Endocardial Cushion Defects Flashcards
TOF, Truncus Arteriosus, DORV, Transposition
Endocardial cushion defects (ECD’s) are also referred to as:
Atrioventricular canal defect (AVCD) or
Atrioventricular septal defect (AVSD)
The endocardial cushions make up the IVS, IAS, the ____ leaflet of TV and ____ MV leaflet.
Septal; anterior
What are the characteristics of a partial AV canal defect?
- Typically, only the right and left atria are involved
- Primum ASD
- Cleft mitral/tricuspid valve
Outlet, or infundibular VSD, may be present with a ____ ASD.
Primum
A ____ AV canal defect involves all 4 chambers of the heart and the AV valves.
Complete
What are the characteristics of a complete AV canal defect?
- Primum ASD,
- Inlet VSD,
- Common atrioventricular valve (most common)
- MV and TV are merged
- Transitional Canal Defect consist of two distinct atrioventricular annuli with defects in the atrial and ventricular septa.
Complete AVCDs are further divided into sub-categories according to their ____ within the heart.
Attachments
The Three types of complete AVCD’s are:
Type A, Type B, and Type C
With Type A complete AVCD’s, chordal attachments to the ____.
Crest of the IVS
With Type B complete AVCD’s, chordal attachments to an ____.
An anomalous papillary muscle
With Type C complete AVCD’s, there is a ____ with attachments to pap muscles on both sides of IVS.
Free-floating leaflet
AVCDs most frequently seen in children with ____.
Down Syndrome
If pulmonary vascular resistance increases over systemic resistance, the shunting will reverse __-to-__, which can lead to cyanosis and dyspnea.
Right-to-left
If left unrepaired, the increase in pulmonary blood flow will cause progressive obliteration of the ____.
Pulmonary arterioles
Complete AVCD’s usually appear within the first few ____ of life.
Weeks
What are some signs and symptoms of a complete AVCD?
- Dyspnea
- Cyanosis
- Poor appetite and weight gain
- Possible signs of CHF (edema, ascites)
With a partial AVCD, signs may not appear until patient is __-__ years of age.
20 - 30 years
What are some signs and symptoms of a partial AVCD?
Arrhythmias, CHF, and Pulmonary HTN
What are some treatment options for an AVCD?
- Digitalis, Diuretics to treat CHF
- Antibiotic prophylaxis
- Pulmonary Artery Banding (Palliative)
- Close ASD
- Close VSD
- Reconstruct cleft AV valves
- Replace MV
Conotruncal defects affect the hearts ___ tract.
Outflow
Failure of the conotruncal septation will result in an array of defects, including:
- Tetralogy of Fallot (TET, or TOF)
- Pulmonary atresia with VSD
- Double outlet right ventricle (DORV)
- Truncus arteriosus
With all conotruncal defects, the relationship of the IVS and the great vessels is abnormal with one great vessel overriding the ___, allowing blood to exit from both ventricles.
IVS
All ____ defects cause improper circulation of oxygenated and deoxygenated blood.
Conotruncal
____ is the most common cyanotic lesion in the adult population.
Tetrology of Fallot (TOF)
TOF is comprised of 4 defects:
- Overriding aorta (straddles IVS)
- VSD
- Infundibular stenosis (PV) or PS
- RVH
With TOF, since the ____ is overriding, blood flow from either ventricle can exit out the ____.
Aorta; aorta
With TOF, since ____ flow is going out of the pulmonary artery, this reduces the size of the pulmonary artery and valve and increases the size of the aorta
Less
The two types of TOF are ___ and ___.
Cyanotic and acyanotic
Cyanotic TOF has severe PS with predominant __-to-__ shunt.
Right-to-left
Acyanotic TOF has mild PS with predominant __-to-__ shunt
Left-to-right
Most important hemodynamic factor in determining the severity of TOF is the severity of the ____.
PS
In TOF, what is the order of the blood flow?
IVC/SVC > RA/RV > aorta or the pulmonary artery
In TOF, if the PA is of normal size and the pulmonary vascular resistance is lower, there will be more blood going out the ____.
PV
In TOF, if there is severe narrowing of the infundibulum, then more blood will go across the VSD and out the ____.
Aorta
With TOF, the PLAX view will show an overriding aorta and a ____.
VSD
With TOF, you need to determine the size of the ____ and ____ to help in determining the surgical course
RPA and LPA
TOF has a higher likelihood of associated ____ so an assessment of the origins of the coronary arteries must be performed.
Coronary artery anomalies
What are the signs and symptoms of TOF?
- Exercise intolerance
- Squatting episodes (the child squats down because this maneuver increases the venous return, stroke volume, and cardiac output)
- “Tet” spells: episodes of cyanosis, syncope, and hypoxia.
Children with TOF exhibit ___ skin during episodes of crying or feeding.
Bluish
What needs to be done to repair TOF?
- Close the VSD
- PV replacement (PV redo if valve doesn’t grow adequately with child)
The RVH will lessen as the heart grows with the child
____ is when one great vessel coming off of the heart acts as both the pulmonary artery and the aorta.
Truncus arteriosus
With truncus arteriosus, the ____, ____, and ____ all arise from a common trunk.
Coronary arteries, main pulmonary artery, and aortic arch
With truncus arteriosus, it is usually the ____ that developed and the ____ will develop out of this trunk.
Aorta; pulmonary artery
With truncus arteriosus, a large malalignment ____ always present.
VSD
A truncal valve may have __-__ leaflets and may leak and/or be stenotic.
2-6
With truncus arteriosus, ____ in aorta and PA is the same.
Oxygen saturation
With truncus arteriosus, LCA originates from ____ ____ truncus side.
Left posterolateral
With truncus arteriosus, RCA originates from ____ truncus side.
Anterolateral
Truncus arteriosus is usually classified into ____ types, based on how the left and right pulmonary arteries originate from the common trunk.
4
Truncus arteriosus type 1:
The MPA arises from the truncal root and then branches to the RPA and LPA (60%)
Truncus arteriosus type 2:
LPA and RPA arise directly from the posterior portion of the truncal root as separate vessels with separate orifices (20%)
Truncus arteriosus type 3:
Each pulmonary artery arises directly from the lateral aspects of the truncal root as separate vessels with separate orifices (20%)
With truncus arteriosus, the truncal valve may have more than ____ cusps.
Three
With truncus arteriosus, there is a VSD present __% of the time.
100%
With truncus arteriosus, you need to find the placement of the ____.
Pulmonary arteries
To assess the pulmonary arteries, you can use a ____ ____ parasternal short-axis orientation with the transducer angled toward the apex.
High right
For the surgical repair of a truncus arteriosus, the ____ needs to be closed.
VSD
For the surgical repair of a truncus arteriosus, you need to excise the ____ from the truncus and place a valved conduit from the RV to the PA.
Pulmonary arteries
Double Outlet Right Ventricle (DORV), is where the two great vessels both of which arise from the morphologic ____.
RV
With DOVR, a ____ must always be present so the blood can get from the LV to the aorta..
VSD
With DORV, there is no ____ continuity between either AV valve and either semilunar valve.
Fibrous
DORV is often associated with ____ and ____.
PS and MV abnormalities (parachute MV, cleft MV straddling MV, supramitral ring)
Severity of DORV is determined by the type of ____ and the presence or absence of PS.
VSD
A more moderate or severe form of DORV will cause ____ in the patient.
Cyanosis
With DORV, blood flow from the left and right ventricles mixes at the ____ level and exits via both great vessels.
Ventricular
With DORV, the degree of mixing and the relative volumes going to each great vessel depend on the ____ of the VSD and the great vessels.
Position
The diagnosis of DORV is usually apparent from the ____ view.
Subcostal
What are the 3 goals to repairing DORV?
- Establish left ventricle to aorta continuity
- Establish right ventricle to pulmonary artery continuity
- Repair associated defects (VSD repair)
What are the two types of transposition?
D-transposition and L-transposition
What type of transposition includes atrioventricular concordance with ventriculoarterial discordance (A.K.A. complete transposition).
D-transposition (DTGV)
What type of transposition includes atrioventricular discordance and ventriculoarterial discordance (A.K.A. congenitally corrected).
L-transposition (LTGV)
In the PSAX, if the aorta is ____ to the pulmonary artery, the diagnosis is transposition of the great vessel (TGV).
Anterior
With DTGV, there is ____ wrong connection.
One
With ____, the correct atrium is connected to the correct ventricle, but the wrong great vessel is attached to each ventricle.
DTGV
What is the circulation of blood for D-transposition?
- Circulation is as follows: RA ~ RV ~ aorta ~ body ~ RA again.
- Pulmonary veins ~LA ~ LV ~ pulmonary artery ~ lungs ~ LA again.
DTGV has ____ independent parallel circuits.
2
Life is dependent on some intermixing of those two circuits which occurs through a ____.
Shunt - PFO, PDA, or VSD
What drug is used to keep a shunt open?
Prostaglandin
In DTGA, the aorta originates from the RV, carrying ____ blood to the body.
Desaturated
In DTGA, the PA originates from the LV, delivering ____ blood to the lungs
Oxygenated
In DTGA, the aorta is ____ and to the right of the PA.
Anterior
In LTGA, the anatomic RV will be displaced posterior and leftward, becoming the ____ ventricle.
Arterial
In LTGA, the anatomic LV will be displaced anterior and rightward, becoming the ____ ventricle.
Venous
What is the circulation of blood for L-transposition?
- Pulmonary veins ~ LA ~ TV ~ RV ~ AO ~ body
- SVC/IVC ~RA ~ MV ~ LV ~ PA ~ lungs
In LTGV, despite the displacement of the ventricles, the PA arises from the ____ ventricle and the AO arises from the ____ ventricle as they should.
Venous; arterial
