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Paediatric Cardiology > Congenital defects > Flashcards

Congenital defects Flashcards

1
Q

What is a Glenn shunt?

A

Stage 2 of Norwood procedure - direct connection between SVC and RPA for venous flow to lungs.

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2
Q

What condition is the Norwood procedure for?

A

Hypoplastic left heart syndrome
Tricuspid atresia
Double outlet right ventricle

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3
Q

In what spectrum does Hypoplastic left heart syndrome belong?

A

Hypoplastic left heart is in the spectrum of single ventricle defects that has a sequence of staged repairs.

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4
Q

Identify the different stages that can be involved

A
  1. Norwood - neonatal repair
  2. Bidirectional glenn repair - 6 months
  3. Fontan (3-4 yrs)
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5
Q

What is hypoplastic left heart syndrome?

A

Malformation of the LV (small) and aorta = can’t pump blood to the body

so the other ventricle (RV) not only pumps blood to the lungs, but also to the body.

Commonly, also have an ASD = mixing of blood

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6
Q

What occurs as a result of Hypoplastic left heart syndrome?

A

RV now has to pump blood to lungs and body via PDA (kept open by meds) = increased RV workload

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7
Q

Why is the ASD important in hypoplastic left heart syndrome?

A

ASD allows for left to right shunting of blood = mixing of oxy and deoxy blood.

Means RV also pumps little oxygenated blood to body via PDA

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8
Q

Why would a catherisation be important in hypoplastic left heart syndrome?

A

To make ASD bigger = more oxygenated blood to right side to be pumped to body

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9
Q

What is the purpose of the Norwood procedure?

A

Let’s the RV pump blood to body

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10
Q

What happens during the Norwood procedure?

A

Build a new larger aorta - bottom of PA joined to Ao = Ao goes from RV to body

Create a shunt to get blood to lungs = because PA now goes to body

Close the PDA

Make a bigger ASD = more oxygenated blood to RV

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11
Q

What kind of shunts can be used for a Norwood procedure?

A

Blaylock-Taussig-Thomas shunt
Sano shunt

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12
Q

What is the purpose of a Blaylock-Taussig-Thomas shunt?

A

moves blood from (and through) the aorta to the lungs.

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13
Q

What is the purpose of a Sano shunt?

A

moves blood from RV to PA then to lungs.

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14
Q

What does the Glenn procedure do?

A

Before Glenn - RV pumping blood to lungs and body = hard work

After Glenn: blood from upper body goes directly to lungs so RV doesn’t have to pump as hard

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15
Q

Why is a Glenn procedure required?

A

Because the shunts placed during the Norwood procedure require a more permanent solution

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16
Q

What happens during a Glenn procedure?

A

SVC disconnected from heart and connected to RPA = blood from upper body flows directly to PA into lungs

Shunt is removed, if Norwood procedure was performed

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17
Q

What is the purpose of the Fontan procedure?

A

No more mixing of oxygenated and deoxygenated blood

18
Q

What is the outcome of the Fontan procedure?

A

The single ventricle:
only pumps blood to the body
only pumps blood with high oxygen to the body

19
Q

What happens during the Fontan procedure?

A

IVC disconnected from heart and connected to PA via conduit

Fenestration (small hole) made between conduit and RA = allows blood to still blood into heart

20
Q

What is the purpose of the fenestration made during Fontan procedure?

A

Prevents too much blood from flowing to lungs right away giving time to adjust.

Can be closed later during cath case

21
Q

What is aortic stenosis?

A

when the aortic valve (the valve between the left ventricle and the aorta) is too small, narrow, or stiff.

22
Q

What happens in aortic stenosis?

A

aortic valve controls direction of flow.

In aortic stenosis valve is narrow and small = cannot open all the way = LV has to work harder to push blood against abnormal valve = weakens heart

23
Q

Treatment for aortic stenosis

A

Ballon valvuloplasty - balloon dilation of valve

Valve replacement - artificial or donor replacement of valve

24
Q

What consideration are taken in choosing treatment for aortic stenosis?

A

the location and amount of the narrowing
age and size
how well the other valves in the heart are working
previous heart surgery
other medical conditions

25
Q

What is an atrial septal defect (ASD)?

A

an abnormal opening in the dividing wall between the atria

26
Q

What is the anatomy of CCTGA?

A

RV (weaker side) pumps to body
LV (stronger side) pumps to right side & lungs

  • the RV was not built to last as long as the LV
27
Q

What % of patients with CCTGA need pacemakers for complete heart block?

A

45%

28
Q

How the RV affect prognosis of a person with CCTGA?

A

The ability of the RV to pump will determine how well a person with CCTGA is

29
Q

Type of treatment for CCTGA is dependant on what?

A

Type and severity of symptoms
Associated defects

30
Q

What are the most common problems in CCTGA?

A

VSD
Pulmonary stenosis
Leaky tricuspid valve
Heart block

31
Q

What is the issue with ASD?

A

Left to right shunting of blood = increased blood flow to lungs

32
Q

What considerations are taken when placing a PA band?

A

length of PA is sufficient to not impinge the Pulmonary valve, coronary arteries proximally or branch PAs distally.

33
Q

What can be seen upon comparison of the MPA and the aorta during pulmonary overcirculation? Are there any risks involved?

A

MPA is larger in size than Ao
MPA can be thinned by dilatation
= increased risk of tearing

34
Q

What is the pathophysiology of pulmonary overcirculation?

A

congenital defects with left to right shunting and unrestricted pulmonary flow due to drop in pulmonary vascular resistance

35
Q

What is the consequence of pulmonary over circulation in the first year of life?

A

medial hypoertrophy of pulmonary arteriolds and fixed pulmonary hypertension

36
Q

In patients with cardiac defects, what is the benefit of PA banding?

A

reduces shunt volume = improves systemic pressure and cardiac output

Decreased pulmonary blood flow = decreased blood to LV = improved LV function

36
Q

What is the purpose of pulmonary banding?

A

creates a narrowing or stenosing of MPA = decreased blood flow to branch PAs = reduced pulmonary blood flow and PA pressure

37
Q

In what patients is PA banding not tolerated?

A

patients with cardiac defects that depend on mixing of blood to maintain adequate Sats.

38
Q

What what patients can PA banding be indicated?

A

VSDs
Coarctation of Ao
Single ventricle defects (tricuspid atresia)
Cardiac defects requiring homograft conduit

39
Q
A

Paediatric Cardiology (4 decks)

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