2003 ISHLT J Heart Lung Transplant 2003; 22: 610-72. LUNG TRANSPLANTATION Pediatric Recipients.

2003ISHLTJ Heart Lung Transplant 2003; 22: 610-72.

LUNG TRANSPLANTATION

Pediatric Recipients


AGE DISTRIBUTION OF PEDIATRIC LUNG RECIPIENTS (Transplants: January 1986 - June 2002)

0

10

20

30

40

50

60

70

80

90

100

110

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


AGE DISTRIBUTION FOR DONORS OF PEDIATRIC

LUNG RECIPIENTS (Transplants: January 1986 - June 2002)

0

25

50

75

100

125

150

175

0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18-25

26-30

31+

Donor Age (Years)

Nu

mb

er

of

Tra

ns

pla

nts


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

4540

8270

83 85

65 67

57


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


PEDIATRIC LUNG TRANSPLANTATION: Indications (Transplants: January 1991 - June 2002)

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

Cystic Fibrosis 56 34.1%

PPH 7 17.1% 24 14.6%

Congenital Heart Disease 20 48.8% 19 11.6%

IPF 12 7.3%

Pulmonary Vascular Disease 5 12.2% 6 3.7%

Re-TX: Non-OB 3 7.3% 8 4.9%

Re-TX: OB 10 6.1%

OB (Non-ReTX) 6 3.7%

Bronchiectasis 2 1.2%

COPD/Emphysema 2 1.2%

Other 6 14.6% 19 11.6%


3%

67%

4%

8%

9%

6%

3%

Congenital Heat Disease

Cystic Fibrosis

IPF

Other

PPH

ReTX

OB (Non-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


PEDIATRIC LUNG TRANSPLANTATIONActuarial Survival for Congenital Diagnosis (Transplants: January 1990 – June 2001)

0

25

50

75

100

0 1 2 3 4 5 6 7 8 9 10Years

Su

rviv

al (

%)

Eisenmenger's Syndrome (N=12)

Other Congenital (N=33)

Eisenmenger's + Other Congenital (N=45)


PEDIATRIC LUNG TRANSPLANTATIONActuarial Survival by Procedure Type (Transplants: January 1990 - June 2001)

0

25

50

75

100

0 1 2 3 4 5 6 7 8 9 10Years

Su

rviv

al

(%)

Single Lung (N=65)

Bilateral/Double Lung (N=543)

HALF-LIFE Single Lung: 3.4 Years; Bilateral/double Lung: 2.5 Years

P = 0.003


PEDIATRIC LUNG TRANSPLANTATIONActuarial Survival by Age Group (Transplants: January 1990 - June 2001)

0

25

50

75

100

0 1 2 3 4 5 6 7 8 9 10

Years

Su

rviv

al (

%)

<1 Year (N=40)

1-10 Years (N=169)

11-17 Years (N=399)

No comparisons are statistically significant

HALF-LIFE<1 Year: ½-life = 3.1 Years1-10 Years: ½-life = 3.5 Years11-17 Years: ½-life = 3.0 Years


PEDIATRIC LUNG TRANSPLANTATIONConditional Actuarial Survival by Age Group (Transplants: January 1990 - June 2001)

0

25

50

75

100

0 1 2 3 4 5 6 7 8 9 10

Years

Su

rviv

al (

%)

<1 Year (N=25)

1-10 Years (N=105)

11-17 Years (N=240)


HALF-LIFE<1 Year: ½-life = 7.1 Years1-10 Years: ½-life = 5.9 Years11-17 Years: ½-life = 5.6 Years


PEDIATRIC LUNG TRANSPLANTATION Actuarial Survival by Era (Transplants: January 1988 - June 2001)

0

20

40

60

80

100

0 1 2 3 4 5 6 7 8 9 10

Years

Su

rviv

al (

%)

1988-1992 (N=127)

1993-1997 (N=320)

1998-2001 (N=245)

HALF-LIFEUnconditional 1988-1992: 2.6 Years; 1993-1997: 3.5 Years; 1998-2001: 3.1 YearsConditional 1988-1992: 8.2 Years; 1993-1997: 5.7 Years



PEDIATRIC LUNG TRANSPLANTATIONActuarial Survival by Procedure Type

Diagnosis: PPH (Transplants: January 1990 - June 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 = 17) Double Lung (N=52)

P = 0.002


PEDIATRIC LUNG RECIPIENTS Functional Status (Follow-ups: April 1994-June 2002)

0%

20%

40%

60%

80%

100%

1 Year (N = 280) 3 Years (N = 170) 5 Years (N = 96)

No Activity Limitations Performs with Assistance Total Assistance


PEDIATRIC LUNG RECIPIENTS Rehospitalization Post-transplant (Follow-ups: April 1994 - June 2002)

0%

20%

40%

60%

80%

100%

Up to 1 Year (N = 316) Between 2 and 3 Years (N = 186)

Between 4 and 5 Years (N = 103)

No Hospitalization Hospitalized, Not Rejection/Not Infection

Hospitalized, Rejection Hospitalized, Infection Only

Hospitalized, Rejection + Infection


0

10

20

30

40

50

Any Induction (N = 50) Polyclonal ALG/ATG (N = 18) IL2R-antagonist (N = 32)

% o

f p

ati

en

tsPEDIATRIC LUNG RECIPIENTS

Induction ImmunosuppressionFor follow-ups between January 2000 and June 2002


0

10

20

30

40

50

60

Any Induction (N = 22)

Polyclonal ALG/ATG (N = 9)

IL2R-antagonist (N = 13)







% o

f p

ati

en

ts

2000 2001 2002 (6 months)

PEDIATRIC LUNG RECIPIENTSInduction Immunosuppression (Follow-ups: January 2000 - June 2002)


0

20

40

60

80

100

Cyclosporine Tacrolimus Rapamycin MMF Azathioprine Prednisone

% o

f P

ati

en

ts

Year 1 (N = 88)

Year 5 (N = 56)

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

For follow-ups between January 2000 and June 2002

NOTE: Different patients are analyzed in Year 1 and Year 5


0

20

40

60

80

100

Cyclosporine Tacrolimus Rapamycin MMF Azathioprine Prednisone

% o

f P

atie

nts

Year 1 (N = 86) Year 5 (N = 55)

PEDIATRIC LUNG RECIPIENTS Maintenance Immunosuppression at Time of Follow-up




0

20

40

60

80

100

CalcineurinInhibitor

CellCycle Prednisone CalcineurinInhibitor

CellCycle Prednisone

% o

f P

ati

en

ts

CyA

TacTac

CyA

AZA AZA

MMFMMF

PEDIATRIC LUNG RECIPIENTS Maintenance Immunosuppression at Time of Follow-up


1 Year Follow-up (N = 86) 5 Year Follow-up (N = 55)NOTE: Different patients are analyzed in Year 1 and Year 5


0%

20%

40%

60%

80%

100%

Year 1 (N = 86) Year 5 (N = 55)

% o

f P

atie

nts

Other

Tacrolimus + MMF

Tacrolimus + AZA

Cyclosporine + MMF

Cyclosporine + AZA

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




POST-LUNG TRANSPLANT MORBIDITY FOR PEDIATRICS Cumulative Prevalence in Survivors within 1 Year Post-Transplant

(Follow-ups: April 1994-June 2002)

Outcome Within 1 Year

Hypertension 37.7% (N = 313)

Renal Dysfunction 7.6% (N = 315) Abnormal Creatinine < 2.5 mg/dl 5.1% Creatinine>2.5 mg/dl 1.6% Chronic Dialysis 0.6% Renal Transplant 0.3%

Hyperlipidemia 0.9% (N = 326)

Diabetes 21.0% (N = 314)

Bronchiolitis Obliterans 14.7% (N = 286)


POST-LUNG TRANSPLANT MORBIDITY FOR PEDIATRICS Cumulative Prevalence in Survivors within 5 Years Post-Transplant

(Follow-ups: April 1994-June 2002)

Outcome Within 5 Years

Hypertension 73.7% (N = 57)

Renal Dysfunction 24.1% (N = 58) Abnormal Creatinine < 2.5 mg/dl 12.1% Creatinine>2.5 mg/dl 6.9% Chronic Dialysis 3.4% Renal Transplant 1.7%

Hyperlipidemia 1.7% (N = 59)

Diabetes 31.0% (N = 58)

Bronchiolitis Obliterans 20.5% (N = 39)


Freedom from Bronchiolitis Obliterans For Pediatric Lung Recipients (Follow-ups: April 1994 - June 2002)

0

10

20

30

40

50

60

70

80

90

100

0 1 2 3 4 5 6

Years

% F

ree

fro

m B

ron

ch

ioli

tis

O

bli

tera

ns


Freedom from Severe Renal Dysfunction*For Pediatric Lung Recipients (Follow-ups: April 1994 - June 2002)

0

10

20

30

40

50

60

70

80

90

100

0 1 2 3 4 5 6 7Years

% F

ree

fro

m S

ev

ere

Re

na

l D

ys

fun

cti

on

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


MALIGNANCY POST-LUNG TRANSPLANTATION FOR PEDIATRICSCumulative Incidence for Survivors (Follow-ups: April 1994 - June 2002)

Malignancy/Type 1-Year Survivors 5-Year Survivors

No Malignancy 304 (93.8%) 53 (89.8%)

Malignancy (all types combined) 20 (6.2%)

6 (10.2%)

Malignancy Type

Lymph 18 6

Other 2


Freedom from MalignancyFor Pediatric Lung Recipients (Follow-ups: April 1994 - June 2002)

50

60

70

80

90

100

0 1 2 3 4 5 6 7

Years

% F

ree

fro

m M

alig

nan

cy

All malignancy Lymph Skin Other


PEDIATRIC LUNG RECIPIENTSRelationship of Diabetes and Cystic Fibrosis

Transplants: April 1994 – June 2001

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

112

69.6%

49

30.4%

32

54.2%

27

45.8%

Other Diagnosis

145

97.3%

4

2.7%

59

90.8%

6

9.2%

*p < 0.0001 **p < 0.0001


PEDIATRIC LUNG TRANSPLANT RECIPIENTS: Cause Of Death(Deaths: January 1992- June 2002)

CAUSE OF DEATH0-30 Days (N = 45)

31 Days - 1 Year

(N = 75)

>1 Year - 3 Years

(N = 81)

>3 Years - 5 Years

(N = 35)

>5 Years

(N = 11)

BRONCHIOLITIS 5 (6.7%) 30 (37.0%) 16 (45.7%) 5 (45.5%)

ACUTE REJECTION 3 (4.0%) 1 (2.9%)

LYMPHOMA 3 (4.0%) 4 (4.9%) 1 (2.9%) 2 (18.2%)

CMV 5 (6.7%)

INFECTION, NON-CMV

6 (13.3%) 31 (41.3%) 17 (21.0%) 8 (22.9%) 1 (9.1%)

GRAFT FAILURE 20 (44.4%) 14 (18.7%) 16 (19.8%) 3 (8.6%) 2 (18.2%)

CARDIOVASCULAR 6 (13.3%) 2 (2.7%) 2 (2.5%)

TECHNICAL 6 (13.3%) 1 (1.3%)

OTHER 7 (15.6%) 11 (14.7%) 12 (14.8%) 6 (17.1%) 1 (9.1%)

2003 ISHLT J Heart Lung Transplant 2003; 22: 610-72. LUNG TRANSPLANTATION Pediatric Recipients.

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Transcript of 2003 ISHLT J Heart Lung Transplant 2003; 22: 610-72. LUNG TRANSPLANTATION Pediatric Recipients.