Congenital Heart Disease

Question 1

what is congenital heart disease?

Answer 1

= abnormality of structure of the heart

Question 2

when does congenital heart disease present?

Answer 2

= at birth

Question 3

what are the 4 levels to the spectrum of severity?

Answer 3

1) mild
2) moderate
3) severe
4) Major

Question 4

what does mild and moderate congenital heart disease present with?

Answer 4

MILD
= asymptomatic
= may resolve spontaneously
- may progress to moderate or severe in adulthood in some specific conditions

MODERATE
= requires specialist intervention and monitoring in cardiac centre

Question 5

what does severe and major congenital heart disease present with and requruie?

Answer 5

SEVERE
= presents severely ill/die in new-born period or early infancy

MAJOR
= requires surgery within first year of life

Question 6

how can congenital heart disease be picked up?

Answer 6

1) Screening
- antenatal
- newborn baby check

2) well baby with clinical signs

3) unwell baby;
= cyanosis
= shock
= cardiac failure

Question 7

when can congenital heart disease present?

Answer 7

1) Antenatally

2) Soon after birth
e. g. cyanosis

3) Day 1-2 baby check
4) Day 3-7
5) 4-6weeks

6) 6-8weeks
= GP check
= incidental finding of murmurs or other clinical contacts

Question 8

what could be find in a baby with congenital disease check in day 1-2

Answer 8

= murmur
= abnormal pulses
= cyanosis

Question 9

what could be find inn a baby with congenital heart disease in days 3-7?

Answer 9

= sudden circulatory collapse
= shock
= cyanosis
= sudden death

Question 10

what could be found in a baby with congenital heart disease in 4-6weeks?

Answer 10

= signs of cardiac failure
= reduced feeding
= failure to thrive
= breathessness
= sweatiness

Question 11

when is antenatal screening done?

Answer 11

= ultrasound at 18-22 weeks gestation

Question 12

what happens in an antenatal screen?

Answer 12

= 4 chambers heart view and outlaw tract view

Question 13

what should be given if there is a duct dependent lesion?

Answer 13

= prostaglandin infusion

Question 14

when should a clinical examination of a new born be done?

Answer 14

= 24hours of age

Question 15

what should a newborn clinical examination involve?

Answer 15

• femoral pulses
• heart sounds
• presence of murmur

= some includes measurement of pre and post ductal saturations

Question 16

what should a newborn screen be able to detect?

Answer 16

= murmur
= obvious cyanosis
= abnormal pulses

Question 17

give 4 examples of murmurs that cold be detected at baby checks?

Answer 17

1) small muscular VSD
2) murmur early in life
3) no haemo-dynamic consequences
4) may close spontaneously

Question 18

what does cyanosis do to the baby?

Answer 18

= blueish dicolouration

Question 19

what is cyanosis? (2)

Answer 19

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

Question 20

what are 3 differential diagnosis of cyanosis in newborns?

Answer 20

1) cardiac disease
2) respiratory disease
3) persistent pulmonary hypertension of the newborn (PPHN)

Question 21

what do cardiac diseased babies tend to present with?

Answer 21

= blue with little or no respiratory distress

- may have pre-post ducal differential

Question 22

what do respiratory disease babies present with?

Answer 22

= increased work of breathing

= X-ray changes

Question 23

what does PPHN usually present with?

Answer 23

= often seen inn unwell babies.

= large pre and post ductal differential

Question 24

when is the time of collapse when theres duct closure?

Answer 24

between 2-7days

Question 25

what happens when there is duct closure?

Answer 25

• cyanosis or pallor
• tachypnoea
• distress
• rapid deterioration to death

Question 26

what are clinical signs of duct closure?

Answer 26

• pallor
• prolonger CRT
• poor or absent pulses
• hepatomegaly
• crepitations
• increased work of breathing

Question 27

what do babies with duct closure become?

Answer 27

= profoundly acidotic

Question 28

what are 2 differential diagnosis of duct closure?

Answer 28

• sepsis

- metabolic conditions

Question 29

how would you treat duct closure?

Answer 29

• ABC = support airwave & breathings
• prostaglandin E2 to open duct
• multi-system support

Question 30

what are the 2 examples of duct dependent conditions?

Answer 30

1) duct dependent systemic circulation

2) duct dependent pulmonary circulation

Question 31

what causes duct dependent systemic circulation?

Answer 31

• hypo plastic left heart
• critical aortic stenosis
• interrupted aortic arch
• critical coarctation of aorta

Question 32

what causes duct dependent pulmonary circulation?

Answer 32

• tricuspid atresia

- pulmonary atresia

Question 33

what does the left to right shunt in cardiac failure do??

Answer 33

• increases pulmonary flow

- increases ventricular load

Question 34

when does cardiac failure tend to present?

Answer 34

= presents after a few weeks as pulmonary pressures drop

Question 35

what are the clinical signs of cardiac failure in babies?

Answer 35

• failure to thrive
• slow/reduced feeding
• breathlessness (especially when feeding)
• sweatiness
• hepatomegaly
• crepitations

Question 36

what sort of effect does a moderate or large ventricular septal defect have?

Answer 36

= big defect - less gradient
= often no murmur at baby check

• murmur develops as pulmonary pressure drops over first weeks
• increased pulmonary circulative, congestive cardiac failure

Question 37

what is the long term management of major congenital heart disease?

Answer 37

1) surgical management
- repair vs palliation

2) developmental problems
- hypoxia
- bypass time

3) need for further surgery
- valves, stenosis
- transplant

4) emotional/social issues

Question 38

how do you repair patent ductus arteriosus?

Answer 38

1) catheter procedure
- requires follow up to ensure flow stopped and device is in correct position

= go on to completely normal life

Question 39

how would you repair VSD?

Answer 39

= restores anatomical normality to heart

Question 40

how would you repair hyper plastic left heart syndrome (HLHS)?

Answer 40

• 3 stage complex surgery
• significant mortality at each stage and between
• ends with RV supplying systemic circulation
  = will fail over time and you will require a transplant