Fall Work Weekend November 18 2016 James M Meza MD and David M Overman MD Study PI Measures of Inflow Physiology Modified atrioventricular valve index Cohen 1996 and Jegatheeswaran ID: 774935
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Slide1
Baseline AVSD Echo Descriptive Analysis
Fall Work Weekend
November 18, 2016
James M. Meza, MD and David M. Overman, MD (Study PI)
Slide2Measures 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
Slide3Measures 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
Slide4Present Study
Imaging Core Lab (ICL) has been collecting and reading echoes for 2 years
Descriptive
analysis of baseline diagnostic echoes
Slide5AVSD Cohort
Inclusion
c
riteria
Diagnosis of or referral with complete AVSD at a CHSS
institution
within
1
st
year
of life
Admitted to CHSS institution
after
January 1, 2012
Age ≤ 365 days at admission for surgery
Atrioventricular and
v
entriculoarterial
concordance
Includes
Tetralogy of
Fallot
and Double
Outlet Right
Ventricle
Exclusion
c
riteria
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
Slide6Study Population
328 enrolled patients,149 with echoes read (59%)
Mean
Std
Median
IQR
Min-Max
Demographics
Age at echo
48
68
15
1-83
0-544
Height
51.6
6.3
50.0
48.0-56.0
34.0-67.3
Weight
3.6
1.2
3.3
2.8-4.2
1.3-8.3
BSA
0.2
0.05
0.2
0.2-0.3
0.1-0.4
Slide7Clinical characteristics
Frequency
Complete AVSD (unrestrictive VSD)
100%
AV concordance
100%
VA concordance
100%
Normal pulmonary venous connection
99.5% (193/194)
Normal systemic venous connection
99.5% (193/194)
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
0%
Slide8Echo Review Protocol
Single reviewer – Cheryl
Fakhoury
111 parameters
Slide9Atrial and Ventricular Septae
FrequencyMalalignment 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
0.4-0.9
0.1-2.3
Inlet VSD size: From PSSAx (cm)
0.6
0.2
0.6
0.5-0.7
0.2-1.1
Inlet VSD size: From Apical 4 (cm)
0.7
0.2
0.7
0.5-0.8
0.2-1.3
Slide10Common AV Valve
Mean
Std
Median
IQR
Min-Max
Mural leaflet length
1.4
0.4
1.3
1.1-1.6
0.6-2.4
Mural leaflet width
0.9
0.2
0.9
0.7-1.0
0.4-1.5
Total CAVV diameter
2.4
0.4
2.4
2.1-2.7
0.9-3.6
RAVV area
1.7
0.7
1.6
1.2-2.1
0.4-4.8
LAVV area
1.4
0.6
1.2
0.9-1.7
0.4-3.7
Total area
3.1
1.1
3.0
2.2-3.8
1.1-7.9
2D measurement RAVV (apical 4) (cm)
1.4
0.3
1.4
1.2-1.6
0.6-2.3
Color inflow diameter at annulus RAVV (cm)
1.5
0.3
1.5
1.3-1.7
0.9-2.4
Color inflow diameter at mid cavity or at smallest inflow portion RAVV(cm)
0.8
0.2
0.8
0.7-0.9
0.4-1.5
2D measurement LAVV (apical 4) (cm):
1.2
0.2
1.2
1.1-1.4
0.6-1.8
Color inflow diameter at annulus (cm)
1.3
0.3
1.3
1.1-1.5
0.4-2.1
Color inflow diameter at mid cavity or at smallest inflow portion (cm)
0.7
0.2
0.7
0.6-0.8
0.1-1.9
Distance between two papillary muscles: (cm)
1.2
0.3
1.2
1.0-1.5
0.5-2.0
Slide11Inflow Characteristics
MeanStdMedianIQRMin-MaxmAVVI 0.450.100.440.39-0.510.18-0.69RV inflow – LV inflow angle 91.015.889.080.0-101.352.1-132.1Left ventricular inflow index 0.50.10.50.5-0.60.3-1.2
Frequency
Right-dominant
22% (42/194)
Balanced
53% (108/194)
Left dominant
5% (9/194)
Unable to determine (no
mAVVI
)
21% (41/194)
Slide12Slide13Slide14Slide15FrequencyCommon AV Valve Regurgitation Trivial 6% (11/194)Mild68% (132/194)Moderate22% (42/194)Severe5% (9/194)Left AV Valve regurgitation None11% (22/193)Trivial 18% (35/193)Mild62% (120/193)Moderate8% (15/193)Severe1% (1/193)Right AV Valve regurgitation None2% (4/193)Trivial 30% (57/193)Mild50% (97/193)Moderate15% (29/193)Severe3% (6/193)Rastelli Type A79% (66/84)B2% (2/84)C19% (16/84)Papillary muscle abnormality32% (56/189)Too close30% (17/56)One hypoplastic 70% (39/56)Parachute0% Double orifice LAVV0.6% (1/160)
Slide16Right Ventricle
Frequency RV hypoplasia None88% (170/194)Trivial3% (6/194)Mild4% (7/194)Moderate4% (8/194)Severe1% (3/194)RV systolic dysfunction None97% (188/193)Mild2% (4/193)Moderate1% (1/193)Apex-forming RV 93% (179/192)
Mean
Std
Median
IQR
Min-Max
RV Area in End - Diastole (4 chamber view) (cm2)
4.3
1.5
4.0
3.2-5.3
1.5-8.3
RV Area in End - Systole (4 chamber view) (cm2)
2.8
1.0
2.7
2.1-3.4
0.8-5.6
Right Ventricle Fractional Area Change (Calculated field) (%)
35.3
7.7
34.5
30.7-39.1
0-75.9
RV length From AV Valve to apex (at end of QRS complex) (cm)
2.8
0.5
2.8
2.5-3.2
1.5-4.2
RV width (from the crest of the septum- RV side- to the free wall) (cm)
1.6
0.4
1.5
1.3-1.8
0.3-2.5
Slide17Pulmonic Valve
MeanStdMedianIQRMin-MaxPV Annulus in systole (cm)1.00.21.00.9-1.10.3-1.4
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
None
39% (75/194)
Trivial
47% (91/194)
Mild
14% (28/194)
Slide18RVOT
MeanStdMedianIQRMin-MaxPeak gradient (mmHg)36.527.427.215.2-54.46.9-109.8Mean gradient (mmHg)22.117.818.77.5-34.14.1-70.1
Frequency
RVOT Obstruction
9% (16/183)
Subvalvar
44% (7/16)
Valvar
13% (2/16)
Supravalvar
6% (1/16)
Diffuse
38% (6/16)
Slide19Left Ventricle
Frequency
LV hypoplasia
None
90% (174/194)
Trivial
0.5% (1/194)
Mild
3% (6/194)
Moderate
3% (6/194)
Severe
3.5% (7/194)
Apex-forming LV
94% (181/192)
LV dysfunction Systolic
None
95% (183/192)
Mild
3% (6/192)
Moderate
1% (2/192)
Severe
1% (1/192)
Slide20Left Ventricle
Mean
Std
Median
IQR
Min-Max
LVEDD (cm)
1.8
0.5
1.8
1.6-2.1
0.5-3.4
LVESD (cm)
1.1
0.3
1.1
0.9-1.3
0.3-2.5
LVEF (%)
71.1
9.3
72.0
65.8-78.0
33.3-92.9
LV area in End-Diastole (4 chamber view): (cm2)
4.4
1.7
4.4
3.3-5.5
0.9-8.8
LV area in End-Systole (4 chamber view): (cm2)
2.4
0.9
2.3
1.8-3.0
0.6-5.1
Left Ventricle Fractional Area Change (calculated field): (%)
43.3
7.5
43.5
39.6-48.0
18.4-63.4
LV length from AVV to apex (at end of QRS complex) (cm)
2.9
0.5
2.9
2.5-3.3
1.3-4.1
LV width (from crest of septum- LV side- to free wall) (cm)
1.6
0.4
1.6
1.3-1.8
0.4-2.6
Slide21LVOT
Frequency Left Ventricular Outflow Tract Obstruction3% (5/193)LVOTO level of stenosis Sub-valvar80% (4/5)Valvar20% (1/5)LVOTO Type of Subvalvar Stenosis Tunnel 100% LVOTO Type of Supravalvar Stenosis Discrete25% Diffuse 75%
Mean
Std
Median
IQR
Min-Max
Smallest LVOT diameter in systole (cm)
0.7
0.2
0.7
0.6-0.8
0.3-1.1
Peak gradient (mmHg)
5.0
2.2
4.8
2.9-7.3
2.9-7.3
Mean gradient (mmHg)
2.3
1.3
2.3
1.3-3.2
1.3-3.2
Slide22Aortic Valve
Mean
Std
Median
IQR
Min-Max
Aortic Annulus in systole (cm)
0.8
0.2
0.8
0.7-0.9
0.3-1.3
Aortic root in systole (cm)
1.0
0.2
1.1
0.9-1.2
0.6-1.5
Sinotubular
junction in systole (cm)
0.8
0.2
0.8
0.7-0.9
0.4-1.2
Slide23Aortic Valve
Frequency
Aortic Valve Thickening
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
None
96% (184/191)
Mild
2% (3/191)
Moderate
2% (4/191)
Regurgitation
None
67% (129/194)
Trivial
29% (57/194)
Mild
4% (8/194)
Slide24Aorta
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
0.8-0.9
0.4-1.2
Transverse Arch (cm)
0.5
0.1
0.5
0.4-0.6
0.2-1.0
Isthmus (cm)
0.4
0.1
0.4
0.3-0.5
0.2-0.7
Descending aorta (cm)
0.6
0.03
0.6
0.6-0.6
0.6-0.6
Peak Arch gradient (mmHg)
11.1
10.0
6.8
4.8-14.6
1.1-42.0
Mean Arch gradient (mmHg)
6.1
5.6
3.7
2.2-8.5
0.6-22.0
Slide25Aorta
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:
Antegrade
95% (181/190)
Retrograde
1% (2/190)
Bidirectional
4% (7/190)
Slide26Ductus 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
Left to right
99% (114/115)
Right to left
1% (1/115)
Slide27Correlating inflow physiology and ventricular geometry
Slide28Correlations 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
Slide29Correlations – 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
< 0.0001
RV end diastolic area
-0.31
0.0001
LV end diastolic area
0.43
< 0.0001
Slide30Correlations – RV/LV inflow angle and measures of ventricular geometry
Pearson
Correlation
Coefficient
P-value
Color inflow diameter at RAVV annulus
-0.40
< 0.0001
Color inflow diameter at LAVV annulus
-0.36
< 0.0001
RV end diastolic area
0.08
0.26
LV end diastolic area
0.12
0.11
Slide31Correlations – 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
Slide32Next steps
Exploring impact of septal angulation on ventricular geometry
Exploring mural leaflet sizes and
ventricular
geometry
Abnormalities of LAVV apparatus
and ventricular geometry
Slide33THANK YOU
james.meza@sickkids.ca