2002 ishlt j heart lung transplant 2002; 21: 827-840. lung transplantation pediatric recipients

2002ISHLT

J Heart Lung Transplant 2002; 21: 827-840.

LUNG TRANSPLANTATION

Pediatric Recipients

2002ISHLT


AGE DISTRIBUTION OF PEDIATRIC LUNG RECIPIENTS

0

10

20

30

40

50

60

70

80

90

100

0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17

Recipient Age (Years)

Nu

mb

er o

f T

ran

spla

nts

2002ISHLT


AGE DISTRIBUTION FOR DONORS OF PEDIATRIC LUNG RECIPIENTS (1986-2001)

0

30

60

90

120

150

180

210

240

0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18+

Donor Age (Years)

Nu

mb

er o

f T

ran

spla

nts

2002ISHLT


AGE DISTRIBUTION OF PEDIATRIC LUNG RECIPIENTS

By Year of Transplant

0

20

40

60

80

100

11-17 Years1-10 Years

<1 Year

Nu

mb

er o

f T

ran

spla

nts

1 3 4 6

20

4750

4542

8272

85 86

66 67

57

* Numbers may be low due to delayed reporting.

2002ISHLT


NUMBER OF CENTERS REPORTING PEDIATRIC LUNG TRANSPLANTS

0

10

20

30

4019

86

1987

1988

1989

1990

1991

1992

1993

1994

1995

1996

1997

1998

1999

2000

2001

*

Transplant Year

Num

ber

of P

edia

tric

Cen

ters

R

epor

ting

* Numbers may be low due to delayed reporting.

2002ISHLT


PEDIATRIC LUNG TRANSPLANTATION: Indications (1991-2001)

DIAGNOSIS AGE: < 1 Year AGE: 1-10 Years

Cystic Fibrosis 55 34.2%

PPH 7 16.7% 24 14.9%

Congenital Heart Disease 14 33.3% 17 10.6%

IPF 12 7.5%

Pulmonary Vascular Disease 6 14.3% 6 3.7%

Re-TX: Non-OB 3 7.1% 8 5.0%

Re-TX: OB 9 5.6%

OB (Non-ReTX) 6 3.7%

Bronchiectasis 2 1.2%

COPD/Emphysema 2 1.2%

Other 11 26.2% 17 10.6%

2002ISHLT


PEDIATRIC LUNG TRANSPLANTATIONIndications by Age (1991-2001)

11%

37%

8%

13%

16%

11%

4%

Congenital Heart Disease Cystic Fibrosis

Idiopathic Pulmonary Fibrosis Other

Primary Pulmonary Hypertension Retransplant/Graft Failure

Pulmonary Vascular Disease

14%

7%

33%

29%

17%

Age <1 Year

Age 1-10 Years

2002ISHLT


3%

66%

3%

13%

9%

6%

Congenital Heat Disease

Cystic Fibrosis

IPF

Other

PPH

ReTX

DIAGNOSIS IN PEDIATRIC LUNG RECIPIENTSAge: 11-17 Years

0

25

50

75

100

1990 1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001

% o

f Cas

es

PPH Cystic Fibrosis IPF

2002ISHLT


PEDIATRIC LUNG TRANSPLANTATIONActuarial Survival by Procedure Type (1990-2001)

0

25

50

75

100

0 1 2 3 4 5 6 7 8 9 10

Years Post Transplantation

Sur

viva

l (%

)

Single Lung (N=70)

Bilateral/Double Lung (N=567)

P = 0.006

2002ISHLT


PEDIATRIC LUNG TRANSPLANTATIONActuarial Survival by Age Group (1990-2001)

0

25

50

75

100

0 1 2 3 4 5 6 7 8 9 10


Sur

viva

l (%

)

<1 Year (N=44)

1-10 Years (N=172)

1-11 Years (N=421)

<1 Year: ½-life = 3.5 Years1-10 Years: ½-life = 3.5 Years11-17 Years: ½-life = 3.2 Years

2002ISHLT


PEDIATRIC LUNG TRANSPLANTATIONConditional Actuarial Survival by Age Group (1990-2001)

0

25

50

75

100

0 1 2 3 4 5 6 7 8 9 10


Sur

viva

l (%

)

<1 Year (N=26)

1-10 Years (N=98)

1-11 Years (N=222)<1 Year: ½-life = 7.1 Years1-10 Years: ½-life = 5.9 Years11-17 Years: ½-life = 5.4 Years

2002ISHLT


PEDIATRIC LUNG TRANSPLANTATION Actuarial Survival by Era (1988-2001)

0

20

40

60

80

100

0 1 2 3 4 5 6 7 8 9 10

Years Post-Transplantation

1988-1992 (N=127)

1993-1997 (N=326)

1998-2001 (N=276)

Su

rviv

al (

%)

2002ISHLT


PEDIATRIC LUNG TRANSPLANTATIONActuarial Survival by Procedure Type

Diagnosis: PPH (1990-2001)

0

25

50

75

100

0 1 2 3 4 5 6


Sur

viva

l (%

)

Single Lung (N = 17) Double Lung (N=52)

P = 0.002

2002ISHLT


PEDIATRIC LUNG RECIPIENTS Functional Status (April 1994-December 2001)

0%

20%

40%

60%

80%

100%

1 Year (N = 263) 3 Years (N = 156) 5 Years (N = 82)

No Activity Limitations Performs with Assistance Total Assistance

2002ISHLT


PEDIATRIC LUNG RECIPIENTS Rehospitalization Post-transplant (April 1994 - December 2001)

0%

20%

40%

60%

80%

100%

Up to 1 Year (N = 299)

Between 2 and 3 Years (N = 170)

Between 4 and 5 Years (N = 87)

No Hospitalization Hospitalized, Not Rejection/Not Infection

Hospitalized, Rejection Hospitalized, Infection Only

Hospitalized, Rejection + Infection

2002ISHLT


0

10

20

30

40

50

Any Induction (N = 48)

Polyclonal ATG/ALG (N = 16)

OKT3 (N = 1) IL2-R Antagonist (N = 32)

% o

f p

ati

en

tsPEDIATRIC LUNG RECIPIENTS

Induction ImmunosuppressionFor follow-ups between October 1999 and December 2001

2002ISHLT


0

20

40

60

80

100

Cyclosporine Tacrolimus Rapamycin MMF Azathioprine Prednisone

% o

f P

atie

nts

Year 1 (N = 78)

Year 5 (N = 41)

PEDIATRIC LUNG RECIPIENTS Maintenance Immunosuppression at Any Time During Follow-up Period

For follow-ups between October 1999 and December 2001

2002ISHLT


0

20

40

60

80

100

Cyclosporine Tacrolimus Rapamycin MMF Azathioprine Prednisone

% o

f P

atie

nts

Year 1 (N = 76) Year 5 (N = 39)

PEDIATRIC LUNG RECIPIENTS Maintenance Immunosuppression at Time of Follow-up


2002ISHLT


0

20

40

60

80

100

CalcineurinInhibitor

CellCycle Prednisone CalcineurinInhibitor

CellCycle Prednisone

% o

f P

ati

en

ts

CyA

TacTac

CyAAZA AZA

MMFMMF

PEDIATRIC LUNG RECIPIENTS Maintenance Immunosuppression at Time of Follow-up


1 Year Follow-up (N = 76) 5 Year Follow-up (N = 39)

2002ISHLT


0%

20%

40%

60%

80%

100%

Year 1 (N = 76) Year 5 (N = 39)

% o

f P

atie

nts

None Given

Other

Tacrolimus + MMF

Tacrolimus + AZA

Cyclosporine + MMF

Cyclosporine + AZA

PEDIATRIC LUNG RECIPIENTS Maintenance Immunosuppression at Time of Follow-up Report


2002ISHLT


POST-LUNG TRANSPLANT MORBIDITY For Pediatric Recipients (April 1994-December 2000)

Outcome By 1 Year By 5 Years

Hypertension 36.2% (N=298) 71.1% (N=45)

Renal Function (N=300) (N=46) Normal 92.3% 73.9% Renal Dysfunction 5.0% 10.9% Creatinine>2.5 mg/dl 1.3% 8.7% Chronic Dialysis 1.0% 4.3% Renal Transplant 0.3% 2.2%

Hyperlipidemia 0.6% (N=308) 4.3% (N=46)

Diabetes 19.8% (N=298) 28.3% (N=46)

Bronchiolitis Obliterans 14.2% (N=267) 14.8% (N=27)

2002ISHLT


Freedom from Bronchiolitis Obliterans For Pediatric Lung Recipients (April 1994-December 2000)

40

50

60

70

80

90

100

0 12 24 36 48 60

Months

% F

ree

from

Bro

nchi

oliti

s O

blite

rans

2002ISHLT


Freedom from Renal Dysfunction*For Pediatric Lung Recipients (April 1994-December 2000)

50

60

70

80

90

100

0 12 24 36 48 60

Months

% F

reed

om fr

om R

enal

D

ysfu

nctio

n

* Renal dysfunction = Creatinine > 2.5 mg/dl, dialysis or renal transplant

2002ISHLT


MALIGNANCY POST-LUNG TRANSPLANTATION FOR PEDIATRICSCumulative Incidence for Survivors (April 1994-December 2001)

Malignancy/Type 1-Year Survivors 5-Year Survivors

No Malignancy 288 (93.5%) 45 (91.8%)

Malignancy (all types combined) 20 (6.5%) 4 (8.2%)

Malignancy Type

Lymph 18 4

Other 2

2002ISHLT


Freedom from MalignancyFor Pediatric Lung Recipients (April 1994-December 2000)

50

60

70

80

90

100

0 12 24 36 48 60

Months

% F

ree

fro

m M

alig

nan

cy

All malignancy Lymph Skin Other

2002ISHLT


PEDIATRIC LUNG TRANSPLANTATION Actuarial Survival Based on Rejection within 1st Year (1994-2000)

0

20

40

60

80

100

0 1 2 3 4 5 6 7


Rejection within 1st Year (N = 142)

No Rejection within 1st Year (N = 101)

Su

rviv

al (

%)

P= 0.01

2002ISHLT


PEDIATRIC LUNG RECIPIENTSRelationship of Diabetes and Cystic Fibrosis

Transplants: April 1994 – December 2000

Diagnosis

Report of Diabetes Developing Between

Transplant and 1st Year*

Report of Diabetes Developing Between

Transplant and 3rd Year**

No Yes No Yes

Cystic Fibrosis

103

70.1%

44

29.9%

29

55.8%

23

44.2%

Other Diagnosis

137

97.3%

4

2.8%

53

94.6%

3

5.4%

*p < 0.0001 **p < 0.0001

2002ISHLT


PEDIATRIC LUNG RECIPIENTS: Time Of Death (1988-2001)

0%

20%

40%

60%

80%

100%

<1 1-10 11-17Recipient Age (years)

% o

f D

ea

ths

0-30 Days 31 Days -1 Year 1- 3 Years 3 - 5 Years 5+ Years

2002ISHLT


PEDIATRIC LUNG TRANSPLANT RECIPIENTS: Cause of Death (1982-2001)

0%

20%

40%

60%

80%

100%

0-30 Days (N = 45)

31 Days - 1 Year(N=71)

>1 Year - 3Years (N = 66)

>3 Years - 5Years (N = 27)

>5 Years (N = 8)

Per

cent

age

of D

eath

s

Other

Technical

Graft Failure - Other

Primary Graft Failure

Bronchiolitis

Infection, Non-CMV

Acute Rejection

Lymphoma

Cardiac

Timing of Death

2002 ishlt j heart lung transplant 2002; 21: 827-840. lung transplantation pediatric recipients

Documents

ishlt j heart lung transplant

pediatric lung transplants

congenital heart disease1433

delayed reporting

number of centers

ob nonretx63

pulmonary vascular disease614

years cystic fibrosis5534