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262 Cards in this Set
- Front
- Back
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Angiogenesis |
Formation and differentiation of blood vessels. |
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Atrioventricular (AV) Canal |
The canal joining the atria and the ventricles in the primitive heart tube. |
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AV Concordance |
Occurs when morphologic atria are connected to morphologic ventricles (RA attached to RV, LA attached to LV). |
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AV Discordance |
Occurs when morphologic RA is connected to morphologic LV, morphologic LA is connected to morphologic RV. |
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Bulboventricular Loop |
(aka: Cardiac Loop) Very early "heart" Develops when heart tube rotates 180 degrees and folds over. |
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Bulbus Cordis |
Portion of the primitive heart tube that divides into three sections: the primitive RV, ventricular outflow tracts, and truncus arteriosus. |
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Caudad |
Toward the tail or posterior end. |
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Cephalad |
Toward the head or anterior part of the body. |
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Conotruncal (Truncoconal) Region |
Area when great vessels and outflow tracts meet. |
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Conus Cordis |
Ventricular outflow tracts. |
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D-Loop |
Dextro loop (dextro = on or to the right) Heart tube loops anteriorly and to the right Normal looping |
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Ductus Arteriosus |
A vessel in the fetal heart that allows blood to bypass the lungs. Blood is shunted from the pulmonary artery to the descending aorta. Normally closes a few days after birth. |
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Patent Ductus Arteriosus |
When the ductus arteriosus remains open after birth. |
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Ductus Venosus |
A vein passing through the liver that connects the umbilical vein with the IVC in the fetal heart. |
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Endocardial Cushions |
Endocardial tissue that develops into AV orifices and helps close the ostium primum portion of the IAS and the membranous portion of the IVS. |
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Endothelium |
Single layer of thin, flattened cells that lines internal body cavities (the heart). |
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Eustachian Valve |
A "valve" at the entrance of the IVC to the RA in the fetal heart that helps direct blood across the foramen oval. Remnant of eustachian valve can sometimes be seen in the adult heart, but serves no purpose. |
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Foramen Ovale |
An opening in the atrial septum in the fetal heart. Normally closes shortly after birth. |
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Patent Foramen Ovale |
A foramen ovale that does not close after birth. |
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Fossa Ovalis |
Area in mid atrial septum where the foramen oval was in the fetal heart. |
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Ligamentum Arteriosum |
After ductus arteriosus (between MPA and AO) closes, it becomes the ligamentum arteriosum in the postnatal heart. |
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Ligamentum Venosum |
After the ductus venosus (between umbilical vein and IVC) closes it becomes the ligamentum venosum. |
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L-Loop |
Heart tube loops anteriorly and to the left. Heart defects will occur. Abnormal looping. |
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Membranous IVS |
Thinner, more superior portion of IVS. Formed from endocardial cushion. |
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Mesenchymal Cells |
Loosely organized cells that give rise to connective tissue, blood, lymphatics, bone, and cartilage. |
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Morphologic |
Pertaining to form or structure. |
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Muscular IVS |
Thicker, muscular, more inferior portion of IVS. Formed as muscular floor grows toward the cushion. |
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Placenta |
Vascular organ that attaches the embryo to the uterine wall and exchanges nutrients, gases, and wastes between the maternal blood and the embryonic blood. |
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Septum Primum |
Most superior portion of the IAS. |
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Septum Secundum |
Mid portion of the IAS. |
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Sinus Venosus |
Large pouch at caudal end of primitive heart tube. Develops into coronary sinus, IVC, SVC. |
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Truncus Arteriosus |
Part of primitive heart tube that develops into aorta and pulmonary artery. |
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Umbilical Artery |
Artery in the umbilical cord that carries deoxygenated blood from the fetus to the placenta. |
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Umbilical Vein |
Passes oxygenated blood to the fetus from the placenta, passes through the umbilical cord. |
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What is the first fully functional system of the fetus? |
Cardiovascular system |
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When is a fully functioning four chamber heart formed? |
Week 7 (or 8) |
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Week 3 |
Paired heart tubes fuse to form the primordial heart tube. Primitive CV system develops, heart begins to beat, blood flow is unidirectional (caudad-cephalad) |
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Weeks 3-4 |
Heart tube bends upon itself due to an increase in blood volume, static size of the pericardium, and elongation of the heart tube. Forms an S-shaped heart. |
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Weeks 4-5 |
Heart beat heard with Doppler. Cardiac loop is formed (either D-Loop or L-Loop). Heart tube rotates 180 degrees. Heart tube has 7 regions. |
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7 Regions of Heart Tube
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- Sinus Venosus - Primitive Atria - Atrioventricular Canal (AV Canal) - Primitive Ventricle - Bulbus Cordis - Truncus Arteriosus - Aortic Sac and Aortic Arches |
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How many aortic arches are there? Which forms the aortic arch, common and internal carotids, pulmonary arteries, and ductus arteriosus? |
Six arches. 4th arch forms Aortic Arch. 3rd arch forms common and internal carotids. 6th arch forms pulmonary arteries and ductus arteriosus. |
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Weeks 5-6 |
Septation: development of endocardial cushions, AV canal, and truncoconal region. |
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Atrial Septal Formation |
- Completed weeks 5-6 - Divides atrium into right and left halves formation consists of septum primum and septum secundum. |
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Interventricular Septum Formation |
- Formed by swelling and moving together of endocardial cushion - Muscular floor grows towards cushion - Conus cordis swells and helps form mid portion of septum - Completed between 55-65 days |
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Cardiac Valve Formation |
- AV valves form from the AV canal partition - Valves formed from connective tissue - Chordae formed from endocardial tissue - Semilunar valves form from the truncus arteriosus - LVOT and RVOT are formed when truncus separates |
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Fetal Circulation |
- Blood flow is provided from the placenta - Increased pulmonary resistance because lungs are filled with fluid and are not inflated - Right heart pressures are greater than left heart - Blood bypasses the lungs by shunting through the foramen oval and ductus arteriosus. |
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Neonatal Circulation |
- Baby takes first breathe: decreases vascular resistance and increases pulmonary blood flow - Increased pulmonary blood flow increases LA pressure and LA pressure > RA pressure which closes the foramen ovale (becomes fossa ovals). - Systemic pressure > MPA pressure, which closes the ductus arteriosus (becomes ligamentum arteriosum). |
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3 Types of Conotruncal Abnormalities:
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Tetralogy of Fallot
Truncus Arteriosus Transposition of the Great Arteries –– D–transposition, “Simple” TGA (TGA) –– L–transposition, Congenitally CorrectedTGA (CCTGA) |
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Tetralogy of Fallot
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– Most common cyanotic congenital heart lesion
– 4 defects in TOF: VSD, overriding aorta, pulmonary stenosis, RVH |
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Cyanosis
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Bluish skin coloration due to decreased blood oxygen concentration.
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VSD and Tetralogy of Fallot
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– Typically sub aortic/malalignment
– In 1.5% of patients, the VSD is part of a complete atrioventricular septal defect – May be only infundibular, but more commonly extends into the membranous septum |
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Overriding Aorta and Tetralogy of Fallot
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– Occurs secondary to the sub aortic location of the VSD
– A consequence of maldevelopment of the conotruncus |
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Pulmonary Stenosis and Tetralogy of Fallot
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– Key factor in determining the type and amount of dysfunction that a patient with Tetralogy of Fallot displays
– Usually occurs in the right ventricular infundibulum or subvalvular area – Pulmonary valves may be tricuspid, bicuspid, unicuspid, or dome–shaped – Normally the diameter of the pulmonary orifice is greater than 80% of the diameter or the aortic orifice – Hypoplasia of the pulmonary trunk and its branches usually occurs – Pulmonary atresia is the most severe form of pulmonary outflow tract obstruction |
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Right Ventricular Hypertrophy and Tetralogy of Fallot
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– Because of the pulmonary outflow tract obstruction, the RV can demonstrate pressure induced hypertrophy.
– LV is of normal size and caliber – Usually happens sometime after birth when the PDA closes and pressure increases in the RV |
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____% of patients with Tetralogy of Fallot also have associated cardiac anomalies.
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40%
– Right sided aortic arch is the most common – ASD/PSD common – Absence of pulmonary valve and/or pulmonary artery – Pulmonary atresia – Atrioventricular septal defects – Persistent left SVC – Partial and total anomalous pulmonary vein return. |
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Cyanotic Defect (Tetralogy of Fallot)
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Cyanotic:
– With severe PS, VSD shunt is primarily R–L – R–L shunt causes low O2, decreased pulmary flow, and decreased PA size – Clubbing of the fingers and toes |
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Acyanotic Defect (Tetralogy of Fallot)
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Acyanotic:
– With mild PS, VSD shunt in primarily L–R – Symptoms depend on severity of PS (exercise intolerance, "Tet spells"– episodes of faintness with cyanosis) |
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Tetralogy of Fallot Surgical Goal
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To increase blood flow to the lungs.
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Surgeries for Tetralogy of Fallot
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Blalock–Taussig shunt: shunts blood from rt. subclavian artery to pulmonary artery
Waterston shunt: shunts blood from ascending Ao to right pulmonary artery Potts shunt: Descending Ao to left pulmonary artery |
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Complete Surgical Repair of Tetralogy of Fallot
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– Pulmonary stenosis site corrected (remove tissue and repair valve)
– VSD is repaired – Patch/graft is applied to PA to enlarge it |
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Tetralogy of Fallot Prognosis
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– Dependent on the severity of the pulmonary stenosis
– Survival rates of 85% at 5–11 yrs follow up after surgery – When there is Tetralogy of Fallot with pulmonary atresia the prognosis is not as positive (may cause CHF in fetus or infant, sudden death can occur during infancy) – Without surgery, life expectancy is around 12 yrs of age |
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Truncus Arteriosus
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– Caused by failure of the conotruncal ridges to fuse
– Normally, they divide into separate aortic and pulmonary arterial trunks – In Truncus Arteriosus the result is a single great vessel arising from the heart that overrides the ventricular septum |
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Anatomy of Truncus Arteriosus
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One great vessel:
– Supplies blood to systemic, coronary, and pulmonary circulations – Receives blood from both the RV and LV – Pulmonary trunk usually rises from the truncus VSD: – The semilunar valve or truncle valve lies directly above the VSD in 42% of patients (42% over RV, 16% over LV) |
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Truncal Valve in Truncus Arteriosus
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– Truncal valvehas 3 cusps in 60% of patients, 2 cusps in 5% of patients, and 4 – 6 cusps in 25% of patients
– Truncal valve is insufficient in 10 – 15% ofpatients – Truncal valve is rarely stenotic |
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Types of Truncus Arteriosus
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* Type is based on the location of where the pulmonary arteries originate
Type 1: Single pulmonary trunk and ascending aorta arise from TA Type 2: Right or left pulmonary arteries arise close together from dorsal wall of TA Type 3: One or both pulmonary arteries arise independently from either side of TA Type 4: Pulmonary atresia, VSD |
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Truncus Arteriosus: Type 1
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– Most common type
– 48–68% of cases – Single pulmonary trunk and ascending aorta arise from truncus – LPA and RPA arise normally |
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Truncus Arteriosus: Type 2
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– 29–48% of cases
– Right or left pulmonary arteries arise close together from dorsal wall of truncus – Absent pulmonary trunk |
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Truncus Arteriosus: Type 3
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– Least common
– 6–10% of cases – One or both pulmonary arteries arise independently from either side of truncus – Absent pulmonary trunk |
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Truncus Arteriosus: Type 4
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– Pulmonary atresia, VSD
– Lungs are supplied blood flow from smaller arteries arising from ascending or descending aorta |
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Associated Anomalies in Truncus Arteriosus
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– VSD is always present
– Truncal valve (usually 3 leaflets, sometimes 1–6) – Right aortic arch – Interrupted aortic arch – Absent pulmonary artery – Coronary artery anomalies – Branch pulmonary stenosis – ASD¢Persistent L–SVC to coronary sinus |
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Clinical Presentation of Truncus Arteriosus
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– Presents in infancy with mild cyanosis and CHF
– Survival beyond infancy is uncommon – Without surgical correction, median age of death is 1–3 months, 80% mortality by 1 year |
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Surgical Treatment for Truncus Arteriosus
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Rastelli Procedure:
– Pulmonary artery is excised from trunk and the resulting defect is closed – VSD closed with Dacron patch – Valved conduits are used to reconstruct RVOT |
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Transposition of the Great Arteries
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2 Main Types:
1. D–Transposition– dextro–transposition; complete transposition 2. L–Transposition– levi–transposition; congenitally corrected transposition – In both cases, the pulmonary artery is connected to the LV and the aorta is connected to the RV |
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Complete Transposition (D–Transposition)
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– The connections between the atria and theventricles are normal or concordant (AV Concordance)
– The aorta arises from the RV and thepulmonary artery arises from the LV (VA Discordance) – Defect is associated by abnormal division of the truncus arteriosus – More common in males |
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___% of Transposition of the Great Arteries cases are D–Transposition.
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80%
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D–Transposition Clinical Findings
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– Cyanosis
– Tachypnea – Distressed baby – Systolic murmur |
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Treatment for TGA (D–transposition)
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– 1st priority is to stabilize patient (maintain or create shunt flow)
– Prostaglandins to keep ductus open – Balloon septostomy (creates ASD or makes one larger) |
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Treatment for TGA (D–transposition): Senning and Mustard Procedure (Atrial switch)
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– Rarely performed today
– Reroutes atrial blood flow so thatde–oxygenated blood passes to the LV to the lungs and oxygenated blood passesto the RV (systemic ventricle) to the body Complications: – RV dysfunction can occur – Accounts for 20% of the late deathsTV repair or replacement is rarelysuccessful in the long term |
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Treatment for TGA (D–transposition): Jatene
Procedure |
– Surgicallycorrects the arterial–ventricular attachments so that a neoaorta and neopulmonary artery are created to provide outflow to the left and right ventricles
– The coronary arteries are also reimplanted into the neoaorta – This could cause long–term complications relating to coronary artery disease |
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Associated Anomalies in TGA
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– VSDin 20% of patients
– Leftventricular subpulmonic outflow tract obstruction in 5–40% ofpatients |
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Congenitally Corrected TGA
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– Both atrioventricular (AV) andventriculoarterial (VA) have discordant connections
– The morphologic LV is on the right andconnects to the pulmonary artery; the morphologic RV is on the left and is nowthe systemic ventricle and connects with the aorta – Defect is associated with abnormal looping of the bulboventricular heart tube – Rare, and more common in males |
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CCTGA Treatment and Prognosis
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– In absence of other anomalies, no correction is required
– Only need surgical intervention for associated cardiac lesions (VSD, pulmonary stenosis) – Prognosis depends on the nature of associated lesions (systemic heart failure, TR, complete heart block) |
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Truncus Arteriosus
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D–Transposition of the Great Vessels
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Tetralogy of Fallot
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Four types of CHD Anomalies
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1– Malformation
2– Disruption 3– Deformation 4– Dysplasia |
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Malformation
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A morphologic defect of an organ that results from an intrinsically abnormal development process.
– Genetic or chromosome abnormalities (Down Syndrome, Turner Syndrome) – Can be inherited (HCM, Non–Compaction) |
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Deformation
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An abnormal form, shape, or position of a part of the body that results from mechanical forces.
– Intrauterine compression from low amount of amniotic fluid |
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Disruption
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A morphologic defect of an organ that results from the extrinsic breakdown or interference with an originally normal developmental process.
– Morphologic alterations due to exposure to drugs or viruses – Cannot be inherited |
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Dysplasia
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An abnormal organization of cells into tissues and its morphologic results (abnormal tissue formation)
– Marfan Syndrome – Ehlers Danlos |
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Causes of Congenital Abnormalities (%'s)
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– Genetic Factors: 8% (chromosomal abnormalities)
– Environmental Factors: 2% (drugs, viruses, infections, maternal nutrition, radiation) – Multifactorial Inheritance: 90% (genetic and environmental factors acting together) |
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What are the most common defects?
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– Ventricular Septal Defects
– Atrial Septal Defects |
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What are the rarest defects?
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– Unicuspid Aortic Valve
– Absent Tricuspid Valve |
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Pediatric Echo
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– Conscious sedation is used in patients 2–24 months
– Good image quality: high frequency transducer, less calcified bone, no hyperinflated lungs – May be difficult due to: uncooperative patient, chest deformities/other congenital defects |
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CHD in the Adult Echo Patient
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Undiagnosed/untreated defects:
– May have other problems like HTN, CAD, etc. – Incidental finding – Doctor must decide whether they should be treated Treated Defects: – Type of correction is not always clear – Difficult to image due to previous surgery |
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Segmental Approach
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– Step by step approach used to divide the patient's cardiovascular system into individual segments and the connections between those segments
– Established the relationship to the heart chambers and cardia axis to the arrangement of other intrathoracic and abdominal organs – Evaluates venoatrial, atrioventricular, and ventriculoarterial connections |
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Why is the subcostal view often the first window used in the segmental approach?
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It determines situs, which how the heart is positioned in the body.
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Axis
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Describes the rotational orientation of the heart.
The degree that the apex points to the right or the left. Dextro/Levo/Meso–cardia |
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Position
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Describes the translational relationship or overall location of the heart in the chest.
Dextro/Levo/Meso–postion |
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Abnormal Axis
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Is a manifestation of intrinsic congenital heart disease, and is usually associated with complex intracardiac defects.
Cannot change. |
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Abnormal Position
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Result of extracardiac defect.
Can change, because it is due to displacement and abnormal position is often due to other organ malformation (ex. huge liver) |
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Situs
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Spatial position of a structure.
– Situs Solitus – Situs Inversus – Situs Ambiguous |
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Situs Solitus
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– Normal organ arrangment
– Liver on the right side, stomach on the left – Heart in levoposition – Situs Solitus with levocardia is the normal body configuration |
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Situs Solitus with Dextrocardia
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– Also referred to as dextroversion or isolated dextrocardia
– Associated with CHD very common: 95% of cases – Two types: one has normal AV and VA relationships and the other has both AV and VA discordance |
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Situs Inversus
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– Rare
– Mirror image – Stomach on right side, liver on left side |
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Situs Inversus with Dextrocardia
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– RA and RV are on the left side
– LA and LV are on the right side – Physiologic AV and VA connections |
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Situs Ambiguous
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Classified as Heterotaxy Syndrome (hetero: different, taxy: arrangement)
Two distinct syndromes associated: – asplenia – polyspenia |
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Asplenia
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– Both atria have right atrial morphologic features
– Liver is in the midline position – Spleen is absent – Stomach can be in mid–epigastrium or on either side of the abdomen – Associated with CHD in 99–100% of cases |
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Polysplenia
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– Both atria have left atrial features
– Abdominal organs tend to be less symmetric than asplenia – Liver is in the midline in 1/2 of patients – Several spleens – Stomach either midline or to the left – Associated with CHD in 90–95% of cases |
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Distinguishing RA from LA
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RA: Eustachian valve, shorter and broader appendage, can follow IVC to RA
LA: Rounder shape, appendage longer and thinner, can't rely on pulmonary veins emptying into LA |
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Distinguishing RV from LV
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RV: trabeculated, 3 papillary muscles, chordae with septal insertion, infundibular muscle band, moderator band, triangular shape, TV with relative apical insertion
LV: smooth endocardial surface, 2 papillary muscles, ellipsoid shape, MV with relative basal insertion |
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Distinguishing PA from Ao
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PA: Bifurcates into left and right branches, anterior and slightly medial to aortic root, PV slightly more superior in chest than AV
Ao: Follow superiorly to a left sided arch and visualize the innominate artery, left common carotid, and left subclavian |
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Transposition of the Great Vessels
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Concordant: PA arises from morphologic RV, Ao arises from morphologic LV, AV and PV lie in planes perpendicular to each other
Discordant: PA arises from morphologic LV, Ao arises from morphologic RV, AV and PV lie in same plane, Ao and PA will be parallel |
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D–Transposition of the Great Vessels
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– Ventricular relationship is normal
– Morphologic RV is located to the right of morphologic LV – Aorta and PA are switched |
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L–Transposition of the Great Vessels
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– AV discordance is present
– Ventricles are switched (RV lies to the left of LV) – LA connects to RV, RA connects to LV |
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Residua
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Anatomic abnormalities that are cardiac, vascular, or non cardiac that remain after surgery.
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Sequelae
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Physiologic alterations or disorders that remain after surgery are are looked upon as long–term consequences of an operation.
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In CHD, how do they refer to the mitral and tricuspid valves?
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Instead of mitral and tricuspid, they say Right AVV (right atrioventricular valve) and Left AVV (left atrioventricular valve) because they are not always true triscupid and mitral valves.
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Atrial Septal Defect
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Hole in the atrial septum of varying size producing a left to right shunt, resulting in RA and RV dilation.
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Three Locations of ASD's:
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– Secundum
– Primum – Sinus Venosus |
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Secundum Defect
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– Located in the mid–portion of the atrial septum (region of fossa ovalis).
– Most common type of ASD – Usually an isolated defect but can be found with other anomalies – Small ostium secundum defect may be difficult to differentiate from a PFO, especially in infants |
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Primum Defect
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– Caused by failure of endocardial cushions to merge
– Located in the lower atrial septum – Can be associated with other endocardial cushion defects – Associated with abnormalities of the MV, TV, and inlet portion of the IVS |
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Sinus Venosus
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– Least common type of ASD
– Occurs near the junction of the SVC and RA, high in the atrial septum – Frequently associated with partial anomalous pulmonary vein return (where some of the pulmonary veins drain into a structure other than the LA) |
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Coronary sinus ASD
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– Rare
– Roof of the coronary sinus is partially or completely absent creating a left–to–right shunt from the LA to the coronary sinus and then into the RA – Associated with persistent left SVC which may connect to the coronary sinus or LA |
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Common Atrium
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– Rare
– Characterized by the absence or virtual absence of the interatrial septum – Associated with Ellis–van Creveld syndrome (genetic disorder found in Amish Multiple abnormalities and dwarfism) |
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Physiologic Effects of ASD's
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– Shunts are usually left to right causing RVVO, RAE, RVE, paradoxical septal motion
– Some shunts are bi–directional – Valsalva maneuver may induce right to left shunt – Severe PHTN may develop over time – Right sided pressures > left sided pressures – Shunt will reverse, and become right to left (Eisenmenger’s Syndrome) |
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Eisenmenger's Syndrome
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Congenital shunt flow left–to–right that changes right–to–left due to volume overload. It is secondary to irreversible severe PHTN.
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Clinical Findings of ASD's
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– Dyspnea
– Fatigue – Decrease in exercise tolerance – Palpitations – Arrhythmias |
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Complications of ASD's
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– CHF
– PHTN – Eisenmenger’s syndrome – Atrial arrhythmias – CVA (paradoxical embolization) – Infective endocarditis (rare) |
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Echo Findings of ASD's
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– Subcostal view is best, but use any window where you will be parallel to shunt flow
– Trans–septal shunt flow (L – R) – Atrial septal “dropout” – Increased size of RV, RA with abnormal septal motion |
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2D and M–Mode ASD's
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– RAE, RVE
–Paradoxical septal motion – Large shunt : Qp:Qs > 2 : 1, Location with 2D, usually > 3.0 mm |
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CF and Doppler ASD's
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– Direction of shunt
– Velocity of shunt w/ PW – Turbulence seen in receiving chamber – Late systolic to early diastolic flow disturbance |
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Qp/Qs
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– Pulmonary to systemic flow ratio
– Normally, pulmonary and systemic flows should be equal – More flow goes through PV with ASD (or VSD) shunt .785 x RVOT d^2 x RVOT TVI / .785 xLVOT d^2 xLVOT TVI – If Qp/Qs ratio > 1.5 : 1, may consider closure – For PDA, divide LVOT by RVOT (switched) |
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Bubble Study for ASD's
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– 5–10 mL of agitated saline injected into left arm vein or leg vein
– Theapical and subcostal views are prefer – Negative study = no bubbles visualized in left heart – Positive study = bubbles visualized in left heart – If they appear in left heart after 5–10 beats, pt. may be a pulmonary shunt (pulmonary artery to pulmonary vein) *** If they appear in coronary sinus before RA, pt. may have a persistent left SVC *** |
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Treatment of ASD's
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– Surgically with a suture or patch
– Amplatz device – If ASD is found in infancy, surgery is deferred until 4 or 5 years of age if doing well |
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Ventricular Septal Defect
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– Hole of varying size in the interventricular septum allowing left–to–right shunting of oxygenated blood between left & right ventricle
– Blood flows from high to low pressure chamber VSDs are the most common congenital abnormality of the heart –As an increased volume of blood returns from lungs to the left heart, left atrial and ventricular dilatation may result – Pulmonary arteries may dilate as well – May close spontaneously, depending on size |
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Types of VSD's:
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– Membranous, perimembranous, infracristal
– Muscular or trabecular – Inlet, atrioventricular – Outlet, supracristal, infundibular |
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Membranous VSD's
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– Most common type,75–80% of all VSDs
– Located in the LVOT inferior to the Ao and TV (beneath aortic valve) – Perimembranous includes portions of membranous and muscular septum, so extends inferior to true membranous septum – May have associated TV abnormalities – Associated with AR – True membranous VSD most common type in adults – May close spontaneously by aneurysmal formation via the TV septal leaflet |
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Echo Views: Membranous VSD
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– PLAX– may require medial tilt
– PSAX at AV level (9–12 o’clock position) – Apical 5 chamber – Subcostal 5 chamber |
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Muscular VSD
|
– 2nd most common in adults
– Occurs anywhere in the muscular IVS – Usually near the apex – Frequently multiple lesions (swiss cheese effect) – May close spontaneously in childhood – 5–20% of all VSDs (DeWitt) |
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Echo Views: Muscular
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– PLAX
– PSAX of the LV at the level of the papillary muscles – PSAX at the LV at the cardiac apex – Apical 4 chamber – Apical5 – Subcostal 4 chamber, 5 chamber and SAX views |
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Inlet VSD's
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– Located posterior and inferior between the AV valves
– Beneath the septal cusp of the TV – Associated with endocardial cushion defects (atrioventricular septal defects) – 3–5% of all VSDs |
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Echo Views: Inlet VSD's
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– PSAX of the MV
– PSAX of the LV at the level of the papillary muscles – Apical 4 chamber (posterior tilt) – Subcostal 4 chamber (posterior tilt) – Subcostal SAX of the MV and LV – Evaluate for partial or complete atrioventricular septal defect (AV Canal) |
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Outlet VSD's
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– Located in the RVOT inferior to the PV (beneath the PV)
– Associated with AR – Uncommon in Caucasians unless associated with Tetrology of Fallot – Most common in Asian population – ~5% of all VSDs |
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Echo Views: Outlet VSD's
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– PLAX with clockwise rotation of transducer with superior tilt
– PSAX of the AV (12:00 to 3:00 position) – Subcostal 5 chamber with superior tilt – Subcostal SAX view of the AV |
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Echo Assessment of VSD's
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– Use multiple views (Apical 4 chamber and subcostal views are good)
– PSAX– sweep the transducer from the apex of the heart to the base of the heart with color Doppler on – VSD's less than diameter of aortic root is considered small to medium; VSD greater than this diameter is considered medium to large – A low peak systolic velocity across VSD may represent poor Doppler beam angulation, systemic hypotension, increased RV systolic/systolic PAP, or alarge defect |
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Physiologic Effects of VSD's
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– Initially shunt is from LV – RV
– PHTN may develop with significant left to right shunt over time due to the increase in pulmonary pressure, and as the PA pressure rises, RV pressure may equal or exceed LV pressure (shunt may become bi–directional) – Eventually Eisenmenger’s Syndrome may develop when pulmonary pressure > systemic pressure (shunt reverses and becomes right to left) – Irreversible PHTN – Patient cyanotic |
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Color Flow and Doppler: VSD's
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– Color flow excellent for demonstrating a high velocity turbulent (mosaic) systolic L–R shunt flow
– Evaluate for MR, AR, TR, and RVOT obstruction –A low peak systolic velocity across VSD may represent poor Doppler beam angulation, systemic hypotension, increased RV systolic/systolic PAP, or a large defect – Determine Qp/Qs – Can estimate RVSP from VSD velocity |
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2D and M–Mode: VSD's
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– Large shunts LAE, LVE
– Qp:Qs > 2 : 1, 2D > 3.0 mm – Aneurysm formation from spontaneous closure – Systolic bulging toward right – Assess correct position of Aorta within LVOT |
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VSDs are the most common congenital heart defect found in children but representsonly ___% of cases of CHD in the adult.
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10%
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Patent Ductus Arteriosus
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– Persistent patency of the fetal vessel that connects the pulmonary artery and the descending aorta
– With normal pressures, the shunt will flow from L–R; from aorta to PA – Most common extra cardiac shunt – Prostaglandin E may be administered to the infant to keep the ductus arteriosus open to provide flow to the lungs or body sometimes – Continuous flow –– continuous murmur |
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Echo Views for PDA
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– PSAX
– High left PSAX: ductal/coarct view – SSN |
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PDA Treatment
|
– Surgical closure/ligation– Coil closure – in cath lab
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Blood flow across a defect is dependent upon:
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– Size of orifice
– Pressure gradient across the defect – Resistance to flow of vascular beds |
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Velocity of blood depends on:
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The pressure difference between chambers & size of orifice
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Volume Overload and Shunts
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ASD
– left to right – Dilated RA, RV – Paradoxical septal motion PDA (Ao to PA) – Dilated LA, LV – Left to Right VSD Shunt – RV size usually normal due to blood flowing directly into MPA – Dilated LA, LV |
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Sinus Venosus ASD
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Outlet VSD
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Ostium Secundum ASD
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Ostium Primum ASD
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Muscular VSD
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Membranous VSD
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Inlet VSD
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What creates a partial AV canal?
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Primum ASD + cleft mitral valve
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Which side does saline get injected into?
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Left arm or leg vein
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Which defect is often associated with Epstein's anomaly?
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Membranous/perimembranous
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All cyanotic diseases can cause what?
|
Clubbing of the fingers
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How to calculate RSVP from VSD velocity?
|
RVSP = BPsys – 4 (vel VSD)^2
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ASD's affect the _________ side?
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Right
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VSD's affect the __________ side?
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Left
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PDA's affect the _________ side?
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Left
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Vascular Aortic Ring
|
– A ring formed around the trachea and esophagus due to abnormal persistence of aortic arches.
3 Types: – Double aortic arch – Right aortic arch – Interrupted aortic arch |
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Double Aortic Arch
|
– Left aortic arch develops normally
– Abnormal persistence of right aortic arch – Most common vascular ring |
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Symptoms of Double Aortic Arch
|
– Dysphasia: difficulty swallowing
– Stridor: high pitched sound resulting from turbulent gas flow in the upper airway |
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Surgical Repair for Double Aortic Arch
|
Separate off smaller arch (normally left) to relive pressure.
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Right Aortic Arch
|
– Second most common vascular ring
– Aorta is to the right of the spine – Asymptomatic if simple right aortic arch – 25% associated with Tetrology of Fallot, D–Transposition, Truncus Arteriosus – May result in interrupted arch |
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Anatomy of Right Aortic Arch
|
– Aorta arches to the right, and lies on the right of the spine
– Anomalous left subclavian artery lies behind the esophagus – Ductus arteriosus connects the subclavian artery the pulmonary artery (instead of Ao to PA) |
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Interrupted Aortic Arch
|
– Most severe vascular aortic ring defect
– Arch is not connected (normally between the left subclavian and left common carotid) – Blood shunted to lower part of body from the PA through the PDA to the aorta – VSD allows blood to PA to be mixed – Surgical correction is needed w/in 1st few days of life (connect arch with desc. aorta, close VSD, close PDA) |
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Pulmonary Atresia
|
– Rare conotruncal defect after associated with Tetralogy of Fallot
– Also called hypoplastic right heart – PV has not formed and blood is not able to go from RV to PA – Pulmonary blood flow provided by PDA |
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RV/RA in Pulmonary Atresia
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RV: high pressure, thick, small chamber
RA: usually dilated |
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Treatment for Pulmonary Atresia
|
– Prostaglandins keep ductus arteriosus open
– Surgical corrections: – Temporary/pallative– Blalock–Taussig Shunt (if RV is is severely hypo plastic) |
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Coarctation of the Aorta
|
– Narrowing of the aorta
– 98% of the time narrowing is between the origin of the left subclavian artery and the ductus arteriosus – Most commonly associated with bicuspid AV – Presents in newborns or in older children/young adults |
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Names of the 3 Locations of Coarctation of Aorta
|
Preductal: before ductus
Juxtaductal: at ductus Post ductal: distal to the ductus |
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Coarct in the Newborn
|
– Usually associated with other congenital abnormalities
– Narrowing can be severe and life threatening – Blood flow maintained through right–left ductal shunt – Ductal dependent: needs ductus arteriosus to be open in order to get blood flow – When PDA closes, symptoms of cardiogenic shock develop as systemic blood flow becomes compromised when the PDA begins to close – Needs quick echo diagnosis as it is a medical emergency |
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Coarct in the Older Child/Young Adult
|
– Milder form of coarctation
– Not ductal dependent – Usually have upper extremity hypertension and diminished femoral pulses |
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Turner Syndrome
|
– Genetic condition characterized by webbing of neck and short stature
– Often have coarctation of the aorta |
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Symptoms of Coarctation of the Aorta
|
– Elevated blood pressure in arteries proximal to the coarct
– Decreased blood pressure below the coarct – Asymptomatic at times – Congestive heart failure – Poor feeding – Dyspnea |
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Echo Findings of Coarctation of the Aorta: 2D
|
– SSN long axis: best for viewing narrowing
– PLAX: dilation of ascending Ao – Subcostal: Lack of pulsation in abdominal Ao – Assess for bicuspid AV |
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Echo Findings of Coarctation of the Aorta: Doppler
|
– Color flow: turbulence in region of narrowing
– PW SSN: obtain velocity proximal to narrowing – CW SSN: obtain peak velocity distal to narrowing, "saw tooth" pattern, calculate/trace gradient |
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What does the abdominal aorta doppler signal look like when there is coarctation of the aorta?
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Damped systolic flow with a variable degree of diastolic flow (flow during diastole as well)
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Treatment of Coarctation of the Aorta
|
– Balloon angioplasty + stent placement
– Surgical removal of narrowed segment (treatment of choice): Sew together ends of the aorta, or place dacron graft, or use left subclavian artery to repair it (using left subclavian is called the Waldhausen repair) – Repair/replace AV is affected |
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Persistent Left SVC
|
– Left anterior cardinal vein abnormally persists after fetal life, creating a left SVC
– More commonly associated with endocardial cushion defect – Usually connects to coronary sinus, causing coronary sinus to dilate – Will usually have a right and left SVC – Can determine whether it exists by using agitated saline: injected in left arm––if contrast appears in coronary sinus before the right atrium there is a left SVC (use PLAX and apical views) |
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Coronary Fistula
|
Fistula: an abnormal connection between two hollow organs of the body
Coronary Fistula: Coronary artery enters directly into a cardiac chamber or major thoracic vessel, bypassing coronary venous system and the coronary sinus (RCA, LCA, or both can be involved and can drain into RV, MPA, RA, coronary sinus, LA, LV, SVC) |
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Symptoms of Coronary Fistula
|
– Asymptomatic in children
– May present with a continuous murmur – Angina – Fatigue |
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Complications and Treatment of Coronary Fistula
|
Complications:
– CHF – Rupture of fistula – MI Treatment: – Closure of fistula either surgically or with occluding device in the cath lab |
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Partial Anomalous Pulmonary Venous Return
|
– Rare
– One or both pulmonary veins empty into the right heart rather than the LA – May empty into RA, IVC, or SVC – Often associated with a sinus venosus ASD |
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Symptoms of Partial Anomalous Pulmonary Venous Return
|
– Essentially asymptomatic in children, but may present with fatigue with exertion
– May be more pronounced in adulthood due to oxygenated blood being dumped back into right side of the heart rather than circulated to the body – Extra volume may result in RAE, RVA, and pulmonary artery dilation |
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When is surgical repair needed for Partial Anomalous Pulmonary Venous Return?
|
– If more than 50% of pulmonary venous flow empties into the right heart surgery is necessary.
– Shunt or patch made of pericardium carries blood from anomalous PV through the RA to the LA |
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Total Anomalous Pulmonary Venous Return
|
– Also known as Total Anomalous Pulmonary Venous Connection (TAPVC)
– All four pulmonary veins empty into right side of heart or it's vessels – Dilated RA and RV – Must have ASD for blood to circulate to the body––may need to be surgically created – Mostly asymptomatic if no obstruction to PV flow |
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Four Types of TAPVC
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Type 1: Supracardiac Type
Type 2: Cardiac Type Type 3: Infracardiac Type Type 4: Mixed Type |
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Type 1: Supracardiac Type
|
– Most common TAPVR
– Pulmonary veins connected by vessel to SVC – Usually no obstruction of blood flow |
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Type 2: Cardiac Type
|
– Pulmonary veins empty into the coronary sinus or the posterior RA
– Usually no obstruction to flow, although it is possible |
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Type 3: Infracardiac Type
|
– Pulmonary veins form confluence posterior to LA and descending vein extends from the confluence into the portal vein system or hepatic veins, ductus venosus, or IVC
– Usually associated with some degree of obstruction |
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Type 4: Mixed Type
|
– Least common type of TAPVR
– Connections to both IVC and SVC or RA – Most commonly left pulmonary veins drain into the left innominate vein via an anomalous vertical vein and the right pulmonary veins drain into the coronary sinus or directly into the RA |
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Treatment of Total Anomalous Pulmonary Vein Return
|
– Definitive treatment is surgery
– Most surgeons advocate immediate repair as soon as diagnosis is made – Echo is used as a diagnostic procedure – Pulmonary veins are reattached to the LA |
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Congenital Aortic Aneurysm
|
– Rare
– Associated with inherited disorders that affect connective tissue – Marfan Syndrome: enlarged Ao, MV prolapse – Ehlers–Danlos Syndrome: enlarged Ao |
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Three Types of Congenital AS
|
Valvular Stenosis: most common
– unicuspid, bicuspid – thick, dysplastic leaflets Subvalvular Stenosis: 2nd most common – membrane immediately below the AV in LVOT – localized muscular hypertrophy Supravalvular Stenosis: least common – narrowing of the ascending aorta immediately above the aortic sinus |
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Unicuspid Aortic Valve
|
– Rare
– Abnormal, deformed AV – Systolic doming – Doppler criteria same as AS (AVA, peak velocities, mean gradients) |
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Bicuspid Aortic Valve
|
– 2% of general population
– 3–4 : 1 more common on males – Functionally normal unless calcified (AS) – Significant AR less common – May be associated with coarctation of the aorta |
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|
Bicuspid AV M–mode/2D/Doppler
|
M–mode: eccentric closure
2D: assess in systole (PSAX), identify raphe and # of cusps, eccentric closure, systolic doming Doppler: evaluate for AS and AR |
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Types of Bicuspid AV (where the raphe is)
|
R + L fusion: most common
R + N fusion: 2nd most common (15%) L + N fusion: least common (1–2%) |
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Subvalvular Aortic Stenosis (2D/Doppler/M–mode)
|
2D: use PLAX and Apical 3, membrane not always seen on TEE
M–mode: locate peak velocity at site of obstruction, assess for turbulence, pulse through subvalvular area to show where obstruction is located Doppler: decreased aortic valve opening in early systole near obstruction |
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Supravalvular Aortic Stenosis
|
– Obstruction above aortic valve
– May be associated with Williams Syndrome (28–50% of supravalvular AS patients have this, developmental delay + elfin features) |
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Aortic Valve Abnormalities Treatment
|
Subvalvular AS: surgical removal of thickened muscle, may re–grow and require repeat surgery
Valvular AS: surgical or balloon valvuloplasty, valve replacement Supravalvular AS: narrowed portion of aorta is removed |
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|
Pulmonary Valve Abnormalities
|
– Valvular stenosis
– Subvalvular stenosis – Supravalvular stenosis |
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|
Pulmonary Valve Stenosis
|
2D: thickened leaflets with decreased opening and systolic doming, RVH if stenosis is significant
M–mode: increased A wave Doppler: increased velocity and gradient across valve, turbulent flow distal to PV in MPA, may have PI, can't use to calculate RVSP when PS is present with TR |
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Subvalvular Pulmonary Stenosis
|
– Obstruction caused by muscular ridge in RVOT
– Also known as infundibular stenosis 2D: – Muscular ridge seen proximal to PV in RVOT Doppler: – Increase in velocity and gradient in RVOT – Turbulent flow seen in RVOT proximal to PV |
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|
Supravalvular Pulmonary Stenosis
|
– Narrowing of the pulmonary artery or branches
2D: – Decreased size of pulmonary artery lumen Doppler: – Increased velocity and gradient distal to PV – Turbulent flow seen in PA distal to PV |
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|
Congenital Pulmonary Stenosis:
Signs and Symptoms |
Mild:
– Asymptomatic Severe: – Tachypnea – Feed poorly – Tire easily |
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|
Pulmonary Abnormalities: Treatment
|
Valvular Stenosis: balloon valvuloplasty
Subvalvular Stenosis: removal of thickened muscle Supravalvular Stenosis: patch applied to widen PA |
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|
Tricuspid Valve Abnormalities
|
Ebstein's Anomaly: Displacement of the TV leaflets from their normal location at the atrioventricular junction into the right ventricle
Tricuspid Atresia: TV fails to develops and instead there is a musculofibrous plate across the tricuspid annulus, which prohibits blood passage from the RA to the RV |
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|
Ebstein's Anomaly
|
– TV is displaced toward the apex, causing atrialization of the RV (RAE) and subsequent TR
– May include one or all of the TV leaflets – Usually involves 2: septal and posterior – Leaflets are adherent to the RV wall and causes only a small part of the valve cusps to move, contributing to TR – Anterior leaflet is not displaced and may be relatively normal (sail like) – Often associated with ASD and Wolff–Parkinson–White syndrome |
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Ebstein's Anomaly Echo Findings
|
– Atrialization of the RV
– TR – Volume overload of right heart – Associated ASD – Apical views are diagnostic for Ebstein's |
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|
Displacement of _____mm/m^2 is diagnostic of Ebstein's
|
> or = to 8 mm/m^2
|
|
|
Ebstein's Doppler
|
CF imaging:
– Amount and significance of TR – Presence of ASD CW: – TR |
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|
Ebstein's Anomaly
|
– Symptoms depend on placement of TV and degree of TR
– Significant TR causes an increase in RAP which may cause a right to left shunt through ASD |
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|
Ebstein's Anomaly Treatment
|
– Dependent on amount TR and R–L shunt at atrial level (if ASD present)
– Mild cases––no treatment – Surgical treatment: repair associated anomalies, TV repair (Danielson repair), TV replacement, atrial plication |
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Tricuspid Atresia
|
Rare lesion: TV fails to develop and instead there is a musculofibrous plate across the tricuspid annulus, which prohibits blood passage from the RA to the RV
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|
Associated Findings of Tricuspid Atresia
|
– Large ASD
– VSD – Small to hypoplastic RV – Pulmonary stenosis |
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|
TV Atresia Treatment
|
Initial treatment:
– Prostaglandin to keep PDA open – Blalock–Taussig shunt Fontan Procedure: – Very complicated procedure – May be done in stages |
|
|
Atrioventricular Septal Defect
|
– Also called Endocardial Cushion Defect or AV Canal Defect
– Defects resulting from the failure of the embryologic endocardial cushions to fuse resulting in anomalies of the atrial and ventricular septum and adjacent mitral and tricuspid valves – A large defect in the crux of the heart that affects the inferior portion of the atrial septum, the AV valves, and the posterior portion of the ventricular septum |
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|
What is the disease that AV canal defects are very common in?
|
Down Syndrome patients
|
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|
3 Different Forms of AV Canal Defects
|
– Partial: Incomplete fusion of superior and inferior endocardial cushions resulting in a cleft in the midportion of the anterior mitral valve
– Complete: Results in division of the anterior mitral leaflet into separate components – Intermediate (transitional) |
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|
Partial AV Canal Defect
|
– Distint and separate mitral and tricuspid annuli (AV valves insert at the same level in A4C)
– Primum ASD – Cleft anterior mitral valve |
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|
Symptoms/Treatment of Partial AV Canal Defect
|
Symptoms:
– Murmur – Difficulty feeding and breathing – Poor growth Treatment: – MV repair by suturing cleft – ASD closed |
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|
Complete AV Canal Defect
|
– Primum ASD
– Inlet VSD (or membranous) – One common AV valve * Multiple leaflets (usually 5) * Commissures vary in completeness * Inferior displacement of AV valve; valve appears at same level in A4C |
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|
Intermediate (Transitional) AV Canal Defect
|
– Rarest form
– Much like complete form except the anterior and posterior bridging leaflets are fused to the top of the ventricular septum. This then divides the common AV valve into mitral and tricuspid. |
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|
Endocardial Cushion Defects Associated
Anomalies |
– Persistent left SVC
– Secundum ASD – Discreet subaortic stenosis: narrow LVOT – Coarctation of the aorta – PDA |
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|
Endocardial Cushion Defects Echo: 2D
|
– AV valves insert at same level
– Valve morphology: cleft MV, common AV valve – Evaluate AV valve chordal attachments – Primum ASD – Inlet VSD – Evaluate chamber size – Additional defects (secundum ASD, muscular VSD) |
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|
Endocardial Cushion Defects Echo: Doppler
|
– Identity shunt flow and amount of shunting
– Presence and severity of AV valve regurgitation and or stenosis – Rule out subaortic/subpulmonary stenosis – PDA – Estimate RVSP: assess for PHTN |
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|
Endocardial Cushion Defects Clinical Findings
|
As a result of a large increase in pulmonary blood flow and increased pulmonary artery pressure, symptoms usually show in infants prior to age 1
– Heart failure – Dyspnea – Fatigue – Recurrent respiratory infections – Poor growth/failure to thrive |
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|
Endocardial Cushion Defects Physical Findings
|
– Overactive heart: tachycardia
– Tachypnea, congestion – Apical systolic thrill (MR) – Small undernourished appearance – Holosytolic murmur (MR) |
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|
Endocardial Cushion Defects Treatment
|
– Surgical repair recommended between 6–12 months of age in infants with failure to thrive (earlier in child with complete form)– Closure of ASD, VSD, repair or reconstruction of AV valves and repair other associated anomalies
– Palliative repair: pulmonary artery band––lowers pressure in right side |
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|
Hypoplastic Right Heart |
- Pulmonary Atresia with Intact Ventricular Septum - Tricuspid Atresia |
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|
Pulmonary Atresia with Intact Ventricular Septum |
- Completeobstruction of RVOT - Atreticpulmonary valve - Intactventricular septum - TVusually abnormal - Sizeof RV varies (HypoplasticRV – 85%/Normalsize RV – 15%) - R-L shunt at atrial level - PDAsupplies blood to lungs (ductal dependent lesion) - Progressivecyanosis |
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Treatment of PA/IVS |
- Prostaglandinsto keep PDA open - AtrialSeptostomy– blood from RA to LA - Blalock-Taussig Shunt - RVOTreconstruction if RV large enoughto function as a true ventricle - Possiblepulmonary valvotomy/valvectomy - FontanProcedure if RV is not developed enoughto function as a ventricle - Cardiac Transplant |
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|
Tricuspid Atresia |
- Absenceof tricuspid valve - Lackof direct communication between the right atrium and the right ventricle - Hypoplasiaof right ventricle - Needatrial level shunt – ASD or large PFO - PossibleVSD - Cyanosis |
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|
Treatment of Tricuspid Atresia |
- Prostaglandinsto keep PDA open - Atrialseptostomy - Blalock-Taussigshunt – palliative - Fontan– staged procedure; connection of systemic blood to pulmonary system |
|
|
Hypoplastic Left Heart Syndrome Characteristic Features |
- Underdevelopedatresia and/or obstruction of the left heart structures which are unable tosupport the systemic circulation - Severehypoplasia or total atresia of the aortic and mitral valve - Small,hypo plasticLV/Left atrium is usually small - Hypoplasiaof the ascending aorta - Normalcoronary artery orientation, severe hypoplasia of the ascending aorta,coronaries are perfused in retrograde manner via the flow from the ductusarteriosus - Intactventricular septum |
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HLHS Prognosis |
- Lethalcongenital cardiac anomaly - Leftuntreated results in certain death - Untreated95% die within one month - Untreated100% die within one year - Malepredominance (67%) - Itis a “ductal-dependent” lesion, if the ductuscloses the resultant perfusion failure causes changes in other organs |
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|
Blood Flow/Features HLHS |
- Levocardia/Levoposition;Visceral and atrial situssolitus;Concordant atrioventricular and ventriculoarterialconnections - Incompleteclosure of the foramen ovaleor a very small ASD - Bloodis primarily unable to pass through the MV, therefore it travels into the LA,through the foramen ovale,and into the RA - L - R atrial level shunt - Right-to-left(pulmonary to aorta) shunt through PDA |
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|
Right Heart in HLHS |
- RVis usually enlarged and hypertrophied due to increased blood volume andpressure - RVsupplies both systemic and pulmonary circulations - Pulmonaryvalve is usually normal; PA and branches are usually dilated due to increase inblood volume - TVis usually normal although TR may be present due to volume overload - IVCand SVC drain normally into RA |
|
|
Associated Findings of HLHS |
- Coarctationof the aorta in 70% of cases - Completeinterruption of the aortic arch may occur - Anomalouspulmonary veins are common |
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|
Possible reasons for HLHS: |
- Fusionof the endocardialcushions occurs but fails to produce a valve. It may also be due to an overgrowth of the ventral atrioventricularcushion, which deprives the ascending aorta of adequate blood flow. - The causative factor is a rudimentary left atrium and, consequently,diminished blood flow to the mitral portion of the atrioventricular canal. Theleft ventricle and aorta or aortic valve thus fail to develop properly becauseof the decreased blood flow through the lumen. |
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|
HLHS Clinical Features |
- Normalbirth weight baby - Cyanosisat delivery is rare, appears within first few hours/days - AsPDA closes - Dyspnea - Tachypnea - Tachycardia - Hypotension - Enlargedliver due to RV volume overload |
|
|
HLHS Medical Treatment |
- ProstaglandinE: to maintain ductal potency - Ductusneeds to stay open - Atrialseptostomy/septectomy - Arterialduct manipulation (stent in ductusarteriosus)for those awaiting transplantation - 3 Stage Surgery: Norwood, Glenn, Fontan - Transplant: #1 reason for infant heart transplant |
|
|
Shone's Complex |
4 Left-Sided Defects 1. ParachuteMV: both leaflets of MV insert into onepapillary muscle --narrowed opening of MV 2. SupravalvularMitral Ring/Membrane: ridge of connective tissue aroundcircumference of MV on LA side -- obstructs flow 3. Valvular orSubvalvular AS 4. Coarctation ofthe Aorta |
|
|
Kawasaki Disease |
- Diseaseof unknown etiology marked by inflammation in the walls of arteries throughoutthe body, also affects lymph nodes, skin, & mucous membranes in mouth, nose, & throat - Possiblecauses: bacteria, virus, environmental factors? - Asianpopulation has higher rate of having disease - Occurspredominantly in infancy and childhood (< 5 years of age) - ~1 in 5 children with disease develop heart problems; small % have lastingdamage |
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Kawasaki Stages |
FirstPhase: Fever, Redeyes, Skinrash – trunk of body, Red,cracked tongue – “strawberry” tongue, Swollen,red palms of hand and soles of feet, Swollenlymph nodes SecondPhase: Peelingof hands and feet, Jointpain, Diarrhea, Vomiting, Abdominalpain ThirdPhase: Symptomsstart to go away |
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Heart Complications of Kawasaki Disease |
- Coronaryartery aneurysms: Measureand track size by echo - Pericarditis - Myocarditis - Endocarditis - Disruptionof conduction system |
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Kawasaki Treatment |
- Serialecho studies to assess coronary arteries: 3,4, and 12 month follow-up - Intravenousgamma globulin: Immuneprotein that lowers the risk of coronary problems - Aspirin: Decreaseinflammation - Antithromboticagents: Decreaserisk of clots in artery aneurysms - Stents/CABG: Ifpermanent coronary artery damage occurs |
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ECMO |
- Extracorporeal Membrane Oxygenation - Heart and breathing support - Utilized on patients with alife-threatening condition after medicine and ventilator have failed |
