Congenital Heart Disease & Foetal Circulation Flashcards

1
Q

Describe how the inclusion of the placenta changes how the foetal organs function in comparison to an adult body?

A

The placenta plays a role in
- Gas exchange/ Nutrition/ Waste excretion

=> lungs are fluid filled and unexpanded
=> liver has little role in nutrition and waste management
=> gut is not in use

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2
Q

How is the placenta joined to the foetal circulation?

A

Foetal heart pumps blood to the placenta via:
- umbilical arteries (FOETUS TO MUM)

Blood from the placenta flows to the foetus via:
- umbilical vein (MUM TO FOETUS)

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3
Q

What 3 shunts are specific for foetal life and why?

A

Ductus venosus
Foramen Ovale
Ductus Arteriosus

  • needed as the oxygenated blood needs to be distributed from the RHS of the heart
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4
Q

Where is the ductus venosus found and what is its role?

A
  • Connects umbilical vein to the IVC

=> blood flows INTO foetus from MUM and takes this route to the heart

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5
Q

Where is the foramen ovale located and what is its job?

A

Opening in atrial septum connecting RA to LA

=> oxygenated blood can easily flow into the LV and be distributed around the baby’s body

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6
Q

What is the function of the ductus arteriosus and where is it found?

A
  • Connects pulmonary bifurcation to the aorta

- allows more O2 blood to be shunted to the systemic circ. rather than travelling to immature lungs

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7
Q

What allows the ductus arteriosus to remain patent?

A
  • circulating prostaglandin E2

- this is produced by the placenta

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8
Q

Describe how the foetal circulation changes in the first few minutes after birth?

A
  • Pulmonary Vasc. Resistance DECREASES
  • Baby breathes in – lungs expand => lower pressure
  • More of the cardiac output sent to lungs
  • Systemic Vasc. Resistance INCREASES
  • Cord clamped and cut
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9
Q

What forces the foramen ovale to close?

A
  • Systemic vascular resistance increases

=> LA pressure increases and eventually exceeds RA pressure and closes the flap

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10
Q

The foramen ovale can remain open in what percentage of people?

A

Up to 35%

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11
Q

A patent foramen ovale is a risk in what conditions?

A

Stroke

Migraine

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12
Q

How long does it take for the ductus arteriosus to close after birth?

A
  • Functional closure within hours to days
  • Anatomical closure within 7-10 days
  • Ends up as fibrous ligament
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13
Q

What prompts the closure of the patent ductus arteriosus

A
  • Decreased flow due to DECREASED pulmonary vasc. resistance
  • Decreased prostaglandins (PGE2) due to increased lung metabolism
  • Shunt becomes bidirectional then left to right before closing
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14
Q

The ductus arteriosus can remain patent in certain infants. TRUE/FALSE?

A

TRUE

- very common in preterm infants

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15
Q

How can a patent ductus arteriosus be treated?

A
  • wait and see
  • NSAIDs
  • surgery
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16
Q

Some congenital heart diseases, e.g. Aortic Interruption, cause a “duct dependent circulation”. How do we keep the duct open to maintain their circulation?

A

IV prostaglandin E2

- used to keep the duct open until an alternative shunt established or definitive surgery carried out

17
Q

What is meant by Persistent pulmonary hypertension of the newborn (PPHN)?

A

Failure of adaptation from foetal to adult circulation
=> Lung vascular resistance fails to fall
=> Shunts remain
=> Blue baby

18
Q

How is PPHN investigated?

A

Pre and post ductal Oxygen sats

  • large difference between these
  • Pre = R hand and Post = L foot
19
Q

How is PPHN treated?

A
  • Ventilation
  • oxygenation
  • inhaled nitric oxide
  • ECLS
20
Q

Describe the spectrum of congenital heart disease severity.

A

Mild – asymptomatic, may resolve spontaneously OR may progress
e.g. small VSD, ASD, PDA, PFO or bicuspid aortic valve

Moderate – specialist intervention and monitoring in a cardiac centre
e.g. aortic valve disease, larger complex ASD/VSD

Severe – present severely ill, die in newborn period/ early infancy

MAJOR – surgery within 1st year of life

21
Q

How does congenital heart disease present?

A
  • At Screening (Antenatal/ Newborn baby check)
  • Well baby with clinical signs
  • Unwell baby
    => Cyanosis/ Shock/ Cardiac failure
22
Q

When can congenital heart disease present?

A
  • antenatally
  • Soon after birth => Cyanosis
  • Day 1-2 => Murmurs, abnormal pulses, cyanosis
  • Day 3-7 => circulatory collapse, shock, cyanosis, death
  • 4-6 weeks => cardiac failure - poor feeding, failure to thrive, SOB, sweaty
  • 6-8 week GP check => incidental murmur
23
Q

An incidental murmur heard at a newborn baby check may not be pathological. TRUE/FALSE?

A

TRUE
- May be a small muscular VSD
=> Causes a murmur early in life
=> No consequences, Many close spontaneously

24
Q

What is cyanosis and what causes this?

A
  • condition where mixed oxygenated and deoxygenated blood enters the systemic circulation from the heart
  • causes bluish discolouration
25
Q

What are the main differential diagnoses for cyanosis in a newborn baby?

A
  • Cardiac disease
  • Respiratory disease
  • Persistent pulmonary hypertension of the newborn (PPHN)
26
Q

How can you attempt to work out the cause for a baby’s cyanosis?

A

Cardiac babies = blue with little or no respiratory distress

Respiratory = associated with increased work of breathing, X-Ray changes

PPHN = Large pre-post ductal differential

27
Q

What circulations are considered as “duct dependent”?

A

Duct dependent systemic:

  • Hypoplastic left heart
  • critical aortic stenosis
  • interrupted aortic arch
  • critical coarctation of aorta

Duct dependent pulmonary:

  • Tricuspid atresia
  • Pulmonary atresia
28
Q

What is pulmonary atresia?

A

Pulmonary trunk from RV does not form properly

- PDA still exists therefore blood backflows from aorta to pulmonary arteries

29
Q

What clinical signs indicate failure in babies?

A
  • Failure to thrive
  • Slow / reduced feeding
  • Breathlessness (especially when feeding)
  • Sweatiness
  • Hepatomegaly
  • Crepitations
30
Q

What are the consequences of a moderate -> large VSD?

A
  • often no murmur at baby check
  • Murmur develops as pulmonary pressures drop over first weeks
  • Increased pulmonary circulation
  • congestive cardiac failure
31
Q

What is involved in the longer term management of major cardiac diseases?

A

Surgery

  • Repair vs palliation
  • Valves, stenosis
  • Transplant
  • Developmental problems may occur
  • Hypoxia
32
Q

How is a PDA repaired surgically?

A
  • catheter procedure
  • uses device to close this
  • device is left in and followed up on1
33
Q

How is VSD repaired surgically?

A
  • with a patch
34
Q

How is Hypoplastic left heart treated?

A
  • 3 Stage complex surgery
  • Significant mortality at each stage and between
  • Ends with RV supplying systemic circulation
  • Will fail over time
  • Then Transplant is required
35
Q

What happens in Transposition of the Great Vessels?

A

LV connected to pulmonary trunk
RV connected to Aorta
=> baby becomes cyanotic