Congenital Heart Disease

Question 1

What is meant by congenital heart disease?

Answer 1

A gross structural abnormality of the heart or intra-thoracic great vessels that is actually or potentially of functional significance

Question 2

What are some examples of mild congenital heart diseases?

Answer 2

Small Ventricular Septal Defect
Patent Foramen Ovale
Small Atrial Septal Defect
Small Patent Ductus Arteriosus

Question 3

What are some examples of moderate congenital heart diseases?

Answer 3

Large/Complex Atrial Septal Defect
Large Ventricular Septal Defect

Question 4

What are some examples of severe congenital heart diseases?

Answer 4

All cyanotic lesions
All duct dependant lesions

Question 5

How do congenital heart defects usually present?

Answer 5

Through screening tests
Cyanosis
Shock
Cardiac failure

Question 6

When is antenatal screening performed?

Answer 6

Between weeks 18 and 22

Question 7

What is performed during antenatal screening?

Answer 7

An ultrasound of the 4 chambers of the heart

Question 8

When is collapse at duct closure most likely to occur?

Answer 8

2-7 days

Question 9

What are some symptoms of duct closure in duct dependant congenital heart disease?

Answer 9

Severe cyanosis or pallor
Tachypnoea - fast respiratory rate
Distress
Collapse
Rapid deterioration to death

Question 10

What are some treatment options for duct closure in duct dependant congenital heart diseases?

Answer 10

ABC treatment
Prostaglandin E2 IV
Multi-System support
Definitive opening of the ductus arteriosus

Question 11

What are some duct dependant systemic circulation conditions?

Answer 11

Hypoplastic left heart
Critical aortic stenosis
Interrupted aortic arch
Critical correction of the aorta

Question 12

What are some examples of duct dependant venous circulation conditions?

Answer 12

Tricuspid atresia
Pulmonary atresia

Question 13

What occurs in hypoplastic left heart syndrome?

Answer 13

The left ventricle becomes narrow as the wall is stiff
It also results in a narrowed aorta
This means that most of the blood to the aorta comes from the ductus arteriosus
It also results in a hole in the atrial wall that allows oxygenated blood to move into the right ventricle and into the aorta via the ductus arteriosus

Question 14

What can be done to the hole in hypoplastic left heart syndrome?

Answer 14

A balloon can be inflated in the hole in the atrial wall and increase its diameter, meaning more oxygenated blood can mix with the deoxygenated blood in the right ventricle

Question 15

What are some clinical signs of newborn cardiac failure?

Answer 15

Failure to thrive
Reduced feeding
Breathlessness
Hepatomegaly
Crepitations

Question 16

What is the difference between repair and palliation in the long term management of congenital heart disease?

Answer 16

Repair - Returning the heart to normal anatomy and restoring function
Palliation - Returning function to the heart but not to normal heart anatomy

Question 17

How is patent ductus arteriosus surgically treated?

Answer 17

Catheter procedure to occlude the ductus arteriosus
Follow up appointments to show movement of stent

Question 18

How is a large ventricular septal defect treated surgically?

Answer 18

A bypass is provided during surgery, while a patch is placed over the defect to block it off

Question 19

What are the risks of patching a ventricular septal defect?

Answer 19

It can obstruct the bundle of His and cause rhythm problems
As the heart grows, the patch can put strain on the valves which can cause regurgitation later on

Question 20

How is hypoplastic left heart syndrome treated surgically?

Answer 20

It involves a 3 stage complex surgery that ends with the right ventricle supplying the systemic circulation, the right atrium being connected directly to the pulmonary artery and not the right ventricle
This is known as Total Cavo-Pulmonary Circulation

Question 21

Which congenital heart disease is most common in trisomy 21?

Answer 21

Atrio-ventricular septal defect