Atrioventricular Canal (Septal) Defects. Norman H Silverman MD. D Sc (Med),FACC, FAHA

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1 Atrioventricular Canal (Septal) Defects Norman H Silverman MD. D Sc (Med),FACC, FAHA

2 Embryology of the A-V Canal Looping NHS. Formation of the Atrial Septum

3 Embryology of the A-V Canal NHS. Development of the A-V Canal Cono-ventricular development

4 Moorman and Anderson

5

6 Pathology of AVSD PA The Atrioventricular Junction is Common in all cases of AV Canal Defects AO Courtesy of Robert Anderson The aorta is "Sprung" out of its usual position because of the common AV Junction. NHS. ML SBL IBL CS Ant.TL IL

7 Separate valvar orifices For RV & LV Features of AV Canal Courtesy of Diane Spicer.

8 Features of AV Canal The phenotypic feature is the common atrioventricular junction Courtesy of Diane Spicer.

9 Features of AV Canal The phenotypic feature is the common atrioventricular junction Courtesy of Diane Spicer.

10 The Gooseneck Deformity Inlet-outlet disproportion is a feature of all AV Canal Defecgts

11 Cleft Valve and Papillary Muscle Position As papillary muscles support commisures

12 Goals of Ultrasound 1. Define the extent of the atrial communication. 2. Define the type and extent of the ventricular communications. 3. Demonstrate the valve morphology attachments and function. 4. Display the shunting patterns, the magnitude of the shunt. 5. Type of atrioventricular valve regurgitation, magnitude position and direction. 6. Assess the commitment of the atrioventricular junction to the underlying ventricular mass and the size of the underlying ventricle (balance). 7. Recognize associated anomalies.

13 Type C AVSD - UCSF

14 Mayo Clinic Drawings Rastelli A Rastelli B Rastelli C

15 AVSD Concept by Echo

16 Rastelli Classification- AVSD B A C

17 Rastelli Type A PA LV RV Subcostal Oblique

18 Rastelli Type C AO PA LV RV Subcostal Oblique

19 LV Outflow Obstruction

20 The Gooseneck Deformity

21 AV Septal Defect 3-D Left Side

22 AV Septal Defect 3-D Right Side

23 3 D Zoom Technique

24 TYPE A, AVSD: A V Valvar Regurgitation ASD2 ASD1 RA LA A 4 Ch. RV LV PV

25 AVSD Type C : AV valve regurgitation RA LA RV LV A 4 Ch.

26 Entrainment

27 Left Ventricular -Right Atrial Shunt

28 Atrioventricular Septal Defects: Left AV Valvar Leakage

29 Atrioventricular Septal Defects:Associations- Sub AS

30 Ostium Primum ASD. (Same Patient)

31 Ostium Primum ASD.

32 The connecting tongue and the Partial Canal

33 Partial Atrioventricular Septal Defects:Associations - The Tricuspid Pouch Lesion

34 Atrioventricular Septal Defects:Associations - The Tricuspid Pouch Lesion

35 Cleft in the Left Atrioventricular Valve

36 NHS. The So-Called Cleft Mitral Valve

37 The so-called Cleft Mitral Valve

38 Left Atrioventricular Valve & Regurgitation 3D Normal Mitral Valve 3D Cleft Left Atrioventricular Valve Surgical View of Cleft Courtesy of Jeff. Smallhorn

39 New Contributions of 3 D Echocardiography.

40 The So-called Cleft Mitral Valve

41 3 D Zoom Technique

42 Single papillary muscle as a risk factor

43 Single left ventricular papillary muscle Parachute MV RV LV Subcostal Sagittal View

44 Isolated ventricular component of an AVSD

45 Isolated ventricular component of an AVSD

46 Isolated ventricular component of an AVSD

47 Isolated ventricular component of an AVSD

48 AVSD :Secundum ASD Type A. AVSD LA RA LV RV Subcostal Coronal

49 AVSD: Parasternal Short Axis of VSD VSD RV PA RA LA AO

50 Atrioventricular Septal Defects: Associations- DORV

51 Atrioventricular Septal Defects:Associations- Tetralogy of Fallot

52 Dextrocardia, Right Isomerism and R Dom. AVSD ASD2 ASD1 RA LA RV LV R Apex

53 Type A AVSD. Left Isomerism A MRV MLV Apex Rt

54 Unbalanced Atrioventricular Septal Defects

55 Left Dominant AVSD When is the right ventricle too small? This is less clear than for right ventricular dominance. Options include: cheating on the patch a one and a half ventricle repair single ventricle repair

56 Atrioventricular Septal Defects:Right Dominant Associations

57 Atrioventricular Septal Defects:Right Dominant Associations

58 Left Dominant AVSD

59 L Dominant AVSD RA LA EFF RV LV

60 Unbalanced Atrioventricular Canal Defects Repair related to:- Size of the ventricle Volume Length Size of the amount of tissue over the valve Area Quality of the valvar tissue The presence of associated defects Outflow tract Vascular abnormalities NHS.

61 Philosophy for Repair of Unbalanced AVSD s Relative advantages of a one vs. a two ventricle repair. Is a prosthetic valve worse or better than a one ventricle repair. Are there extraneous factors which would prevent a two ventricle repair.

62 Left Dominant AVSD When is the right ventricle too small? This is less clear than for right ventricular dominance. Options include: cheating on the patch a one and a half ventricle repair single ventricle repair

63 R. DOM. AVSD ( L. ISOMERISM) RA LA CS RV A 4 Ch. (L) LV

64 Left Isomerism, AVSD, (Rt. Dom.) PA AVVO LV RV

65 NHS. Questionable LV Size? Cor Triatriatum

66 Short axis change pre- and postop. TAPVR

67 Preoperative/postoperative echo in unbalanced Van Son & Phoon

68 Indexed LVEDV= 14.8±9.1 ml /m 2 Potential Indexed LV EDV= 32.2±18.8 Dimension Measurements Van Son & Phoon Indexed LV long axis dimension =8.9±1.4 cm/m 2 RV/LV Long axis ratio= 0.65±0.1 L AV Valve/Total Valve Ratio = 0.30±0.06 LV/RV Area Ratio= 0.27±0.01 NHS.

69 Postoperative echocardiographic dimensions Postoperative dimensions LV EDV Index 35.6± 3.9 ml. /m 2 LV/RV long ratio 0.88±0.11 L AV Valve / total Valve ratio = 0.42± 0.03 LV/RV area ratio= 0.88 ± 0.18 /m 2 Van Son, Phoon et al. Ann Thorac Surg. 1997;63:1567

70 NHS. LV Volume change real and calculated

71 NHS. Unbalanced AVSD. Cohen et al.

72 Balance :Summary! Balance is a complex issue! Treatment is based on several factors:! morphological factors! direct vs. indirect! surgical preference! philosophical factors! Echocardiography provides one of many determinants! Inspection of the morphology at surgery is the final point of arbitration. NHS

73 Conclusion Balance is a complex issue Treatment is based on several factors: morphological factors direct vs. indirect surgical preference philosophical factors Echocardiography provides one of many determinants Inspection of the morphology at surgery is the final point of arbitration. NHS

74 Postoperative Findings

75 Atrioventricular Septal Defects: Postop. L AV Valve

76 AV Valve Regurgitation- Pre- and Post Op

77 Tetralogy and AVSD ( Type C ): Pre & Post Repair

78 AVSD Postop. Contrast & Color Doppler Studies Subcostal Coronal View DAO RA LA LV RV

79 Postoperative breakdown of left AV valve repair Subcostal coronal view RA LA RV LV

80 Dehisced Sutured Commisure ( Cleft ) P Lax RV LV Cl LA

81 Postop AVVR

82 Postop AVVR

83 NHS. AV Regurgitation & Stenosis

84 NHS. Postoperative Pearls- The AV Septal Defect

85 Annuloplasty

86 Postop AVVR

87 The Normal and Abnormal (Mitral) Annulus Effect of Annular Shape on Leaflet Curvature in Reducing Mitral Leaflet Stress Ivan S. Salgo,

88 Changes in Areal Strain in 3 Shape Valriations.

89 The End

90 .

91 The End

92 Goals of Ultrasound 1. Define the extent of the atrial communication. 2. Define the type and extent of the ventricular communications. 3. Demonstrate the valve morphology attachments and function. 4. Display the shunting patterns, the magnitude of the shunt. 5. Type of atrioventricular valve regurgitation, magnitude position and direction. 6. Assess the commitment of the atrioventricular junction to the underlying ventricular mass and the size of the underlying ventricle (balance). 7. Recognize associated anomalies.

93 Thank You!

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