Cardiac Path II Flashcards
What is the most common valve abnormality ?
calcific aortic stenosis
2% of population
What is calcific aortic stenosis ?
affected valves contain osteoblast-like cells, deposit osteoid-like substance ->ossifies
Prevent complete opening of valve
cusp free edges spared
What causes the wear and tear of calcific aortic stenosis and what can show an accelerated course?
Wear and tear - hyperlipidemia, chronic HTN, inflammation
Bicuspid valve accelerates; responsible for half of stenosis
usually manifest in 60-80 but with bicuspid, 50-70s
What causes mitral annular calcification?
Degenerative, noninflammatory calcific deposits
Describe mitral annular calcificaton
Deposits in fibrous annulus
Does not affect valve function
Nodules may become sites fro thrombus formation or IE
Female, older, and with MVP
What is MVP?
myxomatous degeneration (PG deposit)
valve leaflets prolapse back into LA during systole
leaflets thick, rubbery due to proteoglycan deposits and elastic fiber disruption
Can cause thrombi to form
Who does MVP usually affect?
2-3% adults in US
Female, usually incidental
What are the symptoms and complications of MVP?
usually asymptomatic
Some may have angina-like pain or dyspnea
Complications: rare - IE, mitral insufficieny, Thromboemolism, arrhythmias
What is Rheutamic fever?
multisystem inflammatory disorder follwoing pharyngeal infection with group A streptococcus
Incidence decreased
What is the only cause of acquired mitral valve stenosis?
RHD
What is the pathogenesis of RHD?
immune response to streptococcal M proteins cross reacts with cardiac self antigens
T cells/Abs/and Mo
What is acute RF?
Acute RF occurs 10 days - 6 weeks after grp A strepinfection
Anti-strep O; anti-DNase B
can include pancarditis, migratory polyarthritis, subcutaneous nodules, rash, syndenham chorea
What are cardiac features of Acute RF?
pancarditis, featuring Aschoff bodies (Mo)
Vegetations
Inflammation, fibrinoid necrosis
What are the cardiac features of Chronic RHD?
mitral leaflet thickening, fusion, shortening of commisures, fusion and thickening of tendinous cords,
Mitral stenosis -> LA enlargement-> atrial fib/thrombosis; pulm cong/RHF
Fishmouth
What is infective endocarditis?
An infection of valves and endocardium
Vegetations consisting of microbes and debris, associated with underlying tissue destruction
What is acute infective endocarditis?
rapidly progressing, destructive infection of a previously normal valve
Mainly due to Staph aureus
Requires surgery and Antibiotics
What is subacute IE
Slower-progressing infection of a previously deformed valve
Cured with antibiotics alone
Strep viridans
What are the predisposing conditions of IE?
valvular abnormalities - RHD, prosthetic valves, MV prolapse, calcific stenosis, bicuspid AV
Bacteremia from another site, dental work, needles
What are the classic features of IE?
friable, bulky destructive valvular vegetations
Left-sided valves more common
Septic emboli - can seed at another spot
Invasion of adjacent myocardium or aorta can cause abscesses
What are some classic features of IE in a drug user?
Right sided valves involved
Staph aureus
What are symptoms of IE?
nonspecific - fever, weight loss, fatigue
Murmur with left-sided lesions
What are the organisms involved in IE?
S. viridans - valve abnormal
S. Aureus - normal valves, IV drug, abnormal valves
S. epidermidis - prosthetic valves
HACEK
What is nonbacterial thrombotic endocarditis?
small, sterile thrombi on cardiac valve leaflets, along the line of closure
May be a source of emboli
Associated with malignancies (especially adenocarcinomas), sepsis, or catheter trauma
What is Libman-Sacks disease?
endocarditis of SLE
Small fibrinous sterile vegetations on either side of valve leaflets
what are the complications of prosthetic valves?
IE, occlusion
Mechanical - thromboembolic complications
Bioprosthetic - calcification or tears
What is the most common type of cardiomyopathies?
dilated - 90%
What are some causes of dilated cardiomyopathy?
Familial 30-50%: TTN, AD
Alcohol
Myocarditis
Cardiotoxic drugs: doxorubicin, cobalt, iron overload
What is the morphology of DCM
Dilation of all chambers
Mural thrombi are common - LA and LV
Functional regurgitation of valves
what is the clinical presentation of DCM
age 20-50
progressive CHF->dyspnea, exertional fatique, decrease EF (less than 40%)
Arrhythmias
Embolism
What is takotsubo cardiomyopathy?
broken heart syndrome
Excess catecholamines following extreme emotional or psychological stress
Mostly women 58-75
Symptoms similar to MI
Apical ballooning of LV with abnormal wall motion and contractile dysfunction
what is arrhythmogenic right ventricular cardiomyopathy?
right ventricular failure and arrhytmias
Myocardium of RV wal replaced by adipose and fibrosis
Desmosome gene involved at intercalated disc
Causes ventricular tachycardia and fibrillation, SCD
Familial - AD
What is hypertrophic cardiomyopathy ?
genetic disorder leading to myocardial hypertrophy and diastolic dysfunction, leading to reduced SV and ventricular outflow obstruction
Mutation: b-myosin
collapse of young athlete
What is the morphology of hypertrophic CM?
massive myocardial hypertrophy
Marked septal hypertrophy
Myocyte disarray
banana like ventricle
What are the consequences of extensive hypertrophy with HCM?
foci of myocardial ischemia left atrial dilation and mural thrombus Diminished CO and Increased pulmonary congestion - extertional dyspnea Arrhythmia SCD
What is restrictive cardiomaypathy?
decreased ventricular compliance leading to diastolic dysfunction
may be secondary to deposition of material in wall (amyloid) or increased fibrosis (radiation)
Ventricles normal size but atria can be enlarged
What is amyloid?
extracellular deposition of proteins which form an insoluble beta-pleated sheet
May be systemic (myeloma) or restricted to heart (transthyretin)
When in interstitium of myocardium->RCM
green in plain polarized light (congo red)
What is myocarditis? what is it most commonly due to?
Inflammation of myocardium
Due to virus - Cox A and B
Lymphocyte infiltration
Other infections: trypanosomes (chagas), bacteria or fungi
What is the single most common genetic cause of congenital HD. what are they at increased risk for?
Trisomy 21
40% of pts with own syndrome have at least 1 heart defect
Chronic illness and IE
Describe atrial septal defect
L->
usually asymptomatic until adulthood
Most common congenital cardiac anomalies seen in adults
Fossa ovalis most common
What can left to right shunting cause?
volume overload on right side -> pulmonary HTN, RHF, paradoxial embolization
Describe ventricular septal defect/
L->R
most common form of congenital heart disease
Many small VSDs close spontaneously
Large VSDs may cause shunting -> RV hypertrophy, Pulm HTN which may reverse flow through shunt-> cyanosis
Describe patent ductus arteriosis
L->R
may fail to close when infants are hypoxic, and/or have defects associated with increased pulmonary vascular pressure (VSD)
Harsh, machinery-like murmur
Large shunts increase pulmonary pressure and eventually shunt reversal and cyanosis
What happens with persistent Left to right shunting?
pulmonary HTN forms
Causes Right to left shunting then cyanosis
= eisenmenger syndrome
What causes cyanosis to be seen soon postnatally?
Right to left shunt
Tetrology of Fallot?
VSD
Obstruction of RV outflow tract
Aorta overrides the VSD
RV hypertrophy
Boot shaped heart
Severity depends on subpulmonary stenosis
Right to left shunt with cyanosis
What is transposition of great arteries?
results in two separate circuits when aorta goes to right atrium and pulmonary artery to left
1/3 have VSD
2/3 have PDA, or patent foramen ovale
right ventricle become hypertrophy
Left ventricle atrophies
Dies w/i few months
What is coarctation of the aorta?
Narrowing of the aorta, generally seen with PDA in infants or w/o PDA in adults
M>F
Common in Turners syndrome
Cyanosis in lower half of body
Coarctation without PDA
usually asymptomatic
HTN in upper extremities, Hypotension in LE
Claudication in cold LE
may see LV hypertrophy
Myxomas
most common primary cardiac tumor in adults
Left atria
benign
mostly single
Lipomas
well-circumscribed benign accumulation of adipose tissue
Left venticle, righ atrium or septum
Papillary fibroelastomas
sea-anemone-like lesions On valves can cause emboli usually incidental Core of myxoid
Rhabdomyomas
most common primary heart tumor in children
Valvular or outflow tract obstruction
Half associated with tuberous sclerosis (TSC1/2)
spider clls
Angiosarcoma
malignant neoplasm
Not distiinctive from counterparts in other locations
