Endocardial Cushion Defects

Question 1

Endocardial cushion defects (ECD’s) are also referred to as:

Answer 1

Atrioventricular canal defect (AVCD) or
Atrioventricular septal defect (AVSD)

Question 2

The endocardial cushions make up the IVS, IAS, the ____ leaflet of TV and ____ MV leaflet.

Answer 2

Septal; anterior

Question 3

What are the characteristics of a partial AV canal defect?

Answer 3

• Typically, only the right and left atria are involved
• Primum ASD
• Cleft mitral/tricuspid valve

Question 4

Outlet, or infundibular VSD, may be present with a ____ ASD.

Answer 4

Primum

Question 5

A ____ AV canal defect involves all 4 chambers of the heart and the AV valves.

Answer 5

Complete

Question 6

What are the characteristics of a complete AV canal defect?

Answer 6

• 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.

Question 7

Complete AVCDs are further divided into sub-categories according to their ____ within the heart.

Answer 7

Attachments

Question 8

The Three types of complete AVCD’s are:

Answer 8

Type A, Type B, and Type C

Question 9

With Type A complete AVCD’s, chordal attachments to the ____.

Answer 9

Crest of the IVS

Question 10

With Type B complete AVCD’s, chordal attachments to an ____.

Answer 10

An anomalous papillary muscle

Question 11

With Type C complete AVCD’s, there is a ____ with attachments to pap muscles on both sides of IVS.

Answer 11

Free-floating leaflet

Question 12

AVCDs most frequently seen in children with ____.

Answer 12

Down Syndrome

Question 13

If pulmonary vascular resistance increases over systemic resistance, the shunting will reverse __-to-__, which can lead to cyanosis and dyspnea.

Answer 13

Right-to-left

Question 13

If left unrepaired, the increase in pulmonary blood flow will cause progressive obliteration of the ____.

Answer 13

Pulmonary arterioles

Question 14

Complete AVCD’s usually appear within the first few ____ of life.

Answer 14

Weeks

Question 15

What are some signs and symptoms of a complete AVCD?

Answer 15

• Dyspnea
• Cyanosis
• Poor appetite and weight gain
• Possible signs of CHF (edema, ascites)

Question 16

With a partial AVCD, signs may not appear until patient is __-__ years of age.

Answer 16

20 - 30 years

Question 17

What are some signs and symptoms of a partial AVCD?

Answer 17

Arrhythmias, CHF, and Pulmonary HTN

Question 18

What are some treatment options for an AVCD?

Answer 18

• Digitalis, Diuretics to treat CHF
• Antibiotic prophylaxis
• Pulmonary Artery Banding (Palliative)
• Close ASD
• Close VSD
• Reconstruct cleft AV valves
• Replace MV

Question 19

Conotruncal defects affect the hearts ___ tract.

Answer 19

Outflow

Question 20

Failure of the conotruncal septation will result in an array of defects, including:

Answer 20

• Tetralogy of Fallot (TET, or TOF)
• Pulmonary atresia with VSD
• Double outlet right ventricle (DORV)
• Truncus arteriosus

Question 21

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.

Answer 21

IVS

Question 22

All ____ defects cause improper circulation of oxygenated and deoxygenated blood.

Answer 22

Conotruncal

Question 23

____ is the most common cyanotic lesion in the adult population.

Answer 23

Tetrology of Fallot (TOF)

Question 24

TOF is comprised of 4 defects:

Answer 24

- Overriding aorta (straddles IVS) - VSD - Infundibular stenosis (PV) or PS - RVH

Question 25

With TOF, since the ____ is overriding, blood flow from either ventricle can exit out the ____.

Answer 25

Aorta; aorta

Question 26

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

Answer 26

Less

Question 26

The two types of TOF are ___ and ___.

Answer 26

Cyanotic and acyanotic

Question 27

Cyanotic TOF has severe PS with predominant __-to-__ shunt.

Answer 27

Right-to-left

Question 28

Acyanotic TOF has mild PS with predominant __-to-__ shunt

Answer 28

Left-to-right

Question 29

Most important hemodynamic factor in determining the severity of TOF is the severity of the ____.

Answer 29

PS

Question 30

In TOF, what is the order of the blood flow?

Answer 30

IVC/SVC > RA/RV > aorta or the pulmonary artery

Question 31

In TOF, if the PA is of normal size and the pulmonary vascular resistance is lower, there will be more blood going out the ____.

Answer 31

PV

Question 32

In TOF, if there is severe narrowing of the infundibulum, then more blood will go across the VSD and out the ____.

Answer 32

Aorta

Question 33

With TOF, the PLAX view will show an overriding aorta and a ____.

Answer 33

VSD

Question 34

With TOF, you need to determine the size of the ____ and ____ to help in determining the surgical course

Answer 34

RPA and LPA

Question 35

TOF has a higher likelihood of associated ____ so an assessment of the origins of the coronary arteries must be performed.

Answer 35

Coronary artery anomalies

Question 36

What are the signs and symptoms of TOF?

Answer 36

- 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.

Question 37

Children with TOF exhibit ___ skin during episodes of crying or feeding.

Answer 37

Bluish

Question 38

What needs to be done to repair TOF?

Answer 38

- 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*

Question 39

____ is when one great vessel coming off of the heart acts as both the pulmonary artery and the aorta.

Answer 39

Truncus arteriosus

Question 40

With truncus arteriosus, the ____, ____, and ____ all arise from a common trunk.

Answer 40

Coronary arteries, main pulmonary artery, and aortic arch

Question 41

With truncus arteriosus, it is usually the ____ that developed and the ____ will develop out of this trunk.

Answer 41

Aorta; pulmonary artery

Question 42

With truncus arteriosus, a large malalignment ____ always present.

Answer 42

VSD

Question 43

A truncal valve may have __-__ leaflets and may leak and/or be stenotic.

Answer 43

2-6

Question 44

With truncus arteriosus, ____ in aorta and PA is the same.

Answer 44

Oxygen saturation

Question 45

With truncus arteriosus, LCA originates from ____ ____ truncus side.

Answer 45

Left posterolateral

Question 46

With truncus arteriosus, RCA originates from ____ truncus side.

Answer 46

Anterolateral

Question 47

Truncus arteriosus is usually classified into ____ types, based on how the left and right pulmonary arteries originate from the common trunk.

Answer 47

4

Question 48

Truncus arteriosus type 1:

Answer 48

The MPA arises from the truncal root and then branches to the RPA and LPA (60%)

Question 49

Truncus arteriosus type 2:

Answer 49

LPA and RPA arise directly from the posterior portion of the truncal root as separate vessels with separate orifices (20%)

Question 50

Truncus arteriosus type 3:

Answer 50

Each pulmonary artery arises directly from the lateral aspects of the truncal root as separate vessels with separate orifices (20%)

Question 51

With truncus arteriosus, the truncal valve may have more than ____ cusps.

Answer 51

Three

Question 52

With truncus arteriosus, there is a VSD present __% of the time.

Answer 52

100%

Question 53

With truncus arteriosus, you need to find the placement of the ____.

Answer 53

Pulmonary arteries

Question 54

To assess the pulmonary arteries, you can use a ____ ____ parasternal short-axis orientation with the transducer angled toward the apex.

Answer 54

High right

Question 55

For the surgical repair of a truncus arteriosus, the ____ needs to be closed.

Answer 55

VSD

Question 56

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.

Answer 56

Pulmonary arteries

Question 57

Double Outlet Right Ventricle (DORV), is where the two great vessels both of which arise from the morphologic ____.

Answer 57

RV

Question 58

With DOVR, a ____ must always be present so the blood can get from the LV to the aorta..

Answer 58

VSD

Question 59

With DORV, there is no ____ continuity between either AV valve and either semilunar valve.

Answer 59

Fibrous

Question 60

DORV is often associated with ____ and ____.

Answer 60

PS and MV abnormalities (parachute MV, cleft MV straddling MV, supramitral ring)

Question 61

Severity of DORV is determined by the type of ____ and the presence or absence of PS.

Answer 61

VSD

Question 62

A more moderate or severe form of DORV will cause ____ in the patient.

Answer 62

Cyanosis

Question 63

With DORV, blood flow from the left and right ventricles mixes at the ____ level and exits via both great vessels.

Answer 63

Ventricular

Question 64

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.

Answer 64

Position

Question 65

The diagnosis of DORV is usually apparent from the ____ view.

Answer 65

Subcostal

Question 66

What are the 3 goals to repairing DORV?

Answer 66

- Establish left ventricle to aorta continuity - Establish right ventricle to pulmonary artery continuity - Repair associated defects (VSD repair)

Question 67

What are the two types of transposition?

Answer 67

D-transposition and L-transposition

Question 68

What type of transposition includes atrioventricular concordance with ventriculoarterial discordance (A.K.A. complete transposition).

Answer 68

D-transposition (DTGV)

Question 69

What type of transposition includes atrioventricular discordance and ventriculoarterial discordance (A.K.A. congenitally corrected).

Answer 69

L-transposition (LTGV)

Question 70

In the PSAX, if the aorta is ____ to the pulmonary artery, the diagnosis is transposition of the great vessel (TGV).

Answer 70

Anterior

Question 71

With DTGV, there is ____ wrong connection.

Answer 71

One

Question 72

With ____, the correct atrium is connected to the correct ventricle, but the wrong great vessel is attached to each ventricle.

Answer 72

DTGV

Question 73

What is the circulation of blood for D-transposition?

Answer 73

- Circulation is as follows: RA ~ RV ~ aorta ~ body ~ RA again. - Pulmonary veins ~LA ~ LV ~ pulmonary artery ~ lungs ~ LA again.

Question 74

DTGV has ____ independent parallel circuits.

Answer 74

2

Question 75

Life is dependent on some intermixing of those two circuits which occurs through a ____.

Answer 75

Shunt - PFO, PDA, or VSD

Question 76

What drug is used to keep a shunt open?

Answer 76

Prostaglandin

Question 77

In DTGA, the aorta originates from the RV, carrying ____ blood to the body.

Answer 77

Desaturated

Question 78

In DTGA, the PA originates from the LV, delivering ____ blood to the lungs

Answer 78

Oxygenated

Question 79

In DTGA, the aorta is ____ and to the right of the PA.

Answer 79

Anterior

Question 80

In LTGA, the anatomic RV will be displaced posterior and leftward, becoming the ____ ventricle.

Answer 80

Arterial

Question 81

In LTGA, the anatomic LV will be displaced anterior and rightward, becoming the ____ ventricle.

Answer 81

Venous

Question 82

What is the circulation of blood for L-transposition?

Answer 82

- Pulmonary veins ~ LA ~ TV ~ RV ~ AO ~ body - SVC/IVC ~RA ~ MV ~ LV ~ PA ~ lungs

Question 83

In LTGV, despite the displacement of the ventricles, the PA arises from the ____ ventricle and the AO arises from the ____ ventricle as they should.

Answer 83

Venous; arterial