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| Pregunta | Respuesta |
| What do ASD's result from? | Failure of the IAS to close during fetal development |
| When do most defects happen? | In the first 7 weeks |
| What is the most common ASD? %? | Ostium Secundum, 70% |
| What is the second most common ASD? %? | Ostium Primum, 20% |
| What are the 2 anomalies associated with Ostium Primum? | Deformed MV, Endocardial Cushion Defect |
| What is the 3rd most common ASD? %? | Sinus Venosus, 10% |
| What anomaly is associated with Sinus Venosus? | All pulmonary veins do not enter LA |
| What are the 2 rare ASD's | Coronary Sinus, Common Atrium |
| This ASD is the result of the absence or near absence of the IAS. | Common Atrium |
| The hemodynamic effect of an ASD depends on all of the following except...Compliance of ventricles...Response of pulmonary vascular bed to increase blood flow...Size and direction of shunt...Compliance of the atria. | Compliance of the atria |
| What is the ideal view for visualizing an ASD? Why? | Subcostal, parallel angle |
| What is the formula to calculate the severity of an ASD/VSD? | QP/QS |
| What ratio is considered a large shunt? | 2:1 or higher |
| In the QP/QS equation, what does the QP represent? | Pulmonary flow |
| In the QP/QS equation, what does the QS represent? | Systemic flow |
| What percent of the population have a foramen ovale that remains patent? | 25-34% |
| VSD's range in size from_____ to _____? | 7mm-3cm |
| This type of VSD lies immediately below the PV outflow | Supracristal |
| This type of VSD is located inferior/posterior to the crista supraventricularis. | Infracristal |
| What is the ONLY supracristal defect? %? | Outlet Septum, 3-5% (Hint:easy to remember...First letter of Only and Supracristal (OS)= Outlet Septum :) |
| The outlet septum has an increased incidence with what population? | Asian |
| What is the most common Infracristal VSD? %? | Membranous/Perimembranous, 80% |
| What is the 2nd most common Infracristal VSD? %? | Trabecular, 5-20% |
| What is the 3rd most common Infracristal VSD? %? | Inlet Septum, 3-5% |
| What is a common anomaloy with Inlet Septum? | AV septal defect(endocardial cushion defect) |
| I am a VSD that occurs when 2 portions of the IVS fail to align properly. What am I? | I am Batman!! Or in medical terms, I am a Malalignment Septum |
| What are 2 associated defects with a malalignment septum? | Tetralogy of Fallot, Truncus Arteriosis |
| Ideal view to visualize VSD's? | Subcostal |
| Endocardial Cushion Defect AKA | AV Canal Defect |
| What 3 things make up a complete Endocardial Cushion Defect? | Ostium Primum ASD, Perimembranous VSD, Straddling AV Valves |
| What 2 things are connected in the fetus by the Patent Ductus Arteriosis? | Connects the pulmonary artery to the aorta |
| Functionally, when should the ductus arteriosis close? | At 15 hours |
| Structurally, when should the ductus arteriosis close? | At 2-3 weeks |
| What is the ductus arteriosis called if it fails to close properly? | Patent Ductus Arteriosis |
| What does the ductus arteriosis become after birth. | Ligamentum Arteriosum |
| What is the formula to calculate the ratio pf a PDA? | QP(SV LVOT)/QS(SV RVOT), the opposite of the formula for ASD/VSD's |
| What is the term for when the truncoconal ridges fail to fuse? | Persistent Truncus Arteriosis |
| What are the 4 types of PTA's? This is an extremely difficult question. | Type 1, Type 2, Type 3, Type 4 Omg, who was the genius that came up with these names |
| Describe type 1 PTA | Short main pulmonary artery arises and positioned to left of aorta |
| Describe type 2 PTA | Seperate but close arteries arising from posterior portion of the truncus |
| Describe type 3 PTA | Widely seperated PA's arising laterally from the truncus |
| Describe type 4 PTA | PA's arise from the descending aorta |
| What % of congenital anomalies are from PTA's? | .4-2.8% |
| What is the most common anomaly of the heart? | Bicuspid AoV |
| What other heart abnormality is commonly associated with a Bicuspid AoV? | Coarctation of the aorta, Turner Syndrome |
| What % of the population have a Bicuspid AoV? Male to female ratio? | 1-2%, 2:1 Male to female |
| What age do the symptoms usually begin with a Bicuspid AoV? | Between 20-50 years |
| What causes "blue baby" at birth? | Aortic hypoplasia |
| What else is Aortic hypoplasia associated with? | Hypoplastic Left Heart Syndrome |
| What is an under-developed AoV called | Aortic Atresia |
| In this abnormality, the L heart size is reduced due to restriction of LV In/Outflow | Hypoplastic Left Heart Syndrome |
| What size does the LV need to be to diagnose HLHS. | < 10mm |
| WHat is key for treatment of HLHS? | Early detection!! |
| OB US can see HLHS problems at what point in fetal development? | 16-20 weeks |
| What is administered at birth to maintain opening of PDA/PFO? | Prostaglandin |
| 98% of aortic coarctations are located where? | Between L Subclavian and dustus arteriosis |
| What are the 3 types of aortic coarctations? | Preductal, Juxtaductal, Postductal |
| Preductal = ? | Above the level of the DA/Ligament |
| Juxtaductal = ? | At the level of the DA/Ligament |
| Postductal = | Below the level of the DA/Ligament |
| What is the most common anomaly associated with an aortic coarctation? | Bicuspid AoV |
| What are the other 2 anomalies associated with aortic coarctation? | Transposition of the great vessels, DORV |
| What % diameter reduction is considered severe in an aortic coarc? | 40% |
| When does MV stenosis mostly begin to occur? | Childhood due to rheumatic fever |
| MV Hypoplasia = ? | Small, but normally develpoed |
| Mitral Atresia = ? | Leaflets fail to develop normally |
| Parachute MV = ? | Chordae attached to a single pap muscle |
| Tricuspi Atresia = ? | Absence of functional TV |
| Pulmonary Atresia = ? | Absence of PV |
| Imperforated TV = ? | Leaflets form but fail to seperate |
| Cor Triatriatum = ? | Subvalvular membrane in LA |
| This anomaly is when short fused chordae attach to multiple small or normal pap muscles by a fibrous tissue band. | Arcade MV |
| This anomaly is when a fibrous tissue bridge divides the MV in two. | Double orifice MV |
| What is a Cleft MV associated with? | Trisomy 21, Ostium Primum ASD, Endocardial Cushion Defects |
| What are the 3 types of PS? | Valvular, Subvalvular, Supravalvular |
| This the the most common of PS. | Valvular, stenosis of the PV cusps |
| This type PS is from RVOT stenosis. | Subvalvular |
| This type of PS is stenosis of the main PA above the PV. | Supravalvular |
| This is a rare congenital anomaly of the RV where the RV myocardium is absent or hypoplastic. | Uhl's Anomaly |
| Uhl's Anomaly AKA's | RV Dysplasia, Parchment Heart |
| Which leaflet is the most commonly affected leaflet in Ebstein's Anomaly? | Septal |
| What % of Ebstein's Anomaly are commonly associated with an ASD? | 75% |
| What is responsible for delivering oxygen/nutrients, removing waste, and oxygen/CO2 exchange? | Placenta |
| What is the oxygen saturation of the returning blood? | Approx. 58% |
| At what age is the complete fusion of the foramen ovale usually complete? | 1 year |
| At what age is total obliteration of the umbilical arteries usually complete? | 2-3 months |
| At what age does closure of the umbilical veins and ductus venosus usually ocurr? | Shortly after the closure of the umbilical arteries |
| The RV accounts for how much of the total ventricular output? | 2/3 |
| What is THE most important doppler evaluation of the fetus? | Evaluating for pulmonary artery pressures |
| To be able to evaluate pulmonary artery pressures, the patient must have one of the following circumstances. (3) | PDA, VSD, Tricuspid regurg |
| What equation is used to calculate pulmonary artery pressures? | Modified Bernouli, 4(v)² |
| What are 3 signs of increased pulmonary artery pressures? | RV hypertrophy, RV increased size, Increased PA size |
| Stage 1 begins around what day of gestation? | 22nd day |
| What are the 5 sections the cardiac tube is divided into? | Truncus Arteriosis, Bulbus Cordus, Primitive Ventricles, Atrium, Sinus Venosus |
| Truncus Arteriosis becomes the_____ and the _____. | Aorta/Pulmonary artery |
| The Bulbus Cordis is comprised of what 2 things? | Conus Cordis, Truncus Conus |
| The Conus Cordis becomes the _____. | RVOT/LVOT |
| The Truncus Conus becomes the _____. | RV |
| The Primitive Ventricle becomes the_____. | LV |
| The Sinus Venosus becomes the______. | Proximal Vena Cava...SVC/IVC |
| The Sinus Venosus also creates the_____. | Eustachian valve |
| What is the Eustachian valve responsible for? | Directing flow across the foramen ovale |
| At what day in gestation does stage 2 begin? | Around the 23rd day |
| When does Dextro/D looping happen? | Beginning of stage 2 |
| What is this process of Dextro/D Looping called? | Atrioventriculobulba Loop |
| How long does Dextro/D Looping take to finish? | 1 day |
| At what day is stage 2 complete? | 28 days |
| What also happens at the end of stage 2? | Heart begins to beat |
| What is stage 3 called? | The Septation stage |
| When does the Septation stage occur? | Between the 27th and 37th days |
| When does septation of the great vessels occur? | Week 8 |
| When are the AV and Semilunar valves formed? | Between the 5th and 7th week |
| What is normal heart placement called? Location? | Levocardia, Middle mediastinum/Apex left |
| What are the 3 abnormal heart placements called? Locations? | Dextrocardia, R chest/Apex right......Dextroposition, R chest/Apex left......Mesocardia, Midline/Apex midline |
| Which abnormal heart location(name) is more commonly associated with congenital heart defects? | Mesocardia |
| This type of cardiac situs is considered "normal positioning" where the Descending Aorta, Stomach and LA are to the left, the IVC/Liver are on the right. | Situs Solitus |
| This type of cardiac situs is considered "inverted" where the heart/apex/stomach are on the right, the Liver is on the left. | Situs Inversus |
| What are the 2 syndromes associated with Situs Ambiguous? | Asplenia/Polysplenia |
| Polysplenia/Asplenia...which one typically has more severe cardiac anomalies? | Asplenia |
| Polysplenia/Asplenia...which one is more likely to be associated with a congenital heart defect(99-100%). | Asplenia |
| Polysplenia/Asplenia...which one is L atrial isomersion/Bi-atrial L sidedness/Both atria have L atrial morphological features. | Polysplenia |
| Polysplenia/Asplenia...which one is R atrial isomersion/Bi-atrial R sidedness/Both atria have R atrial morphological features | Asplenia |
| Polysplenia/Asplenia...which one has a 50% incidence of the cardiac apex rotated to the right? | Polysplenia |
| What are the 2 types of ventricular looping? | Dextro/Levo |
| Which ventricular looping has the RV on the left? | Levo |
| Which ventricular looping has the RV on the right? | Dextro |
| What is normal great vessel relationship called. | Dextro, AoV to right of Pulmonary valve |
| What is abnormal great vessel relationship called? AoV position? | Levo, AoV to left of pulmonary valve |
| What are the normal atrioventricular/ventricular arterial connections called? | Concordant |
| What are the abnormal atrioventricular/ventricular arterial connections called? | Discordant |
| What MHz transducer do we use for scanning fetal/pediatric? | 3-7 MHz |
| What 3 things detrermine which transducer we use? | Gestational age, body habitus, amniotic fluid |
| What is the most common indication for doing a fetal echo? | Prior family history of a CHD |
| What are 3 other indications to do a fetal echo? | Known exposure to alcohol/drugs, Chromosomal abnormalities, maternal conditions |
| A chromosomal abnormality such as ________ has a 40-50% increased risk of CHD? | Trisomy 21/Downs |
| A maternal condition such as a ________ has a 12-16% increased risk of CHD? | Diabetic mother |
| In a Apical 4 view of the fetal heart, what chamber is located closest to the spine? Nearest to anterior chest wall? | LA/RV |
| What is seen in the Long axis Aorta view. | Prox. aorta, Anterior aortic wall w/IVS, posterior aortic wall w/anterior MV leaflets |
| What is seen in the Long axis/Pulmonary artery view? | Shortened RV/PV/PA |
| What is seen in the Short axis/Ventricles view? | R/L ventricles, IVS |
| What is seen in the Short axis/Great Vessel view? | R/L atrium, RV/TV, PA, Ductus arteriosis |
| What is seen in the Aortic arch view? | Ascending/Descending aorta, Aorta w/branches, R Pulmonary artery, Foreshortened LA |
| What do we see in the Ductal arch view? | Ductus arteriosis, Aorta, PA,PV, AoV, TV,LA, RVOT |
| What do we see in the IVC/SVC view? | RA/RV/TV...IVC/SVC |
| What MHx transducer for Neo-natal? Pediatric? | 7-7.5MHz, 5MHz or higher |
| What is used to determine normal velocity ranges for different ages of children? | BMI(Body Mass Index) |
| This is when both great arteries arise from the RV. | DORV(Double Outlet Right Ventricle) |
| What are the 4 types of VSD's that can ocurr from the DORV? | Subaortic, Subpulmonary, Doubly committed, Remote |
| The aorta is normally______ to the PA. | Posterior |
| Aorta transposed would have the aorta to the_____ and_____ to the PA. | Right/Anterior |
| A child with a DORV but no PS will have_____ pulmonary blood flow and suffer from_____. | Increased/CHF |
| A child with a DORV and has PS/RVOT obstruction will have_____ pulmonary blood flow and be _____. | Diminished/Cyanotic |
| What is the best view to determine placement/size of a VSD and great vessel relationship? | PSAX |
| What is the most common malformation in children born with cyanotic heart disease? | Tetrology of Fallot |
| What 4 items are associated with ToF? | Misaligned VSD, PS, RVH, Over-riding Aorta |
| Treatment for ToF is surgery, but when do we do that? | When cyanosis begins |
| What are the 2 types of transposition of the great vessels? | Complete, Congenitally corrected |
| What is the most common form of great vessel transposition? %? | Complete, 80% |
| What causes great vessel transposition to happen? | Dextro-Looping |
| If the great vessels are transposed, why is it such a big problem? | The blood can not mix, therefore, no oxygen is being delivered to the body |
| What may mask/hide the great vessel transposition abnormality during fetal development? | The baby has the PFO and Ductus Arteriosis mixing the blood |
| What must happen at birth to a baby with a known great vessel transposition abnormality? | The PFO/DA must be kept open to mix the blood |
| What drug is administered to do that? | Prostaglandin |
| What % of transpositions are Levo-Looping | 20% |
| Levo-Looping = LV where? Connects to? | Right/Pulmonary artery |
| Levo-Looping = RV is where? Connects to? | Left/Aorta |
| What are the 3 kinds of coronary artery anomalies? | Abnormal origins, Abnormal connections, Fistulas |
| Do coronary artery fistulas occur often? | Nope, negatory, nada, most definitely rare |
| When they do occur what is the percentage occurence with the RCA? LCA? Both? | 50%,42%,5% |
| What are the most common connections/drainage sites for coronary fistulas in ORDER of FREQUENCY? | RV-RA-PA-CS-LV-SVC |
| Where are most coronary fistulas discovered(test)? | Angiography |
| What are the 2 types of venous malformations? | Anomalous Pulmonary Veins, Persistent Left SVC |
| With anomalous pulmonary veins, what is happening? | They partially or totally drain into the RA instead of the LA |
| 2 ways anomalous pulmonary veins can present? | Partial, Complete |
| During development of the Sinus Venosus the left horn is obliterated. What is this known as? | Persistent L SVC |
| This type of murmur is ASx in school age children and is NOT associated with any structural heart disease. | Innocent/Functional |
| What % of children have this type of murmur at some point? | 50% |
| what are the 3 timings of murmurs? | Systolic, Diastolic, Continuous |
| Systolic murmur (Functional form), 3 kinds. | Still's murmur, TR 80%, Subclavian bruit |
| Systolic murmur (Pathological form), (5) | VSD/ASD, AS/PS, Coarctation |
| Diastolic murmur (Functional) | NONE! |
| Diastolic murmur (Pathological)(3) | AR/PR/MS |
| Continuous(Functional) | Venous hum |
| Continuous(Patological) (3) | PDA, AV malformation, Ruptured Sinus of Valsava |
| When are the most significant defects found? | Infancy |
| What are the 2 types of CHF? | Forward Failure/Backward Failure |
| Which one is more common in newborns? | Forward Failure |
| Cardiac output Forward vs. Backward | Forward Failure = High, Backward Failure = Low |
| CHF is characterized by what 3 things? | Tachycardia, Tachypnea, Poor feeding |
| What is the leading cause of heart disease in children and young adults? | Rheumatic fever |
| What valves are more commonly affected (in order of how they occur)? | AV valves, usually MV first, Semilunar valves, usually AoV second |
| This disease of the myocardium is not typically seen with any other form of heart disease. | Cardiomyopathy |
| 2 types of cardiomyopathy in pediatrics. | Hypertrophic, Dilated/Congestive |
| What group of chemo drugs are the most damaging to the heart? | Anthracyclines |
| What are 2 common types of anthracyclines? | Adriamyacin, Cerubidine |
| Systemic HTN is not common in children, but if they do have it, what is an example of another disease is it may be associated with? | Renal failure |
| What is most common to happen with Systemic HTN? | Ventricular hypertrophy |
| What is a common PHTN finding in children/young adults? | Cystic Fibrosis |
| How much fluid must be surrounding the heart for a pericardial effusion to be diagnosed? | At least 2mm of fluid |
| Is this common in fetal/pediatric? | No |
| With this disease, the echo can appear "pretty" because of the increase in fluid volume. | End stage renal disease |
| This is a reversal of the congenital shunt from L-R to R-L | Eisenmenger's Syndrome |
| This disease is acute vasculitis that occurs in children < 5 years old. | Kawasaki's Disease |
| Vasculitis occurs within the first_____ days of the disease affecting the coronary arteries. | 10 |
| What are the S&S that patients usually present with in Kawasaki's disease? | Strawberry tongue, Swelling of hands/feet, Total body rash |
| Treatment for Kawasaki's disease? | Early detection to diminish the inflammatory process before any damage can occur |
| To ease or reduce effect or intensity of a defect or disease. | Pallitation |
| Surgically connecting 2 structures | Anastomosis |
| An acqueduct/channel one constructs surgically | Conduit |
| Process of binding | Ligation |
| Opening the orifice of a stenotic valve | Valvotomy/Valvuloplasty |
| Most ASD repairs are done within the first_____ years of life. | 5 |
| What is used to close larger ASD's? | Patch of pericardium or prosthetic material |
| What procedure is used to correct D-transposition of great vessels and DORV with PS? | Damus-Kaye-Stansal procedure |
| Anastomizing the _____ to the _____ will reestablish flow in the D-K-S procedure. | Pulmonary artery/Aorta |
| What is used as a graft to reestablish pulmonary flow in the D-K-S procedure? | A graft from the subclavian artery or the thoracic aorta |
| This procedure is used to correct D-Transposition | Great Artery Switch |
| This procedure is used for tricuspid atresia and ToF. | Glenn Anastomosis |
| The "classic" procedure attaches the _____ to the _____. | SVC/Right pulmonary artery |
| This procedure is used for Tetrology of Fallot. | Blalock-Taussig Anastomosis |
| What are the two types of aortic coarctation repair? | End to End, Subclavian Flap |
| The two ends are sewn together in this aortic coarc repair for older children. | End to End |
| Subclavian artery is ligated and sewn over coarc creating a live tissue graft. Usually used in young children/Infants | Subclavian Flap Procedure |
| This procedure is used for HLHS and D transposition. | Blalock-Hanlon Atrial Septostomy |
| This procedure is used for a PTA, DORV, and a D-transposition with VSD. | Rastelli Procedure |
| This procedure is used for hypoplasia of the aortic annulus or tunnel subaortic obstruction. | Konno/Konno-Rastan Procedure |
| This procedure is used for D-transposition and DORV. | Mustard-Senning Operation |
| This is a procedure for alleviating MV stenosis. | Balloon Valvuloplasty |
| This procedure is used for the complex repair of HLHS. There are _____ stages in this procedure. | Norwood procedure/3 |
| OMG another procedure. This one is used for TV atresia, Single ventricle, and ToF. | Fontan procedure |
| The next few cards are all about what will turn into what. |
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| Truncus Arteriosis becomes the... | Pulmonary artery/Aorta |
| Bulbis Cordis divides into the ... | Conus Cordis/Truncus Conus |
| Conus Cordis becomes the ... | RVOT/LVOT |
| Truncus Conus becomes the ... | RV |
| Primitive Ventricle becomes the ... | LV |
| Atrium become the ... she will never ask this question | Really, you actually had to look at the answer? Haha, ok, ok, R/L atrium |
| Sinus Venosus becomes the ... | Proximal Vena Cava (SVC/IVC) |
| AV Canal becomes the ... | R/L ventricular cavity |
| Cardiac septa develop forming the ... | 4 chamber heart |
| Septum Primum divides the ... | Atrium |
| Two large endocardial cushions fuse resulting in ... | TV/MV orifices |
| Cushions grow and twist around each other forming ... | Aorticopulmonary Septum |
| Septum divides Truncus Arteriosis into... | Aortic and Pulmonary channels |
| 6 aortic arches develop from the ... | Truncus Arteriosis |
| Cushions of conus cordis unite with aorticopulmonary septum and create the... | RVOT/LVOT |
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