9. Heart 2 Flashcards

(152 cards)

1
Q

Arrhythmia from atrium = supraventricular

What is choatic depolarization without functional ventricular contraction?

A

Ventricular fibirillation

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2
Q

What is the most common cause of arrhythmias?

A

Ischemic injury

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3
Q

What occurs when the SA node is damaged leading to bradycardia?

A

Sick sinus syndrome

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4
Q

What occurs when myocytes depolarize independently and sporadically (atrial dilation) with variable transmission through AV node w/ irregular irregular HR?

A

Atrial fibrillation

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5
Q

When the AV node is dysfunctional, it is known as heart block
First degree: prolonged PR interval
Second degree:
Third degree: ?

A

Second: intermittent transmission

Third Degree heart block: complete failure

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6
Q

The following can be due to what?

Ischemic heart dz, dilated cardiomyopathy, myocyte hypertrophy, inflammation (myocarditis) and amyloid…

A

Gap junction abnormalities

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7
Q

Most hereditary heart conditions are autosomal dominant. What are mutations in genes that are required for normal ion channel function? (sometimes assoc w skeletal muscle DOs and diabetes)

A

Channelopathies

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8
Q

What is the most common arrythmogenic syndrome manifests as arryhthmias associated with excessive prolongation of the cardiac repolarization, presenting with stress induced syncope, SCD, assoc w swimming?

A

Long QT syndrome

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9
Q

What syndrome presents with patients having arrhythmias associated with abbreviated repolarization intervals, have palpitations, syncope and SCD?

A

Short QT syndrome

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10
Q

What syndrome manifests as ECG abnormalities (St segment elevations and right bundle branch block) in the absence of a heart DO?

A

Brugada Syndrome

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11
Q

Sudden cardiac death SCD is unexpected deeath from cardiac cause, either without symtpoms or within 1-24 hours of symptom onset. 80-90% of successively resuscitated patients have no?

A

lab or ECG changes

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12
Q

What is the most common underlying etiology, causing 80-90% of SCD?

A

Coronary artery disease CAD

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13
Q

Pt usually have >75% stenosis of 1+ of the coronary As, prior MIs are seen in 40% of the cases… WHat is the first manifestation of Ischemic heart dz? IHD

A

SCD

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14
Q

SCD is typically due to what, which is most frequently a consequence of CAD/ ischemia induce myocardial irritability?

A

fatal arryhtmia/ ventricular fibrillation

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15
Q

Systemic left sided hypertensive heart disease (HHD) occurs when?

A

there is a pressure overload resulting in left ventricular hypertrophy (LVH)

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16
Q

In left sided HHD, the LV wall is concentrically (septum and wall same) thickened (>1.5cm, weight >500gm). The earliest morphologic change of system HHD is?

A

that myocytes show and increase tranverse diameter

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17
Q

What dysnfunction can result in left atrial enlargement leading to atrial fibirillation?

A

diastolic dysfunction

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18
Q

Systemic or left sided HHD may lead to CHF and is a risk factor for?

A

SCD

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19
Q

Pulmonary right sided HHD is isolated right sided HHD that arises in the setting of?

A

pulmonary hypertension

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20
Q

Acute cor pulmonale may arise from a large pulmonary embolus, what can be seen in the heart in this case?

A

marked dilation of RV without hypertrophy (if chronic cor pulomonale will have hypetrophy)

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21
Q

What is the most common cause of pulmonary hypertension?

A

Left sided heart disease

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22
Q

What are two ways one can tell the difference grossly between left sided and right sided hypertensive heart disease?

A

Right sided wall thickened and will be the new apex of the heart in some cases
Left sided wall thickened concentrically

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23
Q

PAthologic changes of cadiac valves are largely of 3 types…
1) damage to collagen weakens leaflets (MVP)
2) Nodular calcification beginning in interstitial cells (calcific aortic stenosis)
3)

A

Fibrotic thickening as seen in rheumatic heart dz (mitral stenosis)

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24
Q

Because cardiac valves are thin enough to be nourished by diffusion from the blood, normal leaflets and cusps have only scant blood vessels limited to?

A

the proximal portion of the valve

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25
When does valvular disease come to clinical attention? 2
due to stenosis and insufficiency/ regurgiation / incompetence
26
What is used to describe the incompetence of a valve stemming from an abnormality in one of its support structures as opposed to a primary valve defect?
function (mitral valve) regurgitation *common in IHD and dilated cardiomyopathy
27
Stenosis is when a valve doesnt open completely occuring chronically, impeding forward flow, sometimes resulting in?
pressure overload hypertrophy (CHF)
28
Insufficiency is when a valve doesnt close completely, occuring acutely and chronically, allowing reversed flow or regurgitation, chornically causing?
volume overload hypertrophy and CHF
29
What is the only cause of mitral stenosis?
Posinflammatory scarring due to rheumatic heart disease***
30
What are two causes of aortic valve disease?
postinflammatory scarring d/t rheumatic heart dz | Calcification of congenitally deformed valve
31
What are two causes of aortic regurgitation?
Rheumatic heart dz | Aortic insufficiency: dilation of the ascending aorta secondary to HTN/aging
32
Syphilitic aortitis and marfan syndrome cause what valvle disease?
Aortic valve disease
33
Abnormalities of tensor apparatus and of the left ventricle and or annulus can lead to?
mitral valve disease
34
What are the two MCC of mitral regurgitation?
Abnormalities of leaflets and commissures | Mitral valve prolapse (myxomatous degen)
35
Calcific aortic stenosis is the most common of all valvular problems whose prevalence increases with age (60-80) and is caused by wear and tear associated with what? 3
chronic HTN hyperlipidemia inflammation
36
Calcific aortic stenosis has a HUGE association with what, which shows an accelerated course due to mechanical stress?
Bicuspid arotic valve (BAV)
37
If someone has a bicuspid aortic valve, there will be clinical sx 1-2 decades earlier.. Affected valves contain osteoblast like cells which deposit?
an osteoid-like substance and ossifies **no commisural fusion (as seen in rheumatic and congential aortic stenosis)
38
Calcific aortic stenosis has mounded calcification in cusps which prevent complete opening of the valve. What are 3 common sx?
angina CHF and syncope
39
LVH develops due to increase pressure of the valve not opening, most pts die in 5 years of devloping angina, in 3 of developing syncope and 2 in developing?
CHF *tx with surgical replacement
40
Biscuspid valves are prone to calcification. Patients can remain asymptomatic until stenosis reaches a critical point when?
CHF ensues.
41
In mitral annular calcification calcific deposits occurs in the?
fibrous annulus-at the base of the leaflets
42
annular calcification normally does not affect valve function however there are expections including regurg, stenosis and?
**arrhythmias and occasionally sudden death by penetration of calcium deposits to a depth sufficient to impinge on the atrioventricular conduction system
43
patients with mitral annular calcification are at an increased risk for thrombus formation or infective endocarditis.. Where is this most commonly seen? 3
Females more than Males greater than 60 y/o WITH mitral valve prolapse
44
Myxomatous degeneration of the mitral valve, OR mitral valve prolapse MVP is where the valve leaflets prolapse BACK into the left atrium during systole. Affects 2-3% of adults affecting M/F more? and what type of murmur?
7Females to 1 Male | *mid systolic click
45
Marfan syndrome (FBN1) causes loss of CT support in the MV leaflets making them floppy. The leaflets in MVP become thickened and rubbery due to?
proteoglycan deposits (myxomatous degeneration) in spongiosa layer and elastic fiber disruption
46
What is the characteristic anatomic change in MVP?
interchordal ballooning of the leaflets
47
Most MVPs are asymptomatic but a minority may experience what two sx?
chest pain mimicking angina | dyspnea
48
MVP has rare complications including infective endocarditis, mitral insufficiency, thromboembolism and?
arrhythmias=SCD *tx with valve replacement
49
Rheumatic fever is a multisystem inflammatory disorder following pharyngeal infection with?
group A streptococcus
50
Incidence of rheumatic fever has decreased with more rapid dx and tx of strep. Acute rheumatic fever may include carditis component and overtime may evolve into?
chronic rheumatic heart disease
51
The pathogenesis of rheumatic fever include immune response to streptococcall M proteins that cross react with?
cardiac self antigens (among others)
52
Acute RF occurs 10 day to 6 weeks after group a strep infection and can be determined by what two titers?
Anti-streptolysin O | Anti-DNAse B
53
acute RF symptoms include pancarditis, migratory polyarthritis, subcutaneous nodules and erythema marginatum (rash). It also involves a neuro DO with involuntary rapid purposeless movements known as?
Sydenham chorea
54
Cardiac features of RF include pancarditis (inflamm of entire heart) with Aschoff bodies contain T cells, plasma cells and plump activated macrophages known as?
Anitschkow cells ***** pathognomonic for RF | central wavy ribbon of chromatin- looks like catepillar
55
Inflammation and fibrinoid necrosis of endocardium on left sided valves seen with what?*
verrucae (vegetations) is seen in RF.
56
What causes mitral leaflet thickening, fusion of commissures with shortenin, fusion and thickening of cords?
Chronic Rhematic Heart disease
57
What is the ONLY cause of mitral stenosis?
chronic RHD
58
RHD causes left atrial enlargement leading to Afib and thromembolic events. It also causes right heart failure leading to?
right ventricular hypertrophy *infective endocarditis as well
59
What is marked by a form of granulomatous inflammation "aschoff nodules' centered around vessels?
acute rheumatic myocarditis
60
Where can one find maccallum plaques as seen in the heart in RHD?
Left atrium
61
Neovascularization is seen in RHD as well as what? which occurs due to calcification and fibrous bridging across the valvular commissures
Fish mouth or button hole stenosis
62
what is an infection of valves or endocardium, characterized by vegetations consisting of microbes and debris associated with underlying tissue destruction?
Infective endocarditis (IE) (acute and subacute)
63
What type of infectious endocarditis is a RAPIDLY progessing destructive infection of a previously normal valve?
ACUTE Infective endocarditis *requries surgery and abx
64
What type of infectious endocarditis is a slower-progressing infection of a previously DEFORMED valve (such as in chronic RHD)
SUBACTE infective endocarditis *tx abx alone
65
Predisposing conditions to IE including valvular abnormalities (RHD, prosthetic valves, MV prolapse) and bacteremia - including?4
***dental work/surgery site infection contaminated needle (IVDU) Compromised epithelium
66
Classic features of IE are friable, bulky, destructive valvular vegetations with what side valves being more common?
Left sided valves EXCEPT in IV drug users- right side more common
67
The friability of the vegetations lead to septic emboli or what if they are on the right side valve?
pulmonary embolism
68
The vegetations are mixtures of fibrin, inflammatory cells and organism, note subacte veges may have?
granulation tissue component
69
Patients with IE present with fever weightloss fatigue and flu-like symptoms. What are the sx for acute endocarditis?
fever, chills, weakness, lassitude
70
What organism affect previouslt damaged or abnormal valves *dental organisms?
Strep Viridans- subacute-not as bad
71
What organism is highly virulent and seen in IV drug users and normal valves?
Staph Aureus ***acute-bad
72
What organism is commonly infecting prothestic valves?
S. Epidermidis
73
``` What is the significance of the following? haemophilus actinobacillus cardiobacterium kingella eikenalla ```
HACEK organisms = commensal bacteria in the oral cavity
74
``` What are the different forms of vegetative endocarditis (warty lesions) in the following? Rheumatic Heart disease Infective endocarditis Nonbacterial thrombotic Endocarditis Libman-Sacks disease ```
Rheumatic Heart disease: warty line along line of closure Infective endocarditis: large and irregular masses on the valve cusps Nonbacterial thrombotic Endocarditis: small, bland vegetations along line of clousre Libman-Sacks disease: small/medium sized on either or BOTH sides of the valve leaflet
75
What is present in 90% of patients with left sided infective endocarditis?
Heart murmur
76
acute IE can cause mortality in less than 6 weeks (50%) and necrotizing ulcerative lesions. Its emboli can cause mycotic aneurysms and what is an outcome grossly?
RING ABSCESS : vegetation erode into underlying myocardium
77
The duke criteria is used for dianosing infective endocarditis which include?
system of labs, clinical, echo and cultures that identify IE
78
What are 4 COMMON important signs of infective endocarditis?
``` subungal splinter hemorrhages (d/t emobli) janeway lesions (red lesions hands/feet) osler nodes (subq painful nodules hands/feet) roth spots (retinal hemorrhaging) ```
79
What is characterized by small, STERILE thrombi on cardiac valve leaflets along the line of closure; loosely attached, not invasive, do NOT illicit inflam reaction?
nonbacterial thrombotic endocarditis (NBTE)
80
NBTE may be a source of emboli (produces infarcts in brain/heart). What is the IMP* malignancy it is associated with? along with spesis or catheter induced endocardial trauma
MUCINOUS ADENOCARCINOMAS
81
nonbacterial thrombotic endocarditis occurs on previously normal valves due to?
a hypercoaguable state
82
Libman sacks endocarditis is due to SLE, mitral valve is more commonly involved causing regurgitation. The cause is due to antiphospholipid syndrome associated with lupus
Not on the test
83
What is a systemic disorder marked by flushing, diarrhea, dermatitis and bronchoconstriction?
Carcinoid syndrome (eg caused by serotonin released from GI carcinoid tumor) - paraneoplastic syndrome!
84
Severity of the cardiac carcinoid symdrome lesions correlate with plasma levels of serotonin and urinary excretion of the serotonin metabolite..?
5-hydroxindoleacetic acid
85
What occurs when 50% of patients with carcinoid syndrome develop cardiac manifestations?
Carcinoid Heart disease
86
Carcinoid heart disease does not occur until there is a massive hepatic burden or damage since the liver normally does what?
catabolizes circulating mediatiors before they effect the heart
87
What heart valves are affects in carcinoid heart disease and why?
The right heart valves (and endocardium) since they are first in contact with the mediators, left side valves are protected due to pulmonary vascular bed degredation of mediators
88
The valvular plaques seen are similar to those in patients taking fenfluramine (appetite suppresant) or ergot alkaloids for migraines - both affect systemic serotonin metabolism. Why serotonin?
it has not been proven why this occurs or if serotonin inhibitors can fix the problem
89
carcinoid heart disease associated with carcinoid syndrome has intimal thickening (acid mucopolysaccharide-rich) and what other characteristic finding?
glistening white intimal plaque like thickenings of endocardial surfaces of the cardiac chambers and valve leaflet
90
What are the two types of valvular prosthese to replace damaged cardiac valves?
``` mechanical valves (nonphysologic material) tissue valves (porcine/bovine) ```
91
60% of valve recipients have serious problems within 10 years. What is a specific complication to mechanical valves?
thromboembolism due to the disruption of laminar flow (long time anticoags)
92
What is a specific complication to tissue valve replacements?
structural deterioration which is the most common for bioprosthetic valves (calcification and tear)
93
Cardiomyopathy means heart muscle disease. What are the three types of cardiomyopathies?
Dilated (MC-90%) Hypertrophic Restrictive
94
What is charcterized by progression cardiac dilation and systolic dysfunction, usually with dilated hypertrophy?
Dilated Cardiomyopathy DCM - most common
95
DCM is thought to be familial in 30-50% of cases (TTN titin mutations may account for 20%- AD). What is strongly linked to DCM? 2
Alcohol | Myocarditis (esp. prior evidence of Coxsackie B Virus)
96
Cardiotoxic drugs and substances are also known to cause DCM including doxorubicin (chemo), cobalt, and iron overload. How does iron do this?
Iron overload from hereditary hemochromatosis (HFE mutation) or multiple transfusions
97
What stain is used to see iron in heart cells?
Prussian blue iron stain
98
DCM causes a large, heavy flabby heart due to dilation of all four chambers, What is common? 2
**mural thrombi and embolism = stroke Also, functional regurgitation of valves
99
DCM usually manifests between ages 20-50yo causing progressive CHF with what sx? 3
dyspnea exertional fatigue dec. ejection fraction by 25%(end stage)
100
What are two important consequences/ outcomes of dialted cardiomyopathy? 2
arrhythmias (sudden death) | embolism
101
Another type of dilated cardiomyopathy which is caused by excess catecholamines from extreme emotional or physiological stress?
Takotsubo cardiomyopathy OR | broken heart syndrome
102
Broken heart syndrome occurs in mainly women aged 58-75. Symptoms are like acut myocardial infarction. What can be seen grossly?
apical ballooning of the left ventricle with abnormal wall motion and contractile dysfunction (octopus trap like)
103
Histologically in DCM, there is hypertrophied muscle cells with nelard nuclei but some are stretched and attenuated, along with a variable degree of?
interstitial and endocardial fibrosis
104
What is the main difference between start and end point of DCM and HCM?
DCM only has 20-50% genetic causes (TITIN) while HCM is caused 100% by genetic mutations in sarcomere patients. Also in HCM, you will see myofiber disarray.
105
What is an inherited disease of myocardium causing right ventricular failure and rhythym distrubances (ventricular tachycardia/ fibrillation) with SUDDEN DEATH?
arrhythmogenic right ventricular cardiomyopathy ARVC
106
in ARVC, myocardium of the right ventricular wall is replaced by **adipose and fibrosis** no muscle left. How do you get this disease?
familial, autosomal dominant - defective cell adhesion proteins in the desmosomes that link adjacent cardiac myocytes
107
What syndrome is ARVC with hyperkeratosis of plantar and palmar skin surfaces?
Naxos Syndrome
108
Naxos syndromes is specifically associated with mutations in the gene encoding the desmosome-associated protein ______?
plakoglobin
109
What is a genetic disorder leading to myocardial hypertrophy and diastolic dysfunction, leading to dec. stroke volume and often ventricular outflow obstruction?
Hypertrophic cardiomyopathy HCM
110
There are numerous mutations known involving sarcomeric proteins, the most common one is?
B-MHC : b-myosin heavy chain
111
HCM presents with massive myocardial hypertrophy often with marked septal hypertrophy, without dilation. What is important of this hypertrophy?
it is not concentric, instead eccentric asymetric septal hypertrophy (ventricular septum more thick than ventricular wall)
112
Microscopically, HCM shows myocyte/fiber disarray (haphazard disarray). What else can be seen?
interstitial and replacement fibrosis and fibrous endocardial plaques
113
What is said about the lumen in HCM, which is due to disproportionate septal and wall thickening without ventricular dilation?
Banana Lumen
114
What symptom of HCM is caused by the ventricular outflow obstruction as the anterior mitral leaflet moves toward the ventricular septum during systole?
HARSH systolic ejection murmur | hypertrophied ventricular septum BLOCKs aorta
115
Consequences of HCM include foci of myocardial ischemia, dec CO and inc pulomnary congestion leading to exertional dyspnea, arrhythmias and what other two important things?
``` SUDDEN DEATH (MC cause of sudden unexplained death in athletes) left atrial dilation & mural thrombus ```
116
What is caused by decreased ventricular compliance (inc stiffness), leading to diastolic dysfunction (impaired filling), while systolic function of the LV remains normal?
Restrictive Cardiomyopathy
117
Restrictive Cardiomyopathy may be secondary to deposition of material within the wall or increased fibrosis, each due to? 2
amyloid (deposition in wall) | radiation (increase fibrosis)
118
Restrictive Cardiomyopathy has patchy diffuse interstitial fibrosis and has normal sized ventricles but?
both atria can be enlarged
119
What is extracellular deposition of proteins which form an insoluble B pleated sheet, sometimes systemic (myeloma) or restricted to the heart (transthyretin)?
Amyloid
120
Certain mutations of transthyretin are more amyloidogenic, and it can involve different parts of the heart but when deposits are in the interstitium of the myocardium....?
resctrictive cardiomyopathy occurs
121
Amyloid can be seen in myocardial interstitium using what stain and light?
congo red stain and using polarized light which shows the amyloid as apple green color
122
Myocarditis is inflammation of the myocardium most commonly due to a virus in the US. Whats the most common virus?
Coxsackie A and B viruses | note viral=lymphocytes/bacterial=neutrophil
123
What disease causes 10% to die during acute attacks, progressing to cardiac insufficiency in 10-20yrs, and causing myocarditis?
Trypanosoma Cruzi (chagas dz)
124
Chagas can be seen with mixed inflammatory cell infiltrate (PMN, lymps, mø, and eosinophils), what can be seen in the myofibers?
parasitization of scattered myofibers (amastigotes to trypomastigotes in the heart muscle)
125
Some noninfectious causes of myocarditis include immune mediated reactions like RH, drug hypersensitivity and?
SLE
126
Active myocarditis is characterized by interstitial inflammatory infiltrate with focal myocyte necrosis, commonly lymphocytic. What can be seen histologically in hypersensitivity myocarditis?
interstitial infiltrates, principally perivascular composed of lymphocytes mø and eosinophils
127
What type of myocarditis is characterized by widespread inflammatory cellular infilitrate containing multinucleate giant cells with lympocytes, eosinophils, mø and plasma cells?
Giant cell myocarditis
128
Normal pericardial fluid is less than 50mL of clear, yellow colored fluid. Slow accumulation of fluid will allow how much and still be asymptomatic?
less than 500mL if slow enough = no sx *globular enlargement of heart on CXR
129
Acute accumulation of 200-300mL rapidly fast is clinically devastating and can cause?
Cardiac tamponade (pericardial effusion)
130
What are the two most common types of pericarditis?
fibrinous and serofibrinous
131
Both sero and fibrinous pericarditis can be due to AMI, post infarction (dresslers), chest irradiation, RF, SLE, trauma and most importantly?
UREMIA
132
Serofibrinous pericarditis is a yellow-brown turbid fluid (chunky) with WBC, RBC and fibrin while fibrinous pericarditis is?
dry, finely granular/rough
133
Some sx of sero/fibrinous pericarditis includ pain (sharp, pleuritic and position dependent), fever, +/- CHF. what is the MOST COMMON feature seen?
*** LOUD pericardial friction rub ***
134
What pericarditis is characteristically produced by noninfectious inflammatory diseases including rheumatic fever, SLE, sleroderma, tumors and uremia?
Serous pericarditis
135
What pericarditis is an active infection caused by microbial invasion of the pericadial space (direct extension, seeding from the blood, lympathic extension, direct introduction)?
Purulent or suppurative pericarditis
136
What pericarditis has an exudate composed of blood mixed with a fibrinous or suppurative effusion?
Hemorrhagic pericarditis | MC d/t spread of malignant neoplasm
137
What pericarditis is tuberculous in origin and infrequently fungal infections evoke the same reaction?
Caseous pericarditis
138
What pericarditis occurs when the heart is encased in a dense, fibrous or fibrocalcific scar that limits diastolic expansion and cardiac output, features that mimic a restrictive cardiomyopathy?
constrictive pericarditis (limits diastole relaxation)
139
Primary cardiac tumors are rare, the top 5 most common are all benign. What are the two most important?
Myxoma | Angiosarcoma
140
Metastatic tumors occur only in 5% of people. What is the most common primary, pedunculated (sessile (on stalk) ) tumor usually in the fossa ovalis (foramen ovale)?
Myxoma (left atrium MC)
141
Familial syndromes associated with myxomas have activing mutations in the GNAS1 gene, encoding what?
subunit of G protein Gsa (associated with mccune albright syndrome)
142
Myxomas range from a globular hard mass mottled with hemorrhade to soft, translucent, papillary, or villous lesion with a ?
gelatinous appearance ***UNIQUE
143
Sx of myxomas include constitutional symptoms (fever malaise d/t IL6), embolization and?
Ball-valve obstruction
144
Pedunculated form of myxoma can cause obstruction during systole of AV valve, which can do what ?
'wrecking ball' does damage to the leaflets | ausculation = tumor "plop"
145
On histology, myxomas are composed of stellate or globular myxoma cells embedded with an abundant acid mucopolysacchride groud substance.. Whats the buzz work for histo?
Multinucleated myxoma cells / giant cells
146
What cardiac tumor is usually incidental, sea-anemone-like lesions, most often identified at autopsy and 80% are located on valves?
Papillary fibroelastoma (look like laml excresecences)
147
What is a tumor of the pediatric heart, 50% due to sporadic mutations and 50% associated with tuberous sclerosis (mut in TSC1 hamartin or TSC2 tuberin)?
Rhabdomyoma
148
What is the only malignant endothelial neoplasm that primarily affects older adult hearts?
Angiosarcoma
149
Cardiac transplants occur about 3000/yr, mostly due to dilated cardiomyopathy and ischemic heart disease. What is the major complication?
Allograft rejection (check via endomyocardial bx)
150
Allograft arteriopathy is the most important long term limitation for cardiac transplant because there is late...?
progressive, diffusely stenosing intimal proliferation
151
Allograft arteriopathy develops in 50% of people in 5 years, and virtually all patients in 10 years. This leads to what?
silent myocardial infarctions (because of the denervated transplanted heart (no angina) )
152
Along with arteriopathy, what is common due to chronic T cell immunosupression?
EBV associated B cell lymphomas