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Admixture lesions in congenital cyanotic heart diseaseRamachandra Barik
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Admixture lesions in congenital cyanotic heart disease
Jaganmohan A Tharakan
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Admixture lesions in congenital cyanotic heart diseaseRamachandra Barik
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Admixture lesions in congenital cyanotic heart disease
Jaganmohan A Tharakan
Department of Cardiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India
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STATEMENT OF NEED
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Target Audience
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Learning Objectives
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Study Resources:
1. Chapter 11, Guyton and Hall Textbook of Medical Physiology, 14th edition
2. Chapter 9, Human Physiology - From Cells to Systems, Lauralee Sherwood, 9th edition
3. Chapter 29, Ganongâs Review of Medical Physiology, 26th edition
4. Electrocardiogram, StatPearls - https://www.ncbi.nlm.nih.gov/books/NBK549803/
5. ECG in Medical Practice by ABM Abdullah, 4th edition
6. ECG Basics, http://www.nataliescasebook.com/tag/e-c-g-basics
6. OBJECTIVES
⢠Identify cardiac segments and components.
⢠Understand and apply segmental approach.
⢠Review variations in cardiac connections.
⢠To see basic imaging techniques.
⢠Employ knowledge with case review.
7. 1960
⢠RICHARD VAN PRAAGH
⢠AMERICA
1960
⢠MARIA VICTORIA DE LA CRUZ
⢠MEXICO
1970
⢠ECHO CARDIOGRAPHY BROUGHT THE TERM SEQUENTIAL
SEGMENTAL ANALYSIS
8. SEGMENTAL ANALYSIS.
⢠Sequential approach to evaluating heart.
⢠Evaluates the cardiovascular system in
âsegmentsâ.
⢠Based on pattern recognition of
Normal.
⢠Allows comprehensive examination of
cardiac structure.
10. THE ANSWER IS ..
⢠Congenital heart defect : Simple to very
complex
⢠Cardiac segment are constant (most of the
time)
⢠Approaching in sequence minimizes error rate.
⢠Pattern recognition of normal is best for
defect detection.
⢠Early defect detection improves clinical
outcomes.
11. ⢠Congenital heart defect : Simple to very complex
⢠Cardiac segment are constant (most of the time)
⢠Approaching in sequence minimizes
error rate.
⢠Pattern recognition of normal is best for defect
detection.
⢠Early defect detection improves clinical
outcomes.
THE ANSWER IS ..
12. THE ANSWER IS ..
⢠Congenital heart defect : Simple to very complex
⢠Cardiac segment are constant (most of the time)
⢠Approaching in sequence minimizes error rate.
⢠Pattern recognition of normal is best
for defect detection.
⢠Early defect detection improves clinical
outcomes.
13. THE ANSWER IS ..
⢠Congenital heart defect : Simple to very complex
⢠Cardiac segment are constant (most of the time)
⢠Approaching in sequence minimizes error rate.
⢠Pattern recognition of normal is best for defect
detection.
⢠Early defect detection improves clinical
outcomes.
14. VAN PRAAG CLASSIFICATION SYSTEM
{ __ , __ , __ }
VISCERO ATRIAL
SITUS VENTRICULAR
LOOP
ORIENTATION
POSITION
AND
RELATION
OF GREAT
VESSELS
19. ATRIAL SEGMENT
â˘PULMONARY VENOUS CONNECTIONS
â˘Usually 4 veins: 2 from each lung
â˘SYSTEMIC VENOUS CONNECTIONS
â˘Single SVC to RA roof.
â˘Hepatic veins convergence with IVC to floor
20. ATRIAL SITUS
ATRIAL SITUS SOLITUS
⢠Position the atria normally occupy
⢠Almost always corresponds to visceral situs
â Viscero atrial situs is often used term
21. ATRIAL SITUS
⢠ATRIAL SITUS INVERSUS
â The viscera and atria are opposite (Mirror Image )
to normal.
22. ATRIAL SITUS
ATRIAL SITUS AMBIGUOS
⢠Atrial isomerism or Heterotaxy syndrome
⢠âBilateral sidednessâ of structures
â Left atrial isomerism
â Right atrial isomerism
23. HETEROTAXY FEATURES- VENOATRIAL
Left Atrial Isomerism
⢠Interrupted IVC
⢠Bilateral SVC
⢠LSVC to CS
⢠Atrial septum primum +
⢠Pulmonary veins ipsilateral
⢠CAVSD
Right Atrial Isomerism
⢠Continuos IVC
⢠Bilateral SVC
⢠Absent CS
⢠Common Atrium
⢠TAPVC
⢠CAVSD
24. VANPRAAG SYSTEM (VISCEROATRIAL)
{X , __ , __ }
VISCERAL SITUS
THORACO-
ABDOMINAL SITUS
ATRIAL SITUS
⢠THREE TYPES OF SITUS â
â SITUS SOLITUS { S,__,__}
â SITUS INVERSUS { I,__,__}
â SITUS AMBIGUOS {A,__,__}
25. VANPRAAG SYSTEM (VISCEROATRIAL)
{X , __ , __ }
⢠Determining the position of visceral
organs.
â SOLITUS (S)
â INVERSUS (I)
â AMBIGUOS (A)
VISCERAL SITUS
THORACO-
ABDOMINAL SITUS
ATRIAL SITUS
26. VANPRAAG SYSTEM (VISCEROATRIAL)
{X , __ , __ }
⢠Pulmonary Sidedness usually
reflects atrial sidedness
⢠Useful for identifying morphologic
RA
VISCERAL SITUS
THORACO-
ABDOMINAL SITUS
ATRIAL SITUS
27. VANPRAAG SYSTEM (VISCEROATRIAL)
{X , __ , __ }
⢠Identify the morphologic RA.
⢠Atrial situs and thoracoabdominal
situs are usually concordant.
VISCERAL SITUS
THORACO-
ABDOMINAL SITUS
ATRIAL SITUS
28. VANPRAAG SYSTEM (VISCEROATRIAL)
{X , __ , __ }
⢠DESIGNATION â
â If ALL letters recorded as S ď { S,__,__}
â If ALL letters recorded as I ď { I,__,__}
â If ANY letter recorded as A ď {A,__,__}
30. ATRIOVENTRICULAR CONNECTION
⢠Type and mode of connection between atria
and ventricles.
⢠Types of AV connection â
â Concordant
â Discordant
â Common
â Double inlet
â Single
36. VENTRICULAR SEGMENT
⢠Described based on morphology
⢠Morphology best defined by internal features.
⢠âLOOPINGâ pertains to the RV position in
relation to the LV.
â Normal is rightward loop (âDâ for dextro)
⢠Describes the spatial position of the
ventricles.
38. VANPRAAGSYSTEM(Ventricular loop orientation)
{__, x , __ }
⢠Relation of Morphologic
RV as compared with
Morphologic LV
â Dextro loop { __, D, __}
â Levo loop { __, L, __}
â Cannot be determined
{__, X, __}
39. VENTRICULOARTERIAL CONNECTION
⢠CONCORDANT
â MPA arises from
morphologic RV
â Aorta arises from
morphologic LV
Irrespective of spatial
location of ventricles
NORMAL â Great Arteries
typically cross
42. ARTERIAL SEGMENT
⢠Position of Aorta in
relation to Pulmonary
Artery.
⢠NORMALLY Aorta is
located posterior and
to the right of MPA ( at
the level of semilunar
valves)
⢠Conus anatomy may
be useful
43.
44. VANPRAAGSYSTEM(POSITIONAND RELATIONOF GREAT VESSELS)
{ __,__,X}
⢠Aorta is posterior to and rightward of MPA
⢠Situs solitus {__,__,S}
⢠Aorta is posterior to but inverted leftward of MPA
⢠Situs Inversus {__,__,I}
⢠Transposition : Usually applies when Aorta from RV
and MPA from LV
â Aorta is anterior and Right of MPA
⢠D â Transposition {__,__, D-TGV}
â Aorta is anterior and left of MPA
⢠L â Transposition {__,__,L-TGV}
45. Example
Step 1 â Determine Viscero-Atrial Situs
Visceral situs
RECORD LETTER âSâ
46. Cont..
⢠Step 1 - Determine Viscero-Atrial Situs
Thoracoabdominal
situs
RECORD LETTER âSâ
47. Cont..
⢠Step 1 - Determine Viscero-Atrial Situs
Atrial situs
RECORD LETTER âSâ
49. Cont..
⢠Step 2 â Determine Ventricular Loop
Orientation
Morphologic RV
on Left : Levo
Loop
{__ ,L, __}
50. Cont..
⢠Step 3 â Position and relation of great vessels
Aorta is anterior
and leftward of
MPA :
L Transposition
{__,__,L-TGV}
51. Cont ..
⢠So, findings are â {S, L , L-TGV}
â Situs solitus with levocardia
â Levotransposition of great vessels
â Atrio-ventricular and Ventriculo arterial
Discordance
ďźDiagnosis â Congenitally Corrected L-TGA.