Segmental approach in congenital heart disease [autosaved].pptx 2.pptx final

Segmental approach in
congenital heart disease
SHABNAM.MOHAMMADZADEH.MD
CARDIOLOGIST
FELLOW OF ADULT CONGENITAL HEART DISEASE

Segmental approach
Step1 • Cardiac position
Step2 • Great veins
Step 3
• Visceral and atrial situs
Step 4 • Venoatrial connection
Step 5 • Ventricular looping
Step 6 • Atrioventricular connections
Step 7 • Relationship between great arteries
Step 8 • Ventriculoarterial connection
Step 9 • Associated anomalies
SHABNAM.MOHAMMADZADEH, MD

Cardiac Orientation
-Relationship or axis of the base to
the apex of the heart .
(Levocardia,Dextrocardia,
Mesocardia)
- If the cardiac apex fails to shift, it
may result in situs solitus with
dextrocardia which is termed
dextroversion or situs inversus
with levocardia called levoversion.

Nomenclature for Thoracic and Visceral
Situs
Situs or sidedness: position or arrangement of structures or organs that are not bilaterally
symmetric.
Solitus, :Normal
Inversus : Mirror image of normal
Ambiguous: (not clearly solitus or inversus). Thoracic and abdominal organs cannot be
lateralized , have neither the normal nor mirror image arrangement.

Situs Deﬁnitions
-Thoracic Situs: The right main
bronchus takes a more vertical course
and branches at an earlier point than
the more horizontally oriented left
bronchus.
-The morphologically left bronchus is
long, and it branches only after it has
been crossed by its accompanying
pulmonary artery, making the
bronchus hyparterial.
-The morphologically right bronchus is
short, and is crossed by its pulmonary
artery only after it has branched,
giving an eparterial pattern of
branching

-Abdominal Situs:

POINTS
Situs inversus with dextrocardia (complete situs inversus, mirror image dextrocardia, usually
occurs without coexisting congenital heart disease. Isolated atrial inversion is rare
 Situs solitus with dextrocardia is only occasionally associated with a structurally normal heart
left-to-right shunts at atrial level or ventricular level usually coexist.
Situs inversus with levocardia is consistently associated with coexisting congenital heart
disease

Situs Inversus with Dextrocardia
-The incidence rate in the general population is estimated at 1/8000 to 1/25,000.
- The heart and the thoracic and abdominal viscera are mirror images of normal .
-The bronchi are inverted
- The heart is right-sided
-the right hemidiaphragm is lower than the left hemidiaphragm
-The descending aorta is on the right
- the ascending aorta, aortic knuckle, and pulmonary trunk are in their mirror image positions;
-L-Loop ventricle.

Situs Solitus with Dextrocardia
The lungs and abdominal viscera are situs solitus,
-The heart is right thoracic (dextrocardia)
-The ascending aorta and aortic knuckle occupy their normal positions and the descending aorta
runs its normal course along the left vertebral border
-The major cardiac shadow lies to the right of midline (dextrocardia), the base to apex axis points
to the right, and the right hemidiaphragm is lower than the left hemidiaphragm

Situs Inversus with Levocardia
The left hemidiaphragm is lower than the right hemi-diaphragm because the
apex is on the left
Inversion of the bronchi ,coincides with inversion of the atria and lungs.
The stomach is on the right, and the liver is on the left

RA LA
-RAA: triangular, broad-based
-Crista terminalis
-The thicker superior limbus of the
atrial septum around the fossa ovalis
-CS entrance
-LAA: smaller, thinner, and ﬁnger-like
-The ﬂap of the valve at the oval
fossa
-CS course

- The superior vena cava (SVC) is not an acceptable criterion
to identify the morphologic right atrium
-Identifying pulmonary venous connections is not a stern
rule for identifying the morphologic left atrium.
- Atrial situs follows abdominal situs in about 70% to 80% of
the cases
-It is often called situs inversus, even though the atrial
chambers are not upside down.
POINTS

Segmental approach
Step1 • Cardiac position
Step2 • Great veins
Step 3 • Venoatrial
Step 4 • Visceral and atrial situs
Step 5 • Ventricular looping
Step 6 • Atrioventricular connections
Step 7 • Relationship between great arteries
Step 8 • Ventriculoarterial connection
Step 9 • Associated anomalies

Deﬁnitions for Cardiac Ventricles
-Subpulmonic conus
-Coarse trabeculation
-Moderator band
-Septal attachment of
TV chorda
-More apical TV
RV
-Fibrous outlet
-Thin trabeculation
-No moderator band
- No septal
attachment of MV
-Higher MV
LV
always

The feature of solitary and indeterminate ventricle
Coarse trabeculation than the morphological right ventricle
There is no other chamber in the ventricular mass
 Rudimentary right ventricle usually situated anterosuperiorly
either leftward or rightward position and rudimentary left ventricle
is positioned inferiorly
The heart where one AV valve confined to the ventricle and the
other AV valve overrides more than 50% will be described as double
inlet ventricle

Ventricular topology
- spatial relationship of one ventricle to the other
- Right hand topology is the normal pattern. Determination of ventricular topology requires first
identification of the morphologic right ventricle. If the palmar surface of right hand can be
placed on the septal surface, thumb in the inlet and the fingers toward the outlet and the wrist
is at the apex, then this is the right –hand pattern. If only the left hand palm can be placed on
the septal surface of the right ventricle in the same manner, then this will be described as left
hand topology. Ventricular topology allows analysis of the atrioventricular junction in hearts
with isomeric arrangement of atrial appendages.
,

LEFT HAND TOPOLOGY

RIGHT HAND TOPOLOGY

Nomenclature for Atrioventricular connection
 Concordant (normal),
Discordant, : RA to the morphologic LV ,LA into the morphologic RV
Ambiguous, : Half atrioventricular junction, concordant,half, discordantly
Double-inlet,
Absent connection.

A-V connection in
functionally
univentricular hearts
1. Double inlet : functional
univentricle connected to two
separate atria with two separate
atrioventicular valves.
2. Single inlet : two separate atria
with only one of the atria
connected to the functional
univentricle via one
atrioventricular valve. The other
atria connection is atretic.
3. Common inlet : both atria are
connected to a functional
univentricle via one atrioventicular
valve.

 Straddling is a feature of the
chordae tendineae of an atrio-
ventricular valve and describes
chordae that cross a ventricular
septal defect and have their
attachments in the opposite
ventricle.
 Overriding is a feature of the
valve annulus which describes an
annulus that crosses a ventricular
septal defect and thus lies “over”
more than one ventricle.

Deﬁnitions for
Great Arteries
 The great vessels are typically described by the terms
solitus, inversus, dextro, and levo.
Solitus : Normal anatomically relationship between the great vessels.
Inversus :mirror image anatomic relationship.
 Dextro : great vessels on the right side of the body
levo : the great vessels are on the left.
 The great arteries :identiﬁed by their branches .
The location of the conus can help to identify the pulmonary artery
 A common arterial trunk : a vessel connected to the ventricle (or ventricles) via a common ventriculoarterial valve.
 A solitary arterial trunk : vessel arising from ventricle or ventricles that does not give rise to intrapericardial pulmonary
arteries. In this anomaly, the blood supply to the lung usually comes from collateral vessels originating from either the
ascending or descending thoracic aorta.

Nomenclature for
Ventriculoarterial
 Concordant,
 Discordant,
 Double-outlet right ventricle,
 Double-outlet left ventricle.
 Ventriculoarterial overriding
:more than half of the area of
the outlet overrides the
ventricular septum.

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