Download Segmental Analysis I

Segmental Analysis
Gautam K. Singh, M.D.
Washington University School of Medicine
St. Louis
Segmental Analysis
Segmental Analysis: From Veins to Ventricles
Segmental Approach to Evaluation of
Congenital Heart Defects
Objectives: Have an organized approach to
evaluation of congenital heart disease (CHD)
by defining


Correct morphology of cardiac segments
Alignment (connections) of cardiac segments
Segmental Approach to Evaluation of
Congenital Heart Defects

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Three main Cardiac Segments:
Atria (and Veins)
Ventricles
Great Arteries
Two Connections:
Atrioventricular Connection (Canal)
Ventriculo-arterial Connection
(Infundibular or Conal Connection)
Segmental Approach to CHD
Three Cardiac Segments
and
Two Connections
Minimum possible
segmental arrangements:
32 x 22 = 36
Segmental Approach to CHD
Three Cardiac Segments
and
Two Connections
Minimum possible
arrangements:
32 x 22 = 36
Van Praagh, in Moss and Adams’,3rd ed, 1989
Segmental Approach to CHD
Cardiac Development
Segmental Approach to CHD
Looping of the Cardiac Tube
Start of looping
Rightward looping
Manner J. Anat Rec 2000
Segmental Approach to CHD
Looping of the Cardiac Tube
Cardiac loop formation as observed embryologically
with the loop rule for ventricular localization
Van Praagh, Moss and Adams’ Heart Diseases, 3rd ed, 1989
Segmental Approach to CHD
Cardiac Looping:
Positional and
morphological changes
of the embryonic heart
tube during the
transformation from the
c-shaped loop into the
s-shaped loop.
Shortening
of
distance between
conus and atrium
Ventricle
shifts
caudally
Manner J. Anat Rec 2000
Segmental Approach to CHD
Cardiac Development: Re-alignments
Manner J. Anat Rec 2000
Segmental Approach to CHD
Cardiac Development: Septation
Ventricles
Formation of
Dhanantwari et al, Circulation 2009
Segmental Approach to CHD
Cardiac Development: Septation
Conotruncus
Formation of
Manner J. Anat Rec 2000
Segmental Approach to CHD – Situs
Body Segment:
 Atrial
Situs:
 Solitus

Bronchial

Inversus

Abdominal

Ambigous/Isomerism
Segmental Approach to CHD – Situs


Body Segments: Atrial, Bronchial, and Abdominal
Situs (Position): Solitus, Inversus, and Ambiguus
Situs Solitus Situs Inversus Situs Ambiguus
Segmental Approach to CHD – Situs
Situs: (a) AtrialSolitus
(b) BronchialSolitus
(c) AbdominalSolitus
Segmental Approach to CHD – Situs
Situs: (a) Atrial, (b) Bronchial, (c) Abdominal:
Inversus and Ambiguus/Isomerism
(a) Atrial situs Inversus
(b) Left Bronchial Isomerism (c) Abdominal Situs Inversus
Courtsey: Atlas of Heart Disease
Segmental Development – Atrial Septation
Dhanantwari et al, Circulation 2009
Segmental Morphology - Atrium
RA: MRI showing IVC connection to RA
Segmental Approach to CHD – Atrium
Situs: (a) Atrial, and (b) Abdominal:
Inversus and Ambiguus/Isomerism
(a) Atrial situs Inversus
(b) Abdominal Situs Inversus
Moss and Adams’, 7th ed, 2008
Segmental Morphology - Atrium
Atrial Morphology: Venous connection, Inner surface
and Appendage
RA: IVC to RA (A),
Fossa Ovalis
(B), Inner
surface with
Crista T, &
Appendage (D)
LA:Other atrum,
Inner surface,
Pulm.Veins, &
appendage (E)
Segmental Morphology - Atrium
Atrial Morphology: Inner surface and Appendage
RA: the limbus of the fossa ovalis (arrowhead), and
LA: the valve of the fossa ovalis (arrows).
Segmental Morphology - Atrium
Atrial Septal Defects


Sinus Venosus Defect
Partial Anomalous Venous Connection of RUPV
Moss and Adams’ Heart Disease, 7th ed,2008
Segmental Alignment - Veins and Atria
Anomalous Atrio-venous connection
Cortriatrium
TAPVC
Courtsey: Atlas of Heart Disease
Segmental Alignment – Veins and Atria
Annomalous Pulmonary Venous Connection
PAPVC
TAPVC
TAPVC
Moss and Adams” Heart Disease, 7th ed,2008
Segmental Development –
Ventricular Septation
Dhanantwari et al, Circulation 2009
Segmental Morphology - Ventricle
Ventricle: Cavity
RV:
Tripatite –
Inflow,
Body, &
Outflow
LV:
Bipatite –
Inflow &
Body merged,
with Outflow
Segmental Morphology - Ventricle
Ventricular Cavity: Trabeculation
RV: heavily trabeculated,
Muscle bands in outflow tract
LV: finely trabeculated,
Smooth outflow tract
Segmental Morphology – Ventricular Septum
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Inlet septum –TV/MV
Trabecular attachments of TV
leaflets to apex and to crista
supraventricularis
Outlet or infundibular from
crista to PV
Membranous divided into two
by the septal leaflet of TV
Segmental Morphology – Ventricular Septum
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The infundibular septum is between PV and AoV above crista.
The membranous septum is between the AoV and TV.
The inlet septum is related to the AV canal.
Segmental Morphology – VSD
Paramembranous VSD
Segmental Morphology – VSD
Outlet VSD (supracristal, conal, infundibular, subarterial…)
Segmental Morphology – VSD
Inlet VSD
Segmental Morphology – VSD
Muscular VSDs
Segmental Morphology - Ventricle
Ventricle: carries its own valve, LV - MV; RV - TV
MV:
Bileaflet,
No septal
attachment
TV:
Trileaflet,
Has septal
attachment
Segmental Morphology - Ventricle
Ventricle: carries its own valve, LV-MV; RV-TV
MV: Basal placed,
leaflets attached to
papillary muscles on
LV free wall.
TV: Apically placed,
leaflets attached to
papillary muscles on
septum and RV
apex and free wall.
Segmental Morphology - Ventricle
Ventricle: carries its own valve, LV-MV; RV-TV
LV and RV: Normal Morphology –
4-Chamber MR image
Segmental Alignment Development –
AV Valve Morphogenesis
Dhanantwari et al, Circulation 2009
Segmental Alignment – Atrio-ventricle
Connection
Atrio-ventricular (AV) Concordance and Discordance
AV Concordance: Normal
Connection
AV Discordance: Ventricular
Inversion
Segmental Alignment – Atrio-ventricle
Connection
Atrio-ventricular (AV) Concordance and Discordance
AV Concordance: Normal
Connection
AV Discordance: Ventricular
Inversion
Segmental Alignment – Atrio-ventricle
Connection
AV concordance
Over-riding
Straddling
Double inlet
Moss and Adams’, 7th ed, 2008
Segmental Alignment – Atrio-ventricle
Connection
AV concordance
Over-riding
Straddling
Double inlet
Moss and Adams’, 7th ed, 2008
Segmental Alignment – Atrio-ventricle
Connection
AV concordance
AV concordance
Over-riding
Straddling TV
Straddling
Double Inlet
Double Inlet LV
Moss and Adams’, 7th ed, 2008
Segmental Alignment – Atrio-ventricle
Connection
AV concordance
Over-riding
Straddling
Double inlet
Moss and Adams’, 7th ed, 2008
Segmental Alignment – Atrio-ventricle
Connection
AV concordance
Over-riding
DILV & “L-TGA”
Straddling
Double inlet
DIRV & “DORV”
Moss and Adams’, 7th ed, 2008
Segmental Alignment – Atrio-ventricle
Connection
Univentricular Atrioventricular (AV) Connections.
Moss and Adams’, 7th ed, 2008
Segmental Alignment – Atrio-ventricle
Connection
Univentricular Atrioventricular (AV) Connections.
Moss and Adams’, 7th ed, 2008
Segmental Alignment – Atrio-ventricle
Connection
Atrio-ventricular (AV) Concordance or Discordance ?
AV canal: Common AV valve
Straddling TV valve
Segmental Analysis
Segmental Analysis: From Ventricles to Arteries
Segmental Alignment Development –
Truncus arteriosus septation
Dhanantwari et al, Circulation 2009
Segmental Alignment – Ventriculoarterial Connection
Ventriculo-arterial Connection:

Concordance: Normal

Discordance: TGV

Double outlet: DORV/DOLV

Truncus Arteriosus
Segmental Alignment – Ventriculoarterial Connection
Ventriculo-arterial Connection:

Concordance: Normal

Discordance: TGV

Double outlet: DORV/DOLV

Truncus Arteriosus
Segmental Alignment – Ventriculo-arterial
Connection
Concordance: Normal
Segmental Alignment – Ventriculoarterial Connection
Ventriculo-arterial Connection:

Concordance: Normal

Discordance: TGV

Double outlet: DORV/DOLV

Truncus Arteriosus
Segmental Alignment – Ventriculoarterial Connection
Ventriculo-arterial Discordance: D Transposition of
Great Arteries (D-TGA)
Position of
Great Arteries
Origins of Great Aarteries
Moss and Adams’, 7th ed, 2008
Relationship of
Great Arteries
Segmental Alignment – Ventriculoarterial Connection
Ventriculo-arterial Discordance: Congenitally
Corrected Transposition of Great Arteries or
L- Transposition of Great Arteries
CC-TGA
L-TGA
Segmental Alignment – Ventriculoarterial Connection
Ventriculo-arterial Discordance: Congenitally
Corrected Transposition of Great Arteries or
L- Transposition of Great Arteries
Origin of Great Arteries
Morphology of ventricles
Position of Great Arteries
Segmental Alignment – Ventriculoarterial Connection
Ventriculo-arterial Connection:
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Concordance: Normal
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Discordance: TGV

Double outlet: DORV/DOLV

Truncus Arteriosus
Segmental Alignment – Ventriculoarterial Connection
Ventriculo-arterial Connection: Double Outlet RV
Development of Conotruncus and DORV
DORV
Normal
Segmental Alignment – Ventriculoarterial Connection
Ventriculo-arterial Connection: Double Outlet RV
Position of the VSD
Moss and Adams’ Heart Diseases , 7th ed, 2008
Segmental Alignment – Ventriculoarterial Connection
Ventriculo-arterial Connection: Double Outlet RV
DORV with Sub-pulomonary VSD
Moss and Adams’ Heart Diseases , 7th ed, 2008
Segmental Alignment – Ventriculoarterial Connection
Ventriculo-arterial Connection: Double Outlet RV
DORV with Side by Side Great Arteries
Segmental Alignment – Ventriculoarterial Connection
Ventriculo-arterial Connection:

Concordance: Normal

Discordance: TGV

Double outlet: DORV/DOLV

Truncus Arteriosus
Segmental Alignment – Ventriculoarterial Connection
Ventriculo-arterial Connection: Double Outlet LV
Different arrangement of Great Arteries and
Relationship with VSD
Van Praagh, Moss and Adams’ Heart Diseases , 3rd ed, 1989
Segmental Alignment – Ventriculoarterial Connection
Ventriculo-arterial Connection: Double Outlet LV
Tricuspid Atresia with Double Outlet LV and Subpulmonary VSD
Segmental Alignment – Ventriculoarterial Connection
Ventriculo-arterial Connection:
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Concordance: Normal
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Discordance: TGV

Double outlet: DORV/DOLV

Truncus Arteriosus
Segmental Alignment – Ventriculoarterial Connection
Ventriculo-arterial Connection: Truncus Arteriosus
Courtesy: Atlas of Heart Diseases
Segmental Alignment – Ventriculoarterial Connection
Ventriculo-arterial Connection: Truncus Arteriosus
Development of Persistent Truncus Arteriosus
Classification of persistent truncus arteriosus. Top: Type I to IV, by Collett and Edwards (1949).
Bottom: Types A1 to A4, by Van Praagh and Van Praagh
Segmental Alignment – Ventriculoarterial Connection
Ventriculo-arterial Connection: Truncus Arteriosus
Persistent Truncus Arteriosus: Type I or A1
Segmental Alignment – Ventriculoarterial Connection
Ventriculo-arterial Connection: Truncus Arteriosus
Persistent Truncus Arteriosus: Type I or A1
Segmental Alignment – Ventriculoarterial Connection
TOF/Pseudotruncus/Pulmonary Atresia
 Aorta
gives
off collaterals
to each lung.
 Absent
central and
branch
pulmonary
arteries
Courtesy: Dr. Pamela Woodard
Segmental Alignment – Ventriculoarterial Connection
Ventriculo-arterial Connection:

Concordance: Normal

Discordance: TGV

Double outlet: DORV/DOLV

Truncus Arteriosus
Tetralogy of Fallot and
Pulmonary Atresia with VSD
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Segmental Alignment – Ventriculoarterial Connection
Ventriculo-arterial Connection: Tetralogy of Fallot and
Pulmonary Atresia with VSD
Segmental Alignment – Ventriculoarterial Connection
Ventriculo-arterial Connection: Tetralogy of Fallot
Segmental Alignment – Ventriculoarterial Connection
Ventriculo-arterial Connection: Tetralogy of Fallot
Segmental Diagnosis of the Day
Segmental Diagnosis of the Day
Segmental Diagnosis of the Day
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Atrial situs Inversus
Atrioventricular Discordance
Ventriculo-arterial Discordance
“D- Transposition of the Aorta”
VSD
LV-to-PA conduit.
Thank You