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Question 1

How many umbilical vessels are there usually?

Answer 1

2 umbilical arteries

1 umbilical vein

Question 2

Path of umbilical artery

Answer 2

Internal iliac artery to Placenta

Question 3

After birth, umbilical arteries become

Answer 3

Medial umbilical ligaments

Question 4

Role of umbilical artery

Answer 4

Blood flows from fetus to placenta

Question 5

High/low Flow and Resistance in placenta?

Answer 5

High flow, Low resistance

Question 6

High/low Flow and Resistance in lungs?

Answer 6

High resistance, Low flow

Question 7

Path of umbilical vein

Answer 7

Placenta to liver/portal vein and ductus venosus to IVC

Question 8

After birth, umbilical vein becomes

Answer 8

Ligamentum teres

Question 9

Role of ductus venosus

Answer 9

Shunt oxygenated blood from UmbV to IVC (to heart and rest of body)

Question 10

After birth, ductus venosus becomes

Answer 10

Ligamentum venosum

Question 11

Role of foramen ovale

Answer 11

Shunt mixed/oxygenated blood from Right heart to Left heart to bypass the lungs and to the baby’s body

Question 12

After birth, foramen ovale becomes

Answer 12

Fossa ovalis

Question 13

Which closes first, foramen ovale or ductus arteriosus?

Answer 13

Foramen ovale (soon after birth)
Ductus arteriosus (approx 2 days)

Question 14

Role of ductus arteriosus

Answer 14

Shunt mixed/oxygenated blood from Pulmonary Artery to the Aorta to bypass the lungs

Question 15

After birth, ductus arteriosus becomes

Answer 15

Ligamentum arteriosum

Question 16

Ductus arteriosus constricts/closes due to which 2 factors;

Answer 16

High O2 and Low prostaglandins

Question 17

Fetal pulmonary circulation has high resistance due to

Answer 17

Hypoxic pulmonary vasoconstriction

Question 18

After birth, umbilical vessels have high/low Flow and Resistance?

Answer 18

High resistance (vessels constrict) and hence low flow

Question 19

Why does foramen ovale close

Answer 19

First breath leads to reduced pressure of Right heart and increased pressure of Left heart

Question 20

Drug to keep holes patent (maintains patency)

Answer 20

Prostaglandins

Question 21

Drug to close holes

Answer 21

Indomethacin (PGE2 antagonist NSAID)

Hence you don’t give pregnant women NSAIDs as they can cause premature closure

Question 22

List the 3 Acyanotic > Obstructive conditions

Answer 22

Coarctation of Aorta
Aortic stenosis
Pulmonary stenosis

Question 23

List the 4 Acyanotic > L to R Shunt conditions

Answer 23

Atrial septal defect
Ventricular septal defect
Patent ductus arteriosus
Atrioventricular septal defect

Question 24

Septal defects are acyanotic or cyanotic

Answer 24

Acyanotic

Question 25

How to remember cyanotic conditions

Answer 25

5 blue TsHirts | 5 T lesions from 1-5

Question 26

List 6 cyanotic conditions

Answer 26

5 Ts and an H 1. Truncus arteriosus (1 vessel) 2. Transposition of Great Arteries TGA (2 vessels) 3. Tricuspid Atresia (3 valve cusps) 4. Tetralogy of Fallot ToF (4 components) 5. Total Anomalous Pulmonary Venous Connection TAPVC (5 words) 6. Hypoplastic left heart syndrome (HLHS) Others: - Ebstein's anomaly - Pulmonary atresia - Interrupted Aortic Arch (IAA)

Question 27

Turner Syndrome and Bicuspid aortic valve assoc with?

Answer 27

Coarctation of aorta

Question 28

2 day old female Suddenly shocked Weak/absent femoral pulse Discolouration of lower limbs compared to upper limbs Continuous/late systolic murmur over thoracic spine

Answer 28

Coarctation of aorta (ductus arteriosus closes at 2 days --> shock) Murmur due to collaterals Radio-femoral delay or difference in blood pressures, cardiomegaly in older children

Question 29

Ex findings of coarctation of aorta

Answer 29

``` Signs of Turner syndrome Shocked Radio-femoral delay Maybe radial-radial delay High BP in arms, low BP in legs Cardiomegaly ```

Question 30

CXR shows rib notching in posterior third of ribs 3-8 | Aorta shows "Figure 3 sign"

Answer 30

Coarctation of aorta Rib notching due to erosion by large collateral intercostal arteries Figure 3: pre- and post-coarctation dilation

Question 31

Rx of coarctation of aorta

Answer 31

If severe, immediate prostaglandin infusion (patent ductus arteriosus) Surgical correction of constricted part (or balloon angioplasty)

Question 32

William's syndrome

Answer 32

Supravalvular aortic stenosis

Question 33

Older child Ejection systolic murmur Syncope, angina and dyspnea Acyanotic

Answer 33

Aortic stenosis | leads to LV hypertrophy

Question 34

Rx for symptomatic aortic stenosis

Answer 34

Balloon valvuloplasty | Valve replacement later in life

Question 35

Noonan syndrome and ejection systolic murmur | Acyanotic

Answer 35

Pulmonary stenosis | leads to RV hypertrophy

Question 36

Pathophys of ASD

Answer 36

Left to Right shunt | Increased flow/volume through R heart and lungs

Question 37

Usually asymptomatic Recurrent chest infections Ejection systolic murmur at left upper sternal border Wide fixed splitting of S2 Acyanotic Dilated pulmonary artery with hilar congestion on CXR

Answer 37

ASD | increased flow across pulmonary valve, leading to delayed closure

Question 38

Risk of embolism

Answer 38

ASD

Question 39

Rx of ASD

Answer 39

If

Question 40

``` 4-6weeks old Recurrent URTIs Poor feeding Tachypnoea Sweating Failure to thrive Harsh pansystolic murmur at left lower sternal border Radiating to axilla and across praecordium Loud P2 Heart failure ```

Answer 40

VSD Louder in smaller defects (more turbulence) Large VSD (aka greater than aortic valve) can lead to heart failure by 2 months old

Question 41

Pathophys of VSD

Answer 41

Left to Right shunt | Increased flow/volume through lungs and Left heart

Question 42

CXR: pulmonary plethora but otherwise normal. | In more severe cases: cardiomegaly, pulmonary oedema, pleural effusion

Answer 42

VSD

Question 43

Rx of VSD

Answer 43

Small VSDs close spontaneously, give proph ABx before surgeries, maintain good dental hygiene. Large VSD: Support feeds: High caloric milk via NG tube Heart failure: Frusemide and ACEi Surgical closure by 12-18months

Question 44

Hx of VSD

Answer 44

``` 4-6weeks old recurrent URTIs Poor feeding Tachypnoea Sweating Failure to thrive ```

Question 45

Ex of VSD

Answer 45

``` Acyanotic FTT Harsh pansystolic murmur at left lower sternal border, radiating to axilla or across praecordium Loud P2 Lungs: pulm edema/crackles Hepatomegaly ```

Question 46

Most common congenital heart defect overall

Answer 46

VSD

Question 47

Most common cyanotic congenital heart defect

Answer 47

ToF

Question 48

Proportion of VSD that will close spontaneously

Answer 48

3/4 (75%)

Question 49

When is ductus arteriosus defined as patent

Answer 49

Failure to close by 1 month after expected date of delivery | normally closes within first couple of days

Question 50

Congenital rubella syndrome assoc with

Answer 50

Patent Ductus Arteriosus

Question 51

Premature likely to have which acyanotic condition?

Answer 51

PDA (hasn't had time to close yet)

Question 52

``` 1 month old, or premature baby Continuous "machinery" murmur at clavicle Bounding femoral pulse (and collapsing) Pulmonary oedema Wide pulse pressure ```

Answer 52

Patent Ductus Arteriosus

Question 53

Rx PDA

Answer 53

Premature: indomethacin | Term infants: catheter or surgical closure

Question 54

Down Syndrome assoc with

Answer 54

Atrioventricular septal defect

Question 55

Atrioventricular septal defect involves a large defect of the

Answer 55

Atrial and ventricular septums | endocardial cushion defect

Question 56

``` Trisomy 21 Dyspnea Poor feeding Sweating May be cyanotic May have a murmur ```

Answer 56

AVSD

Question 57

What is Eisenmenger's syndrome?

Answer 57

A complication of CHD when a L>R shunt leads to pulmonary HTN (Right Ventricular hypertrophy and Pulmonary Artery hypertrophy), causing a SHUNT REVERSAL hence a R>L shunt. Becomes cyanotic.

Question 58

How long after does Eisenmenger's syndrome occur

Answer 58

10-15 yeras later

Question 59

``` Cyanosis in a previously acyanotic patient Clubbing Heart failure Death New early diastolic murmur ```

Answer 59

Eisenmenger's syndrome Murmur from Pulmonary regurgitation

Question 60

Rx of Eisenmenger's syndrome

Answer 60

Heart-lung transplant

Question 61

When do cyanotic lesions often present

Answer 61

After natural closure of ductus arteriosus

Question 62

Differentiating between the causes of 'cyanosis'

Answer 62

``` Anaemia Respiratory (Hyperoxia test, give baby 100% O2 for 10 minutes, if sats do not rise then cardiovascular) Cardiovascular (Pre-ductal O2 sats from RIGHT HAND vs Post-ductal O2 sats from a foot. If these are different, can indicate PDA is being used to maintain sats in a cyanotic R>L shunt) ```