CVS 6 (Congenital heart disease)

Question 1

A shunt in which direction across the heart results in cyanosis?

Answer 1

Right to Left shunt

Question 2

Name 3 left-to-right cardiac shunts:

Answer 2

1) Atrial septal defect (ASD)
2) Ventricular septal defect (VSD)
3) Patent ductus arteriosus (PDA)

Question 3

Name 4 acyanotic obstructive lesions:

Answer 3

1) Aortic stenosis
2) Pulmonary stenosis
3) Coarctation of the aorta
4) Mitral stenosis

Question 4

Is an aortic stenosis cyanotic or acyanotic?

Answer 4

Acyanotic

Question 5

Is a pulmonary stenosis cyanotic or acyanotic?

Answer 5

Acyanotic

Question 6

Is coarctation of the aorta cyanotic or acyanotic?

Answer 6

Acyanotic

Question 7

Is a mitral stenosis cyanotic or acyanotic?

Answer 7

Acyanotic

Question 8

Name the 4 cyanotic shunts:

Answer 8

1) Tetralogy of Fallot
2) Transposition of the great arteries
3) Total anomalous pulmonary venous drainage (TAPVD)
4) Univentricular heart

Question 9

What is Total Anomalous Pulmonary Venous Drainage (TAPVD)?

Answer 9

• Pulmonary veins attach to right heart
• Oxygenated blood mixes with deoxygenated blood and enters left heart via ASD
• Deoxygenated blood enters systemic circulation
• Cyanotic

Question 10

Why does transposition of the great arteries cause cyanosis?

Answer 10

Deoxygenated blood will be pumped into aorta and the systemic circulation so not enough oxygen will reach respiring tissues

Question 11

An atrial septal defect will usually cause a shunt in which direction? Why?

Answer 11

Left-to-right

Pressure in left heart is higher than in the right heart, so blood moves from higher pressure to lower pressure

Question 12

VSD’s most commonly occur where?

Answer 12

In the membrane ventricular septum

Question 13

A ventricular septal defect will usually cause a shunt in which direction? Why?

Answer 13

Left-to-right

Pressure in left heart is higher than in the right heart, so blood moves from higher pressure to lower pressure

Question 14

Is an atrial or ventricular septal defect most likely to cause pulmonary hypertension?

Answer 14

Ventricular septal defect (VSD)

Question 15

What is the most common congenital heart defect associated with Down’s syndrome?

Answer 15

Atrioventricular septal defect

Question 16

What is an atrio-ventricular septal defect?

Answer 16

Defective endocardial cushion formation between both the atria and ventricles

• Atrial septal defect and ventricular septal defect
• Blood moves from left herat to right heart down pressure gradient

Question 17

What is an aortic stenosis?

Answer 17

Narrowing of the aortic valve

Question 18

What is coartation of the aorta?

Answer 18

Narrowing of the aorta, most commonly in the arch of the aorta

Question 19

Is a Tetralogy of Fallot a cyanotic disorder?

Answer 19

Yes

Question 20

Is transposition of the great arteries cyanotic or acyanotic?

Answer 20

Cyanotic

Question 21

Is Total Anomalous Pulmonary Venous Drainage (TAPVD) cyanotic or acyanotic?

Answer 21

Cyanotic

Question 22

Is a univentricular heart a cyanotic or acyanotic disease?

Answer 22

Cyanotic

Question 23

What is a Tetralogy of Fallot?

Answer 23

1) Pulmonary stenosis
2) Ventricular septal defect
3) Right ventricular hypertrophy
4) Over-riding aorta

Question 24

What is tricuspid atresia?

Answer 24

• Incomplete development of tricuspid AV valve

- No RV inlet

Question 25

How does blood flow to the lungs if patient has tricuspid atresia?

Answer 25

1) Via ventricular septal defect (VSD) | 2) Via patent ductus arteriosus (PDA)

Question 26

What is a 'hypoplastic left heart'?

Answer 26

Underdeveloped left ventricle

Question 27

How does blood flow from the heart into the systemic circulation if patient has a hypoplastic left heart?

Answer 27

From the right ventricle into the pulmonary trunk, through a patent ductus arteriosus (PDA) into the aorta

Question 28

2 shunts are obligatory for survival with a hypoplastic left heart. What are they?

Answer 28

1) Patent ductus arteriosus (Right-to-left) | 2) Patent foramen ovale (Left-to-right)

Question 29

Is a hypoplastic left heart a cyanotic or acyanotic disease?

Answer 29

Cyanotic

Question 30

What is meant by 'transposition of the great arteries'?

Answer 30

1) Right ventricle is connected to the Aorta (instead of the pulmonary trunk) 2) Left ventricle is connected to the pulmonary trunk (instead of the aorta)

Question 31

How does a baby with transposed great arteries survive during utero?

Answer 31

In utero tissue is oxygenated via the placenta, not via the lungs.

Question 32

What is pulmonary atresia?

Answer 32

Underdeveloped pulmonary valve = no RV outlet

Question 33

Which 2 shunts are obligatory for survival with pulmonary atresia?

Answer 33

1) Atrial septal defect | 2) Patent ductus arteriosus

Question 34

What is the main complication associated with a ventricular septal defect?

Answer 34

Pulmonary hypertension

Question 35

Name the 4 congenital heart defects which present as neonatal emergencies:

Answer 35

1) Hypoplastic left heart 2) Transposition of great vessels 3) Pre-ductal coarctation of the aorta 4) Pulmonary atresia