Congenital Heart Disease Part 6 Flashcards by Tim Montrief

What is situs solitus?

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.

How well did you know this?

Not at all

Perfectly

What is situs inversus?

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).

How well did you know this?

Not at all

Perfectly

What is situs ambiguous?

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.

How well did you know this?

Not at all

Perfectly

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

Levocardia (normal)
Mesocardia
Dextrocardia

How well did you know this?

Not at all

Perfectly

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

Apex position of the heart

How well did you know this?

Not at all

Perfectly

What is the segment by segment analysis of the heart?

Atria
Atrioventricular valves
Ventricles
Outflow Tracts (Conus = Infundibulum)
Semilunar Valves & Great Vessels

How well did you know this?

Not at all

Perfectly

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

Thoraco-Abdominal situs (Solitus, Inversus, Ambiguous)
Cardiac Position (Levo, Meso, Dextro)
Segment by Segment analysis (Atria, AV, Ventricles, Outflow, Great vessels)

How well did you know this?

Not at all

Perfectly

For the atrium, what is situs solitus?

Normal RA and LA = Situs Solitus

How well did you know this?

Not at all

Perfectly

For the atrium, what is situs inversus?

Mirror image of situs solitus

LA on the Right

RA on the Left

How well did you know this?

Not at all

Perfectly

For the atrium, what is situs ambigous?

Could be one, two atrium.

Grab bag of random

How well did you know this?

Not at all

Perfectly

What are the characteristics of the Right atrium?

Pecinate Muscles

Pectinate muscles outside of the right atrial appendage

How well did you know this?

Not at all

Perfectly

What are the characteristics of the Right atrium:

Right Atrial Appendage

RAA is broad based, triangular, and anterior

How well did you know this?

Not at all

Perfectly

What are the characteristics of the Right atrium?

Septum?

Septum Secundum (limbus of fossa ovalis)

How well did you know this?

Not at all

Perfectly

What are the characteristics of the Right atrium

What is usually present for major vessels and landmarks?

IVC, SVC, CS usually,
Crista Terminalis and tinea sagittalis

How well did you know this?

Not at all

Perfectly

For the Left atrium, where are the pectinate muscles?

Confined to the left atrial appendage

How well did you know this?

Not at all

Perfectly

For the left atrial appendage, what is the description?

Long and narrow (Finger like)

Located more posterior

How well did you know this?

Not at all

Perfectly

What septum is a part of the left atrium?

Septum Primum

How well did you know this?

Not at all

Perfectly

What structures are located in the LA?

Pulmonary veins

(Exception is Anomalous venous return)

How well did you know this?

Not at all

Perfectly

What is seen here?

Complete AV Canal

Primum ASD

Inlet VSD

How well did you know this?

Not at all

Perfectly

What are the types of looping in terms of ventricles?

Which is normal and most common?

D and L loops

D = More common and normal

For D Ventricles

RV is anterior and Rightwaard
LV is posterior and leftward

How well did you know this?

Not at all

Perfectly

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?

Mitral valve attached
Smoother walls
Well defined papillary muscles
No Conus (Muscular outflow tract)
Continuity of the MV and AV

What is seen here?

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.

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

What do 90% of them have?

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

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

Heart Block

Have pads on in the room

What are the other name for L-TGA?

**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

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

They are in the same plane | (see echo clip)

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

90%

What % of L-TGA patients have a VSD?

70-80%

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

30-60%

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

Up to 90%

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

55%

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

**Complete Heart Block** Conduction System is abnormal and unstable

What is reverse differential cyanosis?

**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)

D-TGA Do you have atrio-ventricular *_concordance_* or *_discordance_*?

D-TGA **AV Concordance** - RA dumps into RV and LA dumps into LV

D-TGA Do you have ventriculoarterial *_concordance*_ or _*discordance_*?

D-TGA Ventriculoarterial *_discordance_* ## Footnote *_LV --\> PA_* *_RV --\> PA_*

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

Rarely

What percentage of D-TGA patients have VSD?

50%

L-TGA: What % of patients have VSD?

70-80%

L-TGA: Pulmonary outflow tract obstruction (LVOT obstruction)?

30-60%

L-TGA: What % of patients have Tricuspid abnormalities?

~90%

L-TGA: What % of patients have Mitral valve abnormalities?

55%

L-TGA: What is the most common arrythmia?

complete heart block (conduction system is abnormal and unstable)

L-TGA: What are the 3 other names?

1. Double Discordance 2. Ventricular inversion 3. Congenitally corrected TGA

L-TGA: Is ventriculoarterial discordance present?

Yes

L-TGA: Is atrioventricular discordance present?

Yes

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

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

What procedure can be done with L-TGA patients to improve TR? Explain how this works

**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

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

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

What is the most common cyanotic heart lesion?

Tetralogy of Fallot