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organogenesis 3-5 weeks but probably week 4
heart is formed from single vascular tube :dilation, looping, septation
septum secundum has foramen ovale
septum primum defects are worse
ligamentum teres: with portal HTN, the back flow allows these to open up giving you caput medusae, sister Mary Joseph nodules
1st trimester defects: chromosomal
2nd trimester defects: mechanical
3rd trimester: fetal/placental insufficiencies
congenital malformations: 80% come from 5 diseases: VSD, ASD, coarctation of the aorta, PDA, tetralogy of Fallot (PROVe - Pulmonary stenosis, RV hypertrophy, Overriding Aorta, VSD)
VSD: 50% of small ones close on their own. CHF, cyanosis, pericardial thrill
ASD: no murmur, fixed split of S2, Eisenmenger and atrial fibrillation; Trisomy 21 are septum primum defects, MR, systolic murmur; patent ovale - failure of fusion of valve, paradoxical embolus (R sided thromboembolic clot gets into L sided circulation)
PDA: failure of ductus arteriosus to close, rubella, large defect is L ventricular failure, small is a continuous machinelike murmur. Rx - endomethecin causes decrease of PGE2
coarctation: turner syndrome (XO), differential cyanosis (top is pink, bottom is blue), collateral circulation, moderate size: claudication, BP arm > BP leg; small - HTN, ejection murmur
Tetralogy of fallot: 22q11 deletion syndrome, di George disease, congenital cyanosis, blue baby, clubbing of fingers, ejection murmur of VSD, tet spells of fainting in kids who learn to squat, boot heart CXR
Trisomy 21: (40% have heart defects of septum primum - endocardium cushions)
22q11 ds: T of F
Turner syndrome (XO): bicuspid aortic valve, coarctation of aorta
Cyanosis: bluish colored in skin
peripheral cyanosis - blue fingers and toes - vasoconstriction leads to more O2 extraction; cold, Raynaud's phenomenon, cardiogenic shock
congenital cyanosis - R to L shunt (T o F)
acquired cyanosis - eisenmenger syndrome L to R shunt reversal of shunt due to RVH
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