Embryology - Congenital Heart Disease

Question 1

What is congenital heart disease?

Answer 1

The abnormality of the structure of the heart present at birth. Slide 5

Question 2

How can congenital heart disease be noticed?

Answer 2

Through screening antenatal and newborn baby check.
If a well baby presents with clinical signs e.g. murmur
Unwell baby with clinical signs e.g. cyanosis, cardiac failure or shock. Slide 9

Question 3

In weeks 4-6 what can present?

Answer 3

Cardiac failure: reduced feeding, failure to thrive, breathlessness, sweatiness. Slide 10

Question 4

What is antenatal screening?

Answer 4

An ultrasound at 18-22 weeks gestation and gives a 4 chamber heart view.
The sensitivity can vary as there are many confounding factors e.g. obesity of mother Slide 11

Question 5

What is newborn screening and what is the downfall of it?

Answer 5

Clinical examination at around 24hrs of age.
Check femoral pulses, heart sounds and presence of murmurs.

Only finds heart problems that present at that time, some present weeks later and will no be picked up at 24hrs of age. Slide 14

Question 6

The smaller a musclular ventricular septal defect is the quieter it is nad you hear it later on in life. True or false?

Answer 6

False.

The smaller a VSD is the louder it is due to turbulence and are heard earlier than larger VSDs. Slide 16

Question 7

If a cardiac baby is cyanosed, what is different to a respiratory cyanosed baby?

Answer 7

They tend to have no respiratory distress if they are cardiac cyanosed whereas respiratroy usually hs increased work of breathing. Slide 19

Question 8

What is the most common cyanotic condition that is a cardiac problem?

Answer 8

Transposition of the great vessels.
Where there are 2 circulations working independantly and the blood from the lungs doesn’t mix with the blood to the body apart from through a shunt. Slide 20

Question 9

If babies have a duct dependent ciruclation and the duct closes how do they present?

Answer 9

Between 2-7 days with severe cyanosis, tachypnoea, distress.
If there is no help then they rapidly deteriorate to death.
They will have poor or absent pulses, crepitations and extremely acidotic. Slide 21

Question 10

What is the treatment if babies have a duct dependent ciruclation and the duct closes?

Answer 10

ABC
Prostaglandin E2 to open duct
Multisystem supportive treatment
Transfer to cardiac surgical centre. Slide 22

Question 11

What is duct dependent systemic or pulmonary circulation and what are examples?

Answer 11

Systemic is when there is an obstruction of the outflow from the LV.
e.g. Hypoplastic left heart.
Pulmonary is when there is an obstruction of the outflow from the RV.
e.g. Pulmonary atresia.
Slide 23

Question 12

What are the clinical signs of cardiac failure in babies?

Answer 12

Failure to thrive
Slow/reduced feeding
Breathlessness (esp. when feeding)
Sweatiness
Hepatomegaly
Crepitations. Slide 27

Question 13

What is the longer term problems of major congenital heart diseases?

Answer 13

Surgical (palliation?)
Developmental problems
Need for further surgery
Emotional/social issues. Slide 29

Question 14

What can be done to close a patent ductus arteriosus?

Answer 14

Employ a device through a catheter procedure to block it off. Slide 30

Question 15

How can a VSD be repaired?

Answer 15

Through a patch to close the hole and will require follow up as they grow incase it becomes too small. Slide 31