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clinical congenital heart disease
CVM II
46
Medical
Graduate
04/17/2010

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Term
what is the classic way of classifying congenital heart disease?
Definition
non-cyanotic vs cyanotic
Term
what is the main cyanotic congenital heart disease we are going to look at?
Definition
tetralogy of fallot (relatively common)
Term
what are common congenital defects that will make it to adulthood?
Definition
VSD, ASD, bicuspid Ao valve, PDA, MVP, PS, coarctation, congenital AS, ebsteins anomaly, and tetralogy (cyanotic)
Term
what are VSD, ASD, and PDA referred to as?
Definition
L -> R shunts, and are very common
Term
can PDA be diagnosed easily?
Definition
yes, via auscultation
Term
what is bicuspid aortic valve?
Definition
a congenital problem where pts are born with 2 leaflets instead of 3. the pt presents with a diamond shape, *crescendo/decrescendo murmur* - can can have stenosis across the valve - leads to deterioration of the cardiovascular system
Term
what are some of the more common congential heart defects?
Definition
VSD, pulm stenosis, PDA, and atrial septal defect, etc
Term
what is the approach to diagnosing congenital heart disease?
Definition
incidence (make logical conclusions), natural hx (considere age/background), dx (check shunts, valves, gradients in heart, size of chambers, and extent of disease using ECHO, cardiac catheterization), anatomical variation (many defects are associated with 2ndary defects), tx (shunts can can be installed w/o sx)
Term
what are the general complications of congenital heart disease?
Definition
infective endocarditis, brain abscess (occur in underperfused/cyanotic situations - tetralogy of fallot), arrhythmia (sudden death), *eisemengers* (children with a R->L shunt have a pressure reversal, becomes a L->R), and functional limitations
Term
how can eisemengers be used to treat pulmonary HTN?
Definition
in pulmonary hypertension for children: seem to do better if create a right to left shunt, will develop eisemengers and live longer as opposed to if the shunt wasn’t being made
Term
what is the hemodynamic method os classifying congenital heart disease? what isa gradient?
Definition
shunts: L->R, R->L, or balanced. a gradient is a change in pressure through a valve or shunt.
Term
what might cause a R->L shunt?
Definition
a cyanotic, tetralogy of fallot situation, where pulmonary stenosis and a VSD cause blood to flow from R->L
Term
what might cause a L->R shunt?
Definition
ASD/VSD/PDA
Term
what can cause eisemengers (non-interventional type)? what are symptoms?
Definition
if a dr misses something like ASD, which is initially a L->R shunt, it can reverse to a R->L shunt (b/c of progressively increasing pulm pressure). this will usually become symptomatic in the pts 30s, present with clubbing/cyanosis, and can die relatively young. there is increased incidence of brain abscess (due to cyanosis) and sudden death rate is 14%
Term
what is a good way to position pts for CXR, when trying to look at the heart?
Definition
turning the pt a little to the right from the standard PA view, allows both ventricles to be more visible
Term
what ventricle is seen predominately in the standard PA CXR?
Definition
the L ventricle, which makes up the L border. the R ventricle is coming right out at you, so it is not visible
Term
holosystolic murmurs are always ________? what defects are related?
Definition
holosystolic murmurs are always abnormal. VSD, mitral regurgitation, and tricuspid regurgitation all will cause a holosystolic murmur
Term
what is a mid systolic murmur usually indicative of?
Definition
pulmonary artery or aortic stenosis (from the base of the heart)
Term
what is an early systolic murmur indicative of?
Definition
severe mitral regurgitation (ruptured at this point - nothing left)
Term
what is an late systolic murmur indicative of?
Definition
unusual mitral regurgitation
Term
what is the "tetralogy" of fallot?
Definition
VSD, PS, ->results in-> RVH, and over-riding aorta (aorta doesn’t seem to attach at base of septum)
Term
how do pressures in the R ventricle compare in terms of VSD vs tetralogy of fallot?
Definition
pressure in the R ventricle in tetralogy of fallot is high, while pressure in the R ventricle in VSD is low
Term
what are the different types of VSD?
Definition
membranous, which usually creates a large connection. muscular, which usually creates a high pitched murmur - also *can close spontaneously
Term
where is VSD best auscultated?
Definition
the base of the heart, pulmonary area, and the L sternal border (holosystolic)
Term
what are some kinds of ASD? what is a common risk with this condition?
Definition
patent foramen ovale, sinus venosus. pts are at risk for migranes, emoboli that can cross from the R atrium -> L ventricle and yield a peripheral embolus on the L side of the system
Term
how does ASD present?
Definition
not blatantly apparent, very subtle/standard murmur due to increased flow across the pulmonary valve. pts may be short of breath, with an enlarged heart. upon inspiration, the aortic and pulmonic valve sounds (s2) will separate ("splitting"), but will come back together upon expiration. there are also 2 types: secundum (most common, usually patent foramen ovale) and primum (less common, can involve mitral valve)
Term
what is a patent ductus arteriosus?
Definition
connection between the L pulm artery and aorta
Term
what is significant about auscultating a PDA murmur?
Definition
continuous machine gun murmur - due to flow of high aortic pressure to the pulmonary artery
Term
what is the prognosis for PDA?
Definition
good after usually easy sx
Term
what is the 1/3 rule with bicuspid aortic valve?
Definition
1/3 degenerate to aortic insufficiency, 1/3 degenerate to aortic stenosis, and 1/3 will degenerate to both
Term
what commonly happens to bicuspid aortic valves? what is a fish mouth? might the L ventricle be affected?
Definition
bicuspid aortic valves will usually calcify, become fibrotic, or contract creating a "fish mouth" looking opening. and the L ventricle may also become hypertrophic
Term
why is coarctation of the aorta often missed? what is coarctation of the aorta?
Definition
b/c pts are only treated for HTN. coarctation of the aorta is a narrowing of the aorta distal (before) to the L subclavian artery.
Term
how would coarctation of the aorta appear on a CXR?
Definition
poor flow, high pressure in the upper extremities (should usually be lower in the upper vs lower extremities)
Term
how does coarctation of the aorta sound?
Definition
systolic murmur, the gradient is not at the aortic valve - so the murmur is heard better posteriorly
Term
how is coarctation of the aorta treated?
Definition
with balloon - easy
Term
does pulmonary stenosis favor one gender over another?
Definition
yes it seems to favor women
Term
where is pulmonary stenosis heard? how does it sound? what is the rate of dx?
Definition
at the R sternal border, presenting as a crescendo and decrescendo murmur across the narrowed valve. it often will go undiagnosed, pts will live to adulthood
Term
what is the problem with hypertrophic cardiomyopathy?
Definition
the walls of the heart become over-thickened - outstripping blood supply (angina, SOB b/c pressure is high and this is translated to the lungs) and decreasing capacity. this can cause sudden death via arrhythmias.
Term
what is another name for hypertrophic cardiomyopathy?
Definition
IHSS (idiopathic subaortic stenosis)
Term
how does a heart affected by hypertrophic CMP appear?
Definition
asymmetrically thickened walls
Term
can hypertrophic CMP be inherited?
Definition
yes, this can be autosomally dominant 60% of the time and result in sudden death
Term
how is hypertrophic CMP diagnosed?
Definition
easily via echo - can then measure heart walls - should be ~ 1 cm thick
Term
what can happen during systole with hypertrophic CMP?
Definition
the mitral valve can be pulled up and hit the septum -> creates a gradient
Term
what is the benefit of sx to correct hypertrophic CMP?
Definition
sx slice off some of the hypertrophic muscle of the septum, but there is a high sx mortality and it only makes it a little less symptomatic
Term
can drugs help with hypertrophic CMP?
Definition
yes, beta blockers/Ca+ blockers
Term
what is a new prodecure that has been developed for hypertrophic CMP?
Definition
a catheter is placed in the septum that injects alcohol locally in an attempt to create cell death
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