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

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Appendix II: ECHOCARDIOGRAPHY ANALYSIS

Transcription:

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

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

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

Moorman and Anderson

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

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

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

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

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

Cleft Valve and Papillary Muscle Position As papillary muscles support commisures

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.

Type C AVSD - UCSF

Mayo Clinic Drawings Rastelli A Rastelli B Rastelli C

AVSD Concept by Echo

Rastelli Classification- AVSD B A C

Rastelli Type A PA LV RV Subcostal Oblique

Rastelli Type C AO PA LV RV Subcostal Oblique

LV Outflow Obstruction

The Gooseneck Deformity

AV Septal Defect 3-D Left Side

AV Septal Defect 3-D Right Side

3 D Zoom Technique

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

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

Entrainment

Left Ventricular -Right Atrial Shunt

Atrioventricular Septal Defects: Left AV Valvar Leakage

Atrioventricular Septal Defects:Associations- Sub AS

Ostium Primum ASD. (Same Patient)

Ostium Primum ASD.

The connecting tongue and the Partial Canal

Partial Atrioventricular Septal Defects:Associations - The Tricuspid Pouch Lesion

Atrioventricular Septal Defects:Associations - The Tricuspid Pouch Lesion

Cleft in the Left Atrioventricular Valve

NHS. The So-Called Cleft Mitral Valve

The so-called Cleft Mitral Valve

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

New Contributions of 3 D Echocardiography.

The So-called Cleft Mitral Valve

3 D Zoom Technique

Single papillary muscle as a risk factor

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

Isolated ventricular component of an AVSD

Isolated ventricular component of an AVSD

Isolated ventricular component of an AVSD

Isolated ventricular component of an AVSD

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

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

Atrioventricular Septal Defects: Associations- DORV

Atrioventricular Septal Defects:Associations- Tetralogy of Fallot

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

Type A AVSD. Left Isomerism A MRV MLV Apex Rt

Unbalanced Atrioventricular Septal Defects

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

Atrioventricular Septal Defects:Right Dominant Associations

Atrioventricular Septal Defects:Right Dominant Associations

Left Dominant AVSD

L Dominant AVSD RA LA EFF RV LV

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.

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.

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

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

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

NHS. Questionable LV Size? Cor Triatriatum

Short axis change pre- and postop. TAPVR

Preoperative/postoperative echo in unbalanced Van Son & Phoon

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.

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

NHS. LV Volume change real and calculated

NHS. Unbalanced AVSD. Cohen et al.

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

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

Postoperative Findings

Atrioventricular Septal Defects: Postop. L AV Valve

AV Valve Regurgitation- Pre- and Post Op

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

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

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

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

Postop AVVR

Postop AVVR

NHS. AV Regurgitation & Stenosis

NHS. Postoperative Pearls- The AV Septal Defect

Annuloplasty

Postop AVVR

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

Changes in Areal Strain in 3 Shape Valriations.

The End

.

The End

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.

Thank You!