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Sem4-Path-ConHD
Congenital Heart Disease
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 |
What is the ostium primum? | Small opening at bottom of septum primum, which closes when osteum secondum forms |
What is the septum secondum? | The second layer that forms, dividing the two atria. |
What is the foramen ovale? | The orifice through which blood flows from the RA to LA. Located in the septum secondum |
What is the osteum secondum? | a perforation in the septum PRIMUM |
Where is the osteum secondum located? | In the septum primum (top) |
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 |
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 |
Atrial Ventricular Septal Defect is often comorbid with what genetic disorder? | Trisomy 21 |
Coarctation of the aorta is most commonly associated with what genetic disorder? | Turners. 45, XO |
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. |
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) |
Coarctation of the aorta: Cyanotic or acyanotic? | Acyanotic |
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 |
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 |
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. |
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 |
What is the biggest complication of a R to L shunt | brain infarction / abscess due to paradoxical emboli |
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) |
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 |
What is the an associated disease that cause a patent ductus arteriosis | Congenital rubella infxon. Causes L-> R shunt |
Coarctation of the aorta is associated with what heart defect? | bicuspid aortic valve |
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) |
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, |
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. |
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 |
components of tetrology of fallot | ventricular septal defect, hypertrophy of the right ventricle, overriding aorta, pulmonary stenosis. |
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* |
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 |
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 |