Congenitally Corrected Transposition of the Great Arteries (ccTGA)

Question 1

What is corrected transposition of the great arteries (ccTGA)?

Answer 1

Characterized by abnormal connections between the atrium, ventricles and great arteries (atrioventricular and ventriculoarterial discordance).

Question 2

What does L looped ventricles mean in ccTGA?

Answer 2

Abnormal leftward looping of the primitive heart, resulting in abnormal positioning of the ventricles.

Question 3

Where is the morphologic right ventricle located in ccTGA?

Answer 3

On the left side.

Question 4

Where is the morphologic left ventricle located in ccTGA?

Answer 4

On the right side.

Question 5

What is atrioventricular discordance?

Answer 5

An abnormal connection between the atria and ventricles.

Question 6

How does the morphologic right atrium connect in ccTGA?

Answer 6

Right atrium à mitral valve à left ventricle.

Question 7

How does the morphologic left atrium connect in ccTGA?

Answer 7

Left atrium à tricuspid valve à right ventricle.

Question 8

What is ventriculoarterial discordance?

Answer 8

The pulmonary artery is connected to the right sided morphologic left ventricle, and the aorta is connected to the left sided morphologic right ventricle.

Question 9

What maintains hemodynamically correct blood flow in ccTGA?

Answer 9

Discordant connections at both atrioventricular and ventriculoarterial levels.

Question 10

How are the great arteries described in ccTGA?

Answer 10

As L looped.

Question 11

Where is the aorta positioned in relation to the pulmonary artery in ccTGA?

Answer 11

Anterior and leftward.

Question 12

What is created below the aortic valve in ccTGA?

Answer 12

A well-developed infundibulum.

Question 13

What does the infundibulum create in ccTGA?

Answer 13

Tricuspid and aortic discontinuity.

Question 14

What helps provide supportive findings when diagnosing ccTGA?

Answer 14

The spatial relationship between the great arteries and tricuspid/aortic discontinuity.

Question 15

What are the segments for Congenitally Corrected Transposition of the Great Arteries?

Answer 15

{S, L, L}

S stands for Atrial situs solitus, L for looped ventricles, and L for looped great arteries.

Question 16

What is the significance of the segmental approach in ccTGA?

Answer 16

It assists in accurately diagnosing ccTGA by determining atrial situs, ventricular situs, and great artery arrangement.

This approach is crucial due to the rarity and complexity of the condition.

Question 17

What is the atrial situs in Congenitally Corrected Transposition of the Great Arteries?

Answer 17

Atrial situs solitus

This refers to the normal arrangement of the atria.

Question 18

What type of ventricles are present in Congenitally Corrected Transposition of the Great Arteries?

Answer 18

L looped ventricles

This indicates the orientation of the ventricles in ccTGA.

Question 19

What type of great arteries are found in Congenitally Corrected Transposition of the Great Arteries?

Answer 19

L looped great arteries

This describes the arrangement of the great arteries in this condition.

Question 20

Name an associated defect with Congenitally Corrected Transposition of the Great Arteries.

Answer 20

Ebstein’s anomaly

This is one of several defects that can occur alongside ccTGA.

Question 21

List two types of outflow tract obstructions associated with Congenitally Corrected Transposition of the Great Arteries.

Answer 21

• Left ventricular outflow tract obstruction (sub-pulmonic and/or pulmonary stenosis)
• Right ventricular outflow tract obstruction (sub-aortic and/or aortic stenosis)

These obstructions can complicate the condition.

Question 22

True or False: The segmental approach is optional in diagnosing Congenitally Corrected Transposition of the Great Arteries.

Answer 22

False

The segmental approach is essential for accurate diagnosis.

Question 23

What is the arrangement of the great arteries in the parasternal long axis view?

Answer 23

Parallel arrangement of the great arteries

Question 24

Why is the left ventricle often difficult to visualize in the parasternal long axis view?

Answer 24

Due to the side by side positioning of the ventricles

Question 25

In the parasternal short axis view, where is the aorta positioned relative to the pulmonary artery?

Answer 25

Anterior and leftward to the pulmonary artery

Question 26

How does the interventricular septum appear in the parasternal short axis view?

Answer 26

Runs parallel (straight anteroposterior plane) with side by side ventricles

Question 27

Where is the right ventricle positioned in relation to the left ventricle in the parasternal short axis view?

Answer 27

Leftward to the left ventricle

Question 28

What are the key findings for determining ventricular morphology in apical and subcostal views?

Answer 28

Positioning of the AV valves

Question 29

The right AV valve is described as _______.

Answer 29

septo-phobic (mitral)

Question 30

The left AV valve is described as _______ and more apically displaced.

Answer 30

septo-phyllic (tricuspid)

Question 31

What characterizes the right-sided left ventricle?

Answer 31

Smooth-walled

Question 32

What characterizes the left-sided right ventricle?

Answer 32

Trabeculated

Question 33

What is a characteristic of the endocardial surface of the right ventricle?

Answer 33

Trabeculated endocardial surface ## Footnote The trabeculated surface contributes to the function of the right ventricle by aiding in the contraction and flow of blood.

Question 34

What anatomical feature is present in the right ventricle?

Answer 34

Presence of moderator band ## Footnote The moderator band is a muscular band that helps in the conduction system of the heart.

Question 35

What is the shape of the ventricular cavity in the right ventricle?

Answer 35

Elliptical shape of the ventricular cavity ## Footnote The elliptical shape assists in the efficient pumping of blood.

Question 36

What type of valve is the tricuspid valve?

Answer 36

Trileaflet valve that lies closer to the apex than the MV ## Footnote The tricuspid valve prevents backflow of blood from the right ventricle to the right atrium.

Question 37

What are the chordal attachments of the tricuspid valve in the right ventricle?

Answer 37

Septo-phyllic (chordal attachments to ventricular septum present) ## Footnote These attachments help stabilize the valve during the cardiac cycle.

Question 38

What is a characteristic of the endocardial surface of the left ventricle?

Answer 38

Smooth endocardial surface ## Footnote The smooth surface reduces turbulence and allows for efficient blood flow.

Question 39

What type of valve is the mitral valve?

Answer 39

Chordal attachments are septo-phobic (no attachments to interventricular septum) ## Footnote This characteristic differentiates the mitral valve from the tricuspid valve.

Question 40

Fill in the blank: A right aortic arch can occur in approximately _____ of ccTGA.

Answer 40

18% ## Footnote ccTGA stands for congenitally corrected transposition of the great arteries.

Question 41

What is the significance of the suprasternal notch in relation to a right aortic arch?

Answer 41

A right aortic arch can occur in ~18% of ccTGA ## Footnote The suprasternal notch is an anatomical landmark used in various medical assessments.