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Congenital Heart Disease II pgs. 12-15

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Question
Answer
What is TAPVR (Total Anomalous Pulmonary Venous Return)   When all four pulmonary veins return to the systemic venous circulation or directly to the right atrium resulting in a mixing of systemic and venous blood  
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What defects are associated with TAPVR?   ASD or patent foramen ovale to help maintain left ventricle output  
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What is the management of TAPVR?   Surgical management is often needed in the neonatal period (reattach pulmonary veins to the left atrium  
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What is the main feature of TGA (Transplantation of the Great Arteries)   ventriculoarterial discordance with the aorta arising from the right ventricle and the pulmonary artery arising from the left ventricle  
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When you have TGAs how do you maintain oxygenation of the tissues?   Tissue oxygenation depends on mixing of blood through either an ASD, VSD, or PDA  
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What is d-TGA   Aorta is anterior and to the right of the pulmonary artery with Atrial ventricular concordance (parallel circulation)  
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What is I-TGA   Aorta is anterior and to the left of the pulmonary artery with atrial ventricular discordance resulting in "corrected" transposition (circulation in series)  
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Are infants with d-TGA or I-TGA more likely to be cyanotic in the newborn period and show signs of congestive heart failure within weeks?   d-TGA  
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How do patients with I-TGA often manifest   they are usually asymptomatic but are prone to late right ventricular dysfunction as the right ventricle is not equipped to support the systemic circulation  
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What is initial management of TGA   maintaining patency of the ductus arteriosus and or through atrial septostomy  
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what is Truncus Arteriosus   a single arterial trunk arises from a single semilunar valve and supplies teh aorta, coronary arteries and pulmonary arteries.  
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what component of fetal circulation is usually absent   Ductus Arteriosus  
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why does cyanosis develop with truncus arteriosus   extensive mixing of the oxygenated and deoxygenated blood  
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What are the four components of Tetrology of Fallot?   VSD, Right ventricular outflow obstruction, Overriding aorta, and Right Ventricular hypertrophy  
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what is a Tet spell   paroxysmal hypercyanotic spells that are usually brought on by agitation or feeding (prolonged cyring tachypnea, cyanosis and increased pulmonary stenosis murmur)  
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What is therapy for a Tet Spell   placing the baby's knees to its chest to decrease pulonary venous return and increase systemic vascular resistance.  
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What are the four defects resulting in Acyanosis and increased PBF?   the Ds (Names with a component starting with a D) ASD, VSD, AVSD, PDA  
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what are the defects that result in acyanosis and nml PBF?   the stenosis and Coarcs, Pulmonary and Aortic Stenosis and Aortic Coarctation  
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You have a kid with cyanosis and decreased pulmonary blood flow what do they have?   Pulmonary Atresia, Ebstein's Anomaly, or Tetrology of Fallot  
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