Segmental Analysis. Gautam K. Singh, M.D. Washington University School of Medicine St. Louis
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1 Segmental Analysis Gautam K. Singh, M.D. Washington University School of Medicine St. Louis
2 Segmental Analysis Segmental Analysis: From Veins to Ventricles
3 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
4 Segmental Approach to Evaluation of Congenital Heart Defects Three main Cardiac Segments: Atria (and Veins) Ventricles Great Arteries Two Connections: Atrioventricular Connection (Canal) Ventriculo-arterial Connection (Infundibular or Conal Connection)
5 Segmental Approach to CHD Three Cardiac Segments and Two Connections Minimum possible segmental arrangements: 3 2 x 2 2 = 36
6 Segmental Approach to CHD Three Cardiac Segments and Two Connections Minimum possible arrangements: 3 2 x 2 2 = 36 Van Praagh, in Moss and Adams,3 rd ed, 1989
7 Segmental Approach to CHD Cardiac Development
8 Segmental Approach to CHD Looping of the Cardiac Tube Start of looping Rightward looping Manner J. Anat Rec 2000
9 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, 3 rd ed, 1989
10 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
11 Segmental Approach to CHD Cardiac Development: Re-alignments Manner J. Anat Rec 2000
12 Segmental Approach to CHD Cardiac Development: Septation Ventricles Formation of Dhanantwari et al, Circulation 2009
13 Segmental Approach to CHD Cardiac Development: Septation Conotruncus Formation of Manner J. Anat Rec 2000
14 Segmental Approach to CHD Situs Body Segment: Atrial Situs: Solitus Bronchial Inversus Abdominal Ambigous/Isomerism
15 Segmental Approach to CHD Situs Body Segments: Atrial, Bronchial, and Abdominal Situs (Position): Solitus, Inversus, and Ambiguus Situs Solitus Situs Inversus Situs Ambiguus
16 Segmental Approach to CHD Situs Situs: (a) Atrial- (b) Bronchial- (c) Abdominal- Solitus Solitus Solitus
17 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
18 Segmental Development Atrial Septation Dhanantwari et al, Circulation 2009
19 Segmental Morphology - Atrium RA: MRI showing IVC connection to RA
20 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, 7 th ed, 2008
21 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)
22 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).
23 Segmental Morphology - Atrium Atrial Septal Defects Sinus Venosus Defect Partial Anomalous Venous Connection of RUPV Moss and Adams Heart Disease, 7 th ed,2008
24 Segmental Alignment - Veins and Atria Anomalous Atrio-venous connection Cortriatrium TAPVC Courtsey: Atlas of Heart Disease
25 Segmental Alignment Veins and Atria Annomalous Pulmonary Venous Connection PAPVC TAPVC TAPVC Moss and Adams Heart Disease, 7 th ed,2008
26 Segmental Development Ventricular Septation Dhanantwari et al, Circulation 2009
27 Segmental Morphology - Ventricle Ventricle: Cavity RV: Tripatite Inflow, Body, & Outflow LV: Bipatite Inflow & Body merged, with Outflow
28 RV: heavily trabeculated, Muscle bands in outflow tract LV: finely trabeculated, Smooth outflow tract Segmental Morphology - Ventricle Ventricular Cavity: Trabeculation
29 Segmental Morphology Ventricular Septum 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
30 Segmental Morphology Ventricular Septum 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.
31 Segmental Morphology VSD Paramembranous VSD
32 Segmental Morphology VSD Outlet VSD (supracristal, conal, infundibular, subarterial )
33 Segmental Morphology VSD Inlet VSD
34 Segmental Morphology VSD Muscular VSDs
35 Segmental Morphology - Ventricle Ventricle: carries its own valve, LV - MV; RV - TV MV: Bileaflet, No septal attachment TV: Trileaflet, Has septal attachment
36 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.
37 Segmental Morphology - Ventricle Ventricle: carries its own valve, LV-MV; RV-TV LV and RV: Normal Morphology 4-Chamber MR image
38 Segmental Alignment Development AV Valve Morphogenesis Dhanantwari et al, Circulation 2009
39
40 Segmental Alignment Atrio-ventricle Connection Atrio-ventricular (AV) Concordance and Discordance AV Concordance: Normal Connection AV Discordance: Ventricular Inversion
41 Segmental Alignment Atrio-ventricle Connection Atrio-ventricular (AV) Concordance and Discordance AV Concordance: Normal Connection AV Discordance: Ventricular Inversion
42 Segmental Alignment Atrio-ventricle Connection AV concordance Over-riding Straddling Double inlet Moss and Adams, 7 th ed, 2008
43 Segmental Alignment Atrio-ventricle Connection AV concordance Over-riding Straddling Double inlet Moss and Adams, 7 th ed, 2008
44 Segmental Alignment Atrio-ventricle Connection AV concordance Over-riding Straddling Double Inlet AV concordance Straddling TV Double Inlet LV Moss and Adams, 7 th ed, 2008
45 Segmental Alignment Atrio-ventricle Connection AV concordance Over-riding Straddling Double inlet Moss and Adams, 7 th ed, 2008
46 Segmental Alignment Atrio-ventricle Connection AV concordance Over-riding Straddling Double inlet DILV & L-TGA DIRV & DORV Moss and Adams, 7 th ed, 2008
47 Segmental Alignment Atrio-ventricle Connection Univentricular Atrioventricular (AV) Connections. Moss and Adams, 7 th ed, 2008
48 Segmental Alignment Atrio-ventricle Connection Univentricular Atrioventricular (AV) Connections. Moss and Adams, 7 th ed, 2008
49 Segmental Alignment Atrio-ventricle Connection Atrio-ventricular (AV) Concordance or Discordance? AV canal: Common AV valve Straddling TV valve
50 Segmental Analysis Segmental Analysis: From Ventricles to Arteries
51 Segmental Alignment Development Truncus arteriosus septation Dhanantwari et al, Circulation 2009
52 Segmental Alignment Ventriculoarterial Connection Ventriculo-arterial Connection: Concordance: Normal Discordance: TGV Double outlet: DORV/DOLV Truncus Arteriosus
53 Segmental Alignment Ventriculoarterial Connection Ventriculo-arterial Connection: Concordance: Normal Discordance: TGV Double outlet: DORV/DOLV Truncus Arteriosus
54 Segmental Alignment Ventriculo-arterial Connection Concordance: Normal
55 Segmental Alignment Ventriculoarterial Connection Ventriculo-arterial Connection: Concordance: Normal Discordance: TGV Double outlet: DORV/DOLV Truncus Arteriosus
56 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, 7 th ed, 2008 Relationship of Great Arteries
57 Segmental Alignment Ventriculoarterial Connection Ventriculo-arterial Discordance: Congenitally Corrected Transposition of Great Arteries or L- Transposition of Great Arteries CC-TGA L-TGA
58 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
59 Segmental Alignment Ventriculoarterial Connection Ventriculo-arterial Connection: Concordance: Normal Discordance: TGV Double outlet: DORV/DOLV Truncus Arteriosus
60 Segmental Alignment Ventriculoarterial Connection Ventriculo-arterial Connection: Double Outlet RV Development of Conotruncus and DORV DORV Normal
61 Segmental Alignment Ventriculoarterial Connection Ventriculo-arterial Connection: Double Outlet RV Position of the VSD Moss and Adams Heart Diseases, 7 th ed, 2008
62 Segmental Alignment Ventriculoarterial Connection Ventriculo-arterial Connection: Double Outlet RV DORV with Sub-pulomonary VSD Moss and Adams Heart Diseases, 7 th ed, 2008
63 Segmental Alignment Ventriculoarterial Connection Ventriculo-arterial Connection: Double Outlet RV DORV with Side by Side Great Arteries
64 Segmental Alignment Ventriculoarterial Connection Ventriculo-arterial Connection: Concordance: Normal Discordance: TGV Double outlet: DORV/DOLV Truncus Arteriosus
65 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
66 Segmental Alignment Ventriculoarterial Connection Ventriculo-arterial Connection: Double Outlet LV Tricuspid Atresia with Double Outlet LV and Subpulmonary VSD
67 Segmental Alignment Ventriculoarterial Connection Ventriculo-arterial Connection: Concordance: Normal Discordance: TGV Double outlet: DORV/DOLV Truncus Arteriosus
68
69 Segmental Alignment Ventriculoarterial Connection Ventriculo-arterial Connection: Truncus Arteriosus Courtesy: Atlas of Heart Diseases
70 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
71 Segmental Alignment Ventriculoarterial Connection Ventriculo-arterial Connection: Truncus Arteriosus Persistent Truncus Arteriosus: Type I or A1
72 Segmental Alignment Ventriculoarterial Connection Ventriculo-arterial Connection: Truncus Arteriosus Persistent Truncus Arteriosus: Type I or A1
73 Aorta gives off collaterals to each lung. Absent central and branch pulmonary arteries Segmental Alignment Ventriculoarterial Connection TOF/Pseudotruncus/Pulmonary Atresia Courtesy: Dr. Pamela Woodard
74 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
75 Segmental Alignment Ventriculoarterial Connection Ventriculo-arterial Connection: Tetralogy of Fallot and Pulmonary Atresia with VSD
76 Segmental Alignment Ventriculoarterial Connection Ventriculo-arterial Connection: Tetralogy of Fallot
77 Segmental Alignment Ventriculoarterial Connection Ventriculo-arterial Connection: Tetralogy of Fallot
78 Segmental Diagnosis of the Day
79 Segmental Diagnosis of the Day
80 Segmental Diagnosis of the Day Atrial situs Inversus Atrioventricular Discordance Ventriculo-arterial Discordance D- Transposition of the Aorta VSD LV-to-PA conduit.
81 Thank You
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