Congenital Heart And Other Defects

Question 1

Process of primitive heart tube formation

Answer 1

Cardiogenic mesoderm -> angiogenic cell clusters -> left and right endocardial tubes -> primitive heart tube

Question 2

What is each endocardial tube continuous with

Answer 2

Dorsal aorta
Vitelloumbilical vein

Question 3

What types of folding occurs in the primitive heart tubes

Answer 3

Cranial folding
Lateral folding

Question 4

When does fusion of the 2 endocardial tubes to form the primitive heart tube finish

Answer 4

Day 21

Question 5

What does the primitive myocardium form from

Answer 5

Mesoderm from foregut

Question 6

When does the heart start to beat

Answer 6

Day 22

Question 7

What causes the foramen ovale to close at birth

Answer 7

Left atrium pressure becomes higher than right atrium pressure

Question 8

What does the heart septum form from

Answer 8

Septum primum
Septum secundum

Question 9

How does the outflow tract of the heart partition

Answer 9

Neural crest forms bulbar ridges which grow and spiral

Question 10

How many pairs of aortic arch arteries sprout from the aortic sac

Answer 10

5 (1-6 but 5 disappears)

Question 11

What type of congenital heart defect is linked to migraines

Answer 11

Septal defects

Question 12

Does a septal defect cause left right or right left shunting

Answer 12

Left right

Question 13

How can septal defects be closed with

Answer 13

Fabric
Pericardium

Question 14

Outflow tract defects

Answer 14

Persistent truncus arteriosus
Transposition of the great vessels
Tetralogy of fallot
Coarctation of aorta

Question 15

Persistent truncus arteriosus

Answer 15

Pulmonary artery starts some distance above undivided truncus

Question 16

What causes persistent truncus arteriosus

Answer 16

Truncoconal swellings fail to fuse

Question 17

Transposition of the great vessels cause

Answer 17

Truncoconal septum straight instead of spiralled

Question 18

Most common cyanotic heart defect

Answer 18

Transposition of great vessels

Question 19

What causes tetralogy of fallot

Answer 19

Unequal separation of conus

Question 20

4 characteristic features of tetralogy of fallot

Answer 20

Pulmonary trunk stenosis
ventricular septal defect
Overriding aorta
Right ventricular hypertrophy
VORP

Question 21

2 types of coarctation of the aorta

Answer 21

Pre ductal - before ductus arteriosus
Post ductal - after ductus arteriosus

Question 22

How can the ductus arteriosus be kept open in preductal coarctation of the aorta pts

Answer 22

Alprostadil - prostaglandin E1

Question 23

Which type of coarctation of the aorta causes collateral circulation development

Answer 23

Post ductal

Question 24

How can coarctation of the aorta be surgically treated

Answer 24

End to end flap anastomosis
Subclavian flap
Balloon angioplasty

Question 25

Fetal circulation connections

Answer 25

Foramen ovale Ductus arteriosus Ductus venosus

Question 26

Is resistance higher in the fetal pulmonary circulation or systemic circulation

Answer 26

Pulmonary

Question 27

How long does the ductus arteriosus remain open after birth

Answer 27

24-48hrs Fully within 1 wk

Question 28

How long does the foramen ovale stay open after birth

Answer 28

Closes almost immediately

Question 29

Persistent pulmonary hypertension of the newborn

Answer 29

Elevated pulmonary vascular resistance resulting in right to left shunting of blood and hypoxemia

Question 30

Causes of persistent pulmonary hypertension of the newborn

Answer 30

Parenchymal lung disease Lung hypoplasia Idiopathic

Question 31

Persistent pulmonary hypertension of the newborn treatment

Answer 31

Manage underlying cause Mechanical ventilation Inhaled NO Prostaglandin - PGE1/PGI2 Sildenafil Milrinone

Question 32

Patient colour in cyanotic and Acyanotic defects

Answer 32

Cyanotic - blue Acyanotic - pink

Question 33

neural tube defects

Answer 33

Failure of normal closure of neural tube

Question 34

Examples of neural tube defects

Answer 34

Spina bifilar occulta Meningocoele Myelomeningocoele - spina bifida Encephalocoele Anencephaly

Question 35

Effects of Myelomeningocoele

Answer 35

Loss of bladder control - Incontinence/urinary retention Fecal incontinence Paralysis Loss of sensation in legs

Question 36

Meningocoele

Answer 36

Sac of CSF comes through opening in back

Question 37

MyeloMeningocoele

Answer 37

Sac of CSF containing spinal cord and nerves comes through opening in back

Question 38

Myelomeningocoele treatment

Answer 38

Closure - decr infection Closure in utero - improve outcome

Question 39

Hydrocephalus treatment

Answer 39

VP shunt

Question 40

Gastrischisis

Answer 40

Full thickness defect in abdominal wall lateral to umbilicus allowing bowel to extend outside body

Question 41

Gastroschisis treatment

Answer 41

Reduction and surgical closure

Question 42

Which side of the umbilical cord does gastroschisis occur

Answer 42

Right

Question 43

How is the bowel stopped from drying out in gastroschisis

Answer 43

Put in plastic bag

Question 44

Exomphalos

Answer 44

Failure of umbilicus to close completely combined with failure of mid gut to herniate back into abdo cavity fully Intestines inside membrane

Question 45

Difference between exompahlos and gastroschisis

Answer 45

Exomphalos - bowel covered by membranes of umbilical cord

Question 46

Why must babies with Exomphalos be delivered by c section

Answer 46

Membranes around bowel may burst during vaginal delivery

Question 47

Cleft lip cause

Answer 47

Failure of maxilla to fuse with medial nasal prominence

Question 48

Cleft lip

Answer 48

Cleft between filtrum and rest of upper lip between central and lateral incisors

Question 49

Cleft palate cause

Answer 49

Failure of palatial shelves to fuse