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Congenital Heart Disease

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Question
Answer
What is the septum primum?   The septum that grown from top of primitive atrium down to the endocardiac cushion to divide primitive atrium into L and R atrium  
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What is the ostium primum?   Small opening at bottom of septum primum, which closes when osteum secondum forms  
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What is the septum secondum?   The second layer that forms, dividing the two atria.  
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What is the foramen ovale?   The orifice through which blood flows from the RA to LA. Located in the septum secondum  
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What is the osteum secondum?   a perforation in the septum PRIMUM  
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Where is the osteum secondum located?   In the septum primum (top)  
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What causes the foramen ovale to close?   The foramen ovale is closed by the septum primum due to higher pressure in the LA than the RA  
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What closes the foramen ovale?   Septum primum due to higher pressure in the LA than in the RA. Septum primum is in the LA, and pushes in toward the RA to close the foramen ovale  
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Atrial Ventricular Septal Defect is often comorbid with what genetic disorder?   Trisomy 21  
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Coarctation of the aorta is most commonly associated with what genetic disorder?   Turners. 45, XO  
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What kind of shunt is cyanotic ? Acyonotic?   Cyanotic: R to L shunt. Acyanotic L to R shunt. L to R shunt can progress to an R to L shunt in time making it cyanotic.  
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Give two examples of acyanotic congenital heart defects.   L to R shunt (AVSD, VSD, ASD, PDA) and defects causing a blood flow obstruction (coarctation of the aorta, valvular stenosis of the aorta or pulmonic valves)  
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Coarctation of the aorta: Cyanotic or acyanotic?   Acyanotic  
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Give 4 examples of cyanotic congenital heart diseases   Defects causing a right to left shunt: **THE T's** *T*etrology of fallot, *t*ransposition of the great arteries, persistent *t*runcus arteriosis, and *t*ricuspid atresia  
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What is eisenmenger syndrome?   Reveral of L to R shunt (becomes a R to L shunt --> cyonotic CHD) due to right pressure exceeding left pressure  
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Major complications of a L to R shunt?   Pulm htn with RV hypertrophy, which progresses to a R to L shunt (eisenmenger syndrome) due to pulm a. pressure exceeding aortic pressure.  
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What is a paradoxical emboli? What kind of shunt is it involved in?   A paradoxical emboli is an emboli that originates in a vein and then travels to the right side of the heart where it then crosses to the left side in the presence of a hole b/w the 2 chambers. It then travels to the brain causing a stroke  
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What is the biggest complication of a R to L shunt   brain infarction / abscess due to paradoxical emboli  
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What is the most common kind of atrial septal defect (ASD)   ostium secundum defect (OS is too large either due to absence of septum secundum or excess resorption of septum primum)  
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What are the two mechanisms for ostium secundum defect?   OS is too large due to: 1) absent septum secundum or 2) excess resorption of septum primum  
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What is the an associated disease that cause a patent ductus arteriosis   Congenital rubella infxon. Causes L-> R shunt  
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Coarctation of the aorta is associated with what heart defect?   bicuspid aortic valve  
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What is coarctation of the aorta?   A ridge like infolding of the aorta just opposite to the DA, located either preductally (infantile) or postductally (adult)  
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What are some clinical manifestations of coarctation of the aorta?   LV hypertrophy, dilation of collateral arteries (intercoastal aa and internal mammary a.) made apparent by presents of ib notching on cxr. Delayed/weak femoral pulse, elevated BP in upper body (nosebleeds, berry aneurysms, etc), midsys ejection murmur,  
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What congenital heart disease as associated epistaxis, ha, and elevated BP in upper body, and leg atrophy?   Coarctation of the aorta. Leg atrophy is due to reduced BP in legs.  
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Notching of the ribs is a sign of what congenital heart disease.   coarctation of the aorta. This is due to dilation of arteries leading to PRESSURE ATROPHY of the ribs  
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components of tetrology of fallot   ventricular septal defect, hypertrophy of the right ventricle, overriding aorta, pulmonary stenosis.  
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What are the clinical manifestations of tetralogy of fallot?   Cyanosis ( R to L shunt) **TET SPELLS - cyanosis after crying or feeding**, squatting, pulmonic stenosis with RV hypertrophy. *Boot shaped heart on CXR*  
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What is transposition of the great arteries?   Aorta arises from the RV, pulmonary a. arises from LV. MEDICAL EMERGENCT. due to conotruncal septum failing to follow its normal spiral course... it only runs up and down  
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How is transposition of the great arteries (TGA) managed?   Prostaglandin infusion to maintain DA, followed by balloon catheter to create interatrial foramen, and then surgical switch of the vessels  
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