Tricuspid Atresia
Work Weekend
Nov. 2013
Work Weekend Objectives
• Define Current Cohort
• Present/Evaluate Analysis
• Finalize Analysis Topic
Original Goals
• Describe the impact of patient
characteristics and management
strategies on outcomes of Fontan track
Enrollment Criteria
• Diagnosis of TA with normally related great arteries
• Age <3 months at diagnosis
• Admitted to CHSS institution after 1/1/99
• Excludes AV or VA Discordance, first procedure at non CHSS institution
Previous CHSS Analysis
• Competing risks analysis (2005, N=150)
– Factors for death before CPS: MR, BTS not
originating from innominate
– Factors for reduced transition to CPS:
younger age at admission, non-cardiac
anomalies, larger BTS diameter
• Survival by initial operation among
patients with unrestricted blood flow
– Objective: define late outcomes
Demographics
• N=303
–302 with index procedure
–37 deaths overall (12%)
–1 death prior to intervention
Index Procedure
Total
N=302
SP Shunt
N=189
Alive= 85%
PAB
N=50
Alive= 92%
CPS
N=63
Alive= 95%
Initial Physiology
Total
N=303
Alive=266
(88%)
PV Atresia
N=53
Alive=44
(83%)
Restricted
PBF
N=154
Alive=137
(89%)
Unrestricted
PBF
N=96
Alive=85
(89%)
(Type-1a) (Type-1b) (Type-1c)
All Patients
303
DIED
1 ECMO
2
DIED
1
SPS
186
ANFS
4
SPS
8
CPS
142
DIAPH
1
DIED
7
PDA
3
SPS
1
CPS
1
FONT
1
DIED
1
CPS
4
EXPL
1
FONT
1
ANFS
1
EXPL
1
FONT
3
ANFS
3
EXPL
2
CPS
2
FONT
2
DIAPH
1
ANFS
1
CPS
142
FONT
112
ANFS
98
DIED
3
EXPL
5
ANFS
5
FONT
1
ANFS
1
PACE
5
ANFS
5
ANFS
16
DIED
4
CPS
2
FONT
2
ANFS
2
DIED
2
SPS
2
ECMO
1
DIED
1
DIED
1
FONT
2
DIED
1
ANFS
1
CPS
1
FONT
1
ANFS
1
ANFS
2
ANFS
1
EXPL
6
FONT
2
DIED
1
ANFS
2
ANFS
1
DIED
1
EXPL
1
EXPL
1
FONT
1
PBF
1
EXPL
15
EXPL
2
ECMO
1
SPS
1
CPS
10
SPS
1
DIED
1
DIED
1
CPS
2
EXPL
2
ANFS
1
FONT
1
ANFS
1
DIED
1
ECMO
1
DIED
1
SPS
1
DIED
1
CPS
1
EXPL
2
FONT
2
ANFS
2
FONT
5
ANFS
5
DIED
1
PBF
2
CPS
1
FONT
1
ANFS
1
PAB
1
ANFS
1
CPS
1
CPS
2
FONT
2
ANFS
1
ECMO
1
SPS
2 ANFS
8
CPS
1
DIED
2
EXPL
2
FONT
48
PAB
2
SPS
1
FONT
2
ANFS
43
ECMO
1
EXPL
1
PACE
3 FONT
1
ECMO
1
ANFS
1
ANFS
2
ECMO
1
ECMO
1
ANFS
1
ANFS
1
PACE
1
ANFS
2
ANFS
1
SPS
1
EXPL
1
ECMO x3
1
ANFS
2
EXPL
1
ECMO x6
1
SPS
1
ECMO x3
1
DIED
1
CPS
63
FONT
1
ECMO
1
PAB
50
ANFS
3
CPS
42
EXPL
2
PAB
3
ANFS
10
CPS
1
ECMO
1
FONT
28
PBF
1
CPS
2
CPS
2
ECMO
1
ANFS
1
EXPL
1
EXPL
1
DIED
1
ANFS
24
DIED
1
EXPL
1
ANFS
1
PACE
2
ANFS
2
ANFS
1
FONT
2
ANFS
1
PACE
1
DIED
1
ANFS
1
PACE
1
PACE
1
FONT
1
ECMO
1
ANFS
1
PAB
1
CPS
1
DIED
1
HTX
1
SPS= Systemic-pulmonary shunt
ANFS= Alive, no further surgery
EXPL= sternal exploration/bleeding proc
PBF= other procedure to adjust PBF
DIAPH= diaphragm plication
PBF
1
SPS
1
FONT
1
ANFS
1
Overall Survival From Birth
Years after Birth
% S
urv
ival
Dead = 37
Non-parametric Parametric
N=303
Hazard For Death:
From Birth
Years after Birth
Hazard
N=303
N at risk
2y= 240
4y= 206
6y= 179
Mortality by Stage
• 37 total deaths - denominator=303(12%)
– 1 Prior to surgery
– 13 following SPS- stage 1
– 15 following CPS- stage 2
– 8 following Fontan- stage 3
Definitions for analysis:
-Stage 1= initial PAB or SPS procedure
-Stage 2= initial CPS
-Stage 3= initial Fontan
PV Atresia
N=53
SPS
53
ANFS
3
SPS
4
CPS
35
DIED
1
EXPL
6
PBF
1
PDA
3
SPS
1
CPS
1
FONT
1
DIED
1
CPS
1
EXPL
1
EXPL
1
FONT
1
ANFS
1
EXPL
2
CPS
2
FONT
2
ANFS
2
ANFS
3
CPS
1
FONT
1
ANFS
1
DIED
1
EXPL
1
DIED
1
FONT
29
ANFS
25
DIED
2
EXPL
1
ANFS
1
PACE
1
ANFS
1
CPS
4
SPS
1
DIED
1
EXPL
1
FONT
1
FONT
2
ANFS
2
ECMO
1
SPS
1
DIED
1
EXPL
1
CPS
1
EXPL
1
FONT
1
CPS
1
FONT
1
ANFS
1
ANFS
1
CPS
2
FONT
2
ANFS
2
DIED
1
ANFS
1
SPS= Systemic-pulmonary shunt
ANFS= Alive, no further surgery
EXPL= sternal exploration/bleeding proc
PBF= other procedure to adjust PBF
DIAPH= diaphragm plication
EXPL
5
CPS
4
CPS
1
DIED
1
ANFS
1
DIED
1
FONT
2
ANFS
2
EXPL
1
FONT
1
ANFS
1
Restricted
PBF
N=154
DIED
1
SPS
111
ANFS
1
SPS
4
CPS
3
FONT
3
ANFS
3
CPS
91
ANFS
11
SPS
2
DIED
2
CPS
1
FONT
1
ANFS
1
DIED
2
FONT
72
DIED
6
FONT
2
ANFS
1
DIED
1
CPS
39
ANFS
5
DIED
2
FONT
31
ANFS
28
PBF
1
SPS
2
ANFS
2
EXPL
1
PACE
2
ANFS
1
ANFS
1
PACE
1
EXPL
1
ANFS
1
DIAPH
1
ANFS
1
ANFS
64
DIED
1
PACE
3
FONT
1
ANFS
1
EXPL
3
ANFS
3
ANFS
3
PAB
1
CPS
1
ANFS
1
DIED
1
ECMO
1
PBF
1
EXPL
1
FONT
1
ANFS
1
ECMO
2
SPS
1
CPS
1
FONT
1
ANFS
1
SPS= Systemic-pulmonary shunt
ANFS= Alive, no further surgery
EXPL= sternal exploration/bleeding proc PBF=
other procedure to adjust PBF
DIAPH= diaphragm plication
Unrestricted
PBF
N=96
SPS
22
DIAPH
1
CPS
1
FONT
1
ECMO
1
DIED
1
EXPL
4
CPS
2
FONT
1
ANFS
1
DIED
1
SPS
1
DIED
1
CPS
24
ANFS
3
CPS
1
SPS
1
ANFS
1
EXPL
1
FONT
1
ANFS
1
FONT
17
ANFS
15
ECMO
1
ECMO
1
ECMO
1
ANFS
1
PACE
1
ANFS
1
PAB
2
FONT
1
ANFS
1
ECMO
1
SPS
1
EXPL
1
ECMO x3
1
EXPL
1
ECMO x6
1
SPS
1
ECMO x3
1
DIED
1
FONT
1
CPS
16
ANFS
2
DIED
1
EXPL
1
FONT
1
DIED
1
FONT
11
ANFS
9
PACE
1
ANFS
1
EXPL
1
EXPL
1
FONT
1
EXPL
1
ANFS
1
ANFS
1
ANFS
1
PBF
1
ECMO
1
DIED
1
PAB
50
ANFS
3
CPS
42
ANFS
10
CPS
1
ANFS
1
ECMO
1
EXPL
1
EXPL
1
DIED
1
FONT
28
ANFS
24
DIED
1
EXPL
1
ANFS
1
PACE
2
ANFS
2
EXPL
2
CPS
2
FONT
2
PACE
1
DIED
1
ANFS
1
PAB
3
CPS
2
PACE
1
ANFS
1
PACE
1
FONT
1
ANFS
1
ECMO
1
ECMO
1
PAB
1
CPS
1
HTX
1
DIED
1
ANFS
1
PBF
1
SPS= Systemic-pulmonary shunt
ANFS= Alive, no further surgery
EXPL= sternal exploration/bleeding proc PBF=
other procedure to adjust PBF
DIAPH= diaphragm plication
1. Competing Risk Analysis
Transition to Fontan vs. Death
Research Question
• How does timing of CPS affect the
successful transition to Fontan or death
before Fontan?
• What procedural, morphologic, and
demographic factors are associated with
successful transition to Fontan?
Competing Risks:
Transition to Fontan from Birth Alive w/o Fontan (N=51)
Died before Fontan (N=29)
Survived to Fontan (N=215)
Years after Birth
% In
each
Gro
up
N=303
3 yr:
44% alive w/out Fontan
9% died w/out Fontan
46% survived to Fontan
5 yr:
11% alive w/out Fontan
10% died w/out Fontan
79% survived to Fontan
Achievement of Fontan:
From Birth
Years after Birth
% F
ree f
rom
Fo
nta
n
Late phase:
20 events >6 years
N=303 Achievement of
Fontan: N=223
Death Before Fontan:
From Birth
Years after Birth
% F
ree f
rom
Death
Death before
Fontan=29 N=303
At risk:
1y N=269
2y N = 193
5 y N=14
Age at Operation
• Median Age At CPS (N=277)
.54 +/- .52y (.12 - 6.7)
• Median Age At Fontan (N=223)
2.8 +/- 1.2y (.9 - 8.5)
• 6 Children alive > 5yrs w/out Fontan
(47, 73, 77, 198, 210, 220)
Competing Risks:
Completion of CPS from Birth
Alive w/o CPS (N=12)
Died before CPS( N=14)
Survived to CPS (N=277)
Years after Birth
% In
each
Gro
up
N=303
At 6 mo:
56% alive w/out intervention
41% survived to CPS
3% died w/out CPS
At 1 year:
8% alive w/out intervention
90% survived to CPS
4% died before CPS
Competing Risks:
Transition to Fontan from CPS
Years after CPS
% In
each
Gro
up
Alive w/o Fontan (N=40)
Died before Fontan(N=15)
Survived to Fontan(214)
N=277
At 2 yr:
62% alive w/out Fontan after CPS
5% died w/out Fontan after CPS
33% survived to Fontan after CPS
At 4 yr:
10% alive w/out Fontan after CPS
5% died w/out Fontan after CPS
85% survived to Fontan after CPS
Time Related Competing Risks
Model • Time Zero
– DOB
• Outcomes – Achievement of Fontan
– Death Before Fontan
• Time varying co-variables – Age at operation
– Surgical procedures and associated variables
– Morphology based on baseline echo and echo prior to CPS
Univariate Analysis Results
• Increased Age at CPS resulted in
increased transition to Fontan – when
unadjusted
– P= .046
• Increased shunt size resulted in increased
risk for death – when unadjusted
– P= .016
Multivariate Analysis Results
• Transition to Fontan
– BCPA as CPS procedure increased
transition to Fontan P<.001
– Larger RPA diameter on Echo prior to CPS
decreased transition P=.03
• Survival
– Having a BTS as 1 stage procedure increased
risk of death P=.03
Summary
• Timing of CPS does not appear to have a
significant affect on mortality before
Fontan, or transition to Fontan
• As previously shown, patients who have a
BTS as a first procedure have an
increased risk of death.
2. Functional result of Fontan
Repeated measures of LV function
and AV-valve function
0 2 4 6 8 10 12
Grade of LV dysfunction post-Fontan 559 echos on 170 patients
Years post-Fontan
Normal
Mild
Moderate
Severe
0 2 4 6 8 10 12
Grade of LV dysfunction post-Fontan 559 echos on 170 patients
10 patients
Years post-Fontan
Normal
Mild
Moderate
Severe
0 2 4 6 8 10 12
Grade of AV-valve regurgitation post-Fontan 505 echos on 160 patients
Years post-Fontan
Normal
Trivial
Mild
Moderate
0 2 4 6 8 10 12
Grade of AV-valve regurgitation post-Fontan 505 echos on 160 patients
Years post-Fontan
Normal
Trivial
Mild
Moderate 5 patients
3. Practical question
When doing a mBTS for tricuspid
atresia, is it better to leave the
native mPA open?
Sub-analysis:
All patients with type Ib or Ic (patent RVOT) who required a mBTS
N = 135
Univariate analysis of mPA ligation:
Overall survival: P = 0.45
Transition to Fontan: P = .85