congenital heart disease - lecture

Question 1

what is CoHD?

Answer 1

an abnormality of the structure of the heart which is present at birth

Question 2

42% of ____ deaths attributable to congenital heart disease

Answer 2

42% of infant deaths attributable to congenital heart disease

Question 3

what indicates mild severity CoHD?

Answer 3

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

Question 4

give an example of a mild CoHD?

Answer 4

patent DA

Question 5

what indicates moderate severity CoHD?

Answer 5

requires specialist intervention and monitoring in a cardiac centre

Question 6

what indicates severe severity CoHD?

Answer 6

present severely ill/ die in newborn period or early infancy

Question 7

what indicates Major CoHD?

Answer 7

requires surgery within the first year of life.

Question 8

how do you screen for CoHD?

Answer 8

antenatal - detailed check

newborn baby check

Question 9

everyone Get an ultrasound at ___ - ____ weeks gestation

Answer 9

Get an ultrasound at 18-22 weeks gestation

Question 10

why is the USS pick up rate very variable?

Answer 10

• depends on who is doing it

Question 11

what is done if a problem is detected? 3

Answer 11

• there is the need for expert follow up/detailed echocardiography and counselling
• Increase in termination for major cardiac disease
• Choices around delivery

Question 12

why are outcomes of survival better if you detect chd anternatally?

Answer 12

i) avoid cardiovascular collapse
ii) minimise hypoxia
iii) improve condition at time of gioing to surgery

Question 13

the newborn baby check is a clinical examination at around __ hours of age

Answer 13

24

Question 14

what things do you do in the newborn baby check?

Answer 14

• Check femoral pulses, heart sounds and presence of murmurs
• Pre and post ductal saturations

Question 15

what could low overall sats for newborn baby check indicate?

Answer 15

cardiac or respiratory problem

Question 16

what does a difference of 3% or more between pre and post ductal saturations indicate?

Answer 16

right to left shunts at ductus

Question 17

babies may present well with clinical signs or unwell

Answer 17

.

Question 18

what are may unwell babies present with? 3

Answer 18

• cyanosis
• shock
• cardiac failure

Question 19

cyanosis- easy to miss in normal babies but also in non-caucasian babies. If in doubt - measure ___

Answer 19

sats

Question 20

what is cyanosis ?

Answer 20

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

Question 21

what indicates cardiac disease in a cyanosed baby?

Answer 21

• Cardiac babies tend to be blue with little or no respiratory distress.
• May have pre-post ductal differential

Question 22

what indicates respiratory disease in a cyanosed baby?

Answer 22

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

Question 23

what may cause persistent pulmonary hypertension of the newborn?

Answer 23

Large pre-post ductal differential.

Question 24

Another important cyanotic condition is total _____ ____ _______

Answer 24

Another important cyanotic condition is total anomalous pulmonary atresia

Question 25

what is total anomalous pulmonary atresia?

Answer 25

this is when the drainage of blood from the lungs comes to the right side rather than the left

Question 26

what is usually associated with cardiac failure of the newborn? 3

Answer 26

Usually seen with moderate to large left to right shunts

Question 27

give an example of a moderate to large left to right shunt

Answer 27

large VSD

Question 28

what does the left to right shunt cause?

Answer 28

increased pulmonary flow and increased ventricular load

Question 29

what are the clinical signs of caridac failure in babies?

Answer 29

• failure to thrive
• slow/reduced feeding
• breathlessness
• sweatiness
• hepatomegaly
• crepitations

Question 30

what are duct dependent lesions?

Answer 30

Any condition in which the pulmonary or systemic circulation is dependent on the patency of the ductus arteriosus

Question 31

if DDLs are not detected earlier what do they present with?

Answer 31

circulatory collapse when the duct closes

Question 32

Diagnosis before baby becomes unwell is very desirable in __ _____ ____

Answer 32

Diagnosis before baby becomes unwell is very desirable in duct dependent conditions

Question 33

what should the baby be commenced on and what should be done if a DDL is detected?

Answer 33

Commence low dose prostaglandin E2 immediately to maintain duct patency

Watch for apnoea but avoid intubation and ventilation if possible

Move to cardiac surgical centre

Question 34

what is the typical presentation if DDL goes undiagnosed?

Answer 34

• 3-5 day old infant suddenly pale,

- tachypnoeic and distressed

Question 35

what are the clinical signs of an undiagnosed DDL? 6

Answer 35

pallor, prolonged CRT, poor or absent pulses, hepatomegaly, crepitations, increased work of breathing

Question 36

undiagnosed DDL babies are profoundly acidotic, true/false

Answer 36

true

Question 37

what should the diff diagnosis be for undiagnosed DDL?

Answer 37

sepsis, metabolic conditions

Question 38

what is the treatment for DDL?

Answer 38

• ABC- support airway and breathing

- Prostaglandin E2 to open duct

Question 39

give some examples of duct dependent systemic circulation conditions

Answer 39

• hypoplastic left heart,
• Critical aortic stenosis,
• interrupted aortic arch
• critical coarctation of the aorta

Question 40

give some examples of duct dependent pulmonary circulation conditions

Answer 40

tricuspid atresia

pulmonary atresia