congenital heart defects

Question 1

what condition its VSD most associated with?

Answer 1

fetal alcohol syndrome

Question 2

what condition is PDA most associated with?

Answer 2

congenital rubella

Question 3

what condition is ASD associated with?

Answer 3

ASD (ostium primum type) is associated with Down’s syndrome.

Question 4

what condition is transposition of the great vessels associated with?

Answer 4

maternal diabetes

Question 5

what condition is turners syndrome most associated with?

Answer 5

coarctation of the aorta -causes hypertension in upper extremities and weak pulses in the lower extremities.

Question 6

what happens in eisenmengers syndrome?

Answer 6

Left to right shunts (ASD, VSD, and PDA) will cause increased flow in the pulmonary circulation leading to pathologic remodelling of vasculature and pulmonary hypertension.

RVH occurs to compensate and the shunt is reversed (now right to left). This will present as late cyanosis, polycythaemia and clubbing.

Question 7

how is polycythaemia treated?

Answer 7

venesection

Question 8

how long does the PDA take to close in term babies?

Answer 8

72hrs

Question 9

what are the acyanotic heart conditions?

Answer 9

1. ventricular septal defect
2. atrial septal defect
3. coarctation of the aorta
4. patent ductus arteriosus

• all cause left to right shunting

Question 10

what are the cyanotic heart defects?

Answer 10

• tetralogy of Fallot
• transposition of the great arteries
  3, truncus arteriosus

Question 11

what type of murmur is present with atrial septal defect?

Answer 11

ejection systolic murmur, fixed splitting of S2- because of prolonged emptying of the right ventricle which delays pulmonic closure

Question 12

what type of murmur is present with ventricular septal defect?

Answer 12

pan-systolic murmurloudest at theleft lower sternal border

palpable thrill - larger VSD can cause parasternal heave and displaced apex beat

Question 13

what type of murmur is present with a patent ductus arteriosus?

Answer 13

continuous crescendo-decrescendo“machinery” murmur

Question 14

what is the effect of pulmonary hypertension on heart sounds?

Answer 14

Loud P2:loud second heart sound due to forceful shutting of the pulmonary valve

Question 15

what type of management is used for VSD when Eisenmenger’s is present?

Answer 15

closure not recommended and can use pulmonary vasodilators like sildenafil

Question 16

which type of ASD is the most common?

Answer 16

ostium secundum

Question 17

which type of ASD is Holt-Orman syndrome associated with?

Answer 17

ostium secundum ASD - characterised by skeletal abnormalities of the hands and arms and heart problems

Question 18

what ECG changes would be present in someone with ostium secundum ASD?

Answer 18

• RBBB

- right axis deviation

Question 19

what ECG changes would be present with ostium primum?

Answer 19

• RBBB
• LAD
• prolonged PR interval

Question 20

how can ASD cause stroke in someone with DVT?

Answer 20

ormally when patients have a DVT and this becomes an embolus, the clot travels to the right side of the heart, enters the lungs and becomes a pulmonary embolism. In patients with an ASD the clot is able to travel from the right atrium to the left atrium across the ASD. This means the clot can travel to the left ventricle, aorta and up to the brain, causing a large stroke.

Question 21

how can patent ductus arteriosus be treated?

Answer 21

IV indomethacin for 3-8 days- blocks the prostaglandins which are keeping the duct open

Question 22

what is the most common cyanotic defect?

Answer 22

tetralogy of Fallot

Question 23

which congenital heart defect is at particular increased risk of endocarditis?

Answer 23

• VSD- give prophylactic antibiotics when operating

Question 24

what are the 4 defects of tetralogy of Fallot?

Answer 24

1. pulmonary stenosis
2. right ventricular hypertrophy
3. overriding aorta
4. ventricular septal defect

pulmonary stenosis and ventricular hypertrophy increases the pressure in the right side and encourages blood through the VSD and into the overriding aorta

more deoxygenated blood going to the body causes cyanosis

Question 25

which defect will show boot shaped heart in CXR?

Answer 25

tetralogy of Fallot

Question 26

what are the 2 types of management for tetralogy of Fallot?

Answer 26

in neonates, a prostaglandin infusion can be used to maintain the ductus arteriosus. This allows blood to flow from the aorta back to the pulmonary arteries. Total surgical repair by open heart surgery is the definitive treatment, however mortality from surgery is around 5%.

Question 27

what is a TET spell?

Answer 27

babies who have tetralogy of Fallot will suddenly develop deep blue skin, nails and lips after crying or feeding, or when agitated

Question 28

how can a tet spell be managed?

Answer 28

1. older children can squat and can do knee to chest for babies- this increases vascular resistance and may encourage blood to go to pulmonary arteries 2. supplementary oxygen 3. beta blockers- relaxes right ventricle and can increase blood flow to pulmonary vessels 4. IV fluids- increases preload and volume of blood to pulmonary vessels 5. morphine- to decrease Respiratory drive and have more effective breathing 6. sodium bicarb- to buffer acidosis 7. phenyl epinephrine- increases systemic vascular resistance

Question 29

what happens in transposition of the great arteries?

Answer 29

- the aorta and the pulmonary artery are switched

Question 30

what are the 3 options for treating transposition of the great arteries?

Answer 30

A **prostaglandin** infusion can be used to maintain the **ductus arteriosus**. This allow blood from the aorta to flow to the pulmonary arteries for oxygenation. **Balloon septostomy** involves inserting a catheter into the **foramen ovale** via the umbilicus, and inflating a balloon to create a large **atrial septal defect**. This allows blood returning from the lungs (on the left side) to flow to the right side of the heart and out through the aorta to the body. **Open heart surgery** is the definitive management. A **cardiopulmonary bypass** machine is used to perform an “**arterial switch**” procedure within a few days of birth. If present, a VSD or ASD can be corrected at the same time.

Question 31

what is truncus arteriosus?

Answer 31

the aorta and pulmonary arteries fail to separate- oxygenated and deoxygenated blood mix

Question 32

what is ebsteins anomaly?

Answer 32

the tricuspid valve doesnt form propery - leading to regurg and right atria enlargememnt - can lead to palpatations and arrhythmias

Question 33

what is a risk factor for ebstiens anomaly?

Answer 33

lithium exposure

Question 34

is ebsteins anomally cyanotic or acyanotic?

Answer 34

cyanotic- causes right to left shunting