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Transcript 
LUNG TRANSPLANTATION
Pediatric Recipients
ISHLT
2002
J Heart Lung Transplant 2002; 21: 827-840.
AGE DISTRIBUTION OF PEDIATRIC
LUNG RECIPIENTS
Number of Transplants
100
90
80
70
60
50
40
30
20
10
0
0
1
2
ISHLT
3
4
5
6
7
8
9 10 11 12 13 14 15 16 17
Recipient Age (Years)
2002
J Heart Lung Transplant 2002; 21: 827-840.
AGE DISTRIBUTION FOR DONORS OF
PEDIATRIC LUNG RECIPIENTS (1986-2001)
Number of Transplants
240
210
180
150
120
90
60
30
0
0
1
2
ISHLT
3
4
5
6
7
8
9
10 11
Donor Age (Years)
2002
J Heart Lung Transplant 2002; 21: 827-840.
12 13
14 15
16 17 18+
AGE DISTRIBUTION OF
PEDIATRIC LUNG RECIPIENTS
By Year of Transplant
11-17 Years
1-10 Years
<1 Year
80
60
85 86
82
72
47
50
66 67
57
45
42
40
20
20
1
3
4
6
0
19
86
19
87
19
88
19
89
19
90
19
91
19
92
19
93
19
94
19
95
19
96
19
97
19
98
19
99
20
00
20
01
*
Number of Transplants
100
ISHLT
2002
J Heart Lung Transplant 2002; 21: 827-840.
* Numbers may be low
due to delayed reporting.
40
30
20
10
Transplant Year
ISHLT
2002
J Heart Lung Transplant 2002; 21: 827-840.
* Numbers may be low due
to delayed reporting.
2001*
2000
1999
1998
1997
1996
1995
1994
1993
1992
1991
1990
1989
1988
1987
0
1986
Number of Pediatric Centers
Reporting
NUMBER OF CENTERS REPORTING
PEDIATRIC LUNG TRANSPLANTS
PEDIATRIC LUNG TRANSPLANTATION:
Indications (1991-2001)
DIAGNOSIS
AGE: < 1 Year
Cystic Fibrosis
PPH
Congenital Heart Disease
AGE: 1-10 Years
55
34.2%
7
16.7%
24
14.9%
14
33.3%
17
10.6%
12
7.5%
IPF
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%
17
10.6%
Other
11
ISHLT
2002
J Heart Lung Transplant 2002; 21: 827-840.
26.2%
PEDIATRIC LUNG TRANSPLANTATION
Indications by Age (1991-2001)
17%
Congenital Heart Disease
Idiopathic Pulmonary Fibrosis
Cystic Fibrosis
Other
Primary Pulmonary Hypertension
Pulmonary Vascular Disease
Retransplant/Graft Failure
7%
29%
14%
16%
13%
11%
4%
8%
11%
33%
Age <1 Year
37%
Age 1-10 Years
ISHLT
2002
J Heart Lung Transplant 2002; 21: 827-840.
DIAGNOSIS IN PEDIATRIC LUNG RECIPIENTS
Age: 11-17 Years
3%
13%
Congenital Heat Disease
Cystic Fibrosis
IPF
9%
66%
6%
3%
Other
PPH
ReTX
% of Cases
100
75
50
PPH
Cystic Fibrosis
IPF
25
0
1990
1991
1992
ISHLT
1993
1994
1995
2002
J Heart Lung Transplant 2002; 21: 827-840.
1996
1997
1998
1999
2000
2001
PEDIATRIC LUNG TRANSPLANTATION
Actuarial Survival by Procedure Type (1990-2001)
100
Survival (%)
Single Lung
(N=70)
Bilateral/Double Lung (N=567)
75
50
P = 0.006
25
0
0
1
ISHLT
2
3
4
5
6
7
Years Post Transplantation
2002
J Heart Lung Transplant 2002; 21: 827-840.
8
9
10
PEDIATRIC LUNG TRANSPLANTATION
Actuarial Survival by Age Group (1990-2001)
100
<1 Year
(N=44)
Survival (%)
1-10 Years (N=172)
75
1-11 Years (N=421)
50
25
<1 Year: ½-life = 3.5 Years
1-10 Years: ½-life = 3.5 Years
11-17 Years: ½-life = 3.2 Years
0
0
1
ISHLT
2
3
4
5
6
7
Years Post Transplantation
2002
J Heart Lung Transplant 2002; 21: 827-840.
8
9
10
PEDIATRIC LUNG TRANSPLANTATION
Conditional Actuarial Survival by Age Group (1990-2001)
Survival (%)
100
75
50
<1 Year
25
(N=26)
1-10 Years (N=98)
<1 Year: ½-life = 7.1 Years
1-10 Years: ½-life = 5.9 Years
11-17 Years: ½-life = 5.4 Years
1-11 Years (N=222)
0
0
1
ISHLT
2
3
4
5
6
7
Years Post Transplantation
2002
J Heart Lung Transplant 2002; 21: 827-840.
8
9
10
PEDIATRIC LUNG TRANSPLANTATION
Actuarial Survival by Era (1988-2001)
100
Survival (%)
1988-1992 (N=127)
80
1993-1997 (N=326)
1998-2001 (N=276)
60
40
20
0
0
1
2
3
4
5
6
7
Years Post-Transplantation
ISHLT
2002
J Heart Lung Transplant 2002; 21: 827-840.
8
9
10
PEDIATRIC LUNG TRANSPLANTATION
Actuarial Survival by Procedure Type
Diagnosis: PPH (1990-2001)
100
Survival (%)
Single Lung (N = 17)
Double Lung (N=52)
75
50
P = 0.002
25
0
0
ISHLT
1
2
3
4
Years Post-Transplantation
2002
J Heart Lung Transplant 2002; 21: 827-840.
5
6
PEDIATRIC LUNG RECIPIENTS
Functional Status (April 1994-December 2001)
100%
80%
60%
40%
20%
No Activity Limitations
Performs with Assistance
Total Assistance
0%
1 Year (N = 263)
ISHLT
3 Years (N = 156)
2002
J Heart Lung Transplant 2002; 21: 827-840.
5 Years (N = 82)
PEDIATRIC LUNG RECIPIENTS
Rehospitalization Post-transplant
(April 1994 - December 2001)
100%
80%
60%
40%
20%
0%
No Hospitalization
Hospitalized, Rejection
Hospitalized, Rejection + Infection
Up to 1 Year
(N = 299)
ISHLT
Hospitalized, Not Rejection/Not Infection
Hospitalized, Infection Only
Between 2 and 3 Years
(N = 170)
2002
J Heart Lung Transplant 2002; 21: 827-840.
Between 4 and 5 Years
(N = 87)
PEDIATRIC LUNG RECIPIENTS
Induction Immunosuppression
50
For follow-ups between October 1999 and December 2001
% of patients
40
30
20
10
0
Any Induction
(N = 48)
ISHLT
Polyclonal ATG/ALG
(N = 16)
2002
J Heart Lung Transplant 2002; 21: 827-840.
OKT3 (N = 1)
IL2-R Antagonist
(N = 32)
PEDIATRIC LUNG RECIPIENTS
Maintenance Immunosuppression at Any Time During Follow-up Period
For follow-ups between October 1999 and December 2001
100
% of Patients
80
Year 1
(N = 78)
Year 5
(N = 41)
60
40
20
0
Cyclosporine
ISHLT
Tacrolimus
Rapamycin
2002
J Heart Lung Transplant 2002; 21: 827-840.
MMF
Azathioprine
Prednisone
PEDIATRIC LUNG RECIPIENTS
Maintenance Immunosuppression at Time of Follow-up
For follow-ups between October 1999 and December 2001
100
Year 1 (N = 76)
Year 5
(N = 39)
% of Patients
80
60
40
20
0
Cyclosporine
ISHLT
Tacrolimus
Rapamycin
2002
J Heart Lung Transplant 2002; 21: 827-840.
MMF
Azathioprine
Prednisone
PEDIATRIC LUNG RECIPIENTS
Maintenance Immunosuppression at Time of Follow-up
For follow-ups between October 1999 and December 2001
100
% of Patients
80
Tac
60
Tac
MMF
MMF
40
20
CyA
CyA
AZA
AZA
0
Calcineurin
Inhibitor
CellCycle
Prednisone
1 Year Follow-up (N = 76)
ISHLT
2002
J Heart Lung Transplant 2002; 21: 827-840.
Calcineurin
Inhibitor
CellCycle
Prednisone
5 Year Follow-up (N = 39)
PEDIATRIC LUNG RECIPIENTS
Maintenance Immunosuppression at Time of Follow-up Report
For follow-ups between October 1999 and December 2001
100%
None Given
80%
% of Patients
Other
60%
Tacrolimus + MMF
Tacrolimus + AZA
40%
Cyclosporine + MMF
20%
Cyclosporine + AZA
0%
Year 1 (N = 76)
ISHLT
Year 5 (N = 39)
2002
J Heart Lung Transplant 2002; 21: 827-840.
POST-LUNG TRANSPLANT MORBIDITY
For Pediatric Recipients (April 1994-December 2000)
Outcome
Hypertension
Renal Function
Normal
Renal Dysfunction
Creatinine>2.5 mg/dl
Chronic Dialysis
Renal Transplant
By 1 Year
By 5 Years
36.2% (N=298)
71.1% (N=45)
(N=300)
92.3%
5.0%
1.3%
1.0%
(N=46)
73.9%
10.9%
8.7%
4.3%
0.3%
2.2%
Hyperlipidemia
0.6% (N=308)
4.3%
Diabetes
19.8% (N=298)
28.3% (N=46)
Bronchiolitis Obliterans
14.2% (N=267)
14.8% (N=27)
ISHLT
2002
J Heart Lung Transplant 2002; 21: 827-840.
(N=46)
Freedom from Bronchiolitis Obliterans
% Free from Bronchiolitis
Obliterans
For Pediatric Lung Recipients (April 1994-December 2000)
100
90
80
70
60
50
40
0
12
24
36
Months
ISHLT
2002
J Heart Lung Transplant 2002; 21: 827-840.
48
60
Freedom from Renal Dysfunction*
% Freedom from Renal
Dysfunction
For Pediatric Lung Recipients (April 1994-December 2000)
100
90
80
70
* Renal dysfunction = Creatinine > 2.5
mg/dl, dialysis or renal transplant
60
50
0
12
24
36
Months
ISHLT
2002
J Heart Lung Transplant 2002; 21: 827-840.
48
60
MALIGNANCY POST-LUNG TRANSPLANTATION FOR PEDIATRICS
Cumulative Incidence for Survivors (April 1994-December 2001)
Malignancy/Type
1-Year Survivors
No Malignancy
288
(93.5%) 45
(91.8%)
Malignancy (all types combined)
20
(6.5%) 4
(8.2%)
Malignancy
Type
ISHLT
Lymph
18
Other
2
2002
J Heart Lung Transplant 2002; 21: 827-840.
5-Year Survivors
4
Freedom from Malignancy
% Free from Malignancy
For Pediatric Lung Recipients (April 1994-December 2000)
100
90
80
70
60
All malignancy
Lymph
Skin
Other
50
0
12
24
36
Months
ISHLT
2002
J Heart Lung Transplant 2002; 21: 827-840.
48
60
PEDIATRIC LUNG TRANSPLANTATION
Actuarial Survival Based on Rejection within 1st Year
(1994-2000)
100
Survival (%)
P= 0.01
80
60
40
Rejection within 1st Year (N = 142)
20
No Rejection within 1st Year (N = 101)
0
0
1
2
3
4
5
Years Post-Transplantation
ISHLT
2002
J Heart Lung Transplant 2002; 21: 827-840.
6
7
PEDIATRIC LUNG RECIPIENTS
Relationship 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
ISHLT
2002
J Heart Lung Transplant 2002; 21: 827-840.
**p < 0.0001
PEDIATRIC LUNG RECIPIENTS:
Time Of Death (1988-2001)
100%
% of Deaths
80%
60%
40%
0-30 Days
31 Days -1 Year
1- 3 Years
3 - 5 Years
5+ Years
20%
0%
<1
1-10
Recipient Age (years)
ISHLT
2002
J Heart Lung Transplant 2002; 21: 827-840.
11-17
PEDIATRIC LUNG TRANSPLANT RECIPIENTS:
Cause of Death (1982-2001)
100%
Percentage of Deaths
Other
80%
Technical
Graft Failure - Other
60%
Primary Graft Failure
Bronchiolitis
Infection, Non-CMV
40%
Acute Rejection
Lymphoma
20%
Cardiac
0%
0-30 Days
(N = 45)
ISHLT
31 Days - 1 Year
>1 Year - 3
(N=71)
Years (N = 66)
>3 Years - 5
Years (N = 27)
Timing of Death
2002
J Heart Lung Transplant 2002; 21: 827-840.
>5 Years
(N = 8)
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