Term
|
Definition
| RA Located on the right, LA on left |
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Term
|
Definition
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Term
|
Definition
| Location of the morphological ventricles |
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Term
|
Definition
| Inutero vintricles twisted rightward, morphological RV on right |
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Term
|
Definition
| Inutero verntricles twisted leftward, morphalogical RV on left |
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Term
|
Definition
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|
Term
|
Definition
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Term
| Atrial Septal Defects (ASD) |
|
Definition
Communication between left and right atrium.
6% of CHD
Spontaneous closure within 2 years but unlikely |
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Term
|
Definition
Sinus venosus (6-8%)
Septum secundum (70%)
Septum primum (20%)
Coronary sinus (least common) |
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Term
|
Definition
| Most superior region near entrance of SVC/IVC |
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Term
| Associated Defect Sinus Venosus ASD |
|
Definition
| Anomalous pulmonary veins |
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Term
|
Definition
| Central portion of septum |
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Term
| Associated Defect Septum Secundum ASD |
|
Definition
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Term
|
Definition
| Inferior portion of septum near AV valves |
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Term
| Associated Defect Septum Primum ASD |
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Definition
| Cleft MV, Endocardial cushion defect |
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Term
|
Definition
| Transcatheter closure, surgical repair with Dacon patch |
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Term
| Patent Foramen Ovale (PFO) |
|
Definition
| Flap is stil open, blood crosses IAS |
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Term
| Ventricula Septal Defects (VSD) |
|
Definition
A hole in IVS
20-25% of CHD
60-70% spontaneously close |
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Term
|
Definition
Membranous
Muscular
Outlet
Inlet |
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|
Term
|
Definition
80% of VSD's
Hole in small thin tussue below AO and above TV |
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Term
|
Definition
5-20% of VSD's
Hole in muscular tissue from membranous septum to apex |
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Term
|
Definition
5-7%
In the RVOT proximal to the PA
Superior to muscular septum inferior to great vessels |
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Term
|
Definition
5-8% of VSD's
Posterior and inferiorly beneath the TV superior region of septum |
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Term
|
Definition
| Dacron patch, transcatheter closur |
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Term
|
Definition
| Coarctation of AO, ASD, PFO, PDA, Eisenmenger's complex and Endocardial cushion defect |
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Term
|
Definition
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|
Term
| Moderate VSD Classification |
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Definition
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Term
|
Definition
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|
Term
| Patent Ductus Arteriosus (PDA) |
|
Definition
Connection between the Aorta and Pulmonary artery
5-10% of CHD
After closure it becomes the Ligamentum Arteriosus |
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Term
|
Definition
Transcatheter coil
Ligation |
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Term
|
Definition
Defect causing communication between AO and PA
Rare 0.2% of CHD
Hole like defect in lateral wall of ascending AO and MPA |
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|
Term
| AO-PA Window Surgical treatment |
|
Definition
| Dacron patch early in life |
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|
Term
| AO-PA Window associated defects |
|
Definition
| Anomalous origin of RCA off of RPA, Right aortic arch, coarctation of AO |
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|
Term
| Atrioventricular Septal Defect (AV Canal) |
|
Definition
| Consists of a Membranous VSD and Septum Primum ASD |
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|
Term
| 3 Types of AV Septal Defects |
|
Definition
Complete
Partial
Unbalanced |
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Term
|
Definition
| Most common, Primum ASD, Inlet VSD, Common AV valve at equal distance from apex |
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|
Term
|
Definition
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Term
|
Definition
| AV valve overides the IVS causing one ventricle to be well developed and other is hypoplastic |
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Term
|
Definition
| Surgical repair necessary, between 3-12 months. Patch ASD and VSD, common AV valve is repaired to make to valves, Pulm Artery banding. |
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|
Term
| AV Canal Associated Defects |
|
Definition
| Down's syndrome (40%), cleft MV, PDA, TOF, PS |
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|
Term
| Left Vertricular Outflow Abnormalities |
|
Definition
| Obstruction of blood flow from LV to aortic arch. 5% of CHD. |
|
|
Term
| 3 types of LVOT Abnormalities |
|
Definition
Valvular (75%)
Subvalvular
Supravalvular |
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|
Term
| Valvular Aortic Stenosis (AS) |
|
Definition
| Obstruction due to abnormal valve leaflets and or annulus |
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|
Term
|
Definition
Unicuspid
Bicuspid (Most common)
Tricuspid
Quadricuspid (Rare) |
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|
Term
|
Definition
| Single leaflet with pinhole of commissural attachment |
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|
Term
|
Definition
|
|
Term
|
Definition
| 3 small restrictive leaflets or hypoplastic |
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|
Term
|
Definition
|
|
Term
|
Definition
| Balloon valvuloplasty (if more than 50mmHg PPG w/out AI), AV replacement, Ross procedure |
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|
Term
| Valvular AS Associated Defects |
|
Definition
| AI, coarctation, PDA, subaortic stenosis |
|
|
Term
|
Definition
| Obstruction occurs between the RV and the PV |
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|
Term
| 3 types of Subvalvular AS |
|
Definition
Discrete fibromuscular ridge encircling the LVOT (most common).
Long fibromuscular narrowing beneath base of AV.
Tunnel-like narrowing of entire LVOT and small AO Root. |
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|
Term
|
Definition
| Surgical resection, AV replacement, Kono procedure. High recurrence risk |
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|
Term
|
Definition
| Obstruction occurs distal to AO Valve |
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|
Term
| 3 types of Supravalvular AS |
|
Definition
Membranous: fibrous diaphragm above sinuses of valsalva.
Hourglass deformity: internal stricture of ascending AO.
Hypoplastic segment: severe narrowing from area above sinuses to innominate artery. |
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|
Term
| Supravavular AS Treatment |
|
Definition
| Surgical excision of fibrous tissue, patch widening |
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|
Term
| Supravalvular AS Associated Defects |
|
Definition
| Frequent with mental retardation, PPS, coarctation of the aorta, PDA |
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|
Term
|
Definition
| A narrowing of the aortic lumen |
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|
Term
| 3 types Coarctation of the Aorta |
|
Definition
Type A: between subclavian and ductal region (isthmus)
Type B: between the left common carotid Artery and the left subclavian artery (most common 55-69%)
Type C: between the innominate artery and left common carotid (Rare <4%) |
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|
Term
|
Definition
| Resection with end-to-end anastomoses, subclavian flap aortoplasty, dacron patch, balloon angioplasty |
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|
Term
| Coarctation Associated Defects |
|
Definition
| Bicuspid AV (46%), VSD, PDA, hypoplastic left sided structures |
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|
Term
| Interrupted Aortic Arch (IAA) |
|
Definition
Lack of continuity between the ascending and descending aorta
<1% of CHD |
|
|
Term
|
Definition
A: distal to left subclavian (30-40%)
B: between left subclavian and left common carotid (55-60%)
C: proximal to left common carotid (5%) |
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Term
|
Definition
| Keep PDA open, anastomosis of 2 segments, closure of VSD and PDA |
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Term
|
Definition
| PDA, VSD, subaortic obstruction, bicuspid AV |
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Term
|
Definition
| Obstruction of blood flow from the RV to the lungs |
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|
Term
|
Definition
|
|
Term
|
Definition
| Balloon valvuloplasty, valvotomy, valve replacement |
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Term
|
Definition
| Obstruction occurs between the RV and PV |
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Term
|
Definition
| Occurs at level of infundibular ostium below PV |
|
|
Term
|
Definition
Hypertrophied muscle bundle crosses RV from septum to free wall creating 2 different pressure systems.
90% have VSD |
|
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Term
|
Definition
| Obstruction occurs distal to PV |
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|
Term
| Supravalvular PS: Thick Fibrous Ring |
|
Definition
| Occurs in MPA, Thick ring or tubular hypoplasia of MPA |
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|
Term
| Supravalvular PS: Tubular hypoplasia of MPA |
|
Definition
| Norrowing past PV due to narrow walls |
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|
Term
| Peripheral Pulmonic Stenosis (PPS) |
|
Definition
|
|
Term
|
Definition
Complete obstruction of flow from RV to lungs.
<1% of CHD |
|
|
Term
| 3 types of Pulmonary Atresia |
|
Definition
Type 1: Normal RV size
Type 2: Small RV
Type 3: Diminutive RV |
|
|
Term
|
Definition
Absence of the tricuspid valve. No communication between the RA and the RV
3% of CHD |
|
|
Term
| Tricuspid Atresia Treatment |
|
Definition
|
|
Term
| Tricuspid Atresia Associated Defects |
|
Definition
PFO/ASD (80%)
TGA (30%)
VSD
Coarctation of the AO |
|
|
Term
|
Definition
Apical displacement of the TV. >20mm from MV annulus.
0.3-0.8% of CHD |
|
|
Term
| Ebstein Malformation Treatment |
|
Definition
TV replacent
ASD closure
If RV is unuseable, Fontan |
|
|
Term
| Ebstein Malformation Associated Defects |
|
Definition
|
|
Term
|
Definition
| A cleft in the anterior leaflet of the MV. Usually part of AV canal |
|
|
Term
| Double-Orifice Mitral Valve |
|
Definition
| Abnormal tissue connects the two MV leaflets, usually in center resulting in two separate MV orifices. |
|
|
Term
| Double-Orifice MV Treatment |
|
Definition
|
|
Term
| Double-Orifice MV Associated Defects |
|
Definition
AV Canal
VSAD
Coarctation of the AO |
|
|
Term
| Congenital Mitral Stenosis |
|
Definition
| Obstruction of flow from the LA to the LV due to MV |
|
|
Term
|
Definition
| Thick, non pliable leaflets, small annulus |
|
|
Term
|
Definition
| All chordae tendineae insert into a single papillary muscle |
|
|
Term
| Supravalvular Mitral Ring |
|
Definition
| Membrane just superior to MV annulus |
|
|
Term
|
Definition
| Balloon Valvotomy, Valve replacement, resection of ring |
|
|
Term
| Congenital MS Associated Defects |
|
Definition
| Hypoplastic LV, Coarctation, AS, PFO, PDA, VSD, component of Shone's complex |
|
|
Term
|
Definition
LA is divided into 2 by a perforated membrane
<1% of CHD |
|
|
Term
| Cor Triatriatum Treatment |
|
Definition
| Excision of obstructive membrane |
|
|
Term
| Cor Triatriatum Associated Defects |
|
Definition
Anomalous pulmonary return
LSVC draining into coronary sinus
PFO, ASD, VSD |
|
|
Term
|
Definition
| Narrowing of one or more of the pulmonary veins where they enter LA |
|
|
Term
|
Definition
Balloon angioplasty
Stent
Pericardial patch |
|
|
Term
| Pulmonary Vein Stenosis Associated Defects |
|
Definition
| MS, Anomalous pulmonary venous return, cor triatriatum, ASD |
|
|
Term
| Total Anomalous Pulmonary Venous Return (TAPVR) |
|
Definition
Pulmonary veins converge and drain into right sided structures
20% of CHD |
|
|
Term
|
Definition
Pulmonary veins-portal system-IVC
20% |
|
|
Term
|
Definition
|
|
Term
|
Definition
Balloon atrial septostomy to temporarilly allow mixing.
Anastomosis of common pulmonary vein to LA. |
|
|
Term
|
Definition
|
|
Term
| Partial Anomalous Venous Return (PAPVR) |
|
Definition
| One, two or three pulmonary veins drain into the RA. |
|
|
Term
| In PAPVR Right PV usually drains into? |
|
Definition
| SVC, RA, IVC, or Coronary Sinus |
|
|
Term
| In PAPVR Left PV usually drain into? |
|
Definition
|
|
Term
|
Definition
| Surgical connection of anomalous vein to LA |
|
|
Term
|
Definition
|
|
Term
| Tetralogy of Fallot (TOF) |
|
Definition
Overriding Aorta
Malalignment VSD (Inlet)
RVH
RVOT obstruction
10% of CHD |
|
|
Term
|
Definition
| B-T shunt, Rastelli, Catheter balloon dialation of PA's, Closure of VSD |
|
|
Term
|
Definition
| Right Aortic Arch, PFO/ASD, VSD, AV Canal |
|
|
Term
| Transposition of the Great Arteries (d-TGA) |
|
Definition
Aorta arises from the RV
Pulmonary artery arises from LV
Aorta is anterior to pulmonary artery
Usually ASD, VSD, PDA
5% oc CHD |
|
|
Term
|
Definition
|
|
Term
|
Definition
VSD (50%)
LVOT obstruction (20%) |
|
|
Term
| Transposition of the Great Arteries (l-TGA) |
|
Definition
Morphological RV on patients left
Morphological LV on patients right
Atrial situs
AO arises from RV
PA arises from LV
0.6% of CHD |
|
|
Term
|
Definition
VSD (80%)
PS (50%)
Pulmonary Atresia (10%)
TR (30%) |
|
|
Term
|
Definition
| One arterial trunk gives rise to pulmonary systemic and coronary circulation. All have VSD |
|
|
Term
| 4 types of Truncus Arteriosus |
|
Definition
Type 1: MPA present, arises from truncal root (60%)
Type 2: RPA and LPA arise separately from posterior portion of truncal root (20%)
Type 3: RPA and LPA arise separately from lateral portion of truncal root (20%)
Type 4: No PA's, bronchial arteries arise from descending AO (10%) |
|
|
Term
| Truncus Arteriosus Treatment |
|
Definition
|
|
Term
| Truncus Arteriosus Associated Defect |
|
Definition
Coronary artery abnormalities
RAA (30-50%)
ASD |
|
|
Term
| Double-Outlet Right Ventricle (DORV) |
|
Definition
Both great vessels arise from RV. Large VSD
<1% of CHD |
|
|
Term
|
Definition
Subaortic (70%)
Subpulmonic
Dubly commited
Noncommitted |
|
|
Term
|
Definition
| Intraventricular tunnel between VSD and AO |
|
|
Term
|
Definition
Coarctation of the Aorta
PS
Anomalies of AV canal
Hypoplastic LV |
|
|
Term
|
Definition
Severely underdeveloped LV
1-2% of CHD
67% Male predominance |
|
|
Term
| Hypoplastic Left Heart Treatment |
|
Definition
| Complete Norwood Procedure |
|
|
Term
|
Definition
| Mucocutaneous Lymph Node Syndrom, Vasculitis of the coronary arteries leading to aneurisms, thrombosis etc. |
|
|
Term
|
Definition
| 90% of untreated results in Coronary aneurysms |
|
|
Term
| Trisomy 21 (Down Syndrome) |
|
Definition
|
|
Term
| Trisomy 18 (Edwards Syndrome) |
|
Definition
|
|
Term
|
Definition
| Coarctation of AO, Bicuspid AoV, AS |
|
|
Term
|
Definition
|
|
Term
|
Definition
| Trunctus Arteriosus, IAA, TOF |
|
|
Term
|
Definition
| Pulmonary valve stenosis (50%), HCM |
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|
Term
|
Definition
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Term
|
Definition
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Term
|
Definition
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Term
|
Definition
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Term
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Definition
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Term
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Definition
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Term
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Definition
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Term
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Definition
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Term
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Definition
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Term
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Definition
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Term
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Definition
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Definition
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Term
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Definition
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Term
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Definition
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Definition
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Term
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Definition
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Definition
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Term
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Definition
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Term
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Definition
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Definition
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Definition
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Definition
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Definition
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Definition
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Definition
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