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