1. ECHO : SEGMENTAL APPROACH Dr Binjo J Vazhappilly SR Cardiology MCH Calicut

2. Segmental Approach to Imaging Congenital Heart Disease (CHD) • Systematic and sequential method to describe the cardiac segments and connections. • Three cardiac segments : atria , ventricle and great vessels. • Connections : specific relationship between the segments .

5. Determine visceroatrialsitus • Situs refers to spatial arrangement of the viscerae . Three types of visceroatrialsitus • S – solitus(normal arrangement) • I – inversus(or the mirror-image arrangement) • A - ambiguus • Situsambiguus: defined as an abnormality in which thoracic and abdominal organs are positioned with respect to each other, in such a way that it cannot be clearly lateralized.

6. SitusSolitus

7. SitusInversus

8. Right Isomerism(Asplenia)

9. Left Isomerism (polysplenia)

10. Cardiac Position & Orientation • Position of the heart in the chest with regard to its location and orientation of its apex. Location of heart in the chest • Levoposition : to the left • Mesoposition : central • Dextroposition : to the right Cardiac orientation is the base to apex orientation of heart • Levocardia : apex directed to left of midline. • Mesocardia : apex oriented inferiorly in the midline • Dextrocardia: apex directed to right of midline.

11. Atrial Morphology The right & left atria are identified morphologically by their respective atrial appendages and veins emptying into them. Left Atrium Narrow, fingerlike posterior appendage . LA receives all 4 pulmonary veins Septum primum lies on LA side . LA is smooth with fewer trabeculations Right Atrium • Triangular, broad based, anterior appendage . • Receives IVC, SVC & coronary sinus. • Septum secundum (limbus of fossaovale) lies on RA side. • CristaTerminalis is in RA.

12. Atrial Morphology

13. Pulmonary veins to LA

14. AtrialSitus • AtrialSitusSolitus (S) • AtrialSitusInversus (I) • AtrialSitus Ambiguous (A) Right isomerism (bilateral right atria) or Left isomerism (bilateral left atria).

15. Ventricular Looping • Embryonic heart is a linear tube with bulbuscordis (future RV ) being cephalad to the developing left ventricle (LV) . • In normal development the tube bends over on itself to right forming a D-loop resulting in positioning of the bulbuscordis to the anatomic right of the left ventricle • Folding to left results in L-loop with the morphologic right ventricle being to the left of the morphologic left ventricle

16. Ventricular Looping

17. D Loop L Loop

18. Identification of the ventricles AV valve morphology directly correlates with ventricular type Tricuspid Valve ⇒ RV • Septalchordal attachments • Apical septal annular attachment • 3 leaflet • Multiple small papillary muscles • Triangular orifice Mitral valve ⇒ LV • No septalchordal attachments • Basal septal annular attachments • 2 leaflet • 2 large papillary muscles • Elliptical orifice.

19. RV Morphology • Large apical trabaculations • Coarse septal surface • Moderator bands • Cresentic in cross section • Tricuspid – pulmonary discontinuity

20. LV Morphology • Small apical trabeculations • Smooth upper surface • No moderator band • Mitral – Aortic continuity

21. Atrio-ventricular Connections • Concordant ( Normal : RA to RV & LA to LV) • Discordant ( RA to LV & LA to RV) • Univentricular AV connections Absent AV connection Common AV valve Double inlet connections

22. Concordant AV Connection

23. Univentricular AV Connections • Absent AV connection (right / left) Tricuspid / mitral atresia • Common AV valve With single ventricle • Double inlet ventricle Connection of both AV valve to the same ventricle Anatomically left Anatomically right Undetermined (rudimentary chamber always present)

24. Univentricular AV connections

25. Univentricular AV connections Absent Rt AV Connection Absent Lt AV connection

26. Univentricular AV connections Double inlet : RV Morphology Double inlet : LV Morphology

27. Overriding & Straddling • Overriding : Biventricular emptying of AV Valve or biventricular origin of a semilunar valve. • Straddling : Chordae or papillary muscle of the valve attach to contralateral ventricle. Involve AV Valves and requires presence of VSD.

28. Arterial Morphology • Aorta : artery that gives rise to the coronary arteries and the brachiocephalic vessels. • Pulmonary artery : branches into two but does not give rise to any vessels.

29. Ventriculo-Arterial Connection • Concordant : Aorta connected to LV and pulmonary artery to RV . • Discordant : Aorta connected to RV and pulmonary artery to LV . • Double Outlet : Both great arteries arise from a single ventricle. • If more than 50% of an artery overrides a ventricle it is said to be committed to it.

30. Commitment • Commitment describes possible abnormalities of flow through valves into ventricles and great vessels. • In TOF, atria, AV valves & ventricles are positioned normally and concordant. Aorta overrides a VSD and is doubly committed to both ventricles. • In univentricular heart both AV valves are usually doubly committed to the single ventricle.

31. Ventriculo-arterial connections • Concordant (normal) Ventricular septal defect, tetralogy of Fallot • Discordant Transposition • Double outlet ventricle With subaortic VSD subpulmonary VSD doubly committed VSD non-committed VSD • Common arterial trunk

32. Conus • Subpulmonary: Absence of subaorticinfundibular free wall, found in normal heart. • Subaortic: Absence of subpulmonaryinfundibular free wall, found in D-loop TGA. • Bilaterally present : In double outlet RV , but rarely in TGA. • Bilaterally Absent: In double outlet LV.

33. Relation b/w semilunar Valves • Solitus : Aorta posterior and to right. • Inversus : mirror image : Aorta posterior and to left • D-malposition : aortic valve anterior and to the right. • L-malposition : aortic valve anterior and to the left. • Anterior malposition : aortic valve anterior in the middle. • Parasternal and high parasternal short axis and subcostal short axis views are used to recognize the positions of AV and PV.

34. Possible Great vessel relations

35. Normal PSX Suprasternal long and short axis

36. d TGA

37. cc TGA

38. DORV

39. TOF

40. TruncusArteriosus

41. Assessment of Associated Anomalies Abnormalities in each segment should be assessed • Septal defects • AV valve abnormalities like Ebstein’s • Outflow tract stenosis • Aortic arch branching and sidedness • Pulmonary artery and branches • PDA • Coronary abnormalities

42. Ebstein anomaly

43. Pulmonary valve pathology

44. LV outflow Subaorticmembrane Supravalvular AS

45. COARCTATION OF AORTA

46. Sequential Segmental Approach • Identify situs of the thoraco-abdominal organs. • Determine cardiac position within thorax. • AtrialSitus. • Ventricular Situs. • Atrioventricular connection and alignment. • Ventriculo-arterial connection and alignment. • Conal (infundibular) anatomy. • Relationship between the great arteries. • Description of associated malformations

47. Segmental Expression 1 . Visceroatrialsitus : S , I or A. 2 . Ventricular situs : D or L 3 . Position of great vessels : S , I , D , L or A • Normal : S , D , S • SitusinversusDextrocardia : I , L , I • D TGA : S , D , D • D TGA with situsinversus : I , L ,L • L TGA with situssolitus : S , L , L

48. QUIZ

49. Interrupted IVC is seen in • Situsinversus • Situssolitus • Right isomerism • Left isomerism

50. 2. Morphological feature of right atrium • Finger like posterior appendage • Smooth surface • Presence of cristae terminalis D) Septum primum lies on RA side