1. Baseline AVSD Echo Descriptive Analysis
Fall Work Weekend
November 18, 2016
James M. Meza, MD and David M. Overman, MD (Study PI)
This is first look at this – really need more involvement, want thoughts

2. Measures of Inflow Physiology
Modified atrioventricular valve index (Cohen 1996 and Jegatheeswaran, 2010)
Portion of AV valve allocated to each ventricular in the subxiphoid saggital ro LAO view
Retrospective CHSS study – identified RV/LV inflow angle as best discriminator of unbalance (Cohen, 2013)
Angle between the base of the right ventricle and left ventricle free wall using the crest of the ventricular septum as the apex of the angle

3. Measures of Inflow Physiology
Left ventricular inflow index = Secondary inflow diameter/Primary inflow diameter (Swazst, 2011)
Primary: level of left AVV annulus
Secondary: color inflow jet diameter at level of papillary muscles

4. Present Study
Imaging Core Lab (ICL) has been collecting and reading echoes for 2 years
Descriptive analysis of baseline diagnostic echoes

5. AVSD Cohort Inclusion criteria Exclusion criteria
Diagnosis of or referral with complete AVSD at a CHSS institution within 1st year of life
Admitted to CHSS institution after January 1, 2012
Age ≤ 365 days at admission for surgery
Atrioventricular and ventriculoarterial concordance
Includes Tetralogy of Fallot and Double Outlet Right Ventricle
Exclusion criteria
Partial or Transitional AVSD
Separate AV valve orifices, restrictive VSD or intact ventricular septum
TAPVC or PAPVC, aortic atresia, Heterotaxy
First Intervention at a non-CHSS institution

6. Study Population 328 enrolled patients,149 with echoes read (59%) Mean
Mean
Std
Median
IQR
Min-Max
Demographics
Age at echo 48 68 15
1-83
0-544
Height
51.6
6.3
50.0
Weight
3.6
1.2
3.3
BSA
0.2
0.05

7. Clinical characteristics
Frequency
Complete AVSD (unrestrictive VSD)
100%
AV concordance
VA concordance
Normal pulmonary venous connection
99.5% (193/194)
Normal systemic venous connection
Additional Diagnosis
Tetralogy of Fallot
7% (13/194)
Systemic venous anomalies
3% (5/193)
LSVC to CS
7% (13/185)
LSVC Unroofed to LA 0%
Bilateral SVCs
6% (11/183)
Interrupted IVC to RSVC/LSVC

8. Echo Review Protocol
Single reviewer – Cheryl Fakhoury
111 parameters

9. Atrial and Ventricular Septae
Frequency
Malalignment of atrial and ventricular septum
15% (25/186)
Right (double outlet LA)
8% (2/25%)
Left (double outlet LA)
92% (23/25)
Ostium primum ASD
99.5% (193/194)
Primum ASD restrictive
2% (4/192)
Mean
Std
Median
IQR
Min-Max
ASD size
0.7
0.4
0.6
Inlet VSD size: From PSSAx (cm)
0.2
Inlet VSD size: From Apical 4 (cm)

10. Common AV Valve Mean Std Median IQR Min-Max Mural leaflet length 1.4
Mean
Std
Median
IQR
Min-Max
Mural leaflet length
1.4
0.4
1.3
Mural leaflet width
0.9
0.2
Total CAVV diameter
2.4
RAVV area
1.7
0.7
1.6
LAVV area
0.6
1.2
Total area
3.1
1.1
3.0
2D measurement RAVV (apical 4) (cm)
0.3
Color inflow diameter at annulus RAVV (cm)
1.5
Color inflow diameter at mid cavity or at smallest inflow portion RAVV(cm)
0.8
2D measurement LAVV (apical 4) (cm):
Color inflow diameter at annulus (cm)
Color inflow diameter at mid cavity or at smallest inflow portion (cm)
Distance between two papillary muscles: (cm)

11. Inflow Characteristics
Mean
Std
Median
IQR
Min-Max
mAVVI
0.45
0.10
0.44
RV inflow – LV inflow angle
91.0
15.8
89.0
Left ventricular inflow index
0.5
0.1
Frequency
Right-dominant
22% (42/194)
Balanced
53% (108/194)
Left dominant
5% (9/194)
Unable to determine (no mAVVI)
21% (41/194)

14. RV/LV inflow angle (degrees) 145+12 113+9 81+9

15. Frequency
Common AV Valve Regurgitation
Trivial
6% (11/194)
Mild
68% (132/194)
Moderate
22% (42/194)
Severe
5% (9/194)
Left AV Valve regurgitation
None
11% (22/193)
18% (35/193)
62% (120/193)
8% (15/193)
1% (1/193)
Right AV Valve regurgitation
2% (4/193)
30% (57/193)
50% (97/193)
15% (29/193)
3% (6/193)
Rastelli Type A
79% (66/84) B
2% (2/84) C
19% (16/84)
Papillary muscle abnormality
32% (56/189)
Too close
30% (17/56)
One hypoplastic
70% (39/56)
Parachute 0%
Double orifice LAVV
0.6% (1/160)

16. Right Ventricle Mean Std Median IQR Min-Max
RV Area in End - Diastole (4 chamber view) (cm2)
4.3
1.5
4.0
RV Area in End - Systole (4 chamber view) (cm2)
2.8
1.0
2.7
Right Ventricle Fractional Area Change (Calculated field) (%)
35.3
7.7
34.5
0-75.9
RV length From AV Valve to apex (at end of QRS complex) (cm)
0.5
RV width (from the crest of the septum- RV side- to the free wall) (cm)
1.6
0.4
Frequency 
RV hypoplasia
None
88% (170/194)
Trivial
3% (6/194)
Mild
4% (7/194)
Moderate
4% (8/194)
Severe
1% (3/194)
RV systolic dysfunction
97% (188/193)
2% (4/193)
1% (1/193)
Apex-forming RV
93% (179/192)

17. Pulmonic Valve Mean Std Median IQR Min-Max PV Annulus in systole (cm)
1.0
0.2
Frequency 
PV stenosis
None
90% (170/194)
Trivial
0.5% (1/194)
Mild
4% (7/194)
Moderate
4% (8/194)
Severe
1.5% (3/194)
PV regurgitation
39% (75/194)
47% (91/194)
14% (28/194)

18. RVOT Mean Std Median IQR Min-Max Peak gradient (mmHg) 36.5 27.4 27.2
Mean gradient (mmHg)
22.1
17.8
18.7
Frequency 
RVOT Obstruction
9% (16/183)
Subvalvar
44% (7/16)
Valvar
13% (2/16)
Supravalvar
6% (1/16)
Diffuse
38% (6/16)

19. Left Ventricle Frequency LV hypoplasia None 90% (174/194) Trivial
None
90% (174/194)
Trivial
0.5% (1/194)
Mild
3% (6/194)
Moderate
Severe
3.5% (7/194)
Apex-forming LV
94% (181/192)
LV dysfunction Systolic
95% (183/192)
3% (6/192)
1% (2/192)
1% (1/192)

20. Left Ventricle Mean Std Median IQR Min-Max LVEDD (cm) 1.8 0.5 1.6-2.1
LVESD (cm)
1.1
0.3
LVEF (%)
71.1
9.3
72.0
LV area in End-Diastole (4 chamber view): (cm2)
4.4
1.7
LV area in End-Systole (4 chamber view): (cm2)
2.4
0.9
2.3
Left Ventricle Fractional Area Change (calculated field): (%)
43.3
7.5
43.5
LV length from AVV to apex (at end of QRS complex) (cm)
2.9
LV width (from crest of septum- LV side- to free wall) (cm)
1.6
0.4

21. LVOT Frequency Left Ventricular Outflow Tract Obstruction 3% (5/193)
LVOTO level of stenosis
Sub-valvar
80% (4/5)
Valvar
20% (1/5)
LVOTO Type of Subvalvar Stenosis
Tunnel
100%
LVOTO Type of Supravalvar Stenosis
Discrete
25%
Diffuse
75%
Mean
Std
Median
IQR
Min-Max
Smallest LVOT diameter in systole (cm)
0.7
0.2
Peak gradient (mmHg)
5.0
2.2
4.8
Mean gradient (mmHg)
2.3
1.3

22. Aortic Valve Mean Std Median IQR Min-Max
Aortic Annulus in systole (cm)
0.8
0.2
Aortic root in systole (cm)
1.0
1.1
Sinotubular junction in systole (cm)

23. Aortic Valve Frequency Aortic Valve Thickening None 92% (178/193)
None
92% (178/193)
Trivial
2% (4/193)
Mild
3% (6/193)
Moderate
3% (5/193)
Number of Leaflets Bi
6% (9/148)
Tri
94% (139/148)
Stenosis
96% (184/191)
2% (3/191)
2% (4/191)
Regurgitation
67% (129/194)
29% (57/194)
4% (8/194)

24. Aorta Mean Std Median IQR Min-Max
Ascending Aorta (measured from parasternal LA view in systole,
just above the ST junction): (cm)
0.8
0.1
0.9
Transverse Arch (cm)
0.5
Isthmus (cm)
0.4
Descending aorta (cm)
0.6
0.03
Peak Arch gradient (mmHg)
11.1
10.0
6.8
Mean Arch gradient (mmHg)
6.1
5.6
3.7

25. Aorta Frequency Coarctation present 18% (29/158)
Location of coarctation
Transverse
7% (2/27)
Isthmus
93% (25/27)
Interrupted Arch 0%
Flow direction in ascending arch:
Antegrade
100%
Flow direction in transverse arch:
95% (181/190)
Retrograde
1% (2/190)
Bidirectional
4% (7/190)

26. Ductus Arteriosus Frequency Ductus patent 61% (116/189)
Ductal flow restrictive
55% (62/113)
Ductal flow in systole
Left to right
3% (3/98)
Right to left
97% (95/98)
Ductal flow in diastole
99% (114/115)
1% (1/115)

27. Correlating inflow physiology and ventricular geometry

28. Correlations of mAVVI with other indices of inflow physiology
Pearson
Correlation Coefficient
P-value
LVII
0.28
0.001
RV/LV inflow angle
0.12
0.13

29. Correlations – mAVVI and measures of ventricular geometry
Pearson
Correlation Coefficient
P-value
Color inflow diameter at RAVV annulus
-0.29
0.001
Color inflow diameter at LAVV annulus
0.41
<
RV end diastolic area
-0.31
0.0001
LV end diastolic area
0.43

30. Correlations – RV/LV inflow angle and measures of ventricular geometry
Pearson
Correlation Coefficient
P-value
Color inflow diameter at RAVV annulus
-0.40
<
Color inflow diameter at LAVV annulus
-0.36
RV end diastolic area
0.08
0.26
LV end diastolic area
0.12
0.11

31. Correlations – LVII and measures of ventricular geometry
Pearson
Correlation Coefficient
P-value
Color inflow diameter at RAVV annulus
-0.26
0.002
RV end diastolic area
-0.10
0.22
LV end diastolic area
0.16
0.04

32. Next steps
Exploring impact of septal angulation on ventricular geometry
Exploring mural leaflet sizes and ventricular geometry
Abnormalities of LAVV apparatus and ventricular geometry
Paplliary muscle abnormalities

33. THANK YOU