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Peds Rot Cardio

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Question
Answer
Syndromes associated with heart disease   Turner, Down, William, Noonan DiGeorge/Velocardiofacial  
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a right to left shunt sign on PE   clubbing  
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Signs of congestive heart failure   extremity edema and hepatomegaly  
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all patients with suspected pathologic murmurs should receive   an electrocardiogram and echocardiogram  
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____ therapy should be started in all unstable infants wtih suspected congenital heart disease   Prostaglandin E1 (PGE1): a medication that will keep the PDA open  
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___ tests are not appropriate for interpretation of the hyperoxia test   pulse oximetry. ABGs are appropriate  
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Truncus arteriosus   a single arterial vessel arising from the base of the heart which gives way to the coronary, systemic, and pulmonary arteries. All the blood gets mixed. A VSD is almost always present. Associated with DiGeorge's and velocardiofacial syndrome.Tx: surgery  
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open connection between the atrium at birth   foramen ovale  
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Transposition of the great vessels (vsd also often present) tx   PGE1 immediately to keep the PDA open.Balloon atrial septostomy to stretch wider the foramen ovale allowing for more mixing of oxygen rich and oxygen poor blood to keep body levels of oxygen in a safe range.Then an arterial switch surgery in the first wk  
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Effects of ASD on the heart   since oxygen rich blood from the pulmonary veins flow from the left atria to the right atria, both atria enlarge. Also, the right ventrical and pulmonary artery enlarge  
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ASD diagnosis   in most children, will cause no sx. a large defect may have sx of CHF: fatigueable, SOB. Dx on PE: Murmur caused by large volume of blood being pumped through the pulmonary artery. Split S2 b/c it takes the R V a bit longer to eject all the blood  
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The main method used to confirm suspicion of ASD   Echo:can show not only the hole and its size, but also any enlargement of the right atrium and ventricle in response to the extra work they are doing.  
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Tx of ASD   small ASD will close on its own in 80% of children. >3yo will not close. Most commonly closed by open heart surgery with suture or if larger, with a patch. Alternative:Amplatzer Septal Occluder  
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ASD facts   F:M ratio of 2:1. 8% of CHD. 3 types. Congestive Sx in first year can be treated wtih diuretics and digoxin  
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The most common congenital heart defect   VSD; accounts for 25% of all CHD  
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Left untreated large VSD may result in   pulmonary vascular obstructive dz, pulmonary HTN, and Eisenmenger's syndrome. In severe cases of Eisenmenger's syndrome, the VSD shunt reverses right to left when the PVR exceeds the SVR  
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The smaller the VSD defect, the _____ the harsh systolic murmur, heard best at the mid-to-lower left sternal border   louder  
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VSD tx   most small VSDs close w/out intervention. For large VSDs w/ significant L to R shunting and CHF, tx is surgical closure before pulmonary vascular changes become irreversible.Dacron patch.Pts with unrepaired VSD require bacterial endocarditis prophylaxis  
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branches off the aorta from R to L   BCS=brachiocephalic, Left carotid artery, L subclavian artery  
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Which syndrome must be considered when coactation of the aorta is present?   Turner Syndrome  
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The most common site of narrowing in coarctation of the aorta   in the descending aorta at the insertion site of the ductus arteriosus.  
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Other findings associated w/ coarctation of the aorta   bicuspid aortic valve, mitral valve anomalies. The coarctation results in obstruction to blood flow and increased left ventricular afterload  
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Prevalence of Coarctation of the aorta   makes up 8% of CHD, M:F ratio of 2:1  
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PE of coarctation of the aorta   weak femoral pulses, upper extremity htn, nonspecific ejection murmur at the heart apex. ECG nl in mild lesions. In more severe obstruction, CXR may show an enlarged aortic knob. RVH more common in the neonate; LVH in older kids  
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Tx of coarctation of the aorta   May be surgical with end to end anastomosis or patch aortoplasty, or interventional, with balloon dilation angioplasty w/ or w/out stent placement. Restenosis not uncommon. BB for persistent HTN may be necessary  
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What does the PDA attach?   the underside of the aorta and the left pulmonary artery just distal to the takeoff of the left subclavian artery from the aorta  
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Blood flow in a PDA   depends on the ratio of SVR to to PVR. If SVR > PVR, then left to right shunt occurs. Sx related to the size of the defect and the direction of the flow  
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Large PDA   L to R flow, CHF sx and failure to thrive. Bounding pulses are palpable. A continuous murmur begins after S1, peaks at S2, and trails off during diastole. CXR shows cardiomegaly, increased pulmonary vascularity, left A and V enlargement.  
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PDA is best seen on   ECHO using Doppler flow mapping. Cyanosis is noted if PVR >SVR  
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Tx of PDA   Indomethacin counteracts PGE1 and is often effective at closing a PDA in the premature neonate. PDA usually closes in term infants in first month of life.  
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Tetralogy of Fallot   VSD, pulmonary stenosis, RVH, Right to Left Shunting of blood, an overriding large ascending aorta  
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CXR of tetralogy of Fallot   heart may be boot shaped; decreased pulmonary vascular markings  
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PE of tetralogy of Fallot   Characteristic periodic episodes of cyanosis,rapid &deep breathing& agitation known as "tet spells" caused by an increase in R ventricular outflow tract resistance which leads to R->L shunting. R V heave,systolic ejection murmur in upper L sternal border  
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Tx of "tet spells" in tetralogy of Fallot   Increasing SVR and decrease pulmonary resistance in order to decrease R->L shunting. Vagal maneuvers, O2, vasoconstrictors, BB. Neonates repaired immediately. 3-6 months also repair  
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Tet spells/ Tetralogy spells   result from a sudden increased constriction of the outflow tract to the lungs so that pulmonary blood flow is further restricted. The lips and skin of babies who have a sudden decrease in arterial oxygen level will appear acutely more blue.  
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Non surgical ASD repair   Amplatzer Septal Occluder. Approved by the FDA in 2001  
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