Paediatric Cardiology - Septal Defects and Vascular Malformation/Defect

Question 1

What are the 3 main types of ventricular septal defects?

Answer 1

• subaortic (near the valve)
• perimembranous
• muscular (near apex)

Question 2

What happens to blood flow in a ventricular septal defect?

Answer 2

can have a left to right shunt

Question 3

What kind of murmur can sometimes be heard with a ventricular septal defect ad where can it be heard?

Answer 3

• pansystolic murmur, sometimes with thrill

- lower left sternal edge

Question 4

What differences in sound of the VSD murmur might you hear depending on the size of the defect?

Answer 4

very small VSDs may present with early systolic murmur

very large VSDs may have a diastolic rumble due to relative mitral stenosis and lots of blood coming through pulmonary circulation

Question 5

What can happen to the heart if large VSDs result in cardiac failure?

Answer 5

biventricular hypertrophy and pulmonary hypertension

Question 6

What is Eisenmenger Syndrome?

Answer 6

development of pulmonary hypertension due to large VSD and left to right shunt of blood results in reversal of shunt i.e. blood flows from right to left

Question 7

How can we treat a VSD?

Answer 7

if very small sometimes can leave alone, otherwise:

• amplatzer device via catheter to close it
• patch closure (Involves open surgery)

Question 8

How are atrial septal defects generally picked up?

Answer 8

• tend to be few clinical signs in early childhood so can often be an incidental finding and with good chance of spontaneous closure
• sometimes detected in adulthood with AF, heart failure or pulmonary hypertension

Question 9

How might you detect an atrial septal defect on auscultation?

Answer 9

wide fixed splitting of 2nd heart sound

Question 10

How can atrial septal defect be treated (if it is required)?

Answer 10

atrial septal defect occlusion device in situ (via catheter)

Question 11

What chromosomal abnormality os atrioventricular-septal (AVSD) defect associated with?

Answer 11

trisomy 21 (Down’s Syndrome)

Question 12

What anatomical defect occurs in AVSD?

Answer 12

• have singular atrioventricular valve (tricuspid and mitral valve are fused) - this is a complete AVSD
• can have varying severities

Question 13

How would pulmonary stenos present tif it was mild stenosis?

Answer 13

asymptomatic

Question 14

How would severe pulmonary stenosis present?

Answer 14

moderate and severe exertional dyspnoea and fatigue

Question 15

Where can you hear the pulmonary stenosis murmur and what does it sound like?

Answer 15

• upper left sternal border

- ejection systolic murmur with radiation to back

Question 16

How is severe pulmonary stenosis treated in children?

Answer 16

balloon valvoplasty to palliate until past puberty and can replace valve

Question 17

How can aortic stenosis present in children?

Answer 17

mostly asymptomatic, if severe then may have reduced exercise tolerance, exertional chest pain, syncope

Question 18

Where ca aortic stenosis best be heard and what does it sound like?

Answer 18

• upper right sternal border with radiation to carotids

- ejection systolic murmur (gets louder and the quiet again)

Question 19

How can you treat severe aortic stenosis in children?

Answer 19

balloon valvuloplasty and later valve replacement

Question 20

Explain foetal blood circulation.

Answer 20

oxygenated, nutrient rich blood from mum—–umbilical vein in cord—-baby IVC—baby RA—foramen ovale—-left atrium—-left ventricle—aorta—blood flow to heart and brain—blood return via SVC to RA—2/3rds blood to left atrium, 1/3 to lungs to keep the growing lungs nourished and oxygenated (but still not functional)—-is also ductus arteriosus which connects the pulmonary trunk to the aorta to help with high to let shunt of blood to bypass the lungs—waste removed by return of deoxygenated, nutrient deplete blood back to umbilical arteries and to mother

Question 21

What changes happen to the foetal circulation at birth?

Answer 21

• pulmonary vascular resistance falls
• pulmonary blood flow rises
• systemic vascular resistance is increased
• ductus arteriosus closes
• foramen ovale closes
• ductus venosus closes

Question 22

In what group of infants is a patent ductus arteriosus very common?

Answer 22

pre-term infants

Question 23

How can you treat patent ductus arteriosus in prettier babies?

Answer 23

• fluid restriction/ diuretics
• prostaglandin inhibitors e.g. IV ibuprofen
• surgical ligation e.g. using umbrella device

Question 24

How would treatment of patent ductus arteriosus differ in term babies?

Answer 24

there is good chance of spontaneous closure within a year and the defect is not prostaglandin sensitive

Question 25

What is coarctation of the aorta and where does it commonly occur?

Answer 25

• narrowing of the aortic wall

- tends to occur where the ductus arteriosus enters into the aorta

Question 26

How can you detect coarctation of aorta?

Answer 26

• radio-femoral delay
• MRI
• echocardiography

Question 27

How would you manage coarctation of the aorta if the child presented collapsed and in shock?

Answer 27

• reopen patent ductus arteriosus with prostaglandin infusion
• may resect area of coarctation with end-to-end anastomoses
• balloon aortoplasty

Question 28

What is transposition of the great arteries?

Answer 28

when the aorta comes off the right ventricle and the pulmonary trunk comes off the left ventricle

Question 29

What are the 4 features of Fallot’s tetralogy?

Answer 29

• VSD
• pulmonary stenosis
• right ventricular hypertrophy
• aorta over-riding the VSD

Question 30

What are signs of Fallot’s tetralogy?

Answer 30

most common cyanotic congenital heart defect

• cyanosis
• dyspnoea
• faints
• clubbing
• thrills
• harsh systolic murmur at left sternal base

Question 31

What sign might you see on x ray of tetralogy of fallot?

Answer 31

‘boot’ shadow of heart