lung transplantation

LUNG TRANSPLANTATION

Pediatric Recipients

2014JHLT. 2014 Oct; 33(10): 1025-1033

Table of Contents

Donor, recipient and center characteristics:

slides 3-18

Post transplant: survival and other outcomes:

slides 19-36

Induction and maintenance

immunosuppression: slides 37-43

Post transplant morbidities: slides 44-60

Multivariable analyses: slides 61-69

2014JHLT. 2014 Oct; 33(10): 1025-1033

Donor, Recipient and Center Characteristics

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsRecipient Age Distribution – Number

(Transplants: January 1986 – June 2013)

1986-1999 (N=675) 2000-6/2012 (N=1,310)0

200

400

600

800

1,000

1,200

1,400

<1 1-5 6-10 11-17

Nu

mb

er

of

Tra

ns

pla

nts

Analysis includes deceased and living donor transplants.

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsRecipient Age Distribution – Percentage


1986-1999 (N=675) 2000-6/2012 (N=1,310)0%

10%

20%

30%

40%

50%

60%

70%

80%

90%

100%

11-17 6-10

1-5 <1

% o

f T

ran

sp

lan

ts


2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsDonor Type Distribution by Transplant Year

(Transplants: January 1986 – December 2012)

1986

1988

1990

1992

1994

1996

1998

2000

2002

2004

2006

2008

2010

2012

0

20

40

60

80

100

120

140

LivingDeceased

Nu

mb

er

of

Tra

ns

pla

nts

NOTE: This figure includes only the lung transplants that are reported to the ISHLT Transplant Registry. As such, the presented data may not reflect the changes in the number of lung transplants performed worldwide.


2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsDonor Type Distribution by Recipient Age Group Within Era


<1 year 1 - 5 years

6 - 10 years

11 - 17 years

<1 year 1 - 5 years

6 - 10 years

11 - 17 years

0

200

400

600

800

1,000

1,200

Living

Deceased

Recipient Age

Nu

mb

er

of

Tra

ns

pla

nts

1986-1999 2000-6/2013


2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsDonor Age Distribution


0%

10%

20%

30%

40%

50%

60%

70%

80%

90%

100%

695

455

324

245

10116

60+ years

50 - 59 years

35 - 49 years

18 - 34 years

11 - 17 years

0 - 10 years

Donor Age (Years)

% o

f T

ran

sp

lan

ts

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsDonor and Recipient Age (Transplants: January 2000 – June 2013)

<1 year 1 - 5 years 6 - 10 years 11 - 17 years0%

20%

40%

60%

80%

100%

0-10 years 11-17 years 18-34 years 35-49 years 50-59 years 60+ years

Recipient Age

% o

f T

ran

sp

lan

ts

Donor Age:

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsRecipient Age Distribution by Year of Transplant

1986

1988

1990

1992

1994

1996

1998

2000

2002

2004

2006

2008

2010

2012

0

20

40

60

80

100

120

140

1 3 5 7

23

45 48 49 52

96

82

95 96

73 73 72 7478

89

97103

108114

125 126

106

93

Total

11-17 years

6-10 years

1-5 years

Nu

mb

er

of

Tra

ns

pla

nts

NOTE: This figure includes only the pediatric lung transplants that are reported to the ISHLT Transplant Registry. Therefore, these numbers should not be interpreted as the rate of change in pediatric lung procedures performed worldwide.


2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung Retransplantsby Year of Retransplant

Only patients who were less than 18 years old at the time of retransplant are included.2014

1986

1988

1990

1992

1994

1996

1998

2000

2002

2004

2006

2008

2010

2012

0

2

4

6

8

10

12

0

4

8

12

16

20

24

N %

Year of retransplant

Nu

mb

er o

f re

tran

spla

nts

% o

f re

tran

spla

nts

JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsNumber of Centers Reporting Transplants by Location

(Transplants: January 1986 – December 2012)

1986

1988

1990

1992

1994

1996

1998

2000

2002

2004

2006

2008

2010

2012

0

5

10

15

20

25

30

35

40

45

50

55

Others

North America

Europe

Transplant Year

Nu

mb

er

of

Ce

nte

rs


2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsNumber of Centers Reporting Transplants

by Pediatric Center Volume

1986

1988

1990

1992

1994

1996

1998

2000

2002

2004

2006

2008

2010

2012

0

5

10

15

20

25

30

35

40

45

50

5520+ transplants

10-19 transplants

5-9 transplants

1-4 transplants

Transplant Year

Nu

mb

er

of

Ce

nte

rs


2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsNumber of Transplants by Pediatric Center Volume

1986

1988

1990

1992

1994

1996

1998

2000

2002

2004

2006

2008

2010

2012

0

20

40

60

80

100

120

14020+ transplants

10-19 transplants

5-9 transplants

1-4 transplants

Transplant Year

Nu

mb

er

of

Tra

ns

pla

nts


2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsIndications by Age Group (Transplants: January 1990 – June 2013)

Diagnosis < 1 Year 1-5 Years 6-10 Years 11-17 Years

Cystic Fibrosis 1 1.0% 6 4.5% 148 52.3% 968 69.7%

Idiopathic Pulmonary Arterial Hypertension 12 12.1% 30 22.6% 26 9.2% 109 7.8%

Retransplant: Obliterative Bronchiolitis 0 7 5.3% 9 3.2% 40 2.9%

Congenital Heart Disease 16 16.2% 11 8.3% 4 1.4% 12 0.9%

Idiopathic Pulmonary Fibrosis 10 10.1% 21 15.8% 16 5.7% 47 3.4%

Obliterative Bronchiolitis, Not Retx 0 10 7.5% 23 8.1% 58 4.2%

Retransplant, Not OB 3 3.0% 4 3.0% 8 2.8% 37 2.7%

Interstitial Pneumonitis 1 1.0% 2 1.5% 2 0.7% 1 0.1%

Pulmonary Vascular Disease 8 8.1% 8 6.0% 4 1.4% 1 0.1%

Eisenmenger’s Syndrome 1 1.0% 5 3.8% 3 1.1% 9 0.6%

Pulmonary Fibrosis, Other 8 8.1% 12 9.0% 15 5.3% 28 2.0%

Surfactant Protein B Deficiency 17 17.2% 5 3.8% 0 0

COPD/Emphysema 4 4.0% 2 1.5% 2 0.7% 10 0.7%

Bronchopulmonary Dysplasia 3 3.0% 3 2.3% 6 2.1% 3 0.2%

Bronchiectasis 1 1.0% 0 2 0.7% 18 1.3%

Other 14 14.1% 7 5.3% 15 5.3% 48 3.5%

Analysis includes deceased and living donor transplants. For some retransplants diagnosis other than retransplant is reported, so the total percentage of retransplants may be greater.

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsAge Distribution by Location


Europe (N=512) North America (N=712) Other (N=86)0%

20%

40%

60%

80%

100%

<1 year 1 - 5 years 6 - 10 years 11 - 17 years

% o

f T

ran

sp

lan

ts


2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsDiagnosis Distribution by Location(Transplants: January 2000 – June 2013)


20%

40%

60%

80%

100%Cystic Fibrosis IPAH IPF OB Other Congenital heart disease Retx

% o

f T

ran

sp

lan

ts

NOTE: Unknown diagnoses were excluded from this tabulation. Analysis includes deceased and living donor transplants.

For some retransplants diagnosis other than retransplant is reported, so the total percentage of retransplants may be greater.

Total number of transplants reported:Europe = 512North America = 712Other = 86

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung Transplants Donor Age Distribution by Location



20%

40%

60%

80%

100%

0 - 10 years 11 - 17 years 18 - 34 years 35 - 49 years 50 - 59 years 60+ years

% o

f D

on

ors

NOTE: Transplants with unknown donor age and living donor transplants were excluded from this tabulation.

Total number of transplants reported:Europe = 512North America = 712Other = 86

2014JHLT. 2014 Oct; 33(10): 1025-1033

Post Transplant:Survival and Other Outcomes

2014JHLT. 2014 Oct; 33(10): 1025-1033

Lung TransplantsKaplan-Meier Survival by Recipient Age Group


0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 180

25

50

75

100

Adult (N=43,501) Pediatric (N=1,751)

Years

Su

rviv

al (

%)

Median survival (years): Adult = 5.6; Pediatric = 5.1

p = 0.4400

2014JHLT. 2014 Oct; 33(10): 1025-1033

Lung TransplantsKaplan-Meier Survival by Recipient Age Group and Procedure

Type (Transplants: January 1990 – June 2012)

0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 170

25

50

75

100Adult, Single (N=17,295)

Adult, Bilateral/Double (N=26,175)

Pediatric (N=1,751)

Years

Su

rviv

al (

%)

Median survival (years): Adult, Single = 4.5; Adult, Bilateral/Double = 7.0; Pediatric = 5.1

Adult, Bilateral/ Double vs. Adult, Single: p<0.0001Adult, Bilateral/ Double vs. Pediatric: p=0.0003Adult, Single vs. Pediatric: p<0.0001

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsKaplan-Meier Survival by Procedure Type


0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 150

25

50

75

100

Single Lung (N=94)

Bilateral/Double Lung (N=1,654)

Years

Su

rviv

al (

%)

Median survival (years): Single Lung = 1.9; Bilateral/Double Lung = 5.5

p<0.0001

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsKaplan-Meier Survival by Diagnosis


0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 170

25

50

75

100

Cystic Fibrosis (N=985)

Non-Cystic Fibrosis (N=706)

Years

Su

rviv

al (

%)

Median survival (years): Cystic Fibrosis = 4.9; Non-Cystic Fibrosis = 5.1

p=0.6070

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsKaplan-Meier Survival by Recipient Age Group


0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 160

25

50

75

100

<1 Year (N=99)1-5 years (N=129)6-10 years (N=257)11-17 years (N=1,266)

Years

Su

rviv

al (

%)

N at risk = 11N at risk = 17

N at risk = 10Median survival (years):<1 year = 6.41-5 years = 6.76-10 years = 6.511-17 years = 4.7

Pair-wise comparisons were not significant at p<0.05.

N at risk = 25

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsConditional Kaplan-Meier Survival by Recipient Age Group


0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 160

25

50

75

100<1 year (N=63)1-5 years (N=90)6-10 years (N=199)11-17 years (N=933)

Years

Su

rviv

al (

%) N at risk = 11

N at risk = 17

N at risk = 10

Conditional median survival (years):<1 year = 11.21-5 years = 10.56-10 years = 9.711-17 years = 7.7

Pair-wise comparisons were not significant at p<0.05.

N at risk = 25

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsKaplan-Meier Survival by Era


0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 160

25

50

75

100

1988-1999 (N=602)2000-2005 (N=446)

Years

Su

rviv

al (

%)

N at risk = 40

N at risk = 14

Median survival (years):Unconditional 1988-1999=3.3; 2000-2005=6.1; 2006-6/2012=5.6Conditional 1988-1999=7.2; 2000-2005=9.0; 2006-6/2012=7.0

2014

1988-1999 vs. 2000-2005: p=0.0041988-1999 vs. 2006-6/2012: p<0.00012000-2005 vs. 2006-6/2012 is not significant at p<0.05.

N at risk = 12

JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsKaplan-Meier Survival by Donor Age for Recipients Age 11-17

Years (Transplants: January 1990 – June 2012)

0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 160

25

50

75

100

0-10 years (N=243)

11-17 years (N=400)

18-34 years (N=284)

Years

Su

rviv

al (

%)

N at risk = 12

N at risk = 11

Median survival (years):0-10 years=5.411-17 years=4.718-34 years=4.735-49 years=4.450+ years=3.9

No pair-wise comparisons were significant at p<0.05.

N at risk = 12

N at risk = 10

N at risk = 11

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsKaplan-Meier Survival by Donor Type for Recipients Age 11-

17 Years (Transplants: January 1990 – June 2012)

0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 160

25

50

75

100

Deceased Donor (N=1,266)

Living Donor (N=85)

Years

Su

rviv

al (

%)

N at risk = 25

N at risk = 10

Median survival (years):Deceased = 4.7Living = 3.8

p = 0.1743

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsKaplan-Meier Survival for Living Donor Transplant Recipients

Age 11-17 Years by Era (Transplants: January 1990 – June 2012)

0 1 2 3 4 5 60

25

50

75

100

1990-1999 (N=49)

2000-6/2012 (N=36)

Years

Su

rviv

al (

%)

N at risk = 16

N at risk = 11

p = 0.8516

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung Retransplants(Retransplants: January 1994 – June 2013)

0-<1 month 1-<12 months 12-<36 months 36+ months Not reported0

5

10

15

20

25

30

35

40

45

Time Between Previous and Current Transplant

Nu

mb

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of

Re

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pla

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2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsKaplan-Meier Survival by Transplant Type


0 1 2 3 4 5 6 70

20

40

60

80

100

Retransplant (N = 106)Primary (N = 1,490)

Years

Su

rviv

al (

%)

N at risk = 15

N at risk = 246

p<0.0001

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsSurvival by Transplant Type and Inter-Transplant Interval


0 1 2 3 4 5 60

20

40

60

80

100

Retransplant/<1 Year (N=34)

Retransplant/≥1 Year (N=82)

Primary (N=1,743)

Years

Su

rviv

al (

%)

N at risk = 11

N at risk = 15

Retransplant/<1 Year vs. Primary: p<0.0001Retransplant/≥1 Year vs. Primary: p=0.0007Retransplant/<1 Year vs. Retransplant/≥1 year is not significant at p<0.05.

Analysis includes deceased and living donor transplants. Only patients who were less than 18 years old at the time of retransplant are included.

2014

N at risk = 388

JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung RetransplantsSurvival by Diagnosis


0 1 2 3 4 5 60

20

40

60

80

100

Obliterative Bronchiolitis (N=60)

Non Obliterative Bronchiolitis (N=66)

Years

Su

rviv

al (

%)

N at risk = 13

N at risk = 10

p = 0.8740

Analysis includes deceased and living donor transplants. Only patients who were less than 18 years old at the time of retransplant are included.

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsFunctional Status of Surviving Recipients

(Follow-ups: March 2005 – June 2013)

0%

20%

40%

60%

80%

100%

1 Year (N=368) 2 Years (N=308) 3 Years (N=251)

10%

20%

30%

40%

50%

60%

70%

80%

90%

100%

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsRehospitalization Post-transplant of Surviving Recipients

(Follow-ups: April 1994 – June 2013)

0%

20%

40%

60%

80%

100%

Up to 1 Year (N=786) Between 2 and 3

Years (N=489)Between 4 and 5

Years (N=299)

No Hospitalization Hospitalized, Not Rejection/Not Infection

Hospitalized, Rejection Hospitalized, Infection Only

Hospitalized, Rejection + Infection

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsRehospitalization Post-transplant of Surviving Recipients


0%

20%

40%

60%

80%

100%

Up to 1 Year (N=786)

Between 1 and 3 Years (N=440)

Between 3 and 5 Years (N=269)

No Hospitalization Hospitalized, Not Rejection/Not Infection

Hospitalized, Rejection Hospitalized, Infection Only

Hospitalized, Rejection + Infection

2014JHLT. 2014 Oct; 33(10): 1025-1033

Induction and Maintenance Immunosuppression

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsInduction Immunosuppression


Any Induction (N = 406) Polyclonal ALG/ATG (N = 87)

IL-2R Antagonist (N = 315)0

10

20

30

40

50

60

70

% o

f p

ati

en

ts

Analysis is limited to patients who were alive at the time of the discharge.2014

JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsInduction Immunosuppression


Analysis is limited to patients who were alive at the time of the discharge.

20012003

20052007

20092011

1/13-6/13

20012003

20052007

20092011

1/13-6/13

20012003

20052007

20092011

1/13-6/13

0

10

20

30

40

50

60

70

80

% o

f p

ati

en

ts

Any Induction Polyclonal ALG/ATG IL-2R Antagonist

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsKaplan-Meier Survival Stratified by Induction Use


0 1 2 3 4 5 6 70

25

50

75

100

Induction (N = 370)

No Induction (N = 227)

Years

Su

rviv

al (

%)

Median survival (years):Induction = 6.5No Induction = 6.1

p=0.8870

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsMaintenance Immunosuppression at Time of Follow-up

(Follow-ups: January 2001 – June 2013)

Cy-closporine

Tacrolimus Sirolimus/Everolimus

MMF/MPA Azathioprine Prednisone0

20

40

60

80

100Year 1 (N = 495) Year 5 (N = 230)

% o

f p

ati

en

ts

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

Analysis is limited to patients who were alive at the time of the follow-up.

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsMaintenance Immunosuppression at Time of Follow-up

(Follow-ups: January 2001 – June 2013)

Calcineurin Inhibitor

CellCycle Prednisone Calcineurin Inhibitor

CellCycle Prednisone0

20

40

60

80

100

% o

f P

ati

en

ts


Tac

CyA

Tac

CyAAZA AZA

MMF/MPA

MMF/MPA

1 Year Follow-up (N = 495) 5 Year Follow-up (N = 230)


NOTE: 0.2% of patients were on both calcineurin inhibitors at different point during the 1-year; these patients are not counted in either group. And no patients were on neither drug during the 1-year. In the 5-year tabulations, 0.4% were reported to be on both drugs during the year and 0.9% (2 patients) was reported to be on neither drugs.

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung Transplants Maintenance Immunosuppression Drug Combinations at

Time of Follow-up (Follow-ups: January 2001 – June 2013)

0%

20%

40%

60%

80%

100%

Year 1 (N = 495) Year 5 (N = 230)

Other

Tacrolimus + Sirolimus/Everolimus

Tacrolimus

Tacrolimus + MMF/MPA

Tacrolimus + AZA

Cyclosporine + MMF/MPA

Cyclosporine + AZA

% o

f P

ati

en

ts



2014JHLT. 2014 Oct; 33(10): 1025-1033

Post Transplant Morbidities

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsCumulative Morbidity Rates in Survivors within 1 Year

Post-Transplant (Follow-ups: April 1994 – June 2013)

Outcome Within 1 Year

Total number with known response

Hypertension 40.9% (N = 727)

Renal Dysfunction 9.4% (N = 756)

Abnormal Creatinine ≤ 2.5 mg/dl 6.3%

Creatinine > 2.5 mg/dl 2.0%

Chronic Dialysis 0.8%

Renal Transplant 0.3%

Hyperlipidemia 5.0% (N = 746)

Diabetes 22.2% (N = 757)

Bronchiolitis Obliterans Syndrome 12.9% (N = 700)

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsCumulative Morbidity Rates in Survivors within 1 Year Post-

Transplant by Induction Use (Transplants: January 2000 – June 2012)

Outcome

Induction No Induction

Within 1 Year


Within 1 Year


Hypertension 43.6% (N = 319) 43.1% (N = 181)

Renal Dysfunction 10.9% (N = 330) 9.7% (N = 196)

Abnormal Creatinine ≤ 2.5 mg/dl 8.2% 6.6%

Creatinine > 2.5 mg/dl 2.1% 2.0%

Chronic Dialysis 0.6% 0.5%

Renal Transplant 0% 0.5%

Hyperlipidemia 5.2% (N = 328) 9.8% (N = 183)

Diabetes 22.1% (N = 330) 26.9% (N = 197)

Bronchiolitis Obliterans Syndrome 12.2% (N = 311) 9.9% (N = 181)

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsCumulative Morbidity Rates in Survivors within 5 Years

Post-Transplant (Follow-ups: April 1994 – June 2013)

Outcome Within 5 Years


Hypertension 69.0% (N = 210)






Hyperlipidemia 17.8% (N = 214)

Diabetes 35.7% (N = 224)


2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsCumulative Morbidity Rates in Survivors within 5 Years Post-

Transplant by Induction Use (Transplants: January 2000 – June 2008)

Outcome


Within 5 Years


Within 5 Years


Hypertension 75.0% (N = 68) 61.7% (N = 60)

Renal Dysfunction 30.3% (N = 76) 35.4% (N = 65)

Abnormal Creatinine ≤ 2.5 mg/dl 28.9% 29.2%

Creatinine > 2.5 mg/dl 1.3% 1.5%

Chronic Dialysis 0% 4.6%

Renal Transplant 0% 0%

Hyperlipidemia 29.6% (N = 71) 20.7% (N = 58)

Diabetes 41.6% (N = 77) 35.9% (N = 64)

Bronchiolitis Obliterans Syndrome 30.2% (N = 63) 46.0% (N = 50)

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsCumulative Morbidity Rates in Survivors within 7 Years Post-

Transplant (Follow-ups: April 1994 – June 2013)

Outcome Within 7 Years








2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsFreedom from Bronchiolitis Obliterans Syndrome


0 1 2 3 4 5 6 7 8 90

10

20

30

40

50

60

70

80

90

100

Years

% F

ree

fro

m B

ron

ch

iolit

is O

blit

-e

ran

s S

yn

dro

me

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung Transplants Freedom from Bronchiolitis Obliterans Syndrome

by Age Group (Follow-ups: April 1994 – June 2013)

0 1 2 3 4 5 6 7 80

10

20

30

40

50

60

70

80

90

100

<1 year (N=60)

1-5 Years (N=75)

6-10 Years (N=121)

11-17 years (N=448)

Years

% F

ree

fro

m B

ron

ch

iolit

is O

blit

-e

ran

s S

yn

dro

me


2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsFreedom from Bronchiolitis Obliterans Syndrome

by Diagnosis (Follow-ups: April 1994 – June 2013)

0 1 2 3 4 5 6 70

10

20

30

40

50

60

70

80

90

100

Cystic Fibrosis (N=377)

IPAH (N=65)

Years

% F

ree

fro

m B

ron

ch

iolit

is O

blit

-e

ran

s S

yn

dro

me

p = 0.0712

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsFreedom from Bronchiolitis Obliterans Syndromeby Induction Use (Follow-ups: April 1994 – June 2013)

0 1 2 3 4 5 6 70

10

20

30

40

50

60

70

80

90

100

Induction (N = 328)

No Induction (N = 365)

Years

% F

ree

fro

m B

ron

ch

iolit

is O

blit

-e

ran

s S

yn

dro

me

p=0.2021

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsFreedom from Severe Renal Dysfunction*


0 1 2 3 4 5 6 7 8 9 1050

60

70

80

90

100

Years

% F

ree

from

Sev

ere

Ren

al D

ysfu

nct

ion

*Severe renal dysfunction = Creatinine > 2.5 mg/dl (221 μmol/L), dialysis or renal transplant

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsCumulative Post-Transplant Malignancy Rates in Survivors


Malignancy/Type 1-Year Survivors

5-Year Survivors

7-Year Survivors

No Malignancy 729 (94.7%) 208 (89.3%) 118 (90.8%)

Malignancy (all types combined) 41 (5.3%) 25 (10.7%) 12 (9.2%)

Malignancy Type*

Lymphoma 38 25 12

Other 2 1 0

Type Not Reported 1 0 0

* Recipients may have experienced more than one type of malignancy so sum of individual malignancy types may be greater than total number with malignancy.

“Other” includes liver and primitive neuroectodermal tumor.

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsFreedom from Malignancy (Follow-ups: April 1994 – June 2013)

0 1 2 3 4 5 6 7 8 9 1050

60

70

80

90

100

All malignancy

Lymphoma

Skin

Other

Years

% F

ree

fro

m M

alig

na

nc

y

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsFreedom from Malignancy by Age Group


0 1 2 3 4 5 6 7 8 9 1050

60

70

80

90

100

<1 Year

1-5 years

6-10 years

11-17 years

Years

% F

ree

fro

m M

alig

nan

cy


2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsFreedom from Malignancy by Induction Use


0 1 2 3 4 5 6 750

60

70

80

90

100


Years

% F

ree

fro

m M

alig

nan

cy

p = 0.8595

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsCause of Death (Deaths: January 1992 – June 2013)

CAUSE OF DEATH 0-30 Days (N =126)

31 Days - 1 Year (N=182)

>1 Year - 3 Years (N=234)

>3 Years - 5 Years

(N=112)

>5 Years (N=115)

BRONCHIOLITIS 0 18 (9.9%) 87 (37.2%) 42 (37.5%) 55 (47.8%)

ACUTE REJECTION 3 (2.4%) 4 (2.2%) 3 (1.3%) 3 (2.7%) 0

LYMPHOMA 0 8 (4.4%) 7 (3.0%) 4 (3.6%) 5 (4.3%)

MALIGNANCY, NON-LYMPHOMA 0 2 (1.1%) 1 (0.4%) 0 5 (4.3%)

CMV 0 6 (3.3%) 0 0

INFECTION, NON-CMV 17 (13.5%) 59 (32.4%) 35 (15.0%) 20 (17.9%) 10 (8.7%)

GRAFT FAILURE 35 (27.8%) 33 (18.1%) 59 (25.2%) 26 (23.2%) 22 (19.1%)

CARDIOVASCULAR 21 (16.7%) 8 (4.4%) 4 (1.7%) 1 (0.9%) 1 (0.9%)

TECHNICAL 15 (11.9%) 5 (2.7%) 6 (2.6%) 3 (2.7%) 2 (1.7%)

MULTIPLE ORGAN FAILURE 13 (10.3%) 22 (12.1%) 11 (4.7%) 4 (3.6%) 6 (5.2%)

OTHER 22 (17.5%) 17 (9.3%) 21 (9.0%) 9 (8.0%) 9 (7.8%)

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung TransplantsRelative Incidence of Leading Causes of Death

(Deaths: January 1992 – June 2013)

0-30 days (N=126)

31 days–1 year (N=182)

>1 year–3 years (N=234)

>3 years–5 years (N=112)

>5 years (N=115)

0

10

20

30

40

50BronchiolitisInfection (non-CMV)Graft FailureCardiovascularMultiple Organ Failure

% o

f D

ea

ths

2014JHLT. 2014 Oct; 33(10): 1025-1033

Multivariable Analyses

2014JHLT. 2014 Oct; 33(10): 1025-1033

Pediatric Lung Transplants (January 1995 – June 2012)

Risk Factors For 1 Year Mortality/Graft Failure

N = 8522014

* Retransplant includes those with a retransplant diagnosis or a previous transplant was reported. JHLT. 2014 Oct; 33(10): 1025-1033



Continuous Factors (see figure)

Recipient age

2014JHLT. 2014 Oct; 33(10): 1025-1033


Risk Factors For 1 Year Mortality/Graft FailureRecipient Age

0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 170.0

0.5

1.0

1.5

2.0

2.5

3.0

Recipient Age

Ha

zard

Ra

tio

of

1 Y

ea

r M

ort

alit

y/G

raft

Fa

ilure

p = 0.0210

2014JHLT. 2014 Oct; 33(10): 1025-1033



N = 6462014

JHLT. 2014 Oct; 33(10): 1025-1033



Continuous Factors (see figures)

Recipient age

Pediatric transplant center volume

2014JHLT. 2014 Oct; 33(10): 1025-1033


Risk Factors For 5 Year Mortality/Graft FailureRecipient Age

0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 170.0

0.5

1.0

1.5

2.0

Recipient Age

Ha

zard

Ra

tio

of

5 Y

ea

r M

ort

alit

y/G

raft

Fa

ilure

p = 0.0095

2014JHLT. 2014 Oct; 33(10): 1025-1033


Risk Factors For 5 Year Mortality/Graft FailureCenter Volume Pediatric Transplants

0 1 2 3 4 5 6 7 8 9 100.0

0.5

1.0

1.5

2.0

Center Volume (cases per year)

Ha

zard

Ra

tio

of

5 Y

ea

r M

ort

alit

y/G

raft

Fa

ilure

p = 0.0051

2014JHLT. 2014 Oct; 33(10): 1025-1033



N = 4412014

NOTE: No continuous factors were significant.JHLT. 2014 Oct; 33(10): 1025-1033

lung transplantation

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