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PTE me > Congenital Heart Disease Part 6 > Flashcards

Congenital Heart Disease Part 6 Flashcards

1
Q

What is situs solitus?

A

Situs solitus (rare plural: sitūs soliti) refers to the normal position of the thoracic and abdominal organs.

This will include a left-sided heart, also known as levocardia.

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2
Q

What is situs inversus?

A

Situs inversus, short form of the Latin “situs inversus viscerum”, is a term used to describe the inverted position of chest and abdominal organs.

It is called situs inversus totalis when there is a total transposition of abdominal and thoracic viscera (mirror image of internal organs normal positioning).

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3
Q

What is situs ambiguous?

A

Situs ambiguous, heterotaxy, heterotaxia

Situs ambiguus is a rare congenital defect in which the major visceral organs are distributed abnormally within the chest and abdomen.

Heterotaxy in general refers to any defect of left-right laterality and arrangement of the visceral organs.

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4
Q

What are the three positions of the heart in the chest?

A
  1. Levocardia (normal)
  2. Mesocardia
  3. Dextrocardia
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5
Q

When you are determining Levocardia (normal), Mesocardia, and Dextrocardia what specifically are you looking at?

A

Apex position of the heart

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6
Q

What is the segment by segment analysis of the heart?

A
  1. Atria
  2. Atrioventricular valves
  3. Ventricles
  4. Outflow Tracts (Conus = Infundibulum)
  5. Semilunar Valves & Great Vessels
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7
Q

What is Tommy Burch recommended sequence of events to identify for complex congenital abnormalities?

A
  1. Thoraco-Abdominal situs (Solitus, Inversus, Ambiguous)
  2. Cardiac Position (Levo, Meso, Dextro)
  3. Segment by Segment analysis (Atria, AV, Ventricles, Outflow, Great vessels)
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8
Q

For the atrium, what is situs solitus?

A

Normal RA and LA = Situs Solitus

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9
Q

For the atrium, what is situs inversus?

A

Mirror image of situs solitus

LA on the Right

RA on the Left

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10
Q

For the atrium, what is situs ambigous?

A

Could be one, two atrium.

Grab bag of random

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11
Q

What are the characteristics of the Right atrium?

Pecinate Muscles

A
  • Pectinate muscles outside of the right atrial appendage
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12
Q

What are the characteristics of the Right atrium:

Right Atrial Appendage

A
  • RAA is broad based, triangular, and anterior
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13
Q

What are the characteristics of the Right atrium?

Septum?

A

Septum Secundum (limbus of fossa ovalis)

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14
Q

What are the characteristics of the Right atrium

What is usually present for major vessels and landmarks?

A
  • IVC, SVC, CS usually,
  • Crista Terminalis and tinea sagittalis
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15
Q

For the Left atrium, where are the pectinate muscles?

A

Confined to the left atrial appendage

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16
Q

For the left atrial appendage, what is the description?

A

Long and narrow (Finger like)

Located more posterior

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17
Q

What septum is a part of the left atrium?

A

Septum Primum

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18
Q

What structures are located in the LA?

A

Pulmonary veins

(Exception is Anomalous venous return)

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19
Q

What is seen here?

A

Complete AV Canal

  1. Primum ASD

&

  1. Inlet VSD
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20
Q

What are the types of looping in terms of ventricles?

Which is normal and most common?

A

D and L loops

D = More common and normal

For D Ventricles

  1. RV is anterior and Rightwaard
  2. LV is posterior and leftward
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21
Q

How can you identify the left ventricle?

  • What valve attached?
  • Comment on the walls?
  • What are well defined in the LV?

What does the outflow tract NOT have?

  • What is in continuity?
A
  1. Mitral valve attached
  2. Smoother walls
  3. Well defined papillary muscles
  4. No Conus (Muscular outflow tract)
  5. Continuity of the MV and AV
22
Q

What is seen here?

A

L - Transposition in the ME 4 chamber

Isolated L-TGA is “physiologically corrected” because systemic deoxygenated venous blood returns to the pulmonary circulation and oxygenated pulmonary venous blood returns to the systemic circulation.

23
Q

Isolated L-TGA patients are at increased risk of what?

What do 90% of them have?

A

Patients with L-TGA are at increased risk for heart failure as adults due to progressive decline in systemic right ventricular (RV) function.

90% have Tricuspid valve dysfunction

24
Q

What do Left Transposition of great vessel patients have a high prediliction for outside of severe TR?

A

Heart Block

  • Have pads on in the room
25
Q

What are the other name for L-TGA?

A

Double discordance

  1. Atrioventricular Discordance = RA –> LV and LA –> RV
  2. Ventriculoarterial Discordance = RV –> Aorta and LV –> PA

Ventricular Inversion

The ventricles are switched

Congenitally corrected transposition of great vessels

Normal in series flow anatomically (Right –> Lungs –> Left –> Systemic circulation

26
Q

What is important to note with transposition patients in regards to aortic and pulmonary valves?

A

They are in the same plane

(see echo clip)

27
Q

What % of L-TGA patients are associated with other lesions?

A

90%

28
Q

What % of L-TGA patients have a VSD?

A

70-80%

29
Q

What % of L-TGA patients have pulmonary outflow obstruction (LVOT obstruction)?

A

30-60%

30
Q

What % of L-TGA patients have tricuspid valve abnormalities?

A

Up to 90%

31
Q

What % of L-TGA patients have mitral valve abnormalities?

A

55%

32
Q

What is the most common arrhythmia in patients with L-TGA?

A

Complete Heart Block

Conduction System is abnormal and unstable

33
Q

What is reverse differential cyanosis?

A

Phenomenon usually only seen with transposition

Post ductal sats > Preductal sats

higher sats in the feet (Post ductal) > lower sats in the RUE (Right arm)

34
Q

D-TGA
Do you have atrio-ventricular concordance or discordance?

A

D-TGA

AV Concordance - RA dumps into RV and LA dumps into LV

35
Q

D-TGA
Do you have ventriculoarterial concordance* or *discordance?

A

D-TGA
Ventriculoarterial discordance

LV –> PA

RV –> PA

36
Q

Do you have D-TGA associated with other non-cardiac anomalies?

A

Rarely

37
Q

What percentage of D-TGA patients have VSD?

A

50%

38
Q

L-TGA:

What % of patients have VSD?

A

70-80%

39
Q

L-TGA:

Pulmonary outflow tract obstruction (LVOT obstruction)?

A

30-60%

40
Q

L-TGA:

What % of patients have Tricuspid abnormalities?

A

~90%

41
Q

L-TGA:

What % of patients have Mitral valve abnormalities?

A

55%

42
Q

L-TGA:

What is the most common arrythmia?

A

complete heart block (conduction system is abnormal and unstable)

43
Q

L-TGA:

What are the 3 other names?

A
  1. Double Discordance
  2. Ventricular inversion
  3. Congenitally corrected TGA
44
Q

L-TGA:

Is ventriculoarterial discordance present?

A

Yes

45
Q

L-TGA:

Is atrioventricular discordance present?

A

Yes

46
Q

What are the 5 cardiac lesions that occur in L-TGA?

A
  1. VSD
  2. Pulm Outflow Obstruction
  3. TV abnormalities
  4. MV abnormalities
  5. Complete HB
47
Q

What procedure can be done with L-TGA patients to improve TR?

Explain how this works

A

Pulmonary Artery banding

Pathophysiology

  • PA Banding increases LV pressure by shifting the septum towards the systemic RV improving RV geometry which decreases TR
  • Alters the LV/RV pressure ratio, reduces RV sphericity aand improves RV geometry –> Decrease TR
48
Q

When would a women be counseled against pregnancy with L-TGA?

A

Systemic Ventricular EF <40% and/or NYHA Class III or IV symptoms

49
Q

What is the most common cyanotic heart lesion?

A

Tetralogy of Fallot

50
Q
A

PTE me (33 decks)

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