Congenital heart disease

Question 1

What is congenital heart disease?

Answer 1

Abnormality of the structure of the heart, present at birth that is potentially of functional significance

Question 2

What is mild congenital heart disease?

Answer 2

asymptomatic, may resolve spontaneously (may progress to moderate or severe in adulthood in some specific conditions)

Question 3

Name some mild congenital heart diseases

Answer 3

Small VSD, PFO, small ASD, small PDA, Bicuspid aortic valve may progress in adulthood to severe AS or AR and need surgery

Question 4

What is moderate congenital heart disease?

Answer 4

require specialist intervention and monitoring in a cardiac centre

Question 5

Name some moderate congenital heart diseases

Answer 5

mild or moderate AS, PS. Larger or complex ASD, VSD

Question 6

What is severe congenital heart disease?

Answer 6

present severely ill/die in newborn period or early infancy

Question 7

Name some severe congenital heart diseases

Answer 7

all cyanotic lesions, all duct dependent lesions, truncus

Question 8

What is major congenital heart disease?

Answer 8

Requires surgery within first year of life

Question 9

When are congenital heart diseases screened for?

Answer 9

Screening
>antenatal
>new born check

Question 10

What are the two common presentations of CHD?

Answer 10

Well baby with clinical signs

Unwell baby

• cyanosis
• shock
• cardiac failure

Question 11

What does CHD present as soon after birth?

Answer 11

Cyanosis

Question 12

What does CHD present as on day 1-2?

Answer 12

Murmurs, abnormal pulses, cyanosis

Question 13

What does CHD present as on days 3-7?

Answer 13

Sudden circulatory collapse, shock, cyanosis, sudden death (usually duct dependant circulations)

Question 14

What does CHD present as in weeks 4-6?

Answer 14

• Signs of cardiac failure - reduced feeding, failure to thrive, breathlessness, sweatiness
• typical age of shunt presentation

Question 15

What does CHD present as in weeks 6-8?

Answer 15

GP check, incidental finding of murmurs at other clinical contacts

Question 16

When is antenatal screening performed?

Answer 16

18-22 weeks gestation

Question 17

Why is the sensitivity so variable?

Answer 17

-operator experience and training
-maternal characteristics
>obese mother
-baby
>moving a lot
lying in wrong position

Question 18

What are the benefits of antenatal screening?

Answer 18

HDLD- may choose to teminate pregnancy?

Birth at specialist unit

Set up palliative care before birth

Question 19

What is the problem with newborn screening?

Answer 19

will detect any condition causing a murmur, obvious cyanosis or abnormal pulses; only at that point in time, a lot of heart conditions will not immediately produce these signs until a few days later

Question 20

Why are murmurs common in newborn babies?

Answer 20

the heart adapts, pressure changes, duct constriction. less than half of the babies are pathological.

Question 21

What causes cyanosis?

Answer 21

any condition allowing deoxygenated blood to bypass the lungs and enter the systemic circulation

any condition where mixed oxygenated and deoxygenated blood enters the systemic circulation from the heart

Question 22

What is the Ddx of cyanosis?

Answer 22

cardiac disease
-tend to be blue with little or no respiratory distress, me have pre-post ductal differential

respiratory disease
-causes usually associated with increased work of breathing, x-ray changes

PPHN
-often seen in otherwise very unwell babies. large pre-post ductal differential

Question 23

What is the most common cyanotic condition?

Answer 23

Transposition of the great arteries

Question 24

What happens to the circulation in transposition of the great vessels?

Answer 24

Two circulations working completely independent of each other

Question 25

Where is there a shunt in transposition of the great vessels?

Answer 25

Across the FO and maybe at the duct

Question 26

What does transposition of the great vessels present as?

Answer 26

Very very blue babies from beginning

Not compatible with life unless there is mixing somewhere

Question 27

What determines the management in transposition of the great vessels?

Answer 27

the atrial hole

Question 28

What is the immediate management of atrial hole in transposition of the great vessels?

Answer 28

thread a chatherer through femoral or umbilical, into RA, across into the left atrium, inflate a balloon and yank it back. allowing baby to be pinker before they get the surgery

Question 29

What does surgery do in transposition of the great vessels?

Answer 29

Puts the heart back to anatomically normal structure

Question 30

What happens when a baby with a duct dependant circulation has the duct closed?

Answer 30

• baby will die very quickly when it closes

- pallor, grunting. poor perfusion, increased work of breathing

Question 31

What are the signs of collapse at duct closure?

Answer 31

pallor, prolonged CRT, poor or absent pulses, hepatomealy, crepitations, increased work od breathing

Question 32

What will happen progressively with collapse at duct closure?

Answer 32

progressively worse, more acidotic, lactate with build up

Question 33

What is the Ddx of collapse at duct closure?

Answer 33

Sepsis, metabolic condition

Question 34

What is the management of collapse at duct closure?

Answer 34

• ABCDE management
• if cardiac disease suspected give PGE2
• multisystem supportive treatment
• transfer to cardiac management centre

Question 35

What are some examples of duct dependant circulations?

Answer 35

Duct dependant systemic circulations
>hypoplastic left heart, critical aortic stenosis, interrupted aortic arch, critical coarctation of the aorta

Duct dependant pulmonary circulation
>tricuspid atresia
>pulmonary atresia
>critical pulmonary stenosis