN**, **hyperlipidemia**, and **inflammation**
46
Calcification, leading to aortic stenosis of previously normal valve differs in onset from calcification of a **abnormal bicuspid aortic valve (BAV)** how?
**Senile calcific aortic stenosis** occurs 60-80 YO, however if the patient has a bicuspid valve, onset occurs **1-2 decades earlier** because it faces **more stress.**
47
How does valve injury of **calcific aortic stenosis** differ from **atherosclerosis**?
Abnormal valves contain **osteoblast-like cells**--\> **make bone matrix** and **deposit Ca2+**=\> **ossifies**
48
In contrast to **rheumatic** (and congenital) aortic stenosis, what are **2 major differences seen in _nonrheumatic_, calcific aortic stenosis?**
1. - **Commissural fusion is NOT usually seen** 2. - **Mitral valve = normal**
49
What are the 3 main symptoms of **calcific aortic stenosis?** What do you see on XR?
1. **Angina** 2. **CHF** 1. Patients with calcific aortic stenosis + CHF will die in **2 years** 3. **Syncope** 4. On XR, patients will have **LV hypertrophy d/t increased pressure**
50
If present, which site on the **cusp** is a major site of Ca2+ deposits in those with **congenital bicuspid aortic valves (BAV)?**
**Midline raphe**
51
**Calcium deposits** in the **mitral valv**e tend to accumulate in the \_\_\_\_\_\_\_, resulting in **_\_\_\_\_\_\_\_\__**
* **Fibrous annulus** * **Mitral annular calcification**
52
As opposed to the **cuspal** involvement in **aortic valve calcificatio**n, where do calcific deposits occur in the mitral valve?
**Mitral annulus**
53
What is the gross morphology of the calcific deposits in **mitral annular calcification?**
* Irregular, stony, hard ulcerated nodules.
54
How does **mitral annular calcification** affect **valve function?**
**Valve function is usually NOT affected**. However, it can cause: * 1. **Stenosis** by preventing opening * 2. **Regurg** by impaiing physio contraction * 3. **Arrhythmia** and sudden death if Ca2+ deposits fuck with the AV node
55
Ca2+ nodules in **mitral annular calcificatio**n may provide a site for what complications? Pt's are at greater risk for what?
for **_thrombi_** or **_infective endocarditis_**
56
**Mitral annular calcification** is most common who?
* **Females** **over 60** with **mitral valve prolapse.**
57
**Mitral valve _prolapse_** has a higher incidence in what gender?
**F (7:1)**
58
**Mitral Valve Prolapse** is often discovered incidentally by hearing what during ausculation?
**Mid systolic click** sometimes followed by **mid-to-late systolic murmur**
59
What occurs to the valve leaflets in **Mitral Valve Prolapse?**
"Floppy" **leaflets** **balloon** back **into** the **LA** during **systole**
60
Which heritable disorder of CT is associated with **Mitral Valve Prolapse?**
**Marfan Syndrome**
61
The leaflets in **Mitral Valve Prolaps**e become **thickened** and **rubbery** due to what?
1. **Deposits of proteoglycans (myxomatous degeneration)** 2. **Fucking up of elastic fibers**
62
What is the **characterstic anatomic change in _mitral venous prolapse?_**
**Interchordal ballooning (hooding**) of mitral leaflets
63
* **Majority** of patients with **Mitral Valve Prolapse** are **_asymptomatic_,** but a small minority may develop which **4 serious, but RARE complications?**
* **_1. Infective endocarditis_** * 2. **Mitral insufficiency** * 3. **Stroke** or **_thromboembolism_** * 4. **Arrythmias**
64
2' changes reflecting the stresses and tissue injury incident to the billowing leaflets in **mitral valve prolapse** include **thickening** of what 3 structures?
- Fibrous thickening of **valve leaflets** - Linear fibrous thickening of **LV endocardial surface** - Thickening of the **mural endocardium of the LV or LA**
65
How can the **diagnosis/confirm** of **mitral valve prolaps**e be made?
* - **Auscultation** * - Confirmed w/ **Echocardiography**
66
What is the most common cause for **mitral valve surgery** in the US?
**MVP (mitral valve prolapse)**
67
**Rheumatic heart disease** is virtually the only cause of what cardiac disorder?
**mitral stenosis**
68
What is **_rheumatic fever_**?
**autoimmune reaction (type 2)** that causes inflammation of joints, muscles and fibrous tissue. I can damage heart muscle =\> lead to rheumatic heart disease.
69
What is the pathogenesis of **RF**?
* **CD4+ T cells t**hat attack **M-protein**s **on group A strep** cross-react with **cardiac self-antigens i**n a processes called molecular mimicry.
70
What bacteria causes r**heumatic fever** and when does it occur?
* **Weeks** after streptococcal pharyngitis “**strep throat**” due to **group A strep.**
71
**Rheumatic heart disease** is most common in who?
chilren
72
When does **_acute RF_** occur and what enzymes can we detect in these patients?
* **10 days – 6 weeks after group A strep** * **Anti-streptolysin O** * **Anti-Dnase B**
73
How do we diagnose rheumatic fever?
* JONES CRITERIA * **Joints**: polyarthritis in at least 5 joints * \<**3**: Carditis: valvulitis, myocarditis, pANcarditis * **Nodules**; subcutaneous * **Erythema marginatu**m (ring rash) on the trunk * **Sydenham chorea**: a jerking movement disorder
74
* **Rheumatic heart disease** is damage to the heart valves by rheumatic fever. __________ is most commonly affected
**mitral valve**
75
What are the distinctive lesions found in the heart during **acute RF,** and what do these lesions consist of?
* **_Pancarditis_** (inflammation of ENTIRE heart) with **_aschoff bodies_** (granuloma inflammation with giant cells) with: * T-cells * Plasma cells * **_Anitschow cells_** (“caterpillar cells” activated MO that look wavy) * **Fibrinoid necrosis of the endocardium** and l**eft sided valves with vegetations (verecae** *
76
Which cells are pathognomonic for **RF**?
* **Anistchow cells (activated MO)** located in aschoff bodies.
77
What changes do we see in the **heart** and **valves** with **acute rheumatic fever.**
1. **Fibrinoid necrosis of endocardium** 2. Vegetation of _fibrin_ and _platelets_ on **left-sided valves (mitral and aortic)** called **verrucae**, that overlie necrotic area.
78
**What heart changes occur in chronic rheumatic heart disease?**
**_mitral stenosis_** * **Mitral leaflets** become **thicker** and can **fuse together (commissural fusion)** * **Chordae tendineae** become thick and short and **fuse** * **_LA enlarges \> a-fib_**
79
With tight mitral stenosis seen in RHD what compensatory changes occur in the left atrium and it may harbor what?
**Left atrium DILATES** and may harbor **mural thrombi** that can embolize
80
**Chronic RHD** can lead to what changes in the **LUNGS**, that will result in...
**Pulmonary congestion =\> RV hypertrophy**
81
Which 2 antibodies to streptococcal enzymes may be detected in the sera of pt with RF?
anti-streptolysin 0 anti DNAse B
82
What is **infective endocarditis?**
* **infection of the endocardium** and **heart valves** that forms **vegetations** (made up of microbes and debris) underlying **tissue damage**.
83
How are **acute IE a**nd **subacute IE** different from one another? and txs
* Acute IE is a R**APID** **infection** of a **previously NORMAL valve.** * Treatment: **surgery + ABX** * Subacute IE is **slower infection** of a **previously DEFORMED valve.** * **Treatment: ABX**
84
How does **acute infective endocarditis** differ from **subacute infective endocarditis** in the type of organism involved?
§ - **Acute IE** = infection by **highly virulent bacteria (i.e., S. aureus)** § **- Subacute IE** = infection w/ **lower virulence bacteria (i.e., Viridians strep)**
85
What are **6 predisposing conditions** which increase the risk of developing **infective endocarditis?**
* **Valvular abnormalities:** such as * **prosthetic valves** * RHD * MV prolapse * calcific stenosis/bicuspid AV * **Bacteremia** (dental work, infection, contaminated needle)
86
The most important among the risk factors for **infective endocarditis** are those that can cause microorganisms to do what?
**get into bloodstream (bacteremia/fungemia)** =\> spread infection
87
Which organism is most often responsible for **infective endocarditis** of native but previously damaged or abnormal valves?
**Streptococcus viridians**
88
Which bacteria is responsible for a majority of the **mortality** associated with **infective endocarditis** and is common in **IV drug abusers**?
* **S. aureus**
89
Which bacteria is causes in**fectious endocarditis** in those with **prosthetic valves?**
* **S. epidermidis (coagulase-neg staph)**
90
Which organisms are implicated in **infective endocarditis**?
* **_HACEK (gram - bacteria)_** * Hemophilus, actinobacillus, cardiobacterium, eikenella, kingella
91
**IE** causes vegetations to build up on endocardium and valves. ## Footnote **What valve is most commonly affected?**
* **Left-**sided valves **(aortic and mitral)** * **Right-sided valves** in **IV drug users** (tricuspid and pulmonary) * **Don’t “tri” drugs.**
92
**Describe the vegetations IE** caues and what they can result in.
* **Friable**, **bulky** and **destructive** valvular vegetations * **Left** sides valves (mitral and aorta) =\> **septic emboli** * **Right** sided valves (tri/pul) =\> **pulmonary empolism**
93
Microscopically, the vegetations of **subacute IE t**ypically exhibit what that is indicative of **healing**?
**granulation tissue, which can fibrosis and calcify**
94
What is the **clinical presentation (signs/sx's)** of i**nfective endocarditis?** What may be heard upon **auscultation in a patient with IE.**
* - Rapidly developing fever + Chills * - Weakness + Malaise * **- Left sided IE = MURMUR**
95
What are the 4 major forms of **vegetative endocarditis**? in RHD, IE, LSE, and NBTE
1. **Rheumatic heart disease** 1. Small vegetations that look like **warts** along the line of valve closure 2. **Infective endocarditis** 1. Large irregular masses on cusps that extend into chordae 3. **Non-bacterial thrombotic endocarditis (NBTE)** 1. Small vegetations on line of closure 4. **Libman-sacks endocarditis (LSE)** 1. Small or medium sized vegetation on both side of valve leaflet (often involving the mitral valve) 2. Assx with SLE
96
What happens if a vegetation in infective endoarditis erodes into underlying tissue?
Ring abcess
97
Which type of infective endocarditis is more likely to form emboli? What can these emboli cause
* **Acute IE** * **Septic infarcts** or **mycotic aneurysms**
98
**What are signs of IE?**
* **_FROM JANE_** * Fever * Roth Spots: retinal hemorrhages in eye w pale centers * Osler nodes (subcutaneous nodes on fingers and toes that persist for hours/days) * Murmur (if left-sided IE) * Janeways lesions: non-tender lesions on palms or soles * Anemia * Nail-bed hemorrhage: splinter * Emboli
99
Although **non-bacterial thrombotic endocarditis** (**NBTE**) has **small, sterile vegetations along the line of closure** that are **_not invasive**_ and _**not inflammatory_**, why can they pose a problem?
* They are loosely attached and can **emboli** =\> produce **infarcts**
100
**NBTE (non-bacterial thrombotic endocarditis)** has a striking association with what?
**MUCINOUS ADENOCARCINOMAS**
101
**carcinoid syndrome;** what is it?
a **paraneoplastic syndrome** that causes **SOB** (bronchoconstriction), **flushing** of face, **diarrhea** and **dermatitis** d/t release of **5HT** from carcinoid tumors
102
50% of pts with carcinoid syndrome develop **carcinoid heart disease,** what part of the heart is more protected and why?
_**Right** **endocardium** and **valves**_ because the **L side is protected** **by the lungs metabolizing 5HT and mediators**
103
**Cardiac lesions of carcinoid syndrom**e ususally only occur after there is a massive burden where and why?
**Liver**, because the liver metabolizes 5-HT before it reaches the heart.
104
What is the morphology of **cardiac lesions** that occur in **carcinoid heart disease?**
* **Mucopolydsacchardie builds up on endocardium and valve**= \> producing glistening plaque-like intimal thickenings
105
What is the major complication associated with the use of **mechanical prosthetic valves?**
**Thromboembolism** --\> **thrombotic** occlusion of the prosthesis or **emboli** released from thrombi formed on the valve
106
Due to the major **risk** of **thromboembolism** associated with **mechanical prosthetic valves**, what long-term therapy must be given?
**Long-term anti-coagulation tx**
107
Which type of prosthetic valve (mechanical or bioprostheses) almost always **undergoes structural deterioration?**
Almost ALL **bioprostheses** eventually become incompetent due to calcification and/or tearing
108
Pt's with **mechanical prosthetic valve**s must be on **long-term anticoagulant therapy** to **prevent thromboembolism, b**ut this puts them at a risk of what complication?
**Hemorrhagic stroke**
109
**Infective endocarditis** is a potentially serious complication associated with what type of prosthetic valve (mechanical or bioprostheses)?
**Both :)**
110
**Cardiomyopathy** is a disease of the _______ (\_\_\_\_\_\_\_disease). * ________ **cardiomyopathy**: occurs when disease of myocardium is genetic or acquired. * _______ **cardiomyopathy**: occurs when cardiomyopathy occurs to **compensate** for another disease.
* **myocardium (heart muscle disease)** * **Primary** * **Secondary**
111
What are our **cardiomyopathies**? 6
* 1. Dilated * 2. Hypertrophic * 3. Restrictive * 4. Takotsubo * 5. Arrhythmogenic RV cardiomyopthy (ARVC) * 6. Amyloid *
112
The mechanism of heart failure in **dilated cardiomyopathy (DCM)** is an impairment in what?
**Contractility (systolic dysfunction)**
113
The mechanism of heart failure in **_hypertrophic_** and **_restrictive cardiomyopathy_** is an impairment in what?
* **Compliance (diastolic dysfunction)** * Compliance=\> how easily something expands when it fills with blood
114
**90%** of cardiomyopathies are of what kind? What is the least common?
**Dilated cardiomyopathy** ## Footnote **_Least: Restrictive_**
115
What is a **dilated cardiomyopathy?**
* **Dilation** of all 4 chambers and dilation hypertrophy =\> causing **systolic dysfunction**, because the **heart now has a problem contracting.**
116
What are the common gross morphological features of **Dilated CM**? Which type of thrombi are common and valvular alterations?
* **All 4 chambers** are **dilated & interstitial fibrosis** * **Mural thrombi** * No innate problems w valves; but **functional regurg** can occur
117
Familial cases of **dilated cardiomyopathy (30-50%)** are linked to mutations in what gene? Major inheritance pattern?
**TTN** **AD**
118
**Dilated cardiomyopathy** typically manifests during what ages? What signs or sxs does it present with?
* **20-50 YO** * **Slowly progressive signs of CHF** =\> dyspnea, easy tired and decrease EJ
119
What does it mean that **DCM causes a systolic dysfunction?**
* If you stretch the heart =\> **will not be able to contract properly =**\> **will not empty** =\> **_biventricular CHF_**
120
**Most commonly, DCM is idiopathic.** Other causes include what?
1. **Alcohol** 2. **Myocarditis** d/t coxsackie B 3. **Drugs**: _doxorubicin_, _cobalt_, _iron overload_ d/t hereditary hemochromatosis (HFE mutation)
121
**Myocarditis** can progress to what type of cardiomyopathy and is often associated with what virus?
* **DCM; coxsackie B**
122
What serious complications are associated with **dilated cardiomyopathy?**
- Thrombus 2' to stasis --\> **embolism (stroke)** - **Arrhythmias** --\> **sudden cardiac death**
123
What is **“broken heart syndrome”?**
* **Takotsubo cardiomyopathy** is a **dilated cardiomyopathy of the LV****(octopus trap**) that occurs after**extreme emotions**or**psychological stress** d/t too much catecholamines.
124
Who is most likely to get broken heart syndrome? Sx are similar to what?
* **90% W that are 58-75** * **Acute MI**
125
How does the \<3 change in Takosubo cardiomyopathy?
* **Apical ballooning of the LV**
126
Which type of **cardiomyopathy** has a 100% genetic cause?
**Hypertrophic cardiomyopath**y d/t **mutations in sarcomere proteins (B-myosin heavy chain)**
127
How is the gross morphology and contractility of **hypertrophic cardiomyopathy** different from dilated cardiomyopathy?
* - Heart is **thick-walled**, **heavy**, and **HYPERcontracting** in HCM - Compared to flabby + HYPOcontractingheart in DCM
128
In classic **hypertrophic cardiomyopathy** what **disproportionate thickening** do we see and what is a possible consequence?
**Ventricular septum** becomes **WAY THICCER** than the **left ventricle free wall,** termed **asymmetric septal hypertrophy =\> can block aorta (outflow)**
129
What does **hypertrophic cardiomyopathy** look like on histo?
* **Septum is hypertrophied** * **Myocyte disarray**
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What do you hear upon ausculatation during **hypertrophic cardiomyopathy and why?**
**Harsh ejection fraction** d/t obstraction in ventricular outflow as the mitral leaflets move toward the ventricular septum during systole
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What is the **most common cause** of s**udden, unexplained death in young athletes?**
**Hypertrophic Cardiomyopathy**
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**Which phase of the cardiac cycle is dysfunctional in Hypertrophic Cardiomyopathy?**
Diastole
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In **hypertrophic cardiomyopathy,** the **LV is poorly compliant =**\> **abnormal diastolic filling** and can lead to intermittent what?
**Ventricular outflow obstruction**
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What is the **essential** morphological feature of **Hypertrophic Cardiomyopathy?**
**Massive myocardial hypertrophy,** usually W/O ventricular dilation
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What are the 3 most important histologic features of **hypertrophic cardiomyopathy?**
**1. Myocyte hypertrophy** **2. Myofiber disarray** **3. Interstitial and replacement fibrosis**
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In hypertrophic cardiomypothay, what is the thickeniing of th eventricular septum called
**Asymmetric septal hypertrophy**
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What is a consequency of hypertrophic cardiomyopathy?
1. Dilation of left atria 2. MURAL THROMBUS =\> emboli and stroke 2. sudden death
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**Arrhythmogenic right ventricular cardiomyopathy (ARVC)** shows what characteristic morphology of the r**ight ventricle**?
**VERY thin** wall replaced **with fat a**nd **fibrosis**
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**Arrhythmogenic right ventricular cardiomyopath**y leads to failure of what and is associated with what rhythm disturbances?
* **RV failure** * **V-tach or v-fib =\> sudden death**
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**Arrhythmogenic right ventricular cardiomyopathy (ARVC)** has what inheritance pattern?
* **AD** * **defect in the desmosomes** that link cardiac myocytes.
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What is **Naxos syndrome?** Caused by what mutation?
* **Arrhythmogenic right ventricular cardiomyopathy,** with **hyperkeratosis** of the sole of foot and palm of hand * **Plakoglobin**: encodes desmosome-assx protein
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What is **restrictive cardiomyopathy**
**Decreased ventricular compliance (stiff),** causing **diastolic dysfunction** (impaired filling). often d/t a**myloid deposit** in the wall or **increased fibrsosis in radiation**
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What do the myocardium, ventricles and atria look like in **Restrictive Cardiomyopathy?**
- **Ventricles** = NL - **Myocardium** = firm and noncompliant - **Atria**: **BOTH dilated** * **have to work harder to fill ventricles**
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**Restrictive cardiomyopathy** is typically due to __________ (deposition of \_\_\_\_\_\_\_\_\_\_) in what part of the heart. _______ is often the misfolded culprit that leads to the disease.
* **Amyloidosis** (deposition of β-pleated sheets). * **INTERSTITIUM OF THE MYOCARDIUM** * **Transthyretin**
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Amyloidosis results from the extracellular accumulation of protein fibrils which form?
**Insoluble** β-pleated sheets
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**Amyloidosis** of the heart can appear as a consequence of what 2 underlying conditions?
- **Systemic amyloidosis** = **myeloma** - **Restricted to hear**t of older pt's w/ senile cardiac amyloidosis via transtheyretin
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Remember that **\_\_\_\_\_\_\_**stain is the stain for amyloidosis. Amyloid appears **\_\_\_\_\_**
Remember that **congo red** stain is the stain for amyloidosis. Amyloid appears **apple green**.
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Inflammation of the myocadrium, most likely d./t virus cocksackie A and B
**Myocarditis**
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Other causes of Myocarditis include what?
* **1. Chagas disease (trypanasoma cruzi)** * **2. Immune mediated (noninfective)** RF, SLE, drug hypersensitivity
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What is the distinct morphology of the **myocarditis** of **Chagas disease?**
**Parasite invasion of** scattered **myofibers:** **trypanosomes** w/ _mixed inflammatory infiltrate_ (**neutrophils**, **lymphocytes**, MO, and some EOS)
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**Chagas disease** is **10% fatal** during the acute attack, and many progress to **cardiac insufficiency in 10 - 20 years. E**osinophils will be present due to the infection, and we can see **\_\_\_\_** in the **myocytes** themselves.
**amistagotes**
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What stain is used for **Chagas**?
**Giemsa stain** typansoma cruzi
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**Lymphocytic** myocarditis ≡ \_\_\_\_\_\_\_ **Eosinophils** myocarditis ≡ \_\_\_\_\_\_\_\_ **Giant cell** myocarditis ≡ \_\_\_\_\_\_\_\_. **Amastigotes** mycardidits ≡ \_\_\_\_\_\_
* **Virus (cocksackie)** * **Chagas/hypersensitivty to drug** * **Chronic myocarditis** * **Chagas**
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What type of infiltrates are seen in **Hypersensitivity Myocarditis?**
Perivascular, composed of lymphocytes, macrophages, and **high proportion of eosinophils**
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**Giant-cell myocarditis** is characterized by widespread inflammatory infiltrates composed of?
**Multinucleate giant cells** + lymphocytes, eosinophils, plasma cells, and macrophages
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Non-infectious causes of myocarditis are due to what conditions?
- **Hypersensitivity** --\> Post-strep (RF), SLE, drug hypersensitivity, and transplant rejection - **Idiopathic** --\> Giant cell myocarditis
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Acute, rapidly developing fluid collections of **200-300 mL** in the pericardial sac can cause what fatal complication?
**Cardiac Tamponade**
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**Serous pericarditis** is characteristically produced by what 5 **non-infectious** inflammatory diseases?
1. **- Rheumatic fever** 2. **- SLE** 3. **- Scleroderma** 4. **- Tumors** 5. **- Uremia**
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What are the most frequent types of **pericarditis**?
**Fibrinous** and **serofibrinous** pericarditis
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What are the most common causes of **Fibrinous and Serofibrinous pericarditis?**
- Acute MI **- Postinfarction (Dressler) syndrome** **- Uremia** - Chest radiation - RF, SLE, and trauma
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* _______ pericarditis is dry, and finely granular. * _______ is charactarized by a **yellow-brown, turbid fluid with WBC, RBC, and fibrin**
* **Finrinous** * **Serofibrinous**
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The most striking finding suggesting **pericardial disease** is a \_\_\_\_\_\_\_\_
**loud pericardial friction rub.**
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**Purulent or suppurative pericarditis** reflecting a**n active infection** is most often a result of microbial invasion via what 4 routes?
* 1. Dir**ect extension** * **2. Seed from blood** * **3. Lymphatic extension** * **4. Direct introduction**
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The intense inflammatory rxn of purulent or suppurative pericarditis eventually causes scarring and commonly leads to what?
**_Constrictive pericarditis:_ heart is encased in fibrous scar =\> limits expansoin in diastole, CO**
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**Constrictive pericareditis** mimics what
**restrictive cardiomyopthy**
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What type of pericarditis is caused by the spread of a m**alignant neoplasm** into the pericardial space?
**Hemorrhagic pericaridits**
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Inflammatory diseases produce ____ pericarditis. Infections produce ____ pericarditis. Tuberculosis produces ____ pericarditis. Cancer produces a ____ pericardidit
* **Serous** * **Purulent/suppurative** * **Caseous** * **Hemorrhagic**
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\_\_\_\_\_\_ exudate is described as "bread and butter" pericarditis
***fibrous;*** ***_AMI, postinfarction, uremia,_***
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the top 5 PRIMARY tumors in the heart are all \_\_\_\_\_
**benign**
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**Top 5 tumors in the heart**
**1. Myxoma** **2. Fibroma** **3. Lipoma** **4. angiosarcoma**
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Myxocoma are the most common primary cardiac tumor. They are originate from the \_\_\_\_\_\_\_, are _______ (sit on a stalk), and can are called a "ball valve" why?
fossa ovalis pedunculated - Often mobile and cause i**ntermittent obstruction of AV valve during systole** - May exert "**wrecking ball" effect, damaging valve leaflets**
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Which cytokine elabortated by **Myxomas** is responsible for the consititutional sx's, such as fever and malaise?
**IL-6**
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what do we hear on auscultation of myxoma?
**tumor plop**
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Histologically myxomas are composed of what cells? Which peculiar structures are characteritics findings?
**- Stellate or globular myxoma cells w many nuclei** - Peculiar vessel-like or gland-like structures
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top malignant cancer of heart
angiosarcoma
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Most common cause of heart transplant \_\_\_\_\_\_\_ Major complication: \_\_\_\_\_\_\_\_\_\_
**Dilated cardiomyopathy or IHD** **Allograft rejection**
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Virtually all **heart transplants** develop ____________ presenting as **intimal proliferation** =\> causes stenosis =\> ischemia
**Allograft arteriopathy**
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What is a **silent MI?**
A transplanted heart is **denervated**, so if the pt has an MI, **they won’t feel it** ## Footnote **No angina either**
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