Heart Part 2 Flashcards
1
Q
Arrhythmias:
What kind of injury is the most common cause of rhythm disorders?
A
Ischemic injury
2
Q
Arrhythmias:
What occurs during Sick Sinus Syndrome?
A
SA node damaged –> BRADYCARDIA
3
Q
Arrhythmias:
Condition where myocytes depolarize independently and sporadically (atrial dilation) with variable transmission thru AV node –> IRREGULAR IRREGULAR HR
A
atrial fibrillation
4
Q
Arrhythmias:
What is a heart block?
A
dysfunctional AV node
5
Q
Arrhythmias:
What do you see with a First degree heart block?
A
prolonged PR interval
6
Q
Arrhythmias:
What do you see with a Second degree heart block?
A
INTERMITTENT transmission
7
Q
Arrhythmias:
What do you see with a Third degree heart block?
A
COMPLETE failure
8
Q
Arrhythmias:
Hereditary conditions for Arrhythmias are dominant or recessive?
A
most autosomal dominant
9
Q
Arrhythmias:
mutations in genes that are required for normal ion channel function
- Can be assoc with skeletal muscle disorders and diabetes also; most common isolated to heart
A
Channelopathies
10
Q
What is the most common inherited arrhythmogenic disease?
A
Long QT syndrome
- Function is related to K+ channel, Na+ channel, and Caveolin
11
Q
What are some Inherited Arrhythmogenic Dzs?
A
- Long QT syndrome
- Function is related to K+ channel, Na+ channel, and Caveolin - Short QT syndrome
- Function is related to K+ channels - Brugada syndrome
- Function is related to Na+ channel and Ca+ channel - CPVT syndrome
- Function is related to diastolic Ca2+ release
12
Q
• Unexpected death from cardiac cause, either:
– WITHOUT symptoms, or
– Within 1-24 HRS of symptom onset
– 80-90% of successively resuscitated pts show NO lab or ECG change
A
Sudden cardiac death (SCD)
13
Q
• ? disease precipitates SCD in 80-90%
– Usually >?% STENOSIS or one or more of the 3 main coronary arteries
– Unfortunately, SCD often the first manifestation of ?
– Healed remote MIs seen in about 40%
A
• Coronary artery disease
- > 75%
- IHD
14
Q
What are some other causes of Sudden cardiac death (SCD) ?
A
cardiomyopathies, myocarditis, congenital abnormalities of the conduction system, myocardial hypertrophy
15
Q
Sudden cardiac death (SCD) is due to what?
A
SCD is due to a FATAL ARRHYTHMIA MOST often arising from ISCHEMIA-INDUCED MYOCARDIAL IRRITABILITY
16
Q
• Pressure overload results in left ventricular hypertrophy
(LVH)
- The LV wall is CONCENTRICALLY thickened (>1.5cm), weight ≥500gm
A
Left-sided (systemic) hypertensive disease
17
Q
Left-sided (systemic) hypertensive disease:
- Diastolic dysfunction can result in LEFT ATRIAL enlargement which can lead to?
- May lead to ?, and is a risk factor for ?
A
- atrial fibrillation (afib)
* CHF, … SCD
18
Q
Right-sided (pulmonary) hypertensive disease:
– Isolated right-sided hypertensive heart disease arises in the setting of?
A
- pulmonary hypertension
19
Q
Right-sided (pulmonary) hypertensive disease:
• Acute COR PULMONALE may arise from what?
- Marked dilation of RV without hypertrophy
A
• a large pulmonary embolus
20
Q
Right-sided (pulmonary) hypertensive disease:
What is the most common cause of pulmonary hypertension?
A
left-sided heart disease
21
Q
What are some disorders predisposing to Cor Pulmonale?
A
• Diseases of the Pulmonary Parenchyma:
- Cystic fibrosis
• Diseases of the Pulmonary Vessels:
- Recurrent pulmonary thromboembolism
- Primary pulmonary hypertension
- Extensive pulmonary arteritis (e.g., granulomatosis with polyangiitis)
- Drug-, toxin-, or radiation-induced vascular obstruction
- Extensive pulmonary tumor microembolism
• Disorders Affecting Chest Movement:
- Kyphoscoliosis
22
Q
Pathologic changes of valves are largely of three types: ?
A
1) damage to COLLAGEN that weakens the leaflets, exemplified by MITRAL VALVE PROLAPSE
2) nodular CALCIFICATION beginning in interstitial cells, as in CALCIFIC AORTIC STENOSIS
3) FIBROTIC THICKENING, the key feature in RHEUMATIC HEART DISEASE
23
Q
Cardiac valves:
Valvular disease can come to clinical attention due to?
A
stenosis, insufficiency
(synonyms: regurgitation or incompetence), or both
24
Q
Cardiac valves:
What term 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?
A
Functional regurgitation
25
Cardiac valves:
– FUNCTIONAL MITRAL VALVE REGURGITATION is particularly common and clinically
important in ?, as well as in ?
- IHD,.... dilated cardiomyopathy
26
Valvular Heart Dz:
• Valve disease may present with ? and/or ?
• Valve disease may present with stenosis and/or insufficiency
27
Valvular Heart Dz:
• ? : valve doesn’t OPEN completely, occurs chronically
– Impedes FORWARD flow
– Chronic stenosis may cause PRESSURE overload hypertrophy --> CHF
• Stenosis
28
Valvular Heart Dz:
• ?: valve doesn’t close completely, may occur acutely or chronically
– Allows REVERSED flow
– Chronic insufficiency may cause VOLUME overload hypertrophy --> CHF
• Insufficiency
29
Etiologies of Acquired Heart Valve Dz:
Mitral Valve Disease
1. Mitral Stenosis = ?
2. Mitral Regurgitation= ?
Aortic Valve Disease
1. Aortic Stenosis = ?
2. Aortic Regurgitation = ?
Mitral Valve Disease
1. Mitral Stenosis = Postinflammatory scarring (rheumatic heart disease) ****
2. Mitral Regurgitation=
- Abnormalities of Leaflets and Commissures ***
- Mitral valve prolapse ***
- Abnormalities of Tensor Apparatus
- Abnormalities of Left Ventricle and/or Annulus
Aortic Valve Disease
1. Aortic Stenosis = Calcification of congenitally deformed valve***
2. Aortic Regurgitation =
- Postinflammatory scarring (RHEUMATIC heart disease) **
- AORTIC INSUFFICIENCY : dilation of the ascending aorta, often secondary to hypertension and/or aging **
- Syphilitic aortitis
- Marfan syndrome
30
What is the most common valve abnormality?
Calcific aortic stenosis
31
Calcific aortic stenosis:
• Prevalence increases with age and usually manifests at what age?
* “wear and tear” is associated with?
* ? valves (BAV) show an ACCELERATED course (increased mechanical stress)
* Affected valves contain ?-LIKE CELLS, which deposit an osteoid-like substance --> ossifies
* ? prevent complete opening of the valve
* 60-80 years
* chronic HTN, hyperlipidemia, inflammation
• Bicuspid valves (BAV) show an accelerated course (increased mechanical stress)
- Bicuspid clinical sx 1-2 DECADES EARLIER than normal valve
* OSTEOBLAST
* Mounded calcifications in cusps prevent complete opening of the valve
32
What are the symptoms of Calcific aortic stenosis?
Treatment?
angina, CHF, or syncope
- LEFT VENTRICULAR HYPERTROPHY (LVH) develops from increased pressure
- Most patients with aortic stenosis will die within *5* years of developing ANGINA, within *3* years of developing syncope, and within *2* years of CHF
onset
- TX: surgical replacement
33
Calcific deposits occur in the FIBROUS ANNULUS
Mitral annular calcification
34
Mitral annular calcification:
• Normally does not affect valve function; however there are exceptions…
- REGURGITATION by interfering with physiologic contraction of the valve ring
- STENOSIS by impairing opening of the mitral leaflets
- ARRHYTHMIAS and occasionally SUDDEN DEATH by penetration of calcium deposits to a depth sufficient to impinge on the atrioventricular conduction system
35
Mitral annular calcification:
– Nodules may become sites for?
thrombus formation or infective endocarditis
36
Mitral annular calcification:
Most common in what gender and age with what condition?
- F > M,
| - > 60 years, with Mitral valve prolapse
37
Valve leaflets prolapse back into LA during systole
Mitral Valve Prolapse (MVP)
| "Floppy"
38
Mitral Valve Prolapse (MVP):
Most common in what gender?
Affects 2-3% adults in US; with a 7:1 = F:M, usually incidental (mid systolic click)
39
What syndrome is it commonly associated with?
Marfan syndrome: FIBRILLIN-1 (FBN-1) mutations; loss of connective tissue support in the mitral valve leaflets makes them soft and billowy, creating a so-called floppy valve
40
Mitral Valve Prolapse (MVP):
• Leaflets become thickened and rubbery, due to proteoglycan deposits (?) and elastic fiber disruption
MYXOMATOUS DEGENERATION
41
Mitral Valve Prolapse (MVP):
• Characteristic anatomic change in MVP is ?
of the mitral leaflets
interchordal ballooning (hooding)
42
What are the symptoms of Mitral Valve Prolapse?
• Most are ASYMPTOMATIC, a minority may experience:
– Chest pain mimicking angina (not exertional in nature)
– Dyspnea
43
What are some serious (but rare) complications of Mitral Valve Prolapse?
– Infective endocarditis
– Mitral insufficiency (+/- chordal rupture)
– Thromboembolism --> stroke or other systemic infarct
– Arrhythmias
44
What is a treatment for Mitral Valve Prolapse?
Valve repair or replacement surgery can be done for symptomatic patients or those with increased risk for significant complications; in USA, MVP is the most common cause for mitral valve surgery
45
a multisystem inflammatory disorder following pharyngeal
| infection with group A streptococcus
Rheumatic fever
46
What is the pathogenesis of Rheumatic fever?
immune response to streptococcal M proteins cross reacts with cardiac (among other) self- antigens
47
What tests are used for Acute Rheumatic fever?
Anti-streptolysin O and anti-DNase B
48
What are the symptoms of Rheumatic fever?
PANCARDITIS, MIGRATORY POLYARTHRITIS (large jts), subcutaneous nodules,
RASH (ERYTHEMA MARGINATUM)
- SYDENHAM CHOREA: neurologic disorder with involuntary rapid, purposeless movements
49
What are the cardiac features of Acute RF?
- PANCARDITIS, featuring ASCHOFF BODIES. Also activated macrophages called ANITSCHKOW CELLS
- Inflammation and FIBRINOID NECROSIS of endocardium and left-sided valves, with VERRUCAE (vegetations)
50
What are the cardiac features of Chronic RF?
• mitral leaflet THICKENING; FUSION of commissures; with shortening, fusion and thickening of cords, --> MITRAL STENOSIS (virtually only cause of mitral stenosis)
- LA ENLARGEMENT --> atrial fib (arrhythmias), thromboembolic events
- pulmonary congestion/RHF; RIGHT VENTRICULAR HYPERTROPHY
51
An infection of valves or endocardium, characterized by vegetations consisting of microbes and debris, assoc with underlying tissue destruction
- Aorta, aneurysms, other blood vessels and prosthetic devices can become infected
Infective endocarditis (IE)
52
• ? infective endocarditis is a RAPIDLY PROGRESSING,
destructive infection of a PREVIOUSLY NORMAL valve
- Treatment?
Acute
- Requires SURGERY in addition to antibiotics
53
• ? infective endocarditis is a slower-progressing
infection of a previously deformed valve (such as in
chronic RHD)
- Treatment?
Subacute
- Can often be cured with ANTIBIOTICS alone
54
What are some predisposing conditions to Infective endocarditis?
1. Valvular abnormalities
- RHD, *PROSTHETIC VALVES*, MV prolapse, calcific stenosis, bicuspid AV
2. Bacteremia
- Another site of infection
- Dental work/surgery
- Contaminated needle
- Compromised epithelium
55
What is a classic feature of Infective endocarditis?
friable, bulky, destructive valvular vegetations
56
Classic features of Infective endocarditis:
- ?-sided valves are more commonly affected
- Right-sided valves often involved in?
- Friability leads to? (PE if right side valve)
- Left-sided
- IV drug abusers
- septic emboli
57
What are the symptoms of Acute Endocarditis?
fever chills, weakness, and lassitude
58
Infective endocarditis:
? are usually present (90%) with LEFT-sided lesions
Murmurs
59
What organisms are often involved with Infective endocarditis
– S. viridans (valve abnormalities)
–S. aureus (normal valves, abnormal valves, IV drug abusers)
–S. epidermidis (prosthetic valves)
– HACEK (Hemophilus, Actinobacillus, Cardiobacterium, Eikenella, Kingella)
60
Infective Endocarditis:
Acute bacterial vs Subacute (SBE)
Acute Bacterial:
• <6 weeks with 50% mortality
• Highly VIRULENT organisms
• NORMAL valves
• Necrotizing, ulcerative DESTRUCTIVE lesions
• Micro: fibrin, inflammatory cells, and organisms
• RAPID onset of symptoms
• Difficult to cure; SURGERY
• Aortic and mitral valves
• Right heart valves if IV DRUG USER
• Ring abscess: vegetation erode into underlying myocardium
• EMBOLI --> septic infarcts or MYCOTIC aneurysm
```
Subacute (SBE):
• >6 weeks, most survive with treatment
• Less virulent organisms; INSIDIOUS
• ABNORMAL valves
• Less destructive lesions
• Micro: granulation tissue, fibrosis, CALCIFICATION, CHRONIC inflammatory cells
• Vague FLU-LIKE symptoms
• ANTIBIOTICS
```
61
Important minor diagnostic criteria for Infective Endocarditis
- subungual/splinter hemorrhages
- Janeway lesions
- Osler nodes
- Roth spots
62
• Small, STERILE thrombi on cardiac valve leaflets, along the line of closure
- Loosely attached, not invasive, do not illicit an inflammatory reaction
Nonbacterial Thrombotic Endocarditis (NBTE)
63
Nonbacterial Thrombotic Endocarditis (NBTE):
* May be a source of ? (produce infarcts in the brain, heart,…)
* **Associated with malignancies (especially ?), sepsis, or catheter-induced endocardial trauma**
* emboli
| * mucinous adenocarcinomas
64
a systemic disorder marked by
FLUSHING, DIARRHEA, DERMATITIS, AND BRONCHOCONSTRICTION; bioactive compounds such as SEROTONIN released by carcinoid tumors
Carcinoid syndrome
65
Carcinoid syndrome:
- plasma levels of serotonin and urinary excretion of the serotonin metabolite ? correlate with the severity of the cardiac lesions
5-hydroxyindoleacetic acid
66
Carcinoid Heart Disease:
What side is usually affected?
- Right endocardium and valves; left side is protected due to pulmonary vascular bed degradation of mediators
67
What are the types of cardiomyopathies?
* Dilated cardiomyopathy
* Takotsubo cardiomyopathy
* Arrhythmogenic right ventricular cardiomyopathy (ARVC)
* Hypertrophic cardiomyopathy • Restrictive cardiomyopathy
* Amyloid
68
What is the most common and least common cardiomyopathy?
- Most common: Dilated Cardiomyopathy ***(90%)
| - Least common: Restrictive Cardiomyopathy
69
What is a cause of phenotype associated with Dilated Cardiomyopathy?
Hemochromatosis
70
Progressive cardiac dilation and systolic dysfunction, usually with dilated hypertrophy
Dilated cardiomyopathy (DCM)
71
Dilated cardiomyopathy (DCM) Pathogenesis:
* Thought to be familial in 30-50% of cases (TTN mutations may account for 20% of all cases); usually autosomal ?
* ? is strongly linked to DCM
* Myocarditis
• Cardiotoxic drugs/substances: doxorubicin, cobalt, ? overload
- Iron overload from ? (HFE mutation) or multiple transfusions
* dominant
* Alcohol is strongly linked to DCM
• IRON
- hereditary hemochromatosis
72
What is the morphology of Dilated cardiomyopathy (DCM)?
– dilation of all chambers
– MURAL THROMBI are common
– functional REGURGITATION of valves
73
What is the presentation for Dilated cardiomyopathy (DCM)?
– usually manifests between ages **20-50yo**
– progressive CHF --> dyspnea, exertional fatigue, Decreased EF (<25% end stage)
–ARRHYTHMIAS (SUDDEN DEATH)
–EMBOLISM
74
“Broken heart syndrome”
Takotsubo cardiomyopathy (a dilated cardiomyopathy)
75
Takotsubo cardiomyopathy (a dilated cardiomyopathy):
What is there an excess of following extreme emotional or
psychological stress?
catecholamines
76
What is the age and gender associated with Takotsubo cardiomyopathy?
>90% women, ages 58-75
77
The symptoms and signs of Takotsubo cardiomyopathy are similar to what condition?
acute myocardial infarction
78
What happens during Takotsubo cardiomyopathy?
APICAL BALLOONING OF THE LEFT VENTRICLE WITH abnormal wall motion and contractile dysfunction
79
What molecular structures are associated with:
1. Dilated Cardiomyopathy
2. Hypertrophic Cardiomyopathy
3. Dilated + Hypertrophic
1. Dilated Cardiomyopathy
- Gamma- Sarcoglycan
- Dystrophin
- Desmin
- Mitochondrial proteins
- Titin
- Lamin A/C
2. Hypertrophic Cardiomyopathy
- Myosin binding protein C
- Myosin light chains
3. Dilated + Hypertrophic
- Beta-Myosin heavy chain
- Actin
- Troponin I/T
- alpha- Tropomyosin
80
What is the percentage of genetic causes for Dilated Cardiomyopathy and Hypertrophic Cardiomyopathy ?
- Dilated Cardiomyopathy = 20-50% genetic causes
| - Hypertrophic Cardiomyopathy = 100% genetic causes (sarcomeric proteins)
81
What is an important phenotype for Hypertrophic Cardiomyopathy?
Myofiber disarray
82
Hypertrophic Cardiomyopathy and Dilated Cardiomyopathy have the same end points. What are they?
Clinical:
- Heart failure
- Sudden death
- Atrial fibrillation
- Stroke
83
• Right ventricular failure and arrhythmias
– Myocardium of the RIGHT VENTRICULAR wall replaced by ADIPOSE AND FIBROSIS
– Causes ventricular tachycardia or fibrillation --> SUDDEN DEATH
Arrhythmogenic right ventricular cardiomyopathy ( ARVC)
84
What are the genetics associated with Arrhythmogenic right ventricular cardiomyopathy ( ARVC)?
• Familial, AUTOSOMAL DOMINANT
– defective cell adhesion proteins in the desmosomes that link adjacent cardiac myocyte
85
What syndrome is associated with Arrhythmogenic right ventricular cardiomyopathy (ARVC)?
• NAXOS SYNDROME: ARVC with hyperkeratosis of plantar palmar skin surfaces***
86
Arrhythmogenic right ventricular cardiomyopathy (ARVC):
- Naxos Syndrome is associated with what mutation?
mutations in the gene encoding the desmosome-associated protein PLAKOGLOBIN
87
A genetic disorder leading to myocardial hypertrophy and diastolic dysfunction, leading to decreased stroke volume and often ventricular outflow obstruction
Hypertrophic cardiomyopathy
88
Hypertrophic cardiomyopathy:
• Numerous mutations known, involving SARCOMERIC proteins; most commonly?
Most commonly Beta-myosin heavy chain (Beta-MHC)
89
What is the morphology of Hypertrophic cardiomyopathy?
– massive myocardial hypertrophy, often with MARKED SEPTAL HYPERTROPHY (usually without dilation)
– Microscopically, MYOCYTE DISARRAY
90
What are the symptoms of Hypertrophic cardiomyopathy?
harsh systolic ejection murmur, caused by the ventricular outflow obstruction as the anterior mitral leaflet moves toward the ventricular septum during systole
91
Hypertrophic cardiomyopathy:
• What are the consequences of extensive hypertrophy?
– Foci of myocardial ischemia may occur
– LEFT ATRIAL DILATION and MURAL THROMBUS
– Diminished cardiac output and increased pulmonary congestion leads to exertional dyspnea
– Arrhythmias
–SUDDEN DEATH (sudden, otherwise unexplained death in young athletes)
92
Decreased ventricular compliance (increased stiffness), leading to DIASTOLIC DYSFUNCTION (impaired filling), while systolic function of the LV remains normal
Restrictive cardiomyopathy
93
Restrictive cardiomyopathy:
* May be secondary to ? within the wall (amyloid), or increased ? (radiation)
* Ventricles are usually of normal size, but both atria can be?
* May be secondary to DEPOSITION OF MATERIAL within the wall (amyloid), or increased FIBROSIS (radiation)
* enlarged
94
Restrictive cardiomyopathy:
What is an extracellular deposition of proteins which form an insoluble Beta-pleated sheet ?
Amyloid
95
Restrictive cardiomyopathy: Amyloid
• May be systemic (myeloma) or restricted to the heart (usually ?)
- Certain mutated versions of transthyretin are more amyloidogenic
- Amyloid can involve different parts of the heart, but when deposits are in the ? of the myocardium, a RESTRICTIVE cardiomyopathy results
• May be systemic (myeloma) or restricted to the heart (usually TRANSTHYRETIN)
- interstitium
96
What staining is involved with Amyloid restrictive cardiomyopathy?
Congo red stain:
| apple green birefringence
97
Inflammation of the myocardium, most commonly due to a virus in US
Myocarditis
98
What viruses are most commonly associated with Myocarditis?
Coxsackie A and B viruses
99
What are some other infectious causes of Myocarditis?
1. Trypanosoma cruzi (Chagas disease)
- 10% die during acute attack
- May progress to cardiac insufficiency in 10-20 years
- PARASITIZATION of scattered myofibers; MIXED INFLAMMATORY CELL INFILTRATE (PMN, lymphs, macrophages, and occ EOSINOPHILS)
2. Various bacteria and fungi
100
What are some noninfectious causes of Myocarditis?
Immune mediated reactions; including RF, SLE, drug hypersensitivity **
101
What stain is associated with Trypanosoma cruzi (Chagas)?
Giemsa-stained
| also look for amastigotes
102
Which disease has:
• Slow accumulation fluid, <500mL asymptomatic if slow enough
– Globular enlargement of heart shadow on CXR
Pericardial Dz
103
What condition is associated with acute Pericardial disease?
200-300mL rapid accumulation --> CARDIAC TAMPONADE
104
What are the most common types of Pericardial disease?
Fibrinous and serofibrinous
105
Fibrinous and serofibrinous Pericardial Disease:
What causes this?
AMI, POSTINFARCTION (Dressler’s), UREMIA, chest irradiation, RF, SLE, trauma…
106
What symptoms are associated with Fibrinous and serofibrinous Pericardial Disease?
pain (sharp, pleuritic and position dependent), fever, +/- CHF
107
What is the most striking feature of Fibrinous and serofibrinous Pericardial Disease?
Loud pericardial friction rub most striking feature
108
Types of Pericarditis:
characteristically produced by noninfectious inflammatory diseases, including rheumatic fever, SLE, and scleroderma, as well as tumors and uremia
Serous pericarditis
109
Types of Pericarditis:
active infection caused by microbial invasion of the pericardial space (direct extension, seeding from the blood, lymphatic extension, direct introduction)
Purulent or suppurative pericarditis
110
Types of Pericarditis:
Exudate composed of blood mixed with a fibrinous
or suppurative effusion
- What is it most commonly caused by?
Hemorrhagic pericarditis
– most commonly caused by the spread of a MALIGNANT NEOPLASM to the pericardial space
111
Types of Pericarditis:
Tuberculous in origin; infrequently, fungal infections
evoke a similar reaction
Caseous pericarditis
112
Types of Pericarditis:
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
113
Primary Cardiac Tumors are rare and the top 5 are all BENIGN. Examples?
MYXOMA, fibromas, lipomas, papillary fibroelastomas, rhabdomyomas and ANGIOSARCOMAS
114
What is the most common primary cardiac tumor?
- PEDUNCULATED (or sessile) usually in region of fossa ovalis
Myxoma
115
Primary Cardiac Tumor:
What mutations and associated syndromes are involved with Myxoma?
Familial syndromes associated with myxomas have activating mutations in the *GNAS1 gene*, encoding a subunit of G protein (Gsα) (in association with MCCUNE-ALBRIGHT SYNDROME)
or null mutations in *PRKAR1A* , encoding a regulatory
subunit of a cyclic-AMP-dependent protein kinase (CARNEY COMPLEX)
116
Primary Cardiac Tumor:
What do Myxomas look like?
**Globular hard mass, mottled with hemorrhage to soft, translucent, papillary or villous with a gelatinous appearance
117
Primary Cardiac Tumor:
What are the symptoms of a Myxoma?
“BALL-VALVE” OBSTRUCTION, embolization or constitutional sx (fever and malaise)
- Pedunculated form cause intermittent obstruction (position dependent) during systole of AV
valve; or “WRECKING BALL” causing damage to the valve leaflets
- Constitutional sx: elaboration by some myxomas of the *IL-6*, a major mediator of the acute-phase response
118
Primary Cardiac Tumor:
What do you hear on auscultation for a Myxoma?
Auscultation: tumor “plop”
119
Which Primary Cardiac Tumor type is this:
localized, well-circumscribed, benign tumors composed of mature fat cells; subendocardium, subepicardium, or myocardium
Lipoma
120
Which Primary Cardiac Tumor type is this:
• Usually incidental, sea-anemone-like lesions, most often identified at autopsy; usually (>80%) located on valves
– They resemble the much smaller, usually trivial, LAMBL EXCRESCENCES that may represent remotely organized thrombus on the aortic valves of older individuals
Papillary Fibroelastoma
121
Which Primary Cardiac Tumor type is this:
most frequent primary tumor of the PEDIATRIC heart
- 50% sporadic mutations
- 50% associated with tuberous sclerosis (mutations in the TSC1 (Hamartin) or TSC2
(Tuberin) tumor suppressor gene)
Rhabdomyoma
122
Which Primary Cardiac Tumor type is this:
malignant endothelial neoplasm that primarily affects
older adults
Angiosarcoma
123
Cardiovascular Effects of Noncardiac Neoplasms:
What are the direct consequences of the tumor?
* Pericardial and myocardial metastases
* Large vessel obstruction
* Pulmonary tumor emboli
124
Cardiovascular Effects of Noncardiac Neoplasms:
What are the indirect consequences of the tumor (complications of circulating mediators)?
* Nonbacterial thrombotic endocarditis
* Carcinoid heart disease
* Pheochromocytoma-associated heart disease
* Myeloma-associated amyloidosis
125
Cardiovascular Effects of Noncardiac Neoplasms:
What are the effects of tumor therapy?
* Chemotherapy
| * Radiation therapy
126
What are the most common diseases associated with cardiac transplantation?
DCM and IHD are the most common
127
What is a major complication of cardiac transplantation?
allograft rejection
| - Routine endomyocardial bx to monitor
128
What is the most important long term limitation to cardiac transplantation?
Allograft arteriopathy
129
Cardiac transplantation: Allograft arteriopathy
– Late, progressive, diffusely ?
– 50% develop in 5 years, and in virtually all patients within 10 years
– Silent MI: denervated transplanted heart (NO angina)
- STENOSING INTIMAL PROLIFERATION
130
Cardiac transplantation:
• Needed for ? due to chronic T-cell immunosuppression
EBV associated B-cell lymphoma
131
What is the overall survival for cardiac transplantation?
Overall survival: 90% at 1 year, >60% at 5 years
132
What are some complications of a heart transplant?
- Lymphocytic infiltrate
| - Intimal thickening
