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119 Cards in this Set
- Front
- Back
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What does Atrial Septal defect (ASD) result from?
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Failure of the interatrial septum to close during fetal development
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What does an ASD allow?
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blood to flow between the left and right atrium
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What is the prevalence of Ostium Primum ASD?
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20%
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Where is Ostium primum ASD located?
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The inferior portion of the intertribal septum
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Why does an Ostium primum ASD occur?
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The septum primum fails to fuse with the endocardial cushion during septation of the atria
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What are associated anomalies of the Ostium Primum ASD
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Deformed Mitral Valve
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What is the most common ASD?
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Ostium secundum 70%
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Where is a Ostium Secundum located?
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Mid portion of the interatrial septum
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What is the prevalence of Sinus Venosus ASD?
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10 %
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Where is a sinus venosus ASD located?
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the superior portion of the interatrial septum near the junction of the superior vena cava and the right atrium.
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What are associated findings with Sinus Venosus ASD?
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Partial anomalous pulmonary venous return (one or more of the pulmonary veins do not return to the left atrium.
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What is the prevalence of Coronary Sinus ASD?
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RARE
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Where does Coronary Sinus ASD occur?
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inferior septal area close to the coronary sinus
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What is a common atrium?
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the absence or near absence of the interatrial septum resulting in a common atrial chamber
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What do hemodynamic effect of the ASD depend on?
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1. size and direction of shunt
2. compliance of ventricles 3. response of the pulmonary vascular bed to the increased blood flow. |
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What does the direction of the shunt depend on
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pulmonary vascular resistance (PVR) and systemic vascular resistance (SVR)
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As the PVR elevates the resistance to pulmonary blood flow increases resulting in what?
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reduction in pulmonary blood flow
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What is Eisenmenger Syndrome?
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When shunt becomes right to left as in utero.
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What will Eisenmenger cause
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less than normal pulmonary flow and cyanosis b/c pulmonary vascular pathology is now reversed
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Qp/Qs shunt ratio equation
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Qp = SV rvot
Qs = SV lvot FYI: opposite of PDA equation |
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What is a VSD
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failure of the inter ventricular septum to close during fetal development, which allows blood to shunt across the inter ventricular septum between left ventricle and right ventricle
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Another name for atrioventricular defect
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Endocardial cushion defect
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What VSD is common with Trisomy 21 (Down Syndrome)
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Endocardial cushion defect
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what is an Endocardial cushion defect
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A shunt between ventricle and atria
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VSDs vary in size from....?
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7mm to 3cm
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Where is a Inlet septal VSD located?
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bordered by tricuspid valve, mitral valve and muscle. (SUPRAVALVULAR)
associated with atrioventricular septal defects. |
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What is the prevalence of Inlet Septal defect
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3-5%
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Where is a Trabecular or muscular septal VSD located?
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between the bodies of the left ventricle and right ventricle. Low on the septal wall in the thicker more muscular portion of the septum close to the apex. "SWISS CHEESE" appearance
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What is another name for Outlet septal VSD?
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SUPRACRISTAL VSD
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What is the prevalence of Outlet septal VSD?
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3-5%
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Where is an Outlet septal VSD located?
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between the left ventricular (SUBVALVULAR) outflow tract and right ventricular outflow tract bordered by the aortic valve, pulmonic valve and muscle
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What VSD has a strong association with aortic valve prolapse and aortic insufficiency?
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Outlet Septal VSD
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What VSD has a strong prevalence with Asian population
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Outlet Septal VSD
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What is the most common VSD with and 80% occurrence
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Membranous septal or perimembranous septal VSD
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Where is the Membranous septal VSD located
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bordered by tricuspid valve, aortic valve and muscle, usually high on the septal wall in the thinner, more flexible portion of the septum, closer to the valves and great vessels
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What VSD si aneurysmal ...thin stretchy portion of tissue extending into RV
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Membranous septal or perimembranous septal VSD
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When does a malalignment septal VSD occur?
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when 2 portions of the inter ventricular septum have failed to align properly during development
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What are examples of malalignment septal VSD
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Tetralogy of Fallot and Truncus Arteriosus
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What occurs with malalignment septal VSD
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Blood through both ventricles mixes together as it exits through a single valve from the heart.
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What is a Truncus Arteriosus
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an abnormality where the aorta and pulmonary artery rise from a common trunk causing blood to mix from both ventricles and exit through a single valve.
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Why does VSD place a burden not the heart?
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volume overload on both ventricles due to large amount of blood ejected into the RV and lungs, flow returns to the LV therefore both ventricles experience overload
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What can a VSD cause?
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PVR resulting in pulmonary hypertension
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VSD IMAGE
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PDA IMAGE
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ASD IMAGE
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When does the ductus arteriosus close?
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15 hours after birth and it is structurally sealed within 2 to 3 weeks
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What does the ductus arteriosus become?
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Ligamentum arteriosus
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What side of the heart is burdened by the PDA
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Right heart
lungs ultimately the left side of the heart |
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What does the flow reversal in a PDA cause
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volume overload in pulmonary artery and lungs as flow returns to the left side. Right side must work harder agains the increased pulmonary vascular resistance
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What kind of murmur is heard with a PDA
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high pitched "machine" murmur heard during diastole and systole
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what is considered a significant PDA
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QpQs ration of 1.5: 1 or greater
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What associated finding and hemodynamic effectswill you find with a PDA?
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left ventricular volume overload and pulmonary hypertension
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What kind of appearance will a Endocardocushion defect have
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butteryfly
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What is an Endocardial Cushion defect
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A hole in the center of the heart where the upper chambers meet the lower chambers allowing oxygenated blood to mix with deoxygenated blood
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If you have a common valve in place of the mitral and tricuspid valve what defect will you have
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Endocardial Cushion defect
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endocardial cushion defect
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endocardial cushion defect
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What is the most common site for a cleft mitral valve leaflet
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anterior mitral valve leaflet
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What are CMVL associated findings
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atrioventricular septal defect
ventricular septal defect atrial septal defect PDA rotation of papillary muscles presence of an accessory papillary muscle or MVL mitral valve prolapse |
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What are the effect of pulmonic stenosis (PS)
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obstructs blood flow between RV and pulmonary artery during systole
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this type of PS is the most common
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Valvular PS
usually due to fusion of the cusp |
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This type of PS involves stenosis in the RV outflow tract creating a RV outflow tract obstruction
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Subvalvular (infundibular) PS
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this type of PS involves stenosis of the main pulmonary artery just above pulmonic valve
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Supravalvular PS
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What changes occur with PS
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cusp become thickened and fibrotic and calcified reducing mobility and increasing obstruction. changes in RV; myocardial fibrosis or subvalvular muscular hypertrophy
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What echo finding must be evaluated with PS
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right heart enlargement
hypertrophy failure and/or infarct |
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What anomalies are found with PS
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atrial septal defect
ventricular septal defect double chambered RV |
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Pulmonic Stensis
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What is coarctation of the aorta
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narrowing or partial obstruction of the aorta, typically in the AORTIC ISTHMUS
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Where is aortic isthmus located
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between the left subclavian artery and the first intercostal artery
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What is coarctation of the aorta associated with
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bicuspid aorta
valvular aortic stenosis subvalvular aortic stenosis PDA ventricular septal defects |
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What are the 3 types of aortic coarctation
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Preductal
Juxtaductal Postductal |
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Where is Preductal coarctation located
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in aorta, superior to the ductus arteriosus or ligamentum arteriosum
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Where is Juxtaductal coarctation located
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in the aorta at the level of ductus arteriosus or ligamentum arteriosum
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Where does the Postductal coarctation occur
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n the aorta inferior to the ductus arteriosus or ligamentum arteriosum
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What are the hemodynamic effects of aortic coarctation
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blood pressure is elevated above the coarctation and decreased below the coarctation.
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What might develop with aortic coarctation
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Collateral vessels develop from the arteries above the coarctation in order to supply blood flow to the lower extremities.
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What is the optimal window to rule out aortic coarctation
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suprasternal
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A coarctation measurement of less than or equal to _____% of the descending thoracic aorta diameter indicates server coarctation
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40
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Postductal Aortic Coarctation
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EPSTEIN ANOMALY
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epstein anomaly with septal defect
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tetralogy of fallot
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dextro transposition
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levo transposition
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What does Epstein Anomaly involve?
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downward displacement of 1,2 or 3 of the tricuspid valve leaflets into the RV. A portion of the RV becomes ATRAILIZED and serves as part of the RA
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What is the result of Epstein Anomaly
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Large RA may cause a PFO and /or sucundum atrial septal defeat , and small RV and TR...may result in heart failure
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What other anomalies are associated with Epstein Anomaly
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ASD (75%)
VSD PFO PDA PS Mitral Stenosis TOF Transposition of Great Arteries |
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What is the most common cyanotic lesion in the adult population?
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Tetralogy of Fallot
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What are the findings with Tetralogy of Fallot
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PS
Right Ventricular Hypertrophy Over-riding Aorta Malalignment |
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With Tetralogy of Fallot what do the symptoms depend on
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Size of the VSD
degree of PS position of the Aorta |
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With Tetralogy of Fallot what do PS and RVH cause
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VSD to shunt right to left producing a decrease in systemic arterial oxygen saturation, cyanosis, reduced pulmonary flow and possibly a hyperplastic PA
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What do children with Tetralogy of Fallot experience
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1. exercise intolerance
2. squatting episodes (incerases venous return and SV and CO 3. Tets episodes- faintness and cyanosis |
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With Dextro-TGA where does the aorta arise from
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anterior ventricle
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With Dextro-TGA where does the PA arise from
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posterior ventricle
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What is the circulation pattern with Dextro-TGA
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systemic flow returns to the RA⇨RV⇨aorta⇨body⇨RA again
Pulmonary Venous Flow returns to the LA⇨LV⇨PA⇨Lungs⇨LA again |
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How does life sustain with Dextro-TGA
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Shunts! intermixing of the 2 circuits with occur through PFO and PDA
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What medications maintains shunts with Dextro-TGA
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Prostaglandins
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What other anomalies are associated with Dextro-TGA
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VSD
ASD PS Right sided Aortic Arch |
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What occurs with Levo-TGA
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1. anatomic RV will be displaced posterior and leftward becoming arterial ventricle
2. anatomic LV will be displaced anterior and rightward becoming venous ventricle |
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What is the circulation pattern with Levo-TGA
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1.Pulmonary Venous returns to LA⇨anatomicRV⇨aorta⇨body
2. Systemic flow returns to RA⇨anatomic LV⇨PA⇨lungs⇨back to LA |
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Why do Levo-TGA survive
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PA arises from venous ventricle
Aorta arises from arterial ventricle as normal |
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How long will a patient with Levo-TGA survive
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With no other defect 15-20 years then systemic ventricle (anatomic RV) will fail
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What is Hypoplastic Left Heart Syndrome? (HLHS)
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Left heart is reduced in size due to restriction of the LV inflow and outflow. Due to MITRAL ATRESIA, AORTIC ATRESIA, HYPOPLASIA OF THE AORTA
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When can you make diagnosis of HLHS
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LV is less than 10mm, the aortic annular size is reduced, mitral valve is distorted or absent
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What assist with circulation with HPHS
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ductus arteries and foramen ovale
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What side of the heart is burdened with HPHS
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Right heart is burdened with volume and pressure working to assist the left heart.
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When can HPHS be diagnosed
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16-20 weeks into pregnancy
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What is needed for survival with HPHS
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Shunts which are kept open with the medication prostaglandin
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ASD
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failure of the interatrial septum to close during fetal development: allows blood to shunt between LA and RA
TYPES: ostium primum, sinus venosus, coronary sinus and common artium |
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VSD
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failure of interventricular septum to close during fetal development: allows blood to shunt between LV and RV
TYPES: inlet, trabecular/muscular, outlet, membranous/perimembranous, malalignment |
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PDA
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failure of the ductus arteriosus to close after birth: results in a communication between the AO (high pressure system) and PA (low pressure system)
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ENDOCARDIAL CUSHION DEFECT
(aka..atrioventricular canal defect or atrioventricular septal defect) |
Combination of anomalies
1. Hole in the center of the heart where the upper chamber set the lower chamber allowing oxygenated blood to mix with deoxygenated blood 2. a common valve in place of the MV and TV: common in patients with Trisomy 21 (Downs Syndrome) |
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CLEFT MITRAL VALVE LEAFLET (CMVL)
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Split or division of the anterior (more common) and or /posterior MV leaflet: evaluate degree of cleft edge fibrosis, retraction of valve anatomy: frequently associated with atrioventriculare septal defect, VSD, ASD, PDA, MVP rotation of the papillary muscles, presence of an accessory papillary muscle or MV leaflet
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PULMONIC STENOSIS (PS)
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Obstruction of blood flow from the RV to the PA (subvalvular, valvular or supravalvular): the PV cusp can become thickened, fibrotic, and calcified which reduces the valve mobility and increases obstruction; changes also occur in the RV such as myocardial fibrosis or subvalvular muscular hypertrophy which can further contribute to the RV outflow tract obstruction.
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COARCTATION OF THE AORTA
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Narrowing of the aorta, typically in the area of the aortic isthmus (preductal, juxtaductal, or post ductal): often associated with BAV, valvular AS, subvalvular AS, PDA and VSD
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EBSTEIN ANOMALY
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downward displacement of one, two or three TV leaflets into the RV, a portion of the RV becomes atrailized and serves as part of the RA: results in a large RA, small RV and TR; may result in RHF; often associated with ASD, VSD, PFO, PDA, PS, MS, TOF and D-TGA
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TETRALOGY OF FALLOT
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Cyanotic lesion consisting of 4 defects
1. PS 2. RV hypertrophy 3. Over-riding Aorta 4. Malaignment children typically experience exercise intolerance, squatting episodes, and "tet" spells |
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D-TGA
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Great arteries are transposed creating two independent parallel circuits; life dependent on some intermixing of those two circuits via PFO, PDA, and possible VSD
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L-TGA
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Ventricles are transposed; if no other defects are present, the patients heart will function normally for 15-20 years but then the systemic ventricle (anatomic RV) will fail.
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HYPOPLASTIC LEFT HEART SYNDROME (HLHS)
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LV is reduced in size due to a restriction of LV inflow and/or outflow; restriction is typically due to mitral atresia, aortic atresia, or hypoplasic of the aorta; the key to treating HLHS is early detection; prostaglandin can be administered in order to maintain the shunts and circulation; keeping the shunts open is the key to survival
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