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Pediatric Anesthesiology Boards Outline > Segmental Approach to CHD > Flashcards

Segmental Approach to CHD Flashcards

1
Q

Who proposed a segmental approach to the heart?

A

Van Praagh

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2
Q

What are the three major segments of the heart according to Van Praagh?

A

Atria

Ventricles

Great Arteries

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3
Q

What are the two major determining embryologic features that are fundamental to Van Praagh’s segmental approach?

A

Visceroatrial Situs

Bulboventricular Loop

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4
Q

Who proposed emphasis on the sequence of connections within the heart to the segmental approach of describing the heart?

A

Shinebourne and Anderson (in the 1970s)

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5
Q

What is the definition of levocardia?

A

The normal position of the heart in the left hemithorax with the apex directed to the left.

So usual that it is often left unstated.

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6
Q

What is the definition of dextrocardia?

A

A term mostly used to describe a heart in the right hemithorax but a few authors restrict the use to hearts in which the apex points to the right.

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7
Q

What is the definition of mesocardia?

A

A heart in the midline in a substernal position.

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8
Q

Is it possible for mesocardiac or dextrocardiac heart to be structurally normal?

A

Yes, but abnormalities of segmental relationships and connections are at least 100 times more common than in the normally positioned (levocardiac) heart.

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9
Q

What is the initial embryologic form of the heart?

A

A straight tube

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10
Q

Draw the initial form of the embryologic heart and label its components.

A

TA = Truncus Arteriosus

BC = Bulbus Cordis

V = Ventricle

A = Atrium

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11
Q

The atrial portion of the primitive heart tube receives blood from what structure(s)?

A

Both the left and right sinus venosus.

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12
Q

When does the primitive heart tube begin to loop?

A

When the embryo is about 11 somites (or 15 days old).

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13
Q

Describe the normal looping of the primitive heart.

A

The looping is anterior and to the right usually resulting in the RV being on the right and the Aorta posterior and to the right of the pulmonary artery.

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14
Q

If there is transposition of the great vessels in a D-loop heart, what is the position of the aorta?

A

Anterior and to the right of the pulmonary artery.

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15
Q

Is transposition of the great vessels an isolated defect or a conotruncal malformation?

A

It is most likely an isolated defect.

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16
Q

Describe an L-loop of the primitive heart.

A

The ventricles are inverted.

Usually there is TGA in the L-loop heart with a high, anterior, and to the left aorta.

Rarely the viscera and atria are inverted and the ventricle and truncus are equally involved resulting in a right hemithorax position with situs inversus and otherwise normal heart.

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17
Q

What structure in the primitive heart tube becomes the right ventricle?

A

The bulbus cordis

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18
Q

What portion of the left cardinal system persists with growth of the embryologic heart?

A

The coronary sinus. The rest of the left cardinal system atrophies.

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19
Q

The development of the proximal IVC is closely linked to the growth of what organ?

A

The liver. So much so that the anatomic right atrium and the liver almost invariably develop on the same side of the body.

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20
Q

What is situs solitus?

A

It is a type of visceroatrial situs. It refers to the normal right-sided liver, SVC and RA.

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21
Q

What is visceroatrial situs inversus?

A

A left-sided liver, left-sided SVC, and left-sided morphologic right atrium.

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22
Q

What Ivemark’s syndrome?

A

Situs ambiguus with cardiosplenic abnormality.

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23
Q

What is situs ambiguus?

A

A term of visceroatrial situs that refers to a midline liver an atrial isomerism.

24
Q

What is associated with right atrial isomerism?

A

Right atrial morphology of both atria and asplenia is usual.

25
Q

What is associated with left atrial isomerism?

A

Both atria have left atrial morphology.

Polysplenia is usual.

26
Q

What is the single most important concept underlying the segmental approach to congenital heart disease?

A

Recognizing that the arrangement of the ventricles does not necessarily follow that of the visceroatrial situs.

When the RA does not connect normally to the RV, it produces a “discordant” AV connection.

27
Q

From what structure do the PA and Aorta derive?

A

The conotruncus

28
Q

The normal partitioning of the conotruncus involves migration of what types of cells?

A

Mesenchymal cells from the neural crest

29
Q

What does the term situs mean?

A

Position, site, location

30
Q

What is the short hand for the normal segmental arrangement of the normal heart

A

S,D,S

31
Q

If a heart is described as S,D,S what does that mean?

A

It means that it is normal.

The first S indicates visceroatrial situs solitus

The D indicates ventricular loop to the right.

The final S indicates solitus relationship of the semilunar valves.

32
Q

Describe the lungs in an situs ambiguus asplenic patient.

A

Asplenia is associated with right atrial isomerism and right bronchial isomerism. Thus often there are two bilobed lungs.

33
Q

Describe the lungs in an situs ambiguus polysplenic patient.

A

Polysplenia is associated with left atrial isomerism and generally left bronchial isomerism. Thus there may be two trilobed lungs.

34
Q

What is more reliable an indication than splenic status of the intracardiac defect in situs ambiguus?

A

Morphology of the atrial appendages

35
Q

What is the incidence of situs inversus and dextrocardia?

A

1 in 10,000 births

36
Q

What is the mode of inheritance of situs inversus with dextrocardia?

A

Recessive mode and attributable to a single gene defect.

37
Q

What is associated with situs inversus with levocardia?

A

Interrupted IVC and complex cardiac defects similar to those seen in situs ambiguus

38
Q

What does the term heterotaxy mean?

A

It literally means “other arrangements” but is used confusingly. Some authors use it to describe discordance between the heart and the viscera (e.g. situs inversus and levocardia), while others use it as a synonym for situs ambiguus.

39
Q

What percentage of children with CHD have situs ambiguus or heterotaxy?

A

2%

40
Q

What is the prevalence of an additional left SVC in the normal population?

A

1 - 2%

41
Q

What is an “unroofed” coronary sinus and under what conditions is it normally found?

A

A wall normally separates the coronary sinus and persistent left superior vena cava from the left atrium. If the wall is absent or fenestrated, left SVC blood can drain into the left atrium. This is an “unroofed” coronary sinus.

42
Q

Describe the SVC and coronary sinus in asplenia.

A

Bilateral SVC are frequent.

The coronary sinus may be absent.

43
Q

Describe the SVC and coronary sinus in right atrial isomerism.

A

Bilateral SVC are frequent.

The coronary sinus may be absent.

44
Q

What is another name for asplenia?

A

Right atrial isomerism

45
Q

What is another name for polysplenia?

A

Left atrial isomerism

46
Q

Describe the great veins in left atrial isomerism.

A

The IVC often fails to connect with systemic atrium and instead drains into the azygos system (i.e. “absent” or “interrupted” IVC)

47
Q

What are the distinguishing morphologic features of the right atrium?

A

Presence of the terminal sulcus and crest.

Rim of the oval foss.

Distinctive shape of the RA appendage (blunt triangle w/ broad junction to the venous component of the atrium)

48
Q

What are the distinguishing morphologic features of the left atrium?

A

flap-valve aspect of the oval fossa

receipt of four pulmonary veins

The shape of the LA appendage is the most reliable feature to evaluate in cases of malposition or situs abnormalities.

49
Q

Describe the right and left atrial appendages.

A

The right is a blunt triangle with a broad junction to the venous component of the atrium

The left is a narrow, crenelated structure with a narrow junction connecting it to the body of the atrium.

50
Q

Describe the distinguishing morphological features of the left and right ventricles.

A

RV has triangular shape and coarse trabeculation.

The LV has a conical shape and fine trabeculations.

51
Q

Where does the SA node normally lie?

A

The junction of the SVC and the RA.

52
Q

Describe the P wave on ECG in a patient w/ situs inversus.

A

Inverted P waves in lead I and the left precordial leads.

53
Q

Describe the P wave in left atrial isomerism.

A

There may be no distinct SA node and junctional rhythms and P wave inversion in leads II and II are present in half of such patients.

The P wave (if there is one) may be directed leftward and superiorly.

54
Q

In a discordant heart, do the AV valves follow the atria or the ventricles?

A

The ventricles.

55
Q

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Pediatric Anesthesiology Boards Outline (4 decks)

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