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congenital cardiac defects

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Answer
Eisenmenger's syndrome   Any left-to-right shunt that becomes right-to-left, or bidirectional. Due to pulm.vascular resistance.  
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Eisenmenger's syndrome : Which side of heart is effected?   RAE, RVE, MPA dilated. Left side unchanged.  
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Eisenmenger's syndrome consist of:   VSD, Dextro-position of Aorta, PHTN, RVH.Measure TR, PR.  
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Hypo-plastic Left Heart Syndrome   Small or non existing (absent morphologic) LV with underdeveloped MV and AV. Also called mitral and aortic atresia. Male predominant.67%  
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Most Common cause of death from heart disease during first week of life.   Hypo-plastic Left Heart Syndrome  
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Hypo-plastic Left Heart Syndrome surgical repair involves:   Ballon atrial septostomy, Norwood I, II, III. possible transplant.  
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Hypoplastic LV echo views:   PLAX, Apical Long axis, PSAX, Apical Subcostal 4ch, supersternal long/short axix.  
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Pulmonary Atresia with intact Ventricular Septum   Complete obstrcdtion of RVOT with atretic PV, intact VentrSeptum, variable hypoplasia of RV and TV  
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Pulmonary Atresia with intact Ventricular Septum also will use Prostaglandin E2 to :   Keep PDA open. PDA helps sustain life until repaired.  
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Pulmonary Atresia with intact Ventricular Septum surgical management includes:   BT shunt, Pulm. valvotomy or valvectomy, RVOT reconstruction(transannular patch), Fontan, (possible transplant)  
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Views for Pulmonary Atresia(intact V-septum)   PLAX of RVOT, PSubcostal SAX of AV, Apical/subcost four chamber.  
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TOF/TET- Tetrology of Fallot defined as combo of 4 cardiac abnormalities   1. Overriding Aorta(bivent ao) 2. VSD(malignment) 3. RVH 4. PS (infundibular) TOF 10 % of all CHD and is the most common cyanotic lesion in adults.  
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TOF/TET caused by underdevelopment of ________ and abnormal separation of _____________.   infundibulum and truncus arteriosus.  
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Two typs of Tetrology of Fallot (TOF/TET)   1. Cyanotic - severe PS with predominant R-L shunt 2. Acyanotic- mild PS with predominant L-R shunt. note(Severity of lesion based on severity of PS)  
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TOF/TET associated disorders may include:   - RtArch30% -Secundum ASD 25% - Persistent SVC11% -Coronary Abn.s, complete AV canal, trabecular VSD,sub AS, PV and/or PA atresia.  
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TOF/TET associated surgeries:   BT shunt, Waterston shunt, Potts shunt, pulm. valvotomy or valvectomy, Close VSD, repair ASD, remove muschle bundles in rvot and Rastellie for pts with pulmonary atresia.  
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Types of Pulmonic Stenosis:   -Subvalvular, sub-infundibular, annular(hypoplasia), bicuspid pv, supravalvular, branch stenosis, pulmonary atresia.  
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Define (TAPR) Total Anomalous Pulmonary Venous Return   pulmonary Veins drain directly into the right atrium. Dialates RV and RA,can cause CHF  
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What is necessary for patient to have in order to survive if born with TAPR.   A PFO or ASD  
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List Cyanotic cardiac defects.    
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TAPR assoc. surgical procedures involve   Rashkind, Park, Blalock-Hanlon, reconnect or anastomosis of Common pulm.vein to LA, close ASD..  
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TAPR 2d/mmode shows   ASD/PFO , RAE, RVE, RVVO pattern, MPA/branching dialitation, dialated vertical vein, innom.v. rt. svc. ivc, and hepatic veins.  
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Most common type of mixed defect in regards to TAPR type IV is a   Connection to coronary sinus and left innom.vein.  
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Tricupsid Atresia   Absence of Tricuspid vavle with hypoplasia of RV  
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Tricupsid Atresia possible associated conditions   PFO/ASD 80% PDA, RT Arch, VSD, Transpo Great Arteries, persistent svc, juxtaposition of atrial appendages, PS  
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TriAtresia assoc.surgeries:   BT shunt, Glenn, PA band( to decrease hight PA flow), fontan  
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Truncus Ateriosis   Absence of normal division of Truncus Ateriosus. Type I,II,III  
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Truncus Ateriosis define:   The coronary arteries, MPA, AoArch all arise from a common truck.Type I,II,III Type I . PA arises from truncal root.60% Type II. Each PA arises directly from posterior side truncal root as seperate vessels,  
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Univentricular heart:   Presence of two atrioventricular valves with only one ventricle chamber or one large dominant ventricle.D-transposistion come with UniVent.heart.  
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Uni-Ventricle assoc.surgeries   BT shunt, Glenn, PA band, fontan partitioning of single ventricle.  
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Kawasaki syndrome   Unknow etiology, also called mudodutaneous lymph node syndrome, strawberry tongue, Involves coronries dilated, ectasia, aneurysms  
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Abnormal coronary artery sizes in child less than 5   > or = to 3mm look for anneurysms and thrombus.  
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Abnormal coronary artery sizes in child 5 and up   > or = 4mm  
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Myocarditis   inflamation of the myocardium caused by virus.  
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Pericardial effusion   collection of fluid between epicardium (visceral) and parietal pericardium.  
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Post-pericardialtomy syndrome   immune response to blood in pericardia lsac  
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PA sling   LPA arises abnormally from RPA passes posteriorly between trache and esophagus causing respiratory distress.  
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PA sling echo views:   PSAX at base tilted posteriorly, Suprast.notch, high left parasternal.  
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PHTN   pulmonary hypertension in pulmonary artery pressure.  
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Causes of PHTN(pulmonary hypertension)   L-R shunt, alveolar hypoxia, airway obstruction, collegen disease, connective tissue disease, high altitude, vascular disease.  
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Vascular ring   Caused by Double Aortic Arch(most common type)and other abnormalities of the aortic arch and vessels, causing a ring encases trachea and esophagus. Causes tracheal compression and respiratory distress.  
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Anomalous Origin of LCA (RARE) (Bland-Garland White syndrome).   When LCA originates from abnormally from PA  
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Cardiac Tamponade   Fast accumulation of pericardial fluid with elevation of venous pressure and pulsus paradoxus.leads to impaired fill and decreased cardiac output. "Swinging Heart" Diastolic collapse.25% resp.variation  
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Most common type of Cardiac benign tumor.   Rhabdomyoma( involves myocardium) assoc.with TB  
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Second most common cardiac tumor   Fibroma intra mural in IVS  
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Myxoma   heart tumor within atrium , has stalk or pedicle attached to wall , most common in females  
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Teratoma cardiac tumor   pedicle attached to base of Great Vessels.  
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Cervical Ao Arch   Ao arch elongates above clavicle into neck  
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CHF   Congestive Heart failure, failure to pump sufficient blood to meet body's demands due to myocardial damage or pressure or volume overload, diastolic dysfuntion.  
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Cardiac pericarditis   fibrotic thickening restricting pericardium and diastolic filling.dialated IVC, hepatics , and lack of IVC collapse on inhale.  
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Dextrocardia )like dextro position Mirror Image dextro Dextro-rotation Dextro-position   Heart in Rt side of chest.(thorax) Heart all structures a mirror image of what norm is. Left apex rotated toward the right from its normal left position. Heart just shifted to right chest all structures normal  
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BT Shunt Blalock-Taussig   proceedure where Rt. Subcv.A. is attached to the RPA (in a left Arch) in order to increase pulmonary blood flow. (In patients with Rt. Arch the Left Subclv. A. is attached to the LPA)  
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What corrective surgery involves attaching right or left subclavian artery to RPA or LPA to increase pulmonary flow in cases that involve PA atresia, TOF, Univentricle heart.   BT Shunt.  
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What defects involve a BT shunt surgery to increase pulmonary flow.   Pulmonary atresia, tetrology of fallot, tricuspid atresia, univentricle.  
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2 D echo views to eval BT shunt.   Supersternal notch(supraclavicle) SAX for a RT. BT Supersternal notch long axis with anterior tilt for LPA and BT.  
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Modified BT Shunt   prosthetic tube used( usually Gortex)  
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Temporal Resolution -Poor tempRes equals poor image quality.   determines frame rate, the time it takes to update all lines in image.  
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Ways to increase or make faster the Temperal Resolution:   -Decrease depth (takes less time for farthest wave to return) -Narrow sector(fewer scan lines) -Lower Density ( fewer scan lines) -Single focal point -Parallel beam forming.  
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Note: M-mode is faster that 2D, 2D faster than 3D   no answer, just a fact  
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Doppler Effect ( Frequency shift is directly proportional to.....   Velocity  
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CW doppler   -Continuous transmission -a Pair of PZ crystals -Range of velocities along entire beam No Distance Range Resolution = Range Ambiguity -Useful to detect max. velocities.  
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PW doppler   -at a single fixed time and place after transmission -Displays velocities at a SPECIFIC distance. (sample volume) _Range gated -limit on maximum velocity, if exceed = aliasing  
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Nyquist Limit   Maximum velocity.  
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Lower Frequency Tx's have a ---------------- Nquist limit.   Higher  
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Shallow depth has a --------------Niquist limit.   Higher  
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Tissue Doppler only detects:   velocity components that are parallel to the beam in pulswave and color doppler display.  
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Higher Frequency Transducers (12)   Better resolution, less depth penetration, more attenuation, lower Nyquist limit or lower max velocity limit with PW.  
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Bernoulli Equation    
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LV Global systolic function (Ejection Fraction) Normal range   55 - 70 % Measured by , Mmode, 2D, 3D 2D modified simpsons  
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limitations on determining EF%   load dependent Apical forshortening poor acoustic windows  
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Diastoic Phases of the Heart are   1. Isovolumetric relaxtion = semilunar valve closure to AV valve flow onset 2. Rapid Filling: atrial pressre > ventriclar pres. PW Ewave.3. Diastasis: equal atrial/ventricular pressures with little flow.4 Atrial contr atrial kick A-pressr >Ventr=pw a wave  
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Decreased myocardial stiffness = a ------------- in volume.   Increase  
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Main parameters for diastolic Ventricular function for Echo are   -PW dop AV valve inflow. (mitral and tricuspid) -PW pulmonary vein flow -TR jet -PW tissu doppler (TDI AV annulus) -Color m-mode (speed of propagation) -Atrial size: reflects duration and severity -RV pressure estimates  
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MV E wave in regards to Ventricular diastolic function, the steeper the slope the -------------   shorter the deceleration time  
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Pulm Vein PW doppler for Ventricular dias.fx.   wave flow shows above baseline S and D peak(systolic and diastolic) and Below baseline A wave Atrial contraction.(shows how much flow goes back up into pulm vein.  
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PW tissue doppler E/A prime wave below base line.diastole, but systolic flow shows   Above the base line.  
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Peds Cardiology TEE indications would be   Poor Transthoracic echo images, large patient, eval for R-L shunting for a pt evaled for stroke or transvenous pacemaker,Vegi,abcess, central line infec.thrombus before cardioversion, intraop , guid for cath procedures.  
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TEE Absolute Contraindications   Unrepaired trach-esph fistula, eshop obstruction, poor airway control , uncooperate unsedative pt. , esophageal varieces, vascular ring poor airway, severe coagulopathy,spine injury  
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peds contrast echo   microbubbles reflect uswaves, for detect PFO R-L, visual of systemic venous drainge(LSVC to LA), AV malfr, define RV enodocardial border,see cath tip  
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Define Aortopulmonary Window   Communication b/n Asc.Aorta and PA above the semilunar valves , Type I -proximal Type II- Distal TypeIII-Complete  
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Waterston Shunt   Waterston Shunt. Not used anymore,, but anastomosis was between Asc.Ao and RPA Problems ,,either too large or too small and destroys RPA as child grows.  
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Classic Blalock -Taussig shunt rarely used but one advantage was   it Grew with the patient.  
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Modifiet BT   most used today and prefered,: , PTFE graft from subclv a or innom a to the RPA  
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Potts shunt reinvented.    
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GERBODE VSD   communication between the LV and RA.. Hard to seperate with a perimembraneous VSD echo wise.  
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Complete AVSD atrial ventricular septal defect.   a common AV valve. VSD, ASD,  
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Rastellie classification Type A, B, C   a common , c. second most comon  
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single patch technique use to repair    
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double patch repair    
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Tricuspid Atresia Surgical management   Cyanosis- neonatal shunt, cath 6 mos and Bidirectional Glen, then Fontatn at 2 - 3 years old  
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Bi Directional Glenn:   Most common, SVC to RPA, Blood goes from SVC to RPA and to over to LPA  
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Glenn types, fenestrated, etc. In what order , and why,    
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Echo shows in Apical 4 view what in regards to chamber size in Restrictive Cardiomyopaythy.   Small Ventricles and Huge Atrias  
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