Congential Heart conditions

Question 1

What is congenital heart disease?

Answer 1

a gross structural abnormality of the heart or intrathoracic great vessels that is actually or potentially of functional significance
present at birth

Question 2

What is a major congenital heart disease?

Answer 2

A defect which will require surgery within the first year of life

Question 3

What are the potential problems to seeing the babies heart in utero?

Answer 3

• maternal obesity

- the way the baby’s sitting

Question 4

What are the benefits of antenatal screening?

Answer 4

Can start prostaglandin infusions if they have a duct dependent lesion
can deliver in a cardiac surgical centre
support from a expert team and ability to plan for the family

Question 5

What is carried out in newborn cardiac screening?

Answer 5

• clinical exam at 24 hours
• femoral pulses, heart sounds and presence of murmurs
• some regions also do measurement of pre and post ductal saturations
• will detect murmurs, obvious cyanosis and abnormal pulses

Question 6

What is the significance of a small muscular VSD?

Answer 6

there will be a murmur early in life but it will have no haemodynamic consequences
Many of these close over spontaneously

Question 7

What causes cyanosis (bluish discolouration)?

Answer 7

any condition causing deoxygenated blood to bypass the lungs and enter the systemic circulation or any condition where mixed oxygenated and deoxygenated blood enters the systemic circulation from the heart

Question 8

What is transposition of the great arteries?

Answer 8

When the aorta goes into the RV and pulmonary artery goes into the LV.
What keeps these babies alive is a the gap between the atria mixing the blood.
ultimately need surgery to fix the anatomy of the heart

Question 9

When does the ductus arteriosis close in duct dependent circulation?

Answer 9

between 2 and 7 days

Question 10

How does a duct dependent baby present when the duct closes?

Answer 10

Severe cyanosis or pallor,
tachypnoea, distress,
rapid deterioration to death
Clinical signs
- pallor,
- prolonged CRT, 
- poor or absent pulses,
- hepatomegaly,
- crepitations,
- increased work of breathing
Profoundly acidotic
(Differential diagnosis includes sepsis, metabolic conditions)

Question 11

Examples of duct dependent conditions

Answer 11

Duct dependent systemic circulation

Duct dependent pulmonary circulation

Question 12

When will babies with cardiac failure present?

Answer 12

6-8 weeks

Question 13

What are the clinical signs of heart failure in babies?

Answer 13

Failure to thrive
Slow / reduced feeding
Breathlessness (especially when feeding)
Sweatiness
Hepatomegaly
Crepitations

Question 14

What is patent ductus arteriosus repair?

Answer 14

• Catheter procedure
• follow up appointments to ensure floww has stopped and the device is in the right place
• discharged from cardiology

Question 15

How is hypoplastic left heart syndrome managed?

Answer 15

• 3 complex surgerys each with high mortality
• in the end rhe RV will be doing the job of the LV
• it will fail over time and a transplant will be needed later in life

Question 16

Give some examples of mild congenital heart conditions in babies

Answer 16

• asymptomatic, may resolve spontaneously or progress to moderate or severe in adulthood depending on the condition
• small VSD
• Patent Foramen ovale
• Patent ductus arteriosus
• bicuspid aortic valve

Question 17

Give some examples of moderate congenital heart conditions in babies

Answer 17

require specialist intervention and monitoring in a cardiac centre

• mild or moderate aortal stenosis or pulmonary stenosis
• larger ASD and VSD

Question 18

Give some examples of severe congenital heart conditions in babies

Answer 18

Present severely ill/die in newborn period or early infancy

• cyanotic lesions
• duct dependent lesions
• truncus arteriosis