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Paediatric cardiology > Congenital key morphology > Flashcards

Congenital key morphology Flashcards

1
Q

Saturations in all limbs in: Normal cardiac anatomy and physiology

A

Right upper: normal
Left upper: normal
Right lower: normal
Left lower: normal

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2
Q

Saturations in all limbs in: Normal cardiac anatomy and persistent pulmonary hypertension in the newborn

A

Right upper: normal
Left upper: mildly low
Right lower: mildly low
Left lower: mildly low
(pHTN forces mixing of blue blood from pulmonary artery into aorta)

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3
Q

Saturations in all limbs in: d-TGA anatomy with pulmonary hypertension

A

Right upper: very low
Left upper: mildly low
Right lower: mildly low
Left lower: mildly low
(pHTN forces mixing of red blood from closed pulmonary loop into aorta)

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4
Q

Saturations in all limbs in: d-TGA anatomy without pulmonary hypertension

A

Right upper: very low
Left upper: very low
Right lower: very low
Left lower: very low
(low pulmonary pressures mean that red blood from pulmonary closed loop does not mix into the aorta - full cyanosis)

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5
Q

Cynanotic congenital heart diseases

A

Tetralogy of fallot
TGA
Truncus arteriosus
TAPVR
Tricuspid or pumonary atresia

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6
Q

Acyanotic obstructive left-sided diseases

A

Aortic stenosis
CoA

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7
Q

Acyanotic non-obstructive shunt lesions, pre-tricuspid

A

ASD

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8
Q

Acyanotic non-obstructive shunt lesions, post-tricuspid

A

VSD
PDA
AP window

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9
Q

3 indications for prostin in neonates

A

Duct dependant systemic circulation (left sided duct dependent lesions)
Duct dependant pulmonary circulation (right sided duct dependant lesions)
TGA

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10
Q

Dosing of prostin

A

Depends on clinical presentation
Guided by echo and blood investigations

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11
Q

While on prostin, monitor for…

A

Apnoea (less likely with doses <15ng/kg/min, usually happen within 1 hr)/
Profound bradycardia
Severe hypotension

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12
Q

Consideration in critical care transfer of babies to Level 1 centres

A

Coordination with cardio / transport team
Consider elective intubation
Ensure good vascular access
Network guidelines

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13
Q

Univentricular circulation explanation

A

Mainly one ventricle supporting both pulmonary and systemic circulation - functionally univentricular

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14
Q

Examples of univentricular circulation

A

HLHS, tricuspid atresia, Unbalanced AVSD, DORV

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15
Q

Management principles for univentricular circulations

A

Duct open
Norwood / PA banding ->
BDGS ->
Fontan ->

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15
Q

Aetiology of congenital heart disease

A

8% genetic (5% chromosomal, 3% single gene)
3% environmental (drugs, toxins, infection, radiation, alcohol)
89% unknown (multifactorial, polygenic)

15% concordance in monozygotic twins

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15
Q

Properly describe normal cardiac morphology

A

Usual atrial arrangement
Concordant atrioventricular connections
Concordant ventriculoarterial connections
No associated lesions

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15
Q

Valve positioning in a structurally normal heart

A

Important to remember that the valves are in different planes in the normal heart
- Pulmonary valve is the most superior, and it is in a coronal plane (horizontal)
- Aortic and mitral valve face anteriorly and leftward, and are side to side with the aorta medial and mitral lateral
- Tricuspid valve is the most inferior, and also faces anteriorly and is more vertically orientated

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16
Q

Normal morphology of RA

A

o Triangular large atrial appendage
o Systemic vena cava come into the back of the right atrial chamber
o Pectinate muscles and terminal crest
o Fossa ovalis
o Coronary sinus

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17
Q

Normal morphology of LA

A

o Finger like LAA; narrow junction with atrial chamber
o Four pulmonary veins
o Smooth walled with narrow junction to LAA

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18
Q

Morphologic features of the RV

A

o Tricuspid valve
o Separated by muscle to pulmonary valve
o Characteristic septal leaflet with characteristic cordae attaching to septum
o Septal marginal traberculation (septal band)
o Moderator band comes off the septal marginal traberculation, crosses RV cavity
o RV has coarse apical traberculations when compared to those found on the left

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19
Q

Morphologic features of LV

A

o Fine apical traberculations
o Smooth septal aspect, no septal muscles or chordae
o Two groups of papillary muscles both attaching close to one another
o Aortic-mitral fibrous continuity

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20
Q

Morphologic features of intraventricular septum

A

o Small part that does not contain muscle – membranous septum
o The tricuspid valve septal leaflet attachment attaches directly to ventricular septum, the hinge line of this septal leaflet crosses the membranous septum in two portions – one above the hinge line in the RA, and one below in the RV

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21
Q

What can you see on short axis view of TTE

A

 RV and LV
 Can obtain one image capturing part of the TV and AV; MV just below this, can see the pulmonary trunk on the side

22
Q

What can you see in parasternal long axis in TTE

A

 LA to LV; LV to Ao; RV on the side
 Might be able to capture the papillary muscles in transection

23
Q

What can you see in apical view in TTE

A

o Visualizes membranous septum well – in 4ch plane where Ao is in the 5Ch
o We can see papillary muscle attachments / moderator band

24
Q

What can you see in subcostal view in TTE

A

o Can obtain 4ch and 5ch: can get a view eith RA/RV and PT and AO side by side,then RA RV LA LV, then start to lose RA chamber
o Can obtain short axis images with Ao, TV, and LA, with PT posteriorly
o On either side can cross section through ventricles, of through the atrial septum (SCV to RA on one side, LA on the other side)

25
Q

What can you see in suprasternal plane in TTE

A

o Aorta with PV running below them

26
Q

What are the heart ‘segments’ for segmental sequential analysis?

A

o 1. Atrial
Right, left, isomerism
o 2. Ventricular
Right, left, solitary and indeterminate
o 3. Arterial
Aorta, pulmonary, common, solitary (absent intrapericardial pulmonary arteries)

27
Q

What are the two ‘junctions’ for segmental sequential analysis?

A

o 1. Atrioventricular junctions
o 2. Ventriculoarterial junctions

28
Q

What is sequential segmental analysis?

A

Systematic analysis of the heart according to morphology of each chamber and assessment of how chambers are connected to one another, then describing lesions identified

29
Q

What are the two main components of sequential segmental analysis?

A
  1. Three segments
  2. Two junctions
30
Q

Atrial types

A

Situs solitus (usual)
Situs inversis (mirror image)
Right isomerism
Left isomerism

31
Q

Describe atrial situs solitus

A

o Spleen on left, liver in right, stomach curves to left
o Three right lung lobes, two left lung lobes, short right main stem bronchus, long left main stem bronchus
o IVC anterior and right, aorta posterior and left of the spine

32
Q

Describe atrial situs inversus

A

o Spleen on right, liver on left, stomach curves to left
o Three left lung lobes, short left main bronchus, two right lung lobes, long right main bronchus
o IVC anterior and left, aorta posterior and right of the spine

33
Q

Describe atrial right isomerism

A

o Midline liver, malrotated gut, asplenia
o Three lung lobes bilaterally, short main stem bronchus bilaterally
o IVC anterior and left, aorta posterior but left of the spint

34
Q

Describe left atrial isomerism

A

o Midline liver, malrotated gut, multiple spleens
o Two lung lobes bilaterally, long main stem bronchus bilaterally
o Aorta anterior and left of the spine, azygos continuation of IVC posterior and further left from aorta

35
Q

Atrioventricular junction types

A

Lateralised atria
Isomeric arrangement
Univentricular connection

36
Q

Describe possible arrangements of lateralised atria

A

o Concordant atrioventricular connections; Usual atrial arrangement
o Concordant atrioventricular connection; Mirror atrial arrangement
o Discordant atrioventricular connection; Usual atrial arrangement
o Discordant atrioventricular connection; Mirror atrial arrangement

37
Q

Describe possible arrangements in isomeric arrangement

A

o Right isomerism, right hand topology (=RV on the right side) == Isomeric RA; RV on right, LV on left
o Right isomerism, left hand topology (=RV on the wrong side) == Isomeric RA; LV on right, RV on left
o Left isomerism, right hand topology == Isomeric LA; RV on right, LV on left
o Left isomerism, left hand topology == Isomeric LA: LV on right, RV on left

38
Q

Describe the changers in Right isomerism, right hand topology

A

Isomeric RA; RV on right, LV on left

39
Q

Describe the changers in Right isomerism, left hand topology

A

Isomeric RA; LV on right, RV on left

40
Q

Describe the changers in Left isomerism, right hand topology

A

Isomeric LA; RV on right, LV on left

41
Q

Describe the changers in Left isomerism, left hand topology

A

Isomeric LA: LV on right, RV on left

42
Q

Describe univentricular connections

A
  1. Absent right connection
    Right atrial chamber is a dead end, only left atrium has connection
  2. Double inlet
    Both atrial chambers connect to ventricular chamber
  3. Absent left connection
    Left atrial chamber is a dead end, only right atrium has connection

The uni-ventricle can be due to a dominant LV with incomplete RV; solitary and indeterminate ventricle, or a dominant RV with incomplete LV

43
Q

What is absent right connection

A

Right atrial chamber is a dead end, only left atrium has connection

44
Q

What is double inlet

A

Both atrial chambers connect to ventricular chamberW

45
Q

What is absent left connection

A

Left atrial chamber is a dead end, only right atrium has connection

46
Q

Possible features of the atrioventricular valve

A
  1. Single valve
  2. Common valve
47
Q

Subtypes of single atrioventricular valve

A

o Both patent
o One (right or left) AV valve is imperforate
o One (right or left) AV valve is stenotic
o One (right or left) AV valve is straddling (Chordae attach to the other side of interventricular septum)
o One (right or left) AV valve is overriding (Valve orifice lies over the top of the intraventricular septum)
o One (right or left) AV valve is straddling and overriding

48
Q

Possible configurations of a single atrioventricular valve

A

o Half of the common valve might be imperforate
o Half of the common valve might be stenotic

49
Q

Arterial segment types

A
  1. Aorta and pulmonary artery
  2. Single outlet ventriculo-arterial connection
50
Q

Possible configurations of arterial segment - aorta and pulmonary artery

A

Concordant
Discordant

51
Q

Possible configurations of single outlet ventriculoarterial connections

A

Common
Solitary - 3 subtypes (with pulmonary atresia, with aortic atresia, with no intrapericardial pulmonary arteries)

52
Q

3 types of solitary single outlet ventrociloarterial connections

A

 Solitary with pulmonary atresia
 Solitary with aortic atresia
 Solitary with no intrapericardial pulmonary arteries

53
Q

Ventriculoarterial junction types

A
  • Concordant
  • Discordant
  • Double outlet ventricles
54
Q

Possible configurations of double outlet ventricles

A

o One ventricle supports outflow to both aortic and pulmonary
o Double outlet right, left, or single indeterminate ventricle
o Common or solitary arterial segments

55
Q
A

Paediatric cardiology (8 decks)

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