Cardio Pathology Flashcards
1
Q
Factor V Leiden
A
- more common in Caucasian
- alters an amino acid residue in factor V and renders it resistant to protein C (failure of protein C to prolong aPTT)
- heterozygotes carry 5-fold increased risk for venous thrombosis, with homozygotes having a 50-fold increased risk
- seen to exist in compound states with other defects (i.e. protein C, protein S, or ATIII deficiency)
- 35 fold incrtease of developing DVT if taking OCPs
2
Q
Prothrombin mutation (G20210A variant)
A
- single-nucleotide substitution
- results in more stable mRNA and increased prothrombin transcription
- three-fold increased risk for venous thromboses
- heterozygotes at increased risk of VTE
- compound heterozygous state in conjunction with other thrombophilias seen
- young age of first thrombosis
- up to 40% of homozygotes may not experience thrombotic event
3
Q
Hyperhomocysteinemia
A
- vitamin B12 deficienc
- folate deficiency
- lack of vitamin B12 or folate leads to decreased conversion of homocysteine to methionine resulting in buildup of homocysteine
- cystathionine beta synthase (CBS) deficiency results in high homocystein elevels with homocystinuria
- CBS converts homocysteine to cystathionine; enzyme build up leads to homocysteine buildup
- characterized by vessel thrombosis mental retardation, lens dislocation, and long slender fingers
4
Q
Heparin-Induced Thrombocytopenic (HIT) syndrome
A
- occurs in up to 5% of patients treated with unfractionated heparin
- development of auto-antibodies that bind complexes of heparin and platelet membrane protein (PF-4)
- antibodies may bind similar complzes present on platelet and endothelial surfaces, resulting in platelet activation, aggregation, and consumption (thrombocytopenia)
- causes prothrombotic state
- fragments of destroyed platelets may activate remaining platelets, leading to thrombosis
5
Q
Antiphospholipid Antibody Syndrome
A
- recurrent thrombosis, repeated miscarriages, cardiac valve vegetations, thrombocytopenia
- associated with autoantibodies directed against anionic phospholipids (e.g. cardiolipin) or , more accurately, plasma protein antigens that are unveiled by binding to such phospholipids (e.g. prothrombin)
- induces hypercoagulable state, perhaps by inducing endothelial injury, by activating platelets or complement directly, or by interacting with the catalytic domains of certain coagulation factors
- secondary antiphospholipid syndrome –> well defined autoimmune disease such as lupus
- primary antiphospholipid syndrome –> hypercoagulable state without evidence of another autoimmune disorder
6
Q
Protein C/Protein S Deficiency
A
- decreases negative feedback on the coagulation cascade
- normally inactivate factors V and VIII
- increased risk of warfarin skin necrosis
- in preexisting C or S deficiency, a severe deficiency is seen at the onset of warfarin therapy increasing risk for thrombosis, especially in the skin
7
Q
Antithrombin III Deficiency
A
- decreases the protective effect of heparin-like molecules produced by the endothelium
- increases risk of thrombus
- heparin-like molecules normally activate ATIII, which inactivates thrombin and coagulation factors
- in ATIII deficiency, PTT does not rise with standard heparin dosing because heparin levels cannot activate limited ATIII
- high doses of heparin activate limited ATIII; coumadin is then given to maintain an anticoagulated state
8
Q
Thrombotic Thrombocytopenic Purpura (TTP)
A
- pathologic formation of platelet microthrombi in small vessels
- platelets are consumed in the formation of microthrombi
- RBCs are sheared as they cross microthrombi, resulting in hemolytic anemia with schistocytes
- due to decreased ADAMTS12, an enzyme that normally cleaves vWF multimers into smaller monomers for eventual degradation
- large uncleaved multimers lead to abnormal platelet adhesion, resulting in microthrombi
9
Q
Warfarin Induced Skin Necrosis
A
- microvascular thrombosis
- may occur in patients with heterozygous protein C or S deficiency if a high initial dose is used or heparin overlap is inadequate
10
Q
Bernard-Soulier syndrome
A
- due to a genetic GPIb deficiency
- platelet adhesion is impaired
- blood smear shows mild thrombocytopenia with enlarged platelets
11
Q
Glanzmann Thrombasthenia
A
- genetic GPIIb/IIa deficiency
- platelet aggregation impaired
12
Q
Aspirin
A
- irreversibly inactivates cyclooxygenase
- lack of TXA2 impairs aggregation
13
Q
Uremia
A
- disrupts platelet function
- both adhesion and aggregation are impaired
14
Q
Hemophilia A
A
- genetic factor VIII deficiency
- X-linked recessive
- deep tissue, joint, and postsurgical bleeding
- increased PTT; normal PT
- normal platelet count and bleeding time
- treatment: recombinant FVIII
15
Q
Hemophilia B
A
- genetic factor IX deficiency
- resembles hemophilia A
16
Q
Von Willebrand Disease
A
- genetic vWF deficiency
- most common inherited coagulation disorder
- mild mucosal and skin bleeding
- increased bleeding time
- increased PTT; normal PT (decreased VIII half-life)
- abnormal ristocetin test
17
Q
Ristocetin
A
- induces platelet agglutination by causing vWF to bind platelet GPIb
- lack of vWF –> impaired agglutination –> abnormal test
18
Q
Vitamin K Deficiency
A
- vit. K is activated by epoxide reductase in the liver
- activated vit. K gamma carboxylates factors II, VII, IX, X, and Proteins C and S
- deficiency in newborns (lack of GI colonization)
- deficiency in long-term antibiotic use
- malabsorption causes deficiency
19
Q
Disseminated Intravascular Coagulation (DIC)
A
- pathologic activation of the coagulation cascade
- widespread microthrombi result in ischemia and infarction
- consumption of platelets and factors results in bleeding
- secondary to OB complications, sepsis (endotoxins), adenocarcinoma, acute promyelocytic leukemia, rattlesnake bite
- decreased platelet count
- increased PT/PTT
- decreased fibrinogen
- elevated D-dimer
20
Q
Temporal (Giant Cell) Arteritis
A
- large-vessel vasculitis
- granulomatous vasculitis of branches of carotid artery
- headache (temporal artery), visual disturb. (ophthalmic artery, jaw claudication
- flu sxs and joint pain (polymyalgia rheumatica) - ESR elev.
- inflammed vessel wall with giant cells and intimal fibrosis
- treatment: corticosteroids
- high risk of blindness without treatment
21
Q
Takayasu Arteritis
A
- large-vessel vasculitis
- granulomatous vasculitis of aortic arch at branch pts
- adults )
- ESR elevated
- treatment: corticosteroids
22
Q
Polyarteritis Nodosa
A
- medium vessel vasculitis
- necrotizing vasculitis involving multiple organs
- lungs are spared
- HTN in young adults (renal artery involvement)
- abd pain with melena (mesenteric artery)
- neurologic disturbances and skin lesions
- serum HBsAg
- early lesions: transmural inflamm. with fibrinoid necrosis
- late lesions: heal with fibrosis, producing “string of pearls” appearance on imaging
- treatment: corticosteroids and cyclophosphamide
- fatal if not treated
23
Q
Kawasaki Disease
A
- medium vessel vasculitis
- Asian children < 4 years old
- nonspecific sxs: fever, conjunctivitis, erythematous rash of palms and soles, enlarged cervical lymph nodes
- coronary artery involvement is common
- thrombosis with MI
- aneurysm with rupture
- treatment: aspirin and IVIG
24
Q
Buerger Disease
A
- medium vessel vasculitis
- necrotizing vasculitis involving digits
- ulceration, gangrene, autoamputation of fingers/toes
- Raynaud phenomenon often present
- highly associated with heavy smoking
- treatment: smoking cessation
25
Wegener Granulomatosis
- small vessel vasculitis
- necrotizing granulomatous vasculitis involving nasopharynx, lungs, kidneys
- middle-aged male with sinusitis or nasopharyngeal ulceration, hemoptysis with bilateral nodular lung infiltrates, and hematuria (rapidly dev. glomerulonephritis)
- treatment: cyclophosphamide and steroids
26
Microscopic Polyangiitis
- small vessel vasculitis
- necrotizing vasculitis involving multiple organs
- commonly the lungs and kidneys
- similar to Wegener, but nasopharyngeal involvement and granulomas are absent
- treatment: corticosteroids and cyclophosphamide
27
Churg-Strauss Syndrome
- small vessel vasculitis
- necrotizing granulomatous inflammation with eosinophils involving mult. organs, (lungs and heart esp.)
- asthma and peripheral eosinophilia are often present
28
Henoch-Schonlein Purpura
- small vessel vasculitis
- due to IgA immune complex deposition
- most common vasculitis in children
- palpable purpura on buttocks and legs
- GI pain and bleeding
- hematuria (IgA nephropahty)
- usually occurs following URI
- treatment: steroids, if severe
29
Atherosclerosis
- intimal plaque that obstructs blood flow
- necrotic lipid core with fibromusc. cap
- often undergoes dystrophic calcification
- large and medium sized arteries
- damaged endothelium - lipids leak into intima
- lipids consumed by macrophages - foam cells
- inflammation and healing - deposition of ECM and proliferation of smooth muscle
- begins as fatty streaks (arise early in life)
- peripheral vascular disease (e.g. popliteal)
- angina (coronary arteries)
- ischemic bowel disease (mesenteric arteries)
- MI and stroke due to plaque rupture
- aneurysm due to weakening vessel wall (e.g. abd aorta)
30
Hyaline Arteriolosclerosis
- narrowing of small arterioles
- hyaline: proteins leaking into the vessel wall causing vascular thickening; proteins seen as pink hyaline
- consequence of long-standing benign HTN or diabetes
- reduced vessel caliber with end-organ ischemia
- glomerular scarring that slowly progresses to chronic renal failure
31
Hyperplastic Arteriolosclerosis
- narrowing of small arterioles
- thickening of vessel wall by hyperplasia of smooth muscle ("onion-skin" appearance)
- consequence of malignant hypertension
- reduced vessel caliber with end-organ ischemia
- fibrinoid necrosis of the vessel wall with hemorrhage
- acute renal failure with characteristic "flea-bitten" appearance
32
Monckeberg Medial Calcific Sclerosis
- calcification of the media of muscular (medium-sized) arteries
- non-obstructive
- not clinically significant; seen as incidental finding on CXR or mammography
33
Aortic Dissection
- intimal tear with dissection of blood through media
- proximal 10 cm of aorta (high stress) with preexisting weakness of the media
- hypertension and inherited defects of connec. tissue
- hypertension results in hyaline arteriolosclerosis of the vasa vasorum, which decreases flow; atrophy to media
- Marfan syndrome and Ehlers-Danlos syndrome
- sharp, tearing CP that radiates to back
- complications: pericardial tamponade (most common cause of death), rupture with fatal hemorrhage, obstruction of branching arteries with end-organ ischemia
34
Thoracic Aneurysm
- balloon-like dilation of the thoracic aorta
- weakness in aortic wall
- tertiary syphilis: endarteritis of the vasa vasorum results in luminal narrowing, decreased flow, and atrophy of the vessel wall
- results in "tree-bark" appearance of the aorta
- dilation of aortic valve root (aortic valve insufficiency)
- compression of mediastinal structures (airway or esophagus) and thrombosis/embolism
35
AAA
- balloon-like dilation of the abd aorta
- below the renal arteries, but above the aortic bifurcation
- primarily due to atherosclerosis, which increases the diffusion barrier to the media, resulting in atrophy and weakness of the vessel wall
- pulsatile abd mass that grows with time
- complications: rupture, especially when >5 cm in diameter
- triad of hypotension, pulsatile abd mass, flank pain
- compression of local structures (e.g. ureter) and thrombosis/embolism
36
Hemangioma
- benign tumor comprised of blood vessels
- commonly present at birth; often regresses during childhood
- most often involves skin and liver
37
Angiosarcoma
- malignant proliferation of endothelial cells
- highly aggressive
- common sites: skin, breast, liver
- liver angiosarcoma is associated with exposure to polyvinyl chloride, arsenic, and Thorostrast
38
Kaposi Sarcoma
- low-grade malignant proliferation of endothelial cells
- associated with HHV-8 (Kaposi's sarcoma-associated herpes virus)
- purple patches, plaques, and nodules of the skin
- may also involve visceral organs
- older Eastern European males (tumor remains localized to skin; txt involves surgical removal)
- AIDS: tumor spreads early; treatment is antiretroviral agents)
- Transplant recipients: tumor spreads early; txt involves decreasing immunosuppression
39
Stable Angina
- CP that arises with exertion or emotional stress
- coronary arteries with >70% stenosis
- CP lasting <20 min
- ST segment depression (subendocardial ischemia)
- relieved by rest and NO
40
Unstable Angina
- rupture of an atherosclerotic plaque with thrombosis and incomplete occusion of a coronary artery
- reversible injury to myocytes (no necrosis)
- ST segment depression (subendocardial ischemia)
- relieved by NO
- high risk of progression to MI
41
Prinzmetal Angina
- coronary artery vasospasm
- reversible injury to myocytes (no necrosis)
- ST segment elevation due to transmural ischemia
- relieved by NO or Ca2+ channel blockers
42
Myocardial Infarction (MI)
- necrosis of cardiac myocytes
- usually due to rupture of an atherosclerotic plaque with thrombosis and complete occlusion of coronary artery
- also by coronary artery vasospasm (Prinzmetal angina or cocaine use), emboli, and vasculitis (e.g. Kawasaki dis.)
- severe, crushing CP (>20 min)
- not relieved by NO
43
Location of MI
- usually LV (right RV and both atria generally spared)
- occlusion of LAD: anterior wall and anterior septum (45% of cases)
- occlusion of RCA: posterior wall, posterior septum, papillary muscles of the LV (2nd most common)
- occlusion of LCX: lateral wall of LV
44
Initial Phase of MI
- subendocardial necrosis involving <50% of the myocardial thickness (subendocardial infarction)
- ST segment depression
45
Continued Phase of MI
- transmural necrosis
| - ST segment elevation
46
Laboratory Tests (Troponin I)
- most sensitive and specific marker
| - levels rise 2-4 hours after infarction, peak at 24 hours, and return to normal by 7-10 days
47
Laboratory Tests (CK-MB)
- useful for detecting reinfarction that occurs days after an initial MI
- levels rise 4-6 hrs after infarction, peak at 24 hrs, and return to normal by 72 hrs
48
MI Treatment
- aspirin and/or heparin (limits thrombosis)
- supplemental O2 (minimized ischemia)
- nitrates (vasodilate veins and coronary arteries)
- beta-blocker (shows heart rate, decreased O2 demand and risk for arrhythmia)
- ACE inhibitor (decreases LV dilation)
- fibrinolysis or angioplasty (opens blocked vessel)
- reperfusion or irreversibly-damaged cells results in calcium influx, leading to hypercontraction of myofibrils (contraction band necrosis)
- return of O2 and inflammatory cells may lead to free radical generation, further damaging myocytes (reperfusion injury)
49
MI (<4 hrs)
- no gross changes
- no microscopic changes
- complications: cardiogenic shock (massive infarction), CHF, and arrhythmia
50
MI (4-24 hrs)
- gross changes (dark discoloration)
- coagulative necrosis
- complications: arrhythmias
51
MI (1-3 days)
- gross changes (yellow pallor)
- neutrophils
- complications: fibrinous pericarditis; presents as CP with friction rub
52
MI (4-7 days)
- gross changes (yellow pallor)
- macrophages
- complications: rupture of ventricular free wall (leads to cardiac tamponade), interventricular septum (leads to shunt), or papillary muscle (leads to mitral insufficiency)
53
MI (1-3 weeks)
- gross changes (white scar)
- fibrosis
- complications: aneurysm, mural thrombus, or Dressler syndrome (immune system response leading to pericarditis)
54
Sudden Cardiac Death
- usually due to fatal ventricular arrhythmia
- without symptoms or <1 hour after sxs arise
- acute ischemia
- less common causes: mitral valve prolapse, cardiomyopathy, cocaine abuse
55
Left-Sided HF
- causes: ischemia, HTN, dilated cardiomyopathy, MI, restrictive cardiomyopathy
- decreased forward perfusion and pulmonary congestion
- pulmonary edema (dyspnea, PND, orthopnea, rales)
* ** intraalveolar hemorrhage marked by hemosiderin-laden macrophages ("heart failure cells") ***
- decreased flow to kidneys leads to fluid retention (exacerbates CHF)
- mainstay of treatment: ACE inhibitor
56
Right-Sided HF
- commonly due to left-sided HF
- also caused by left to right shunt and chronic lung disease (cor pulmonale)
- JVD
- painful hepatosplenomegaly with characteristic "nutmeg" liver; may lead to cardiac cirrhosis
- dependent pitting edema
57
VSD
- defect in septum that divides RV and LV
- most common congenital heart defect
- associated with fetal alcohol syndrome
- left-to-right shunt
- large defect can lead to Eisenmenger syndrome
- treatment: surgical closure; small defects may close spontaneously
58
ASD
- defect in septum between RA and LA
- most common type: ostium secundum
- ostium primum type is associated with Down Syndrome
- left-to-right shunt
- fixed split S2
- paradoxical emboli are an important complication
59
Patent Ductus Arteriosus (PDA)
- failure of ductus arteriosus to close
- associated with congenital rubella
- left-to-right shunt between the aorta and the pulmonary artery
- asymptomatic at birth with "machine-like" murmur
- may lead to Eisenmenger syndrome, with lower extremity cyanosis
- treatment: indomethacin, which decreases PGE, resulting in PDA closure (PGE maintains patency)
60
Tetralogy of Fallot
- stenosis of the right ventricular outflow tract
- right ventricular hypertrophy
- VSD
- aorta that overrides the VSD
- right-to-left shunt leads to early cyanosis
- pts learn to squat in response to cyanotic spell (increased arterial resistance decreases shunting and allows more blood to reach the lungs)
- "boot-shaped" heart on x-ray
61
Transposition of the Great Arteries
- pulmonary artery arising from the LV and aorta arising from the RV
- associated with maternal diabetes
- early cyanosis; pulmonary systemic circuits don't mix
- creation of shunt (allowing blood to mix) after birth is required for survival
- PGE can be administered to maintain PDA until surgery
- results in hypertrophy of RV and atrophy of LV
62
Truncus Arteriosus
- characterized by a single large vessel arising from both ventricles (truncus fails to divide)
- early cyanosis
- deoxygenated blood from the RV mixed with oxygenated blood from the LV before pulmonary and aortic circulations separate
63
Tricuspid Atresia
- tricuspid valve orifice fails to develop; RV ventricle is hypoplastic
- often associated with ASD, resulting in right-to-left shunt
- presents with early cyanosis
64
Coarctation of the Aorta (infantile form)
- narrowing of the aorta
- associated with PDA; coarctation lies after (distal to) the aortic arch, but before the PDA
- presents as lower extremity cyanosis in infants, often at birth
- associated with Turner syndrome
65
Coarctation of the Aorta (adult form)
- not associated with PDA
- coarctation lies after (distal to) the aortic arch
- presents as hypertension in the UEs and hypotension with weak pulses in the LEs
- classically discovered in adulthood
- collateral circulation develops across the intercostal arteries; leading to "notching" of ribs on CXR
- associated with bicuspid aortic valve
66
Acute Rheumatic Fever
- systemic complication of pharyngitis due to group A beta-hemolytic steptococci
- affects children 2-3 wks after episode of strep throat
- molecular mimicry; bacteral M protein resembles proteins in human tissue
- diagnosis based on JONES criteria
- acute attack usually resolves, but may progress to chronic rheumatic heart disease; repeat exposure to group A strep results in relapse of the acute phase and increases risk for chronic disease
67
Jones Criteria For Acute Rheumatic Fever
- J: joints; migratory polyarthritis (large joints)
- O: obvious (cardiac): endocarditis (mitral valve more common than aortic); myocarditis with Aschoff bodies (foci of chronic inflammation), reactive histiocytes with slender, wavy nuclei (Anitschkow cells), giant cells, and fibrinoid material
- N: Nodules: subcutaneous nodules
- E: Erythema marginatum: annular, nonpruritic rash with erythematous borders, commonly involving trunk and limbs
- S: Sydenham chorea: rapid, involuntary muscle mov'ts)
68
Chronic Rheumatic Fever
- valve scarring
- stenosis with "fish-mouth" appearance (mitral stenosis)
- almost always involves MV; leads to thickening of chordae tendineae and cusps
- occasionally involves the aortic valve; leads to fusion of the commissures
- infectious endocarditis
69
Aortic Stenosis
- narrowing of the aortic valve orifice
- fibrosis and calcification from "wear and tear"
- late adulthood (>60 years)
- increased risk with bicuspid valve (increased "wear and tear" with only two valves)
- possible consequence of chronic rheumatic fever
- coexisting mitral stenosis and fusion of the aortic valve commissures distinguish rheumatic disease from "wear and tear"
- cardiac compensation: prolonged asymptomatic stage with systolic ejection click followed by crescendo-decrescendo murmur is heard
- complications: concentric LVH; angina; microangiopathic hemolytic anemia (RBCs are damaged (producing schistocytes) while crossing the calcified valve
- treatment: valve replacement
70
Aortic Regurgitation
- backflow of blood from aorta into LV during diastole
- aortic root dilation (e.g. syphilitic aneurysm and aortic dissection) or valve damage (e.g. infectious endocarditis)
- early, blowing diastolic mrumur
- hyperdynamic circulation: increased pulse pressure (diastolic pressure decreases due to regurg, while systolic pressure increases due to increased SV)
- bounding pulse (water-hammer pulse), pulsating nail bed (Quincke pulse), and head bobbing
- LV dilation and eccentric hypertrophy (volume overload)
- treatment: valve replacement once LV dysfunction develops
71
Mitral Valve Prolapse
- ballooning of mitral valve into LA during systole
- due to myxoid degeneration (accumulation of ground substance) of the valve, making it floppy
- may be seen in Marfan or Ehlers-Danlos syndrome
- incidental mid-systolic clikc followed by regurg murmur
- usually asymptomatic
- click and murmur become softer with squatting (increased systemic resistance decreases left ventricular emptying)
- complications (rare): infectious endocarditis, arrhythmia, severe mitral regurgitation
- treatment: valve replacement
72
Mitral Regurgitation
- reflux of blood from LV to LA during systole
- arises as a complication of MV prolapse
- also caused by LV dilatation (e.g. left-sided HF), infective endocarditis, acute rheumatic heart disease, and papillary muscle rupture after a MI
- holosystolic "blowing" murmur; louder with squatting (increased systemic resistance decreases LV emptying) and expiration (increased return to left atrium)
- results in volume overload and left-sided HF
73
Mitral Stenosis
- narrowing of the mitral valve orifice
- usually due to chronic rheumatic valve disease
- opening snap followed by diastolic rumble
- volume overload leads to dilatation of the LA
- pulmonary congestion
- pulmonary HTN and eventual right-sided HF
- atrial fibrillation with associated risk for mural thrombi
74
Endocarditis
- inflamm. of endocardium that lines the surface of cardiac valves (usually bacterial infection)
- strep viridans most common overall (low virulence that infects previously damaged valves); results in small vegetations that do not destroy the valve (subacute endocarditis)
- damanged endocardial surface develops thrombotic vegetations
- transient bacteremia leads to trapping of bacteria in the vegetations
- Staph aureus most common in IVDA (high virulence; most commonly tricuspid); large vegetations that destroy valve
- Staph epidermidis: prosthetic valves
- Strep bovis: endocarditis with underlying colorectal carcinoma
- HACEK (haemophilus, actinobacillus, cardiobacterium, eikenella, kingella)
- fever (due to bacteremia)
- murmur
- Janeway lesions (erythematous non-tender lesions on palms and soles)
- Osler nodes (tender lesions on fingers or toes)
- splinter hemorrhages
- Roth spots
- anemia of chronic disease (due to chronic inflamm.)
75
Nonbacterial Thrombotic Endocarditis
- due to sterile vegetations that arise in association with hypercoagulable state or underlying adenocarcinoma
- vegetations arise on the mitral valve along lines of closure and result in mitral regurgitation
76
Libman-Sacks Endocarditis
- due to sterile vegetations that arise in association with SLE
- vegetations are present on the surface and under-surface of the mitral valve and result in mitral regurgitation
77
Dilated Cardiomyopathy
- dilation of all 4 chambers of the heart
- most common form of cardiomyopathy
- systolic dysfunction, leading to biventricular CHF
- complications: mitral and tricuspid valve regurgitation and arrhythmia
- most commonly idiopathic
- other causes: genetic mutation, myocarditis (usually coxsackie A or B) lymphocytic infiltrate the myocardium, alcohol abuse, drugs (doxorubicin), pregnancy (late pregnancy or soon (weeks to months) after childbirth), hemochromatosis
- treatment: heart transplant
78
Hypertrophic Cardiomyopathy
- massive hypertrophy of the LV
- usually due to genetic mutations (AD) in sacromere proteins
- decreased cardiac output (LV dysfunction leads to diastolic dysfunction (ventricle cannot fill))
- sudden death due to ventricular arrhythmias (common cause of sudden death in young athletes)
- syncope with exercise (subaortic hypertrophy of the ventricular septum results in functional aortic stenosis)
- biopsy shows myofiber hypertrophy with disarray
79
Restrictive Cardiomyopathy
- decreased compliance of ventricular endocardium
- restriction of filling during diastole
- amyloidosis, sarcoidosis, endocardial fibroelastosis (children), and Loeffler syndrome (endomyocardial fibrosis with an eosinophilic infiltrate and eosinophilia)
- presents with CHF
- low voltage ECG with diminished QRS amplitude
80
Myxoma
- benign mesenchymal tumor with a gelatinous appearance and abudant ground substance on histology
- most common primary cardiac tumor in adults
- usually forms a pedunculated mass in the LA that causes syncope due to obstruction of the mitral valve
81
Rhabdomyoma
- benign hamartoma of cardiac muscle
- most common primary cardiac tumor in children; associated with tuberous sclerosis
- usually arises in the ventricle
82
Metastasis (cardiac tumor)
- metastatic tumors are more common in the heart than primary tumors
- common metastases to the heart include breast and lung carcinoma, melanoma, and lymphoma
- most commonly involve the pericardium, resulting in a pericardial effusion
