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Paediatric cardiology > Morphology > Flashcards

Morphology Flashcards

1
Q

Components of the cardiac septum

A

o Floor of the fossa ovalis (thin)
o Muscular rims around it
o Coronary sinus orifice
o Ventricular septum
o Tricuspid and mitral valve attachments

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2
Q

Proportion of CHD represented by ASD, AVSD and VSD

A

o ASD (8% CHD)
o AVSD (4% CHD)
o VSD (32% CHD)

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3
Q

Key features to note about a VSD

A

Size
Location
Tissue
Associated malformations
Proximity to important structures (valves and conduction system)

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4
Q

Nomenclature of VSDs

A
  1. Size 2. Tissue 3. Location

e.g., 3mm Perimembranous Outlet

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5
Q

Are VSDs the same size on RV and LV aspect?

A

No

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6
Q

Possible VSD locations

A

o Inlet
o Outlet
o Apical/trabercular

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7
Q

Possible VSD tissues

A

o Muscular
o Perimembranous
o Doubly committed / juxtaarterial

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8
Q

Key features of muscular VSD

A
  • Often hard to see in pathological specimens, hidden in muscle layers
  • Usually small, not associated with other congenital abnormalities
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9
Q

Key features of perimembranous VSD

A
  • Associated with other congenital malformations
  • Close to AV conduction bundle
  • Involve any remnant of the membranous septum, therefore usually have a part muscular and part fibrous border
  • Called multiple names: Infracrister/Kirklin II & III, Kawashima type 2, Tatsuno type 2
  • Perimembranous inlet defects tend to extend further back into the inlet portion of the RV
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10
Q

Key features of doubly committed VSD

A
  • Called multiple names: infundibular, supracristal, subpulmonary, Kawashima Type 1, Tatsuno Type 1
  • Sits right underneath the semilunar valves, no muscle in between
  • Can have perimembranous extension
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11
Q

Describe ASD

A

Communication between atrial chambers

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12
Q

Key features to note about an ASD

A
  • Where in the septum
  • Size of the defect
  • The margins
  • Proximity to important structures
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13
Q

Key thing about septum orientation to remember when assessing ASD

A

When visualizing in short axis images, the septum is ‘oblique’ rather than perfectly transverse. It is also relatively close to the aorta, this is something important to bear in mind when considering device closure, don’t want to ‘clip’ the aorta

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14
Q

Normal interatrial septum features

A
  • From the RA, we can see the IVC entrance coming in
  • We can see the fossa ovalis, and the muscular contour of the fossa
  • Atrial defects are defects in the fossa or surrounding areas
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15
Q

List the types of ASD, starting SUPERIORLY

A
  • Superior sinus venosus
  • Oval fossa (secundum)
  • Vestibular
  • Atrioventricular (primum)
  • Inferior sinus venosus
  • Coronary sinus
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16
Q

ASD: Key features of secundum ASD (oval fossa)

A

o Persistence of the ‘ostium secundum’
o Deficient flap valve closing the ostium (flap valve = septum primum)
o Often has fenestrations
o Amenable to device closure, size variable
o Can have windsock configuration

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17
Q

ASD: Key features of superior sinus venosus defects

A

o Superior to the oval fossa
o Near where SVC enters the RA
o Associated nearly always with partial anomalous pulmonary venous connection
o PV entering the SVC
o Sinus node is close slightly anterior
o Therefore at risk during surgical procedures to repair

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18
Q

ASD: Key features of inferior sinus venosus defects

A

o Arises near where the IVC empties into the RA
o Relatively close to AV node

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19
Q

ASD: Features of coronary sinus defects

A

o Lesion at the position of the coronary sinus orifice
o Close to AV node
o Commonly associated with persistence of left SVC

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20
Q

ASD: Key features of vestibular defects

A

o In the region leading to the right AV valve orifice
o Between AV node and oval fossa
o In the sinus septum region of the RA
o Tend to be quite small

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21
Q

Describe atrioventricular septal defects

A

Also known as AV canal defect, ostium primum defect, endocardial cushion defect

There is a loss of offset between the atrioventricular valves, instead there is a hole in the septum

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22
Q

Key features characterising AVSD

A
  1. Common AV junctions
  2. Biventricular AV connections (2 atria, each connected to one ventricle)

Due to lack of AV junction, there is displacement of LVOTO to more anterior than normal (usually it sits in the anterior groove)

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23
Q

Valve configuration in AVSD

A

o 5-leaflet AV valve guards AV junction
o Superior and inferior bridging leaflets are distinctive
o Then three other leaflets with different configurations depending on the configuration of AVSD

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24
Q

Two main configurations of AVSD

A
  1. Common valve orifice (aka AV canal defect)
  2. Separate valve orifice (aka ostium primum)
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25
Q

Key characteristics of common valve orifice AVSD

A

 One single opening between the five leaflets
 Three-leaflet configuration for the morphologic atrioventricular connection

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26
Q

Key characteristics of separate valve orifices AVSD

A

 Two separate openings, with fused superior and inferior bridging leaflets
 Cleft between superior bridging and inferior bridging leaflets
 Superior and inferior arrangement of the papillary muscles (usually obliquely arranged in morphological LV

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27
Q

What influence does AVSD have on inlet and outlet proportions

A

Leads to inlet-outlet disproportion

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28
Q

What are the consequences of the inlet outlet disproportion seen in AVSD

A

o Anterior displacement and elongation of LVOTO mean that outlet distance is longer than inlet distance (gooseneck deformity)
o Leads to unwedged aortic valve – AV displaced away from usual position

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29
Q

How do AVSDs influence the AV node placement

A

Displaced

o Triangle of Koch not identifiable
o Usually displacement of AV node inferior and toward IVC – important to know where to avoid heart block at time of surgical repair

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30
Q

Name the leaflets in AVSDs

A

1 – superior bridging
2 – inferior bridging
3- left mural
4- right anterosuperior leaflet
5- right inferior

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31
Q

Shunting in partial or incomplete AVSD (p-AVSD, ostium primum)

A

 Inter-atrial shunting (defect above the atrioventricular valve attachment)

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32
Q

Shunting in Complete AVSD (canal)

A

 AVSD with common valvular orifice
 Interatrial and interventricular shunting – best appreciated in diastole
 Tethering of superior and inferior bridging leaflets are not anchored at the level of the septum

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33
Q

Shunting in Transitional/intermediate AVSD

A

 Separate valvular orifices because superior/inferior bridging leaflets are fused, but not anchored to septum (variation of complete AVSD)
 Atrial and ventricular shunts

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34
Q

Shunting in Intraventricular AVSD

A

 Bridging leaflets attached to the atrial septum
 Interventricular shunting onlyd

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35
Q

Additional congenital defects associated with AVSD

A

RVOTO
LVOTO
Isomeric arrangement of atrial appendages
Other valvular abnormalities, coarctation
Deviation of ventricular or atrial septum
Chamber disproportion (‘unbalanced’ AVSD)

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36
Q

What does an unbalanced AVSD mean prognostically / for surgical planning

A

 Makes surgical repair complex – if AVSD closed, LV might be too small to maintain adequate CO (and vice versa for pulmonary flow in RA, though the potential issues are more limited in this setting)

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37
Q

Rastelli classification for complete AVSD

A

Based on the relationship of the superior bridging leaflet and chordal attachments

Type A - Divided leaflets and attached to the crest of ventricular septum (multiple chordae). Associated with left-sided obstruction.

Type B - Partly divided into two components, not attached to crest of septum. Chordae from superior leaflet attached to papillary muscle in RV on septal surface. Least common form of the complete atrioventricular septal defect

Type C - Undivided and attached to ventricular septal crest (‘free floating’); attachments to papillary muscle and Associated with Tetralogy of Fallot and other complex congenital heart diseases

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38
Q

Rastelli Type A Complete AVSD

A

Type A - Divided leaflets and attached to the crest of ventricular septum (multiple chordae). Associated with left-sided obstruction.

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39
Q

Rastelli Type B Complete AVSD

A

Type B - Partly divided into two components, not attached to crest of septum. Chordae from superior leaflet attached to papillary muscle in RV on septal surface. Least common form of the complete atrioventricular septal defect

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40
Q

Rastelli Type 3 Complete AVSD

A

Type C - Undivided and attached to ventricular septal crest (‘free floating’); attachments to papillary muscle and Associated with Tetralogy of Fallot and other complex congenital heart diseases

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41
Q

Classification used for complete AVSD

A

Rastelli classification (Type A B C)

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42
Q

List the four left-to-right dominant lesions OUTSIDE the cardiac septum

A

PDA
CAT
APW
P-TAPVC

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43
Q

Relative proportion of
PDA
CAT
APW
P-TAPVC
among CHD

A
  • PDA 7%
  • CAT 1%
  • APW <0.5%
  • P-TAPVC 1%
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44
Q

Summary of PDA

A

Fetal structure coming off the pulmonary trunk and entering the aorta past the brachiocephalic > left common carotid > left subclavian

Usually enters the aorta just past the left subclavian

Exits the PT superiorly at the level of the bifurcation, enters the aorta inferiorly

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45
Q

Duct closure timings

A

Ductal closure begins in the immediate period of birth
Functional closure occurs in days/weeks, then full closure via growth of intimal cushions within months

Should be closed by 1 month in term infants
Might close as late as 3 months in premature infants

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46
Q

Importance of PDA in cyanotic disease

A

Can be useful as source of shunting in complex congenital cyanotic disease e.g., PAIVS, aortic atresia / hypoplastic left heart

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47
Q

How does PDA support circulation in PAIVS and Aortic Atresia

A

In HLH/Ao atresia, the aortic outflow will be small

So PDA will be the primary source of blood to the systemic circulation, including to the neck and arms and to the coronary arteries by retrograde flow

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48
Q

When can the duct be absent?

A

in common arterial trunk (CAT)

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49
Q

What is Common Arterial Trunk (CAT)?

A

Condition where there is one single vessel providing both systemic and pulmonary blood flow

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50
Q

Four types of CAT depending on origin of the pulmonary arteries

A

Depending on origin of pulmonary arteries

o Type 1 – Single posterior origin that bifurcates
o Type 2 – Two distinct PA origins posteriorly
o Type 3 – Two distinct PA origins on the sides of the aorta
o Type 4 - no pulmonary trunk, lungs supplied via systemic to pulmonary collaterals

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51
Q

Details of Type I CAT

A
  • Pulmonary artery arises from the posterior aspect of the ascending aorta
  • Truncal valve overriding the septum
  • Often associated with muscular VSD, dysplastic truncal valve, and left aortic arch
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52
Q

Details of Type II CAT

A
  • Separate origins of pulmonary arteries posteriorly
  • Often associated with muscular VSD, can be associated with dysplastic truncal valve
  • Can be associated with interruption of aortic arch
  • Interruption of the aortic arch is PDA dependent, PDA supplies blood flow between the pre and post interruption segment
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53
Q

Details of Type III CAT

A
  • Can be associated with interruption of the aortic arch
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54
Q

Details of Type IV CAT

A
  • No evidence of intrapericardial pulmonary arteries, pulmonary flow fed by collaterals
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55
Q

Typical lesions associated with CAT

A

Right arch with mirror imaged branching (30% of patients)

Arch interruption (15% of patients)

Double aortic arch (<1% of patients

Ventricular origin of the common trunk
o Balanced 75%
o Entirely RV 20%
o Entirely LV 5%

Ventricular septal defects
o Muscular outlet VSD is common
o The importance of the VSD depends on the origin of the CAT
o Atrioventricular septal defects might also be present

Truncal valve dysplasia / defects
o Number of leaflets, dysplasia

Aortic arch anomalies
o Right sided, interrupted, coarctation, vascular ring formation

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56
Q

Describe aortopulmonary window (APW)

A

Condition where a communication exists between the lumen of the aorta and the pulmonary arteries

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57
Q

Details about APWs

A
  • Two sets of semilunar valves, then window superior to the semilunar valves in the wall between aorta and pulmonary trunk
  • High risk of pulmonary vascular disease due to sizeable shunt
  • Location and size of window varies from case to case
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58
Q

How are APWs described based on location of window?

A

Based on location of window

Proximal - window the origin of the vessels, just past valves
Intermediate - tissue above and below, window in middle
Distal - close to the point of division of the pulmonary trunk
Confluent - no tissue between Ao and PA until the pulmonary trunk separation

In confluent, there is often anomalous origin of right pulmonary artery orifice
Can have both originating after the separation, or overriding both semilunar valves, or can arise from the back of the aortic wall

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59
Q

Typical lesions associated with APWs

A
  • Aortic arch stenosis or interruption
  • Ventricular septal defect
  • Complete transposition
  • Tetralogy of Fallot
  • Pulmonary atresia
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60
Q

What is anomalous pulmonary venous return

A

Condition where one, more than one (noncyanotic) or all (cyanotic) of the pulmonary veins do not drain into the left atrium

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61
Q

Key details to think about in APVR

A
  • Proportion of anomalous connection
  • Site of the connection
  • Is anomalous pathway obstructed
  • Any other associated defects
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62
Q

Where might the anomalous connection drain in APVR

A

o Right atrium
o SVC
o IVC
o Coronary sinus
o Brachiocephalic vein

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63
Q

Typical associated defects

A

o Superior or inferior sinus venosus defect
o ASD
o Persistent left SVC
o AVSD
o Atrial isomerism

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64
Q

What is Scimitar syndrome

A

o Anomalous PV draining part or all of the right lung to the IVC most commonly
o Associated with hypoplasia of right lung and right PA
o Sometimes anomalous systemic arterial supply from the descending aorta

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65
Q

Haemodynamic consequence of PAPVR

A

If IVC/SVC/RA drainage: Pulmonary hypertension due to high RV preload

If CS: dilation of coronary sinus

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66
Q

Key features of TAPVR

A
  • None of the pulmonary veins enter the LA
  • Systemic blood flow is dependent on an interatrial communication (ASD)
  • The shunt is from right to left to maintain life
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67
Q

Classification of TAPVR based on location of entry of PVs into the right sided circulation

A

Supracardiac
 Azygos, right SVC, or brachiocephalic

Cardiac
 Direct to RA or to left SVC and coronary sinus
 CS gets dilated when through sinus

Infracardiac
 To IVC or hepatic portal vein
 Can be associated with stenosis of the vein at the level of the diaphragm, and with isomerism

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68
Q

Cyanotic congenital heart diseases

A

TOF
TGA
TAPVR
Truncus arteriosis
Tricuspid atresia / PAIVS

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69
Q

Frequency among CHD of TOF, PAIVS and TGA

A
  • TOF 5%
  • Pulmonary atresia with intact ventricular septum 2%
  • Complete transposition 4%
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70
Q

TOF characteristics

A

Tetralogy of: VSD, Subpulmonary stenosis, overriding aorta, RV hypertrophy

Might have pulmonary stenosis or atresia

71
Q

What contributes to RVOTO in TOF?

A
  1. Septo-marginal trabeculation [structure that divides near the RVOT into an anterior limb (towards OT) and posterior limb (towards TV)] can become hypertrophic
  2. Outlet septum [a muscular structure that divides the PV from the AV] is deviated anterocephelad in TOF squeezing the RVOT
  3. Septoparietal trabeculations (SPT) that in the TOF setting hypertrophy and therefore add to sub-pulmonary obstruction
72
Q

Potential ventriculoarterial configurations in TOF

A

o Aorta predominantly supported by LV (effectively concordant connections)
o Aorta predominantly supported by RV (essentially double outlet connection)

73
Q

Potential levens of pulmonary obstruction/stenosis in TOF

A

o At mouth of infundibulum – stenosis or atresia
o Pulmonary valve - stenosis to atresia
o Within RV
o Distally within PA

74
Q

Typical other malformations associated with TOF

A

o Pulmonary atresia
o Absent leaflets of PV
o Straddling TV
o AVSD
o Anomalous coronary arteries
o Right sided aortic arch in 3-5%
o Muscular inlet VSD
o Arterial wall anomalies

75
Q

Key features of TOF with pulmonary atresia

A
  • Also called pulmonary atresia with ventricular septal defect
  • RV outlet to aorta via VSD
  • Variable mature of atretic outlet
  • Variable types of VSD
  • Other intracardiac defect
  • Variable lung vascular supply
76
Q

Potential types of mature atretic outlet in TOF with PA

A

o Muscular atresia (no remnant of pulmonary valve at all)
o Imperforate membrane
o Solitary arterial trunk (no existing pulmonary trunk)

77
Q

Potential types of VSD in TOF with PA

A

o Perimembranous
o Muscular
o Multiple

78
Q

Possible pulmonary vascular supply in TOF with PA

A

o Unilateral or bilateral duct
o Systemic to pulmonary collateral arteries (SPCA)
o APW, fifth aortic arch, coronary/pulmonary fistula

79
Q

Key features to summarize in the assessment of TOF

A
  1. Extent of aortic override
  2. RV margins of VSD
  3. Nature and anatomy of subpulmonary obstruction
  4. Pulmonary valve malformations
  5. Associated other malformations
80
Q

Describe PAIVS

A

Cyanotic congenital defect where there is no flow from the RV to the PA due to (1) pulmonary atresia and (2) lack of VSD

81
Q

Key features of PAIVS

A

o Systemic venous return MUST cross ASD, exit via aorta, duct, PA
o Pulmonary supply is therefore duct dependent
o Pulmonary arteries are usually good in size
o Pulmonary trunk hypoplastic if the atresia is muscular, normal in size if atresia is imperforate in origin

82
Q

What does RV cavity typically look like in PAIVS

A

o Hypoplastic to varying degree (can rarely be normal in morphology)
o RV wall hypertrophy is the rule
o Tricuspid valve abnormalities are commeon

83
Q

Key features to report in PAIVS

A
  • Nature of pulmonary atresia (muscular or imperforate valve)
  • Degree of hypoplasia of RV cavity
  • Morphology of tricuspid valve
  • Fistulous communications of coronary arteries
84
Q

Typical lesions associated with PAIVS

A

ASD
Coronary arterial anomalies
o Anomalous origin
o Sinusoids
o Atretic segments in coronaries

85
Q

Key features of d-TGA

A
  • The aorta is to the right of the pulmonary artery
  • D-TGA is the most common form of TGA and is also called ‘complete’ TGA
  • This type of TGA almost always needs to be repaired in the first year of life.
86
Q

Key features of L-TGA

A
  • The aorta is to the left of the pulmonary artery
  • Another term for L TGA is congenitally corrected TGA
  • This type of TGA, in the absence of other heart defects, may not require repair early in life
87
Q

Sequential segmental analysis description of d-TGA

A

o Usual or mirror-imaged atrial arrangements
o Atrioventricular concordance
o Ventriculoarterial discordance

88
Q

What happens in the absence of a shunt in d-TGA after birth

A

After birth if the ducts close if there are no communications between the right and left heart this would be immediately lethal

89
Q

Two types of d-TGA

A

Simple vs Complex

90
Q

Simple d-TGA features

A

Intact IVS, no further malformations

However, might not be so simple: there can be
* Anomalies in the coronary arteries E.g., RCA coming from left hand sinus, LCx coming from left hand sinus, LAD from right hand sinus running between the Ao and PA and then descending
* Abnormal arterial courses
* Infundibular morphology might be abnormal

91
Q

Complex d-TGA features

A

VSD
- 40-45% of cases, with misalignment of the outlet septum

Subpulmonary or subaortic obstruction - 25% of cases

Subaortic obstruction (RVOTO) may arise from
o Muscular infundibulum
o Abnormal muscle bundles
o Anomalous tension apparatus
o Deviated outlet septum with VSD
o Aortic coarctation will contribute to poor aortic flow

Subpulmonary (LVOTO) may arise from:
o Valvular stenosis
o Aneurysmal tissue tags
o VSD with deviated septum
o Fibrous diaphragm or tunnel
o Abnormal insertions of mitral valve

Valvular abnormalities
- Atrioventricular or arterial

Aortic coarctation
- 5% of cases

92
Q

List four obstructive congenital heart defects and relative % among CHD

A
  • Pulmonary stenosis 7%
  • Aortic stenosis 4%
  • Aortic arch malformations 5%
  • Vascular ring <1
93
Q

Key features to determine when examining PS

A

Level of stenosis
Association with chromosomal or genetic abnormalities

94
Q

Potential levels of stenosis in PS

A

Supravalvar
 Discrete or diffuse stenosis of PA

Valvar
 Most common isolated lesion

Subvalvar
 Associated with VSD, Fallot, subdivision of RV

95
Q

Three genetic/chromosomal associations of PS

A

Noonan, Williams, Alagille

96
Q

Valvular PS key fatures

A

Most common type

Different potential morphologies
o Dome shaped subtotal fusion of zones of apposition, pinhole orifice, leaflets not thickened - Most common
o Three thick leaflets, no fusion of zones of apposition
o Three thick leaflets with partial fusion of zones of apposition
o Bicuspid (bifoliate)
o Unicuspid (unifoliate)

97
Q

Subvalvar PS key features

A
  • Double chamber RV
    o Shelf coming across the cavity of the RV
    o Can either be high or low on the septum
98
Q

Possible levels of stenosis in AS

A

Supravalvar
Valvar
Subvalvar
 Anomalous valvular insertion
 Septal hypertrophy, shelf, tunnel

99
Q

Valvar AS: key features to distinguish

A

o Number of leaflets – usually three, two if bicuspid, rarely four leaflets
o Character of leaflets – thickened, proportion, calcifications

100
Q

Valvar AS: key specific veatures for bicuspid aortic valve

A

o Variable levels of obstruction depending on how well formed, proportionate and pliable the leaflets are
o Might not lead to any issues throughout life
o Can sometimes see a remnant of the raphe in the bicuspid leaflets
o Rarely can be unicuspid
o Can be dome shaped

101
Q

Supravalvar AS: three potential configurations of obstruction

A
  • Hourglass
  • Diaphragm
  • Diffuse hyperplasia
102
Q

Characteristics of hourglass supravalvar AS

A

o Dilated distal portion of ascending aorta
o Sinotubular junction constricted
o Aortic valve leaflets thickened and deep
o Associated with isolation of coronary orifice: Left coronary artery tries to enter in aortic sinus but free margin of the valvular leaflet might be plastered across the coronary orifice

103
Q

Characteristics of diaphragm supravalvar AS

A

o We see a flap sitting above the valvular leaflets at the sinotubular junction

104
Q

Characteristics of diffuse hypoplastic supravalvar AS

A

o Characterised by narrow but thickened vessels, including the aorta and neck and arm arteries

105
Q

Key features to assess in aortic coarctation or interruption

A
  1. Site and morphology of obstruction
  2. Associated lesions
  3. Arterial duct
106
Q

Types of coarctation

A

Coarctation (waist lesion)
Coarctation (discrete shelf lesion) with tubular hypoplasia
Tubular hypoplasia of isthmus

107
Q

Describe Coarctation waist lesion

A

 Discrete obstruction due to narrowing in vessel
 Can be preductal, paraductal, or post ductal
 Present in 4-10% of CHD patients
 80% of coarctations are sole lesions

108
Q

Describe coarctation discrete shelf lesion

A

 Discrete obstruction due to a shelf/encircling of lumen in the inner vessel

109
Q

Describe coarctation tubular hypoplasia of isthmus

A

 Segment of obstruction at the isthmus of ductus
 Location relating to the isthmus (area of the aorta between origin of the L subclavian and the entrance of the duct)
 Can occur at the isthmus, affecting region from the left common carotid until insertion of the duct, or can involve the whole transverse aortic arch

110
Q

Complications of coarctation

A

 Rupture of the aorta
 Aortic aneurysms post coarctation lesion

111
Q

Arch interruption types depending on site

A

At isthmus
Between left common carotid and left subclavian
Between right and left common carotid

112
Q

Key features of arch interruption

A

Can be complete (no flow past) or atretic (some remnant of flow but insufficient to sustain life, still duct dependent)

Account for 1.3% of critical congenital anomalies

Lethal within first few weeks after birth

Classified by location relating to carotid/subclavians

Ventriculoarterial connections are variable in patients with arch interruptions
* Might be concordant
* Might have common arterial trunk
* Might be discordant
* Might have double outlet right ventricle

113
Q

Typical associations for CoA + interruption

A

o Mitral stenosis
o Aortic stenosis (VSD with malalignment of outlet septum)
o Bicuspid aortic valve (20-40% of CoA)

114
Q

Describe vascular rings

A

Part of the aorta or its branches form a ring around the trachea, the oesophagus or both, potentially leading to tracheo/esophageal obstruction

115
Q

Three different configurations of vascular rings

A

 Ring
 Sling
 Loop (partial ring)

116
Q

Normal embryonic development of the aortic arch

A

Ascending aorta develops as a component of the primitive heart tube.

The primitive heart develops from five dilations:
 the truncus arteriosus,
 conus cordis,
 primitive ventricle,
 primitive atrium, and
 the sinus venosus.

The truncus arteriosus forms the basis for developing the ascending aorta and pulmonary trunk, beginning during the fifth week of development.

The truncus starts as a single outflow tract from the right and left ventricles but is eventually divided by the aorticopulmonary septum into separate vascular outflow channels.

The truncal and conal ridges are invaded by neural crest cells, leading to spiraling that forms the aorticopulmonary septum.

The arch of the aorta develops from multiple structures.
 The portion of the arch proximal to the brachiocephalic trunk arises directly from the aortic sac.
 The medial area of the arch, between the brachiocephalic trunk and the left common carotid artery, arises from the left 4th aortic arch.
 The portion of the arch distal to the left common carotid artery arises from the dorsal aorta.
 The descending aorta arises from the dorsal aortae.

Early in development, paired right and left dorsal aortae are confluent with the aortic sac.
 The right and left dorsal aortae later fuse along vertebral levels T4 to L4, forming a single, continuous dorsal aorta.
 The dorsal aorta ultimately gives off many vital branches, including intersegmental, splanchnic or visceral, and umbilical arteries. T
 he dorsal aorta in this region is later referred to as the descending thoracic and abdominal aorta

NB the ductus arteriosus develops from the left 6th aortic arch

117
Q

Aorta develops as component of the

A

primitive heart tube

118
Q

The primitive heart develops from five dilations:

A

 the truncus arteriosus,
 conus cordis,
 primitive ventricle,
 primitive atrium, and
 the sinus venosus.

119
Q

When does the aorta development begin

A

week 5

120
Q

Truncus arteriosus forms the basis for

A

Ao and PA

121
Q

The truncus starts as

A

a single outflow tract from the right and left ventricles but is eventually divided by the aortopulmonary septum into separate vascular outflow channels

122
Q

The truncal and conal ridges are invaded by

A

neural crest cells, leading to spiraling that forms the aorticopulmonary septum

123
Q

The arch of the aorta develops from the following structures

A

 The portion of the arch proximal to the brachiocephalic trunk arises directly from the aortic sac.
 The medial area of the arch, between the brachiocephalic trunk and the left common carotid artery, arises from the left 4th aortic arch.
 The portion of the arch distal to the left common carotid artery arises from the dorsal aorta.
 The descending aorta arises from the dorsal aortae.

124
Q

Early in development, paired right and left dorsal aortae are

A

confluent with the aortic sac.

125
Q

The right and left dorsal aortae later fuse along vertebral levels …

A

T4 to L4, forming a single, continuous dorsal aorta.

126
Q

The dorsal aorta ultimately gives off many vital branches, including …

A

intersegmental, splanchnic or visceral, and umbilical arteries. The dorsal aorta in this region is later referred to as the descending thoracic and abdominal aorta

127
Q

The ductus arteriosus develops from the following aortic arch

A

6th

128
Q

Aberrant embryological development of aorta in vascular rings - right sided aortic arch

A

Right sided aortic arch (mirror image)
 Right sided 4th aortic arch has persisted (instead of left sided 4th aortic arch), leading to downstream right sided dorsal aorta persistence
 Associated with TOF (25%) and common arterial trunk (50%)
 Sequentially from LVOTO, the first branch to come off is the left brachiocephalic, then right common carotid, then right subclavian

129
Q

Aberrant embryological development of aorta in vascular rings - left sided aortic arch

A

 In this configuration, we have LVOTO > R common carotid > common carotid > L subclavian > R subclavian
 The aberrant right subclavian loops backwards and around the back of trachea and oesophagus and might therefore lead to compression

130
Q

Aberrant embryological development of aorta in vascular rings - double aortic arch

A

 Persistence of both right and left 4th aortic arch
 Usually independent right and left artery origins (4 in total)
 Usually associated with bilateral duct
 Join into the descending or dorsal aorta

131
Q

Key features of R aortic arch with vascular ring

A
  • 12-15% of vascular rings
  • From RVOTO, first branch is L common carotid, then R common carotid, then R subclavian, finally the aberrant L subclavian posteriorly, travelling behind the oesophagus and trachea, and might connect to the left duct to complete the ring
  • Might also be associated with a remnant of the dorsal aorta (Diverticulum or Kommerell)
  • Persistent ductus arteriosus or dutal ligament can anchor down the L subclavian, leading to a kinking that might worsen trachea-esophageal compression
132
Q

Key features of double aortic arch

A

Usually
* L arch 2/3 size of the R arch
* L arch tubular hypoplasia or atresia
* 20% associated with other CHD – commonly CAT
* L arch anterior to trachea / esophagus, R arch posterior
* Sometimes L arch referred to as the ‘anterior’ and R arch referred to as ‘posterior’

Can be a partial double arch
* Persistence of part of the right dorsal aorta (diverticulum of Kammerell) in the setting of the right sided arch (see below)

133
Q

Describe a PA sling

A
  • Embryologic vascular anomaly where the origin of the left pulmonary artery arises from the right pulmonary artery, instead of the main pulmonary artery
  • This compresses the distal trachea and right mainstem bronchus between the trachea and oesophagus towards supplying the left lung, known as vascular sling
  • Not true vascular ring, but symptoms similar to full vascular ring
134
Q

Key characteristics of PA sling

A

o Narrow affected pulmonary artery
o Narrow trachea
o Small lung on affected side

135
Q

Types of complex CHD and % among CHD

A
  • DORV counts for 2% CHD
  • Hearts with single ventricle physiology overall (e.g., HLHS) count for 4%
136
Q

Key features of DORV

A

Congenital lesion where the great arteries are for the major part supported by the morphological RV

137
Q

Potential morphology in DORV

A

o Any pattern of atrial arrangement and situs
o Any pattern or atrioventricular connection
o Variable infundibular morphology
o Rarely without a VSD

Most commonly usual atrial arrangement and concordant atrioventricular connections

138
Q

Other names for DORV

A

o Taussig-Bing malformation
o Tetralogy of Fallot with extreme dextroposition of the aorta
o Eisenmenger’s anomaly (Eisenmenger’s VSD)

139
Q

Physiology of DORV

A

Varied
- Simple VSD-like
- Fallot
- Transposition
- Single ventricle

140
Q

VSD types in DORV

A

Subaortic
Subpulmonary
Doubly commmitted
Noncommitted

141
Q

Subaortic VSD in DORV

A

o Located inferior to the aorta above the septomarginal trabeculation but below the ventriculoinfundibular fold
o Can be associated with sub pulmonary stenosis
o There might be fibrous continuity between the aortic, tricuspid and mitral valve (lack of conus/infundibulum)

142
Q

Subpulmonary VSD in DORV

A

Transposition type

Can be muscular VSD
 Outlet septum fuses with VIF posteriorly or SMT posterior limn

Can be peri membranous
 Outlet septum fuses with VIF, VIF not fused to SMT
 Pulmonary-mitral-tricuspid continuity

Associated malformations
 Aortic stenosis
 Arch hypoplasia
 Restrictive VSD
 Abnormal TV attachments

143
Q

DOuble committed and noncommitted VSD in DORV

A
  • Doubly committed VSD
    o Lack of outlet septum
    o Muscular postero-inferior rim
  • Non-committed VSD
    o Remote VSD or cannot be connected to a specific outlet
    o Below SMT, e.g., inlet VSD
    o Can be an AVSD
144
Q

Key features to note in DORV patients

A
  • Analyse DORV independent of position of great arteries and bilateral infundibulums
  • Note location and size of VSD
  • Presence or absence of pulmonary or aortic OTO
  • Fallot with DORV when aorta is mainly from RV
  • Other associated malformations e.g., in subpulmonary VSD
145
Q

Types of functionally univentricular hearts

A
  1. Hearts with solitary or indeterminate ventricle
  2. Hearts with unbalanced ventricular mass
146
Q

Subcategories of functionally univentricular hearts with unbalanced ventricular mass

A

o Hearts with univentricular AV connection
o Hearts with straddling AV valves
o Hearts with unbalanced AVSD
o Hearts with intact ventricular septum and critical stenosis or atresia of outflow tracts

147
Q

Assessment of morphology in functionally univentricular hearts

A

o Atrial arrangement: usual, mirror, right isomerism, left isomerism
o AV connection univentricular: absent right, double inlet, absent left
o Ventricular morphology: dominant left with rudimentary right, solitary and indeterminate, dominant right with rudimentary left

148
Q

Solitary indeterminate ventricle: morphology

A

o Any atrial arrangement
o Double inlet AV connection
o Solitary and indeterminate ventricle

149
Q

Solitary indeterminate ventricle: features

A
  • Ventricle might have a small posterior ridge
  • Trabeculations in single indeterminate ventricle are very coarse
150
Q

Unbalanced ventricular mass with univentricular AV Connection: morphology

A

o Any atrial arrangement
o Any AV connection
o Ventricles either dominant left or right

151
Q

Hearts with univentricular AV connection: Any atria, double inlet, dominant left with rudimentary right

A

o Most common
o Pulmonary trunk from the rudimentary RV, Aorta from dominant LV
o Rudimentary RV directly anterior to the LV
o Might have two separate AV valves, or might have a common AV valve especially in the setting of right isomerism
o Double inlet AV connections to LV, concordant VA connections (DILV)

152
Q

Hearts with univentricular AV connection: - Any atria, double inlet, dominant right with rudimentary left ventricle

A

o Double inlet AV connections to RV (DIRV)
o Straddling of the left AV valve is common

153
Q

Hearts with univentricular AV connection: Any atria, absent right sided AV connection, dominant LV with rudimentary RV

A

Type 1: Imperforate tricuspid valve = tricuspid atresia
 Biventricular AV connection but TV imperforate

Type 2: Total absence of AV valve (not technically atresia)
 Univentricular AV connection

Associated with ASD +/- VSD
Pulmonary trunk arises form the rudimentary RV

154
Q

Hearts with univentricular AV connection:- Any atria, absent left sided AV connection, dominant RV with rudimentary LV

A

Type 1: Imperforate mitral valve = mitral atresia
 Biventricular AV connection but MV imperforate

Type 2: Absent AV valve
 Univentricular AV connection

Associated with ASD +/- VSD

155
Q

Hearts with intact ventricular septum and critical stenosis or atresia of outflow tracts: list the underlying types

A

Pulmonary atresia
Aortic atresia or stenosis
Mitral atresia or stenosis

156
Q

Hearts with intact ventricular septum and critical stenosis or atresia of outflow tracts: pulmonary atresia

A

 Stenosis of the RVOTO at the level of the valve
 Type 1: Valvar or membranous atresia (imperforate PV
 Type 2: Muscular atresia: blind-ending outflow tract with absence of the tissue valve

Associated with persistent PDA which fully feeds pulmonary flow

157
Q

Hearts with intact ventricular septum and critical stenosis or atresia of outflow tracts: aortic atresia or stenosis, mitral atresia or stenosis are commonly known as…

A

Hypoplastic left heart

158
Q

Hypoplastic left heart refers to any of the underlying pathologies of:

A

aortic atresia or stenosis, mitral atresia or stenosis

leading to heart with intact ventricular septum and critical stenosis or atresia of outflow tracts

159
Q

how common is HLHS

A

o 0.2 per 1000 live births

160
Q

Key features of HLHS

A

 RV expands to form the apex
 LV endocardial fibroelastosis
 Stenotic MV and AoV
 Fibrous communications with coronary arteries
o Coronary arteries are fed via backward flow from PT > DA > Ao > CA
o There might be minimal flow via the hypoplastic left heart
o HLHs is duct dependent – only minimal Ao flow except via duct

161
Q

Coronary artery flow in HLHS

A

Coronary arteries are fed via backward flow from PT > PDA > Ao > CA

162
Q

Associations of HLHS

A

 LV fibroelastosis
 Fibrous connections between LV and CA
 Atrial septal defects
 Abnormalities in AV junction
 Abnormal mitral valve
 Abnormal tricuspid valve
 Ventriculoarterial junction abnormalities
 Aortic arch abnormalities

163
Q

How to distinguish RV and LV in univentricular hearts

A

Trabeculations
Moderator band
Septal attachments of AV valve

In univentricular hearts
o In dominant LV, the dominant ventricle should be seen posteriorly and the rudimentary RV anterior and superiorly
o Similarly in dominant RV, the rudimentary LV will be sitting posteroinferiorly

164
Q

Key features of isomerism of atrial appendages

A

Associated with complex cardiac malformations approximately 30% of times
- Associated with malrotation of gut 70%
- Midline defects (MSK, genitourinary, cleft palate in 50%
- Varied vessel arrangement

165
Q

Possible vascular arrangement in isomerism of atrial appendages

A

o Usually vessels posterior situated, one to either side of spine, vein anterior, aorta posterior
o Left isomerism vein and artery both to the left of the spine, aorta anterior and vein posterior
o Right isomerism vein and artery to the same side, vein anterior, aorta posterior

166
Q

Right isomerism of atrial appendages: Lung and spleen morphology

A

Bilateral short bronchi (epiarterial) 89%

Asplenia 57%
i. Solitary 14%
ii. Multiple 3%

167
Q

Left isomerism of atrial appendages: Lung and spleen morphology

A

Bilateral long bronchi (hyparterial) 98%
Multiple spleens 64%
i. Solitary 7%
ii. Absent 3%
Biliary atresia 10%

168
Q

Examples of isomerism with univentricular circulation

A

Right atrial isomerism, double inlet solitary indeterminate ventricle

Left atrial isomerism, AVSD, double outlet RV with rudimentary LV
o Associated with azygos continuation of IVC

169
Q

Describe Ebstein’s anomaly

A

Congenital abnormality characterised by apical and downward displacement of the tricuspid valve (rarely mitral valve or common AV valve)

170
Q

Ebstein’s key features

A
  • Apical and downward displacement of the tricuspid valve (rarely mitral valve or common AV valve)
  • Reduced separation of the leaflet from the myocardium, leading to lack of delamination, such that the leaflet is not upwardly mobile to the level of the annulus (hinge line)
  • Likely to result from a halting in the delamination process during development
  • Pathognomic feature is origin of part of the hinge line of the TV leaflets within the RV cavity rather than at the AV junction
171
Q

Different types of Ebstein’s

A

o Can be isolated displacement
o Can be associated with dysplasia of valve
o Can be associated with thinning of the ventricular wall above the displaced hinge line, ‘atrialisation’ of ventricular tissue

172
Q

Quantifying degree of displacement and damage in Ebstein’s

A

Degree of displacement
o Can be linked to the MV insertion
o Normal offset between MV and TV <0.8cm/m^2 BSA

Degree of damage to valve
o How many leaflets are affected (one to three, septal and posterior are the most commonly affected)
o How badly are the leaflet affected
o Associated anomalies of leaflet

173
Q

Typical associations in Ebstein’s

A

Atrial septal defects

Pulmonary atresia with intact septum

Discordant atrioventricular connections (ccTGA)
- Usual or mirror arrangement, discordant AV connections AND discordant VA connections (figure below)
- So essentially only the ventricle is switched (unless mirror image)
- Note AV conduction bundle is abnormal, and more prone to fibrosis / heart block
- Characterised on echo by reversed offset of AV valves
* Tricuspid valve on the left side, mitral valve on the right side
* Therefore right sided valve higher than left sided valve
* Can be further aggravated by Ebstein’s of the TV (which is now on the left side)

Ventricular pre-excitation
 In 25% of Ebstein’s due to lack of separation of conduction at TV level

Paediatric cardiology (8 decks)

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