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Embryology and Congenital Heart Disease Flashcards

1
Q

what layer is the heart derived from?

A

visceral mesoderm

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2
Q

What type of folding brings the heart to its final position?

A

Cranio-Caudal folding

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3
Q

Name the 4 heart tube regions from top to the bottom

A

Truncus Arteriosus
Bulbus Cordis
Ventricle
Atrium

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4
Q

what heart tube region is responsible for aorta and pulmonary trunk?

A

truncus arteriosus

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5
Q

what heart tube region is responsible for RV and outflow of both ventricles?

A

bulbus cordis

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6
Q

what heart tube region is responsible for LV?

A

ventricle

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7
Q

what heart tube region is responsible for both atria?

A

Atrium

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8
Q

How many dilatations does the heart tube develop?

A

5

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9
Q

What is the direction of BF through the primitive heart?

A

caudal to cranial

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10
Q

What are the right and left horns called in atrium?

A

sinus venosus

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11
Q

d: sinus venosus

A

smooth part R atrium

Coronary sinus

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12
Q

What happens to atrium heart tube in looping and folding?

A

becomes dorsal and cranial

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13
Q

What happens to ventricle heart tube in looping and folding?

A

displaced left

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14
Q

What happens to bulbus Cordis heart tube in looping and folding?

A

inferiorly, ventrally to right

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15
Q

f: the placenta in utero (8)

A 
Fetal homeostasis
Gas exchange
Acid base balance
Nutrient transport to fetus
Waste product transport from fetus
Hormone production
Transport of IgG
PGE2
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16
Q

How does the circulation differ in the fetus?

A 
The placenta is included in the circulation:
Gas exchange
Nutrition
Waste excretion
Homeostasis
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17
Q

How do the lungs differ in the fetus?

A

they are unexpanded and fluid filled

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18
Q

How does the liver differ in the fetus?

A

has little role in nutrition and waste management

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19
Q

Is the gut in use in the fetus?

A

no

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20
Q

Describe fetal circulation route?

A

placenta to the R side of the heart to brain and body back to placenta etc

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21
Q

What arteries does the foetal heart use to pump blood to the placenta?

A

umbilical

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22
Q

How does blood from the placenta return to the foetus?

A

via umbilicial vein

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23
Q

How does oxygenated, nutrient rich blood return from the placenta to the right side of the heart and get distributed to the growing foetus?

A

3 “shunts” specific to foetal life:
Ductus venosus
Foramen Ovale
Ductus Arteriosus

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24
Q

what does the ductus venosus do?

A

connects the umbilical vein to the inferior vena cava

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25
what does the foramen Ovale do?
opening in atrial septum connecting RA to LA
26
What does the ductus arteriosis do?
connects pulmonary bifurcation to descending aorta
27
WHere does the ductus venosus carry the majority of placental blood to and how?
straight into IVC bypassing portal circulation
28
where does the foetus get its nutrients?
placenta
29
Name 2 things the foramen ovale allows?
Allows blood to flow from right to left atrium Allows the best oxygenated blood to enter left atrium then on to LV, ascending aorta, carotids Membrane flap on left atrium side
30
what percentage of blood goes to the lungs in foetus? where does the rest go and how?
7% | The rest goes via ductus arteriosus to join descending aorta
31
How is patency maintained? how is this produced?
maintained by circulating prostaglandin E2 produced by placenta
32
what happens immediately after baby born?
Massive changes in the few minutes following birth Baby inflates lungs and cries Goes from blue to pink Cord clamped and cut
33
describe the circulatory changes after birth?
``` Pulmonary Vascular Resistance decreases: -breath in - lungs -physically expand increased SV -cord clamped and cut more CO to the lungs foramen ovale close ```
34
why does the foramen ovale close?
As PVR falls and SVR rises the LA pressure exceeds the RA pressure The flap is pushed closed
35
Describe the adaption of the ductus arteriosus after birth?
Functional closure within hours to days Anatomical closure within 7-10 days Ends up as fibrous ligament – ligamentum arteriosum
36
How does the ductus arteriosus constrict after birth?
Decreased flow due to decreased pulmonary vascular resistance Increased pO2 – oxygen sensitive muscular layer Decreased circulating PGE2 due to increased lung metabolism Shunt becomes bidirectional then left to right
37
What babies more commonly have failure to close from ductus arteriosus?
preterms
38
treatments for failure of duct to close?
include wait and see, NSAIDs and surgery
39
How can duct be kept open for duct dependent circulation until surgery carried out?
IV prostaglandin E2 can be used to keep the duct open until an alternative shunt established
40
what happens to pulmonary resistance after birth?
continues to drop until reaches normal adult levels by 2-3months
41
Name some of the consequences failures of adaptation
Persistent pulmonary hypertension of the newborn More likely in sick babies Sepsis Hypoxic ischaemic insult Meconium aspiration syndrome Cold stress Can be related to underlying anatomical abnormality such as congenital diaphragmatic hernia
42
pathophysiology for Persistent pulmonary hypertension in newborn's(PPHN)?
Lung vascular resistance fails to fall Shunts remain Right to left flow at PFO Right to left flow at PDA
43
What colour are babys with PPHN?
BLUE
44
large/small difference between pre and post ductal O2 sats?
large
45
treatment of PPHN?
Ventilation, oxygenation, high systemic blood pressure, inhaled nitric oxide, ECLS
46
d: congenital heart disease
Abnormality of the structure of the heart or intrathoracic great vessels Present at birth
47
How do you find congenital heart disease?
``` Screening -Antenatal -Newborn baby check Well baby with clinical signs Unwell baby -Cyanosis -Shock -Cardiac failure ```
48
describe the 3 grades of congenital heart disease?
Mild = asymptomatic, Moderate = specialist intervention, Severe = present severely ill, Major = Surgery in 1st year
49
Symptoms of congenital HD?
Unwell baby, cyanosis, shock, cardiac failure
50
What is done to predict congenital defects?
antenatal screening
51
d: blue baby syndrome
Cyanosis (blue baby) – anything where heart allows deoxygenated blood to enter circulation
52
name some of the differntial diagnoses for blue baby syndrome?
Differential: cardiac disease, resp. disease, Persistent Pulmonary Hypertension of the Newborn (PPHN)
53
Name the most common newborn cyanosis presenting example
transposition of great vessels | pulmonary and aortic vessels swapped
54
clinical signs of failure in babies?
- Failure to thrive - Slow/ reduced feeding - Breathlessness - Sweatiness - Hepatomegaly - Crepitations
55
what is hypoplastic left heart?
left side of the heart underdeveloped
56
what is pulmonary atresia and what is it coupled with?
underdeveelopment of right ventriclular outflow | often with VSD
57
What is VSD?
ventricular septal defect
58
treatment of duct closure issues?
ABC – support airway and breathing as necessary Prostaglandin E2 to open duct Multisystem supportive treatment Transfer to cardiac surgical centre for definitive management
59
name the duct dependent systemic circulation?
Hypoplastic left heart, critical aortic stenosis, interrupted aortic arch, critical coarctation of aorta
60
name the duct dependent pulmonary circulation
Tricuspid atresia | Pulmonary atresia
61
is there an initial murmur for large VSD?
Often no murmur at baby check | Murmur develops as pulmonary pressures drop over first weeks
62
if theres a big VSD what is the gradient more/less?
less
63
how is patent ductus arteriosus repaired?
cathether procedure
64
What does VSD repair require?
surgery
65
HLHS requires what for treatment?
3 Stage complex surgery Significant mortality at each stage and between Ends with RV supplying systemic circulation Will fail over time Transplant

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