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Path IM CHD Valve Disease Heart Tumor
KYCOM Block 13
146
Medical
Graduate
01/26/2014

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Term
what is the most comon birth defect in cards
Definition
congenital heart disease
Term
what is the most common heart disease
Definition
congenital
Term
what is the cause of congenital heart disease
Definition
enivornment and genetics
90% unknown
Term
with what types of heart problems are mothers advised not to reproduce 4
Definition
aortic diseases: marfans, coarctation
cyanotic heart disease
pulmonary HTN
Term
how is the severity of a left to right shunt determine
Definition
the bigger the shunt the more blood moves through into the pulmonary circut
Term
what are the 3 left to right shunts
Definition
ASD
VSD
PDA
Term
explain what eisnmenger's syndrome is and the complications of it
Definition
In left to right shunt blood from the systemic circuit is forced into the lower pressure pulmonary circuit. Evuntally the pulmonary circuit will form HTN and it will be lower pressure for it to shunt back into the systemic circuit
This will cause deoxygenated blood to enter the systemic circuit prematurely and this late cyanosis
Term
what are 7 signs of esienmenger's syndrme
Definition
pulmonary vascular disease
pulmonary HTN
exercise syncope
arrhythmia
hemoptysis
stroke
secondary polycythemia
Term
what is polycythemia
Definition
cyanosis activates erythropoetin which increases RBC and blood viscosity
Term
what is the tx for eisnmenger's syndrome
Definition
avoid more viscosity: no oral contraceptives
theraputic phlebotomy
Term
whan is theraputic phlebotomy done, what do you do
Definition
Hb>20 or HCT>65 then you can do it
muscle replace Fe and volume to prevent syncope
Term
what is the most common congenital heart defect in adilts
Definition
ASD 10%
Term
ASD: cause
Definition
open septum between atria allows high pressure blood from the LA into the RA which increases delivery of blood to the lungs
Term
ASD: locations, their prevelance
Definition
sinus venosus: near the SVC
ostium secondum: midlline, fossa ovalia, most common
ostium primum: near AV valves
Term
what location of ASD is associated with another birth defect disorder, what disorder is it
Definition
ASD ostium primum
down syndrome
Term
what are the two complications of ASD, explain why each happen
Definition
paradoxical emboli: DVT normally lodges in lungs, but because there is an opening between the atria it could go RA to LA then to systemic circulation and lodge in brain

eismenger's syndrome: the shunt is delivering lots of blood to the lungs, after a while the lungs will develop HTN and become higher pressure than the systemic circut causing reversal of the shunt and deoxygenated RA blood to be allowed into the circulation causing LATE CYANOSIS
Term
what are the heart sounds associated with a , explain why you heart them 6
Definition
wide fixed S2 split: there is more blood in the RA making it hard for the pulmonary valve to close on time

systolic ejection murmur: the increase RV blood is forced through the pulmonary valve

diastolic rumble: increased blood volume moving across the trucuspid in filling

RV heave: the RV has more blood to push out

ostium primum: mitral regurgitation

ostium secondum: Afib in 50s
Term
other than the heart sounds, what exam finding would there be, why
Definition
prominent jugular venous pressure: traffic jam, extra blood in RA
Term
EXG findings for ASD 2
Definition
RBBB
rSr'
Term
CXR findings for ASD 3
Definition
pulmonary HTN
RV hypertrophy
LV hypertrpohy in primum ASD
Term
ECHO findings for ASD 3
Definition
RA and RV dilation
paradoxical septal movement
Term
what is the most frequent congenital heart disease
Definition
VSD
Term
what congenital heart diseas is associated with fetal alcohol syndrom
Definition
VSD
Term
what is the cause of VSD
Definition
opening in muscular septum allows blood from high pressure LV into lower pressure RV which shunts more blood into the pulmonary circut
Term
2 complications of VSD
Definition
pressure and volume hypertrophy

eismenger's syndrome: more blood in pulmonary circut forms HTN and the systemic circut becomes less pressure so shunt reverses and deoxygenated blood from the RV moves to LV causing LATE CYANOSIS, secondary polycythemia, and CLUBBING
Term
how can you tell if a VSD is small or big
Definition
small: LOUD systolic murmur, thrill, palpable, found early

big: soft diastolic mitral outflow rumble
Term
tx VSD
Definition
surgical closure of shunt (small may close on its own)
Term
who gets PDA 2
Definition
girls > boys
associated with MATERANL RUBELLA
Term
cuase of PDA
Definition
normal ductus arteriosis closes by 3mo due to oxygen induced release of PGE

blood shunts from high pressure aorta to low pressure pulmonary artery which delivers more blood to the lungs and less to the systemic circut
Term
what is a complication of PDA
Definition
eismenger's syndrome: more blood is being delivered to the pulmonary circut so it develops HTN and the aorta becomes less pressure so the shunt reverses and deoxygenated blood moves from pulmonary artery to aorta AFTER THE BRANCHES WHICH CAUSES LE CYANOSIS
Term
exam finding in PDA
Definition
machinery murmur
cyanosis of the LE after reversal
Term
treatment of PDA
Definition
small defect could calcify and close as adult
surgery is curative
antibotic prophylaxis, NSAIDS prior to shunt development, removal of PGE
Term
what are the 4 right to left shunts
Definition
hypoplastic left heart syndrome
tricuspid atresia
tertaolgy of fallot
truncus arteriosis
transposition of the great vessels
Term
what are two conditions we are worried about in all conditions with a right to left shunt
Definition
cyanotic congenital heart disease: right to left shunts present in infancy (blue baby) as clubbing, cyanosis, dyspnea

emboli from peropherial veins can enter the circulation by bypassing the lungs
Term
what is the most common right to left shunt
Definition
tertaolgy of fallot
Term
what is the cause of tertaology of fallot, how is the severity determined
Definition
VSD
displacement of aorta over VSD
subpulmonic stenosis: smaller opening more shunting and more cyanosis
thickening of ventricle wall
Term
5 exam findings in tertaolgy of fallot
Definition
bluish skin
squatting: increases TPR and pressure on right side forcing blood through the stenosis and to the lungs
crying
poor feeding
CXR: boot shaped heart
Term
explain the flow of blood in a tertolgy of fallot
Definition
blood enters RV and the pulmonary valve is hard to get through due to stenosis so instead it goes through VSD and into the aorta that is right next to it and out to the peripherial circulation before it is oxygenated causing EARLY CYANOSIS
Term
what is the cause of truncus artriosis
Definition
maternal diabetes
developmental failure in seperation of truncus into aorta and pulmonary artery
Term
explain the flow of blood in truncus arteriosis
Definition
common aorta/pulmonary artery allows mixing of oxygenated and deoxygenated blood which causes early cyanosis
Term
what conditions is truncus arteriosis associated with 2
Definition
maternal diabetes
VSD
Term
transposition of the great vessels: flow of blood, complications
Definition
aorta comes from RV and pulmonary artery from LV

this creates two seperate pulmonary and systemic circuts and oxygenated blood cannot reach the systemic circut causing EARLY CYANOSIS

the baby is alive because some oxygenated blood goes from the pulmonary artery to the aorta through the PDA
Term
tx transposition of the great vessels
Definition
PDA MUST remain open for the baby to stay alive
surgical corrections
Term
6 obsturction of outflow conditions
Definition
spuravalvular aortic stenosis
coarctation of the aorta
valvular aortic stenosis
aortic regurgitation
mitral stenosis
mitral regurgitation
Term
what condition is supravalvular stenosis associated with, what are other signs 3
Definition
WILLIAMS SYNDROME (elvin facies, MR, hypercalcemia)
Term
cause of supravalvular stenosis
Definition
diaphragmatic (hour glass) stenosis ABOVE the aortic valve PROXIMAL to the coronary artery lesses blood delivery to the ENTIRE systemic circulation
Term
exam findings in supravalvular stenosis
Definition
systolic murmur in suprasternal notch
Term
what 2 other conditions is coarctation of the aorta associated with
Definition
TURNER SYNDROME
bicuspid aorta
Term
who gets coardctaiton of the aorta
Definition
males > females
7% of cardiac birth defects
Term
what is wrong in coarctation of aorta
Definition
narrowing of aortic arch distal to left subclavian but proximal to PDA
Term
explain the flow of blood in infantile coarctation of the aorta
Definition
blood from the RV enters the PDA to the aorta because it is lower pressure after the stenosis causing CYANOSIS TO THE LE
Term
explain the flow of blood in adult coarctation of the aorta
Definition
blood from the LV has a difficult time passing through the stenosis so more goes out the aortic branches causing UE HTN AND LE HYPOTENSION
Term
3 complications of coarctation of aorta and why
Definition
heart failure: excess work of LV pushin against stenosis
anrueysm in circle of willis: due to UE HTN
aortic dissection/rupture: due to pressure on stenotic area
Term
exam findings in coarctation of aorta 6
Definition
HTN in UE
hypotension in LE
reduced pulses in LE
radiofemoral delay
3 sign
rib notching due to formation of collateral circulation
Term
treatment and prognosis of coarctation of aorta 3
Definition
death in middle age if not corrected with surgery of percutaneous balloon dilation
contaol HTH (may rise after surgery)
often die of CAD, stroke, rupture, CHF
Term
what is the most common obstructive congenital heart disease
Definition
valvular aortic stenosis
Term
what are the 3 causes of valvular aortic stenosis, explain each, and what age they come about
Definition
congenital bicuspid: <55yo, increased risk of Ca build up due to wear/tear

degenerative aortic stenosis: >55yo, dystrophic Ca build up due to wear/tear makes it so cusps dont line up

CHRONIC rheumatic fever (40-50yo, 15y after infection): always occurs with mitral stenosis, aortic effect uncommon. due to chronic rheumatic fever causing scaring and FUSING of the valve (fish mouth)
Term
aortic valve stenosis effects on the heart
Definition
obstruction of LV out flow causes hypoplastic left heart and LV hypertrophy
Term
5 exam findings in aortic stenosis and why
Definition
often asympatomatic until adult due to compensation mechanicms

ejection click and diastolic murmur: pressure behidn stenosis blows open valve (click) and high pressure blood rushes through (crescendo) and then slows (decrescendo)

syncope and angina with exercise: limited blood to brain and heart

pulse parvus (volume): radial pulse weak

pulse tarvus (timine): radial pulse not felt in time with heart
Term
how can the severity of an aortic stenosis be determined
Definition
SOUND OF MURMUR INCREASES WITH SEVERITY
Term
2 EKG signs of aortia stenosis
Definition
LVH
LBBB
Term
tx of aortic stenosis
Definition
if severe (<0.75cm2 or >50mmHG) needs surgery
Term
causes of aortic regurgitation 8
Definition
congenital bicuspid degeneration: <55yo
degeneration: >55yo
rheumatic fever: uncommon
endocarditis: cauces valve damage and incomplete closure
seronegative arthritis
ankylosing spondylitis
RA
margans: aortic root dilation pulls edges of valve away from eachother
Term
when should a marfans patient get surgery for the aortic regurgitation
Definition
>5.5cm, dyspnea, fatigue
Term
2 exam findings in aortic regurgitation
Definition
short duration murmur: blood quickly goes back through the valve in diastole to equalize pressures (worse severity) making decrescendo murmur

hyperdyanmic circulation signs
Term
explain hyperdynamic circulation and the outcomes
Definition
when blood regurgitates into the LV next cycle will push more blood out (inc SV and systolic pressure) but some will come back in (dec diastolic pressure) widening pulse pressure

bounding pulses, pulsating nail beds, nead nodding
Term
ECHO/EEG signs in aortic regurgitation
Definition
LB hypertrophy
Term
how is severity of aortic regurgitation determined
Definition
ECHO/EKG!
NOT loudness
Term
tx aortic regurgitation
Definition
valve replacement
Term
cause of mitral stenosis
Definition
chronic rheumatic fever causing scaring the FUSION of the cusps
Term
heart sounds in mitral stenosis and why
Definition
1st is loud: when LA pushes open stenotic valve in diastole it causes snap followed by murmur when blood is pushed through
Term
how is severity of mitral stenosis detchermined
Definition
longer rumble after 1st heart sound, more severe
Term
what is a complication of mitral stenosis, why
Definition
AFib: dilation in LA causes changes in conduction leading to stasis and mural thrombi
Term
CXR, EKG, ECHO signs of mitral stenosis
Definition
CXR: straightening of right heart border, kearly B lines (straight line son CXR in severe stenosis)

EKG: RV hypertrophy

ECHO: shows severity, diagnosis, gradient
Term
when is a mitral stenosis considered severe
Definition
symptomatic, >12mmHg
Term
tx mitral stenosis 3
Definition
surgery if severe
AFib: pacemaker or meds
balloon valvuloplasty if valve is pliable and not too crusty and stenosed
Term
cause of mitral regurgitation 4
Definition
acute rheumatic fever due to endocarditis vegetations
mitral valve prolapse
endocarditis
papillary muscle ischemia: Post MI papillary mucle dies and it cant keep valve closed
Term
where is the most common location of a papillary muscle ischemia and why
Definition
posteriomedial papillary muscle due to flow of blood
Term
5 exam findings in mitral regurgitation and why
Definition
holocystolic murmur: LV contracts and some goes back in
oblideration of 1st heart sound
wide split 2nd heart sound
low pitch early diastolic rumble
dyspnea due to LV pressure increase
Term
how can you make a mitral regurgitation louder 3, why
Definition
squat: increased resistance on atria forces blood into LA

expiration: increased blood in LA increases blood in LV causing more regurgitation
Term
what kind of cancer is most likley in the heart
Definition
secondary tumors, primary are very rare
Term
how often in metastis to the heart
Definition
occurs in 5% of dying cancer patients
Term
what percent of primary cardiac tumors are malignant
Definition
25%
Term
what are the 6 primary tumors of the heart
Definition
myoxmas
lipomas
rhabdomyomas
angiosarcomas
papillary fibroblastoma
fibroma
Term
myoxma: gender, age, metastic?, location, prevelance
Definition
female
50s
MOST COMMON PRIMARY CARDIAC TUMOR
40% benign
90% aorta (usually by fossa ovalis)
75% in LA
Term
cause of myoxmas
Definition
mesenchyme proliferation causes gelatenous ground substance
Term
signs of myexoma
Definition
mitral heart disease (ball value effect)
murmurs in heart
systemic emboli
large peduncukared
Term
what is the effect of myexoma on the mitral valve
Definition
tumor is on wall of LA causing blockage to the mirtal valve and decreased systemic blood and syncope when less reaches the brain
Term
hwart lipoma locations 3
Definition
subendocardium
subepicardium
myocardium (poorly localized in encapsulated masses)
Term
rhabdomyoma: associated disease, prevelance
Definition
most common primary cardiac tumor in infants and kids (40% of tumors in that age group)

associated with tuberous sclerosis
Term
cause of rhabdomyomas, location, size
Definition
fetal harmatomas
large
90% ventricular myocardium
Term
what is the most common primary malignant tumor of the heart
Definition
angiosarcoma
Term
what is the most frequent cardiac tumor, what is the most frequent complication
Definition
metastasis from another cancer to the parenchyma causes effusion
Term
what are 4 common malignancies that metastasize to the heart
Definition
lung, breast, melanoma, lymphoma
Term
what are 5 manuvers that can help with diagnosis of valve disease
Definition
inspiration
valsalva
hangrip
squatting
change from supine to upright
Term
what does inspiration do the the CV
Definition
increased venous return
increased right sided murmur
Term
what does valsalve to to the CV
Definition
increases intrathoracic pressure
decreases venous return to heart
most sound decreased due to decreased LV filling and decreased CO (EXCEPT HYPERTROPHIC OBSTRUCTIVE CARDIOMYOPATHY (SUBVALVULAR AORTIC STENOSIS) AND MITRAL STENOSIS)
Term
what does handgrip do to the CV
Definition
increases CO and systemic vascular resistance
Term
what does squatting do the to CV
Definition
increases peripherial vascular resistance
increases venous return
Term
what does changing from supine to upright do to the CV
Definition
decreases venous return and SV
increases HR and systemic vascular resistance
Term
mitral valve prolapse: prevelance, ages, gender
Definition
3-5% of the population
20-30yo
women > men
Term
causes of mitral valve prolapse 7
Definition
degeneration of mitral valve due to
marfans
elhers danlos
ruptured papillary m or chordae tendinae
endocarditis
arrhythmia
thombi
Term
what condition is mitral valve prolapse often combined with
Definition
mitral valve regurgitation
Term
explain what happens to the valve in mitral valve prolapse
Definition
balooning of the valve into LA in systole due to degenerating loppy valve
Term
symptoms of mitral valve prolapse
Definition
95% asymptomatic
Term
complications of mitral valve prolapse 5
Definition
severe mitral regurgitation
infective endocarditis
arrhythmia
thrombi
Term
what are te six infections of the heart
Definition
rheumatic heart disease
non-bacterial thrombotic endocarditis
libman sacs endocarditis
infective endocarditis
myocarditis
pericarditis
Term
rheumatic heart disease: locations, prevelance, most common dysfunction
Definition
3% of GAS will cause rheumatic heart failure
mitral valve > women
aortic valve > men (overall more rare)
stenosis > regurgitation
Term
cause of rheumatic heart disease
Definition
slight familial predisposition

pt gets pharyngitis via GAS pyogenes which has M proteins that resemble human tissue
the body makes antigens to the M proteins and they attack the GAS and the body (molecular mimicry)
Term
what are the 3 changes in the heart tissue with acute rheumatic heart disease
Definition
pancarditis: inflammatory infiltrate of all three layers

endocarditis: vegitations on mv causes regurgitation

myocarditis: most common cause of death in acute phase

pericarditis: causes friction rub
Term
what are two signs of rheumatic myocarditis
Definition
aschoff bodies: chronic inflammation, giant cells, fibrnoid

anichkow cells: histocytes with catipillar nuclei in bodies
Term
what changes in the heart with chronic rheumatic heart disease
Definition
scarring and fibrnoid necrosis of the valves causes fusion and thickening leaving valve stenosed with "fish mouth" appearance
Term
explain how rheumatic fever is diagnosed
Definition
1. evidence of strep infection (ASO, anti-DNAase)
2. 2 major or 1 major + 2 minor
Term
what are the 5 major criteria for rheumatic fever
Definition
Joint- migratory polyarthritis
O - pancarditis
Nodules in SC
Erythema migrinatum: rash on trunk with red borders
Syndehams chorea: rapid involuntary movement
Term
non-thrombotic bacterial endocarditis, define, aka, location
Definition
marantic endocarditis
50% mitral
sterile deposition of fibrin, platelets, blood components on line of closure vegetations
Term
non-thrombotic bacterial endocarditis causes 2
Definition
hyperviscosity: elevated platelets, adenocarcinomas
subtle endothelial abnormalities
Term
non-thrombotic bacterial endocarditis complication
Definition
embolic events common
Term
libman-sacs endocarditis: cause, common presentations
Definition
SLE attacks tricuspid, mitral, cords, endocardium causing fibrnoid necrosis on BOTH sides of valves
Term
libman-sacs endocarditis tx and prognosis
Definition
decrease incidence with steroid therapy
embolic event sin valve fibrosis common
Term
4 microbes causing infective endocarditis, their virulence, most common pt, type of endocarditis
Definition
S. viridans: low virulence, small vegetations, don't destory valve (subacute)

S. aureus: high virulence common in iv drug user, large vegetations destory valve (acute)

S. epidermiditis: common on presthetic valves

S. bovis: common with colorectal cancer
Term
what predisposes someone for endocarditis
Definition
heart lesion, IV drug use, any exposed collagen on valve allows for thrombotic vegitations on heart valve that can trap bacteria
Term
what valves are most often infected in infective endocarditis
Definition
mostly aortic and mitral
tricuspid in S aureus IV drug user because it is very virulent and tricuspid is the first valve it hits
Term
signs of acute endocarditis
Definition
rapid onset, fever, chills, weakness
Term
signs of subacute endocarditis
Definition
fever, fatigue, flu like
Term
signs of a septic emboli from endocarditis 3
Definition
janeway lesions: red lesions on palms and soles
osler lesions: painful lesions on finger
splinter hemorrhages: on nail beds
Term
treatment of infectious endocarditis
Definition
get positive blood cultyre (major criteria)
6 mo antibiotics
Term
viral causes of myocarditis 5
Definition
most common
cosackie, echo, influenza, HIV, CMV
Term
bacterial causes of myocarditis 5
Definition
chalmydia (C. psittaci)
rickettsia (R. typhi)
corynebacterium dyptheriae
N. meningitidis
B. burdgferi
Term
fungal, protzoa, worm causes of myocarditis 4
Definition
canditia
T. cruzi, toxoplasmosis
helminths
Term
autoimmune causes of myocarditis 5
Definition
post viral (post-strep)
SLE
drug hypersensitivity: methylopa, sulfa
transplant rejection
sarcoidosis
giant cell arteritis
Term
signs of myocarditis non-acute 5
Definition
asymptomatic
fever
fatigue
CP
palpitations
Term
signs of myocarditis acutte 3
Definition
arrythmia
dilated cardiomyopathy
mitral regurgitation
Term
what is the normal cause of pericarditis
Definition
virus
Term
signs of acute pericarditis
Definition
fibrnous exudate
resolves or professes to chronic
Term
signs of chronic pericarditis
Definition
delicate fibrous adhesions or fibrous scaring (causes constrictive pericarditis)
Term
what are the 3 types of pericardial effusions and their cause
Definition
serous: decreased protein (cirrhosis) or increased pressure

serosangiunous: decreased protein with blood (trauma, cancer)

chyloys: lymph obstruction (cancer)

sanguinous: trauma or acute events (dissection/rupture)
Term
what is a cardiomyopathy
Definition
primary mycardial abnormalities (intrinsic disease)
myocarditis that causes cardiac dysfunction
Term
what are the 3 types of cardiomyopathy, which is most common
Definition
dilated 90% flabby
hypertrophic
restrictive
Term
dilated cardiomyopathy: changes in heart and effects
Definition
atria and ventricles dilated decreasing contraction and thus emptying which causes biventricular eccentric hypertrophy and heart failure

dilation stretches the tissue changing conduction causing AFib

dilation stretches the mitral and tricuspid valve causing regurgitation
Term
5 causes of dilated cardiomyopathy
Definition
myocarditis: cosackie late complication
pregnancy: 3rd trimester, after birth
alcohol
drugs (cocaine)
inherited muscular dystrophy
Term
cause of hypertrophic cardiomyopathy
Definition
genetic mutation in sarcomere proteins causes wall to have much more muscle so it cant contract decreasing CO
Term
complications of hypertrophic cardiomyopathy
Definition
changes in contraction causes VFib and sudden death

syncope with exercise: increased muscle causes stenosis within the LV decreasing blood to the brain and heart

septal hypertrophy

intermittent ourflow obstruction
Term
how is hypertrophyc cardiomyopathy diagnosed
Definition
biopsy: myocytes disorganized (dissarray)
Term
define restrictive cardiomyopathy
Definition
idiopathic fibrosis thickens ventricles and decreases their heart wall causing fibrosis
Term
causes of restrictive cardiomyopathy
Definition
loffler's syndrome: eosinophils and inflammation in heart wall cause fibrosis

endocardial fibroelastosis: fibrin and elastin replace epicardium decreasing stretch
Term
complications of restrictive cardiomyopathy
Definition
CHF: blood cant leave the heart causing failure
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