Structural Heart defects

Question 1

What is the most commonly inherited heart defect?

Answer 1

Bicuspid aortic valve

Question 2

What does a bicuspid aortic valve mean?

Answer 2

Will degenerate faster than a normal tricuspid aortic valve and will become regurgitative earlier

Question 3

What structure does an atrial septal defect involve?

Answer 3

Patent foramen ovale

Question 4

Will a patient with an atrial septal defect be cyanotic?

Answer 4

No as blood will be shunted from the left side back to the right

(Unless severe and causes Eisenmenger syndrome and shunt reverses)

Question 5

An atrial septal defect may cause overload in the ___ _______ and lead to ___ _______ __________

Answer 5

RHS circulation
right ventricular hypertrophy

Question 6

In severe cases of atrial septal defect, the RVH can cause pulmonary hypertension. This can cause a r_____ of s____

Answer 6

reversal of shunt

Question 7

What is it called when there is a reversal of the shunt in atrial septal defect so blood is going from the right atria straight to the left atria due to the pulmonary hypertension?

Answer 7

Eisenmenger syndrome

Question 8

In Eisenmenger syndrome in severe cases of atrial septal defect, ________ blood is pumped systemically

Answer 8

deoxygenated

Question 9

In an older patient with atrial septal defect there is more shunting causing what symptom?

Answer 9

Dyspnoea

Question 10

How is an atrial septal defect diagnosed?

Answer 10

ECHO

Question 11

How is atrial septal defect treated?

Answer 11

Sometime there is spontaneous closure
Otherwise closure via cardiac catheterisation/ heart surgery

Question 12

What direction is the shunt in Ventricular septal defect?

Answer 12

Left to right (non-cyanotic shunt)

Question 13

What is there a risk of in ventricular septal defect?

Answer 13

Eisenmenger syndrome (shunt reverses due to pulmonary hypertension) and RVH

Question 14

A small ventricular septal defect is often asymptomatic.
A large ventricular septal defect can cause…

Answer 14

Exercise intolerance
Failure to thrive
Harsh pan systolic murmur

Question 15

How is a ventricular septal defect diagnosed?

Answer 15

ECHO

Question 16

What is treatment for ventricular septal defect?

Answer 16

Sometimes there is spontaneous closure
Can have closure via heart surgery or cardiac catheterisation

Question 17

Describe an atrioventricular septal defect

Answer 17

Hole down middle of heart, no atrial or interventricular septum

Question 18

What is atrioventricular septal defect associated with?

Answer 18

Down’s syndrome

Question 19

What can atrioventricular septal defect cause?

Answer 19

Dyspnoea
Exercise intolerance
Eventually Eisenmenger’s syndrome

Hard to treat

Question 20

Where is a patent ductus arteriosus?

Answer 20

Opening between the aorta and pulmonary artery which fails to close after birth

Question 21

Describe the blood shunt that occurs in a patent ductus arteriosus

Answer 21

Blood shunts from the aorta to the pulmonary trunk.
This increases risk of pulmonary overload and hypertension which leads to Eisenmenger’s syndrome

Question 22

What are symptoms of patent ductus arteriosus?

Answer 22

Dyspnoea
Failure to thrive
Machine-like murmur

Question 23

How is a patent ductus arteriosus diagnosed?

Answer 23

CXR
ECG
ECHO (gs)

Question 24

How is patent ductus arteriosus treated?

Answer 24

A prostaglandin inhibitor like indomethacin may induce closure
Otherwise consider surgery

Question 25

What is the congenital heart condition involving 4 specific abnormalities occurring together called?

Answer 25

Tetralogy of Fallot

Question 26

What are the 4 abnormalities in Tetralogy of Fallot?

Answer 26

Ventricular septal defect
Overriding aorta
RVH
RV outflow obstruction (pulmonary stenosis)

Question 27

What is an overriding aorta?

Answer 27

A congenital heart defect where the aorta is directly over a ventricular septal defect rather than over the LV

This means some deoxygenated blood from RV can enter systemic circulation

Question 28

Does Tetralogy of Fallot cause cyanosis?

Answer 28

Yes

Question 29

What is a characteristic CXR finding in Tetralogy of Fallot?

Answer 29

Boot shaped heart

Question 30

How is Tetralogy of Fallot diagnosed?

Answer 30

ECHO
CXR showing boot shaped heart

Question 31

What position do infants with Tetralogy of Fallot often acquire?

Answer 31

Knee to chest squatting position

Increases preload and afterload (by forcing blood from legs to return to heart) so improves cyanosis

Question 32

What is it called when a baby with Tetralogy of Fallot suddenly develops deep blue/grey skin, nails and lips?

Answer 32

Tet spells

Often happens when baby cries, eats or is upset. Caused by rapid hypoxia

Question 33

What is treatment for Tetralogy of Fallot?

Answer 33

Full surgical repair within 2 years of life
Then have good prognosis

Question 34

Where is a coarctation of aorta?

Answer 34

When the aorta narrows at/just distally to the ductus arteriosus (hole between aorta and pulmonary artery)

Question 35

What happens in a coarctation of aorta?

Answer 35

Blood is diverted massively through aortic arch branches so there is increased perfusion to the upper body compared to lower body

Question 36

What are symptoms of coarctation of aorta?

Answer 36

Scapular bruits (due to hypertension in collaterals)
Upper body hypertension

Question 37

How is coarctation of aorta diagnosed?

Answer 37

CXR shows “notched ribs” due to dilated intercostal vessels

CT angiogram

Question 38

How is coarctation of aorta treated?

Answer 38

Surgical repair or stenting