LUNG TRANSPLANTATION Pediatric Recipients 2015 JHLT 2015 Oct

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LUNG TRANSPLANTATION Pediatric Recipients 2015 JHLT. 2015 Oct; 34(10): 1255 -1263

LUNG TRANSPLANTATION Pediatric Recipients 2015 JHLT. 2015 Oct; 34(10): 1255 -1263

Table of Contents < Donor, recipient and center characteristics: slides 3 -18 < Post

Table of Contents < Donor, recipient and center characteristics: slides 3 -18 < Post transplant – survival and other outcomes: slides 19 -33 < Induction and maintenance immunosuppression: slides 34 -40 < Post transplant morbidities: slides 41 -58 < Multivariable analyses: slides 59 -69 < Early Graft Failure (EGF): slides 70 -86 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033

Donor, Recipient and Center Characteristics 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263

Donor, Recipient and Center Characteristics 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033

Pediatric Lung Transplants Recipient Age Distribution – Number (Transplants: January 1988 – June 2014)

Pediatric Lung Transplants Recipient Age Distribution – Number (Transplants: January 1988 – June 2014) 800 Number of Transplants 700 <1 1 -5 6 -10 11 -17 600 500 400 300 200 100 0 1988 -1999 (N=666) 2000 -2007 (N=691) 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 2008 -6/2014 (N=730) Analysis includes deceased and living donor transplants.

Pediatric Lung Transplants Recipient Age Distribution – Percentage (Transplants: January 1988 – June 2014)

Pediatric Lung Transplants Recipient Age Distribution – Percentage (Transplants: January 1988 – June 2014) 100% 90% 11 -17 % of Transplants 80% 70% 6 -10 60% 50% 1 -5 40% 30% 20% <1 10% 0% 1988 -1999 (N=666) 2000 -2007 (N=691) 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 2008 -6/2014 (N=730) Analysis includes deceased and living donor transplants.

Pediatric Lung Transplants Donor Type Distribution by Transplant Year (Transplants: January 1986 – December

Pediatric Lung Transplants Donor Type Distribution by Transplant Year (Transplants: January 1986 – December 2013) 120 Living Deceased 100 80 60 40 20 0 19 8 19 6 8 19 7 8 19 8 8 19 9 9 19 0 9 19 1 9 19 2 9 19 3 9 19 4 9 19 5 9 19 6 9 19 7 9 19 8 9 20 9 0 20 0 0 20 1 0 20 2 0 20 3 0 20 4 0 20 5 0 20 6 0 20 7 0 20 8 0 20 9 1 20 0 1 20 1 1 20 2 13 Number of Transplants 140 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 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. Analysis includes deceased and living donor transplants.

Pediatric Lung Transplants Donor Type Distribution by Recipient Age Group Within Era (Transplants: January

Pediatric Lung Transplants Donor Type Distribution by Recipient Age Group Within Era (Transplants: January 1988 – June 2014) Number of Transplants 600 Living 500 Deceased 400 300 200 100 0 <1 1 -5 6 -10 11 -17 <1 1988 -1999 1 -5 6 -10 11 -17 2000 -2007 <1 1 -5 6 -10 11 -17 2008 -6/2014 Recipient Age (Years) 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 Analysis includes deceased and living donor transplants.

Pediatric Lung Transplants Donor Age Distribution (Transplants: January 1988 – June 2014) 100% 90%

Pediatric Lung Transplants Donor Age Distribution (Transplants: January 1988 – June 2014) 100% 90% % of Transplants 80% 1 13 59 85 96 119 70% 60% 4 42 14 53 119 112 121 50% 173 193 30% 18 - 34 years 11 - 17 years 280 229 227 2000 -2007 (N=652) 2008 -6/2014 (N=718) 10% 0% 1988 -1999 (N=570) 50 - 59 years 35 - 49 years 40% 20% 60+ years 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 0 - 10 years

Pediatric Lung Transplants Donor and Recipient Age (Transplants: January 2008 – June 2014) Donor

Pediatric Lung Transplants Donor and Recipient Age (Transplants: January 2008 – June 2014) Donor Age: 0 -10 years 11 -17 years 18 -34 years 35 -49 years 50 -59 years 60+ years 100% % of Transplants 80% 60% 40% 20% 0% <1 year 1 - 5 years 6 - 10 years Recipient Age 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 11 - 17 years

Pediatric Lung Transplants Recipient Age Distribution by Year of Transplant 140 127 126 11

Pediatric Lung Transplants Recipient Age Distribution by Year of Transplant 140 127 126 11 -17 years Number of Transplants 120 114 6 -10 years 1 -5 years 100 124 94 96 95 <1 year 80 80 60 46 48 48 96 89 72 73 72 74 101 107 106 95 79 52 40 23 20 3 5 7 19 8 19 6 8 19 7 8 19 8 8 19 9 9 19 0 9 19 1 9 19 2 9 19 3 9 19 4 9 19 5 9 19 6 9 19 7 9 19 8 9 20 9 0 20 0 0 20 1 0 20 2 0 20 3 0 20 4 0 20 5 0 20 6 0 20 7 0 20 8 0 20 9 1 20 0 1 20 1 1 20 2 13 0 1 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. 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 Analysis includes deceased and living donor transplants.

Pediatric Lung Retransplants by Year of Retransplant 10 20 18 8 16 7 14

Pediatric Lung Retransplants by Year of Retransplant 10 20 18 8 16 7 14 6 12 5 10 4 8 3 6 2 4 1 2 0 0 19 8 19 6 8 19 7 8 19 8 8 19 9 9 19 0 9 19 1 9 19 2 9 19 3 9 19 4 9 19 5 9 19 6 9 19 7 9 19 8 9 20 9 0 20 0 0 20 1 0 20 2 0 20 3 0 20 4 0 20 5 0 20 6 0 20 7 0 20 8 0 20 9 1 20 0 1 20 1 1 20 2 13 Number of retransplants % % of retransplants N 9 Year of retransplant 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 Only patients who were less than 18 years old at the time of retransplant are included.

Pediatric Lung Transplants Number of Centers Reporting Transplants by Location (Transplants: January 1986 –

Pediatric Lung Transplants Number of Centers Reporting Transplants by Location (Transplants: January 1986 – December 2013) 50 Others 45 North America 40 Europe 35 30 25 20 15 10 5 0 19 8 19 6 8 19 7 8 19 8 8 19 9 9 19 0 9 19 1 9 19 2 9 19 3 9 19 4 9 19 5 9 19 6 9 19 7 9 19 8 9 20 9 0 20 0 0 20 1 0 20 2 0 20 3 0 20 4 0 20 5 0 20 6 0 20 7 0 20 8 0 20 9 1 20 0 1 20 1 1 20 2 13 Number of Centers 55 Transplant Year 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 Analysis includes deceased and living donor transplants.

Pediatric Lung Transplants 55 50 40 20+ transplants 10 -19 transplants 5 -9 transplants

Pediatric Lung Transplants 55 50 40 20+ transplants 10 -19 transplants 5 -9 transplants 1 -4 transplants 35 30 25 20 15 10 5 0 19 8 19 6 8 19 7 8 19 8 8 19 9 9 19 0 9 19 1 9 19 2 9 19 3 9 19 4 9 19 5 9 19 6 9 19 7 9 19 8 9 20 9 0 20 0 0 20 1 0 20 2 0 20 3 0 20 4 0 20 5 0 20 6 0 20 7 0 20 8 0 20 9 1 20 0 1 20 1 1 20 2 13 Number of Centers 45 Number of Centers Reporting Transplants by Pediatric Center Volume Transplant Year 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 Analysis includes deceased and living donor transplants.

Pediatric Lung Transplants Number of Transplants by Pediatric Center Volume 140 20+ transplants 10

Pediatric Lung Transplants Number of Transplants by Pediatric Center Volume 140 20+ transplants 10 -19 transplants 5 -9 transplants 100 1 -4 transplants 80 60 40 20 0 19 8 19 6 8 19 7 8 19 8 8 19 9 9 19 0 9 19 1 9 19 2 9 19 3 9 19 4 9 19 5 9 19 6 9 19 7 9 19 8 9 20 9 0 20 0 0 20 1 0 20 2 0 20 3 0 20 4 0 20 5 0 20 6 0 20 7 0 20 8 0 20 9 1 20 0 1 20 1 1 20 2 13 Number of Transplants 120 Transplant Year 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 Analysis includes deceased and living donor transplants.

Pediatric Lung Transplants Indications by Age Group (Transplants: January 2000 – June 2014) Diagnosis

Pediatric Lung Transplants Indications by Age Group (Transplants: January 2000 – June 2014) Diagnosis < 1 Year 1 -5 Years 6 -10 Years 11 -17 Years Cystic Fibrosis 0 5 5. 7% 99 50. 5% 726 69. 1% Idiopathic Pulmonary Arterial Hypertension 7 19 21. 8% 20 10. 2% 83 7. 9% Retransplant: Obliterative Bronchiolitis 0 4 4. 6% 6 3. 1% 33 3. 1% Congenital Heart Disease 8 14. 8% 7 8. 0% 3 1. 5% 8 0. 8% Idiopathic Pulmonary Fibrosis 4 7. 4% 11 12. 6% 8 4. 1% 29 2. 8% Obliterative Bronchiolitis, Not Retx 0 8 9. 2% 21 10. 7% 48 4. 6% Retransplant, Not OB 0 4 4. 6% 3 1. 5% 24 2. 3% Interstitial Pneumonitis 0 2 2. 3% 2 1. 0% 1 0. 1% Pulmonary Vascular Disease 2 5 5. 7% 2 1. 0% 1 0. 1% Eisenmenger’s Syndrome 0 1 1. 1% 1 0. 5% 4 0. 4% Pulmonary Fibrosis, Other 7 13. 0% 10 11. 5% 15 7. 7% 28 2. 7% Surfactant Protein B Deficiency 11 20. 4% 4 4. 6% 0 COPD/Emphysema 0 Bronchopulmonary Dysplasia 4 Bronchiectasis 0 Other 11 13. 0% 3. 7% 0 7. 4% 2 2. 3% 0 20. 4% 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 5 0 1 0. 5% 6 0. 6% 3 1. 5% 3 0. 3% 14 1. 3% 43 4. 1% 0 5. 7% 12 6. 1% Analysis includes deceased and living donor transplants. For some retransplants, a diagnosis other than retransplant is reported, so the total percentage of retransplants may be greater.

Pediatric Lung Transplants Age Distribution by Location (Transplants: January 2008 – June 2014) <1

Pediatric Lung Transplants Age Distribution by Location (Transplants: January 2008 – June 2014) <1 year 1 - 5 years 6 - 10 years 11 - 17 years 100% % of Transplants 80% 60% 40% 20% 0% Europe (N=324) North America (N=353) 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 Other (N=53) Analysis includes deceased and living donor transplants.

Pediatric Lung Transplants Diagnosis Distribution by Location (Transplants: January 2008 – June 2014) Cystic

Pediatric Lung Transplants Diagnosis Distribution by Location (Transplants: January 2008 – June 2014) Cystic Fibrosis IPAH IPF OB Other Congenital heart disease Retx % of Transplants 100% 80% 60% 40% 20% 0% Europe (N=311) North America (N=351) For some retransplants, a diagnosis other than retransplant is reported, so the total percentage of retransplants may be greater. 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 Other (N=52) Total number of transplants reported: Europe = 324, North America = 353, Other = 53 NOTE: Unknown diagnoses were excluded from this tabulation. Analysis includes deceased and living donor transplants.

Pediatric Lung Transplants Donor Age Distribution by Location (Transplants: January 2008 – June 2014)

Pediatric Lung Transplants Donor Age Distribution by Location (Transplants: January 2008 – June 2014) 0 - 10 years 11 - 17 years 18 - 34 years 35 - 49 years 50 - 59 years 60+ years 100% % of Donors 80% 60% 40% 20% 0% Europe (N=321) North America (N=352) NOTE: Transplants with unknown donor age and living donor transplants were excluded from this tabulation. 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 Other (N=45) Total number of transplants reported: Europe = 324, North America = 353, Other = 53

Post Transplant: Survival and Other Outcomes 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255

Post Transplant: Survival and Other Outcomes 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033

Lung Transplants Kaplan-Meier Survival by Recipient Age Group (Transplants: January 1990 – June 2013)

Lung Transplants Kaplan-Meier Survival by Recipient Age Group (Transplants: January 1990 – June 2013) 100 Adult (N=47, 382) Pediatric (N=1, 856) Survival (%) 80 60 p = 0. 1448 40 20 Median survival (years): Adult = 5. 6; Pediatric = 5. 3 0 0 1 2 3 4 5 6 7 8 9 Years 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 10 11 12 13 14 15 16 17 18

Lung Transplants Kaplan-Meier Survival by Recipient Age Group and Procedure Type (Transplants: January 1990

Lung Transplants Kaplan-Meier Survival by Recipient Age Group and Procedure Type (Transplants: January 1990 – June 2013) 100 Adult, Single (N=17, 944) Adult, Bilateral/Double (N=29, 097) Pediatric (N=1, 856) Survival (%) 80 Adult, Bilateral/Double vs. Adult, Single: p<0. 0001 Adult, Bilateral/Double vs. Pediatric: p=0. 0013 Adult, Single vs. Pediatric: p<0. 0001 60 40 Median survival (years): Adult, Single = 4. 4; Adult, Bilateral/Double = 7. 0; Pediatric = 5. 3 20 0 0 1 2 3 4 5 6 7 8 9 Years 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 10 11 12 13 14 15 16 17 18

Pediatric Lung Transplants Kaplan-Meier Survival by Procedure Type (Transplants: January 1990 – June 2013)

Pediatric Lung Transplants Kaplan-Meier Survival by Procedure Type (Transplants: January 1990 – June 2013) 100 Single Lung (N=88) Bilateral/Double Lung (N=1, 762) Survival (%) 80 60 p<0. 0001 40 20 Median survival (years): Single Lung = 2. 2; Bilateral/Double Lung = 5. 6 0 0 1 2 3 4 5 6 7 8 9 Years 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 10 11 12 13 14 15 16 17 18

Pediatric Lung Transplants Kaplan-Meier Survival by Diagnosis (Transplants: January 1990 – June 2013) 100

Pediatric Lung Transplants Kaplan-Meier Survival by Diagnosis (Transplants: January 1990 – June 2013) 100 Cystic Fibrosis (N=1, 049) Non-Cystic Fibrosis (N=743) 80 Survival (%) p=0. 6278 60 40 20 Median survival (years): Cystic Fibrosis = 5. 2; Non-Cystic Fibrosis = 5. 3 0 0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 Years 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033

Pediatric Lung Transplants Kaplan-Meier Survival by Recipient Age Group (Transplants: January 1990 – June

Pediatric Lung Transplants Kaplan-Meier Survival by Recipient Age Group (Transplants: January 1990 – June 2013) 100 Pair-wise comparisons were not significant at p<0. 05. (N=93) 1 -5 years (N=139) 6 -10 years (N=277) 75 Survival (%) <1 Year 11 -17 years (N=1, 347) 50 N at risk = 10 Median survival (years): <1 year = 6. 4 1 -5 years = 7. 2 6 -10 years = 6. 6 11 -17 years = 4. 8 25 N at risk = 10 N at risk = 11 N at risk = 13 0 0 1 2 3 4 5 6 7 8 9 10 Years 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 11 12 13 14 15 16 17 18 19

Pediatric Lung Transplants Conditional Kaplan-Meier Survival by Recipient Age Group (Transplants: January 1990 –

Pediatric Lung Transplants Conditional Kaplan-Meier Survival by Recipient Age Group (Transplants: January 1990 – June 2013) 100 Pair-wise comparisons were not significant at p<0. 05. <1 year (N=63) 1 -5 years (N=99) 6 -10 years (N=212) Survival (%) 75 11 -17 years (N=999) N at risk = 10 50 N at risk = 11 N at risk = 10 Conditional median survival (years): <1 year = 9. 2 1 -5 years = NA 6 -10 years = 9. 7 11 -17 years = 7. 7 25 N at risk = 13 0 0 1 2 3 4 5 6 7 8 Years 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 9 10 11 12 13 14 15 16

Pediatric Lung Transplants Kaplan-Meier Survival by Era (Transplants: January 1988 – June 2013) 100

Pediatric Lung Transplants Kaplan-Meier Survival by Era (Transplants: January 1988 – June 2013) 100 Median survival (years): Unconditional 1988 -1999=3. 3; 2000 -2007=5. 8; 2008 -6/2013=NA Conditional 1988 -1999=7. 1; 2000 -2007=8. 8; 2008 -6/2013=NA Survival (%) 75 1988 -1999 vs. 2000 -2007: p=0. 0004 1988 -1999 vs. 2008 -6/2013: p<0. 0001 2000 -2007 vs. 2008 -6/2013: p=0. 5546 N at risk = 13 50 N at risk = 11 25 1988 -1999 (N=597) 2000 -2007 (N=652) N at risk = 13 2008 -6/2013 (N=619) 0 0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 Years 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033

Pediatric Lung Transplants Kaplan-Meier Survival by Donor Age for Recipients Age 11 -17 Years

Pediatric Lung Transplants Kaplan-Meier Survival by Donor Age for Recipients Age 11 -17 Years (Transplants: January 1990 – June 2013) 100 Median survival (years): 0 -10 years=5. 4 11 -17 years=4. 8 18 -34 years=4. 7 35 -49 years=5. 6 50+ years=3. 9 No pair-wise comparisons were significant at p<0. 05. Survival (%) 75 50 N at risk = 10 0 -10 years (N=252) N at risk = 10 11 -17 years (N=421) 25 N at risk = 13 N at risk = 11 18 -34 years (N=304) N at risk = 10 35 -49 years (N=236) 50+ years (N=116) 0 0 1 2 3 4 5 6 7 8 9 Years 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 10 11 12 13 14 15 16 17

Pediatric Lung Transplants Kaplan-Meier Survival by Donor Type for Recipients Age 11 -17 Years

Pediatric Lung Transplants Kaplan-Meier Survival by Donor Type for Recipients Age 11 -17 Years (Transplants: January 1990 – June 2013) 100 Median survival (years): Deceased = 4. 8 Living = 3. 8 Survival (%) 75 p = 0. 1515 50 N at risk = 13 25 N at risk = 12 Deceased Donor (N=1, 347) Living Donor (N=85) 0 0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 Years 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033

Pediatric Lung Retransplants (Retransplants: January 2000 – June 2014) Number of Retransplants 35 30

Pediatric Lung Retransplants (Retransplants: January 2000 – June 2014) Number of Retransplants 35 30 25 20 15 10 5 0 0 - <1 month 1 - <12 months 12 - <36 months 36+ months Not Reported Time Between Previous and Current Transplant 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 Analysis includes deceased and living donor transplants.

Pediatric Lung Transplants Kaplan-Meier Survival by Transplant Type (Transplants: January 2000 – June 2013)

Pediatric Lung Transplants Kaplan-Meier Survival by Transplant Type (Transplants: January 2000 – June 2013) 100 Retransplant (N = 65) Primary (N = 1, 206) 80 Survival (%) p<0. 0001 60 N at risk = 50 40 N at risk = 10 20 0 0 1 2 3 4 5 Years 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 6 7 8 9 10

Pediatric Lung Transplants Functional Status of Surviving Recipients (Follow-ups: January 2008 – June 2014)

Pediatric Lung Transplants Functional Status of Surviving Recipients (Follow-ups: January 2008 – June 2014) 100% 10% 20% 30% 40% 50% 60% 70% 80% 90% 100% 80% 60% 40% 20% 0% 1 Year (N=282) 2 Years (N=243) 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 3 Years (N=205)

Pediatric Lung Transplants Rehospitalization Post-transplant of Surviving Recipients (Follow-ups: January 2008 – June 2014)

Pediatric Lung Transplants Rehospitalization Post-transplant of Surviving Recipients (Follow-ups: January 2008 – June 2014) 100% 80% 60% 40% 20% No Hospitalization Hospitalized, Not Rejection/Not Infection Hospitalized, Rejection Hospitalized, Infection Only Hospitalized, Rejection + Infection 0% Up to 1 Year (N=315) Between 2 and 3 Years (N=227) 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 Between 4 and 5 Years (N=156)

Pediatric Lung Transplants Rehospitalization Post-transplant of Surviving Recipients (Follow-ups: January 2008 – June 2014)

Pediatric Lung Transplants Rehospitalization Post-transplant of Surviving Recipients (Follow-ups: January 2008 – June 2014) 100% 80% 60% 40% 20% No Hospitalization Hospitalized, Not Rejection/Not Infection Hospitalized, Rejection Hospitalized, Infection Only Hospitalized, Rejection + Infection 0% Up to 1 Year (N=315) Between 1 and 3 Years (N=212) 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 Between 3 and 5 Years (N=139)

Induction and Maintenance Immunosuppression 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025

Induction and Maintenance Immunosuppression 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033

Pediatric Lung Transplants 90 Induction Immunosuppression (Transplants: January 2008 – June 2014) 80 %

Pediatric Lung Transplants 90 Induction Immunosuppression (Transplants: January 2008 – June 2014) 80 % of patients 70 60 50 40 30 20 10 0 Any Induction (N = 244) Polyclonal ALG/ATG (N = 48) 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 IL-2 R Antagonist (N = 197) Analysis is limited to patients who were alive at the time of the discharge.

Pediatric Lung Transplants Induction Immunosuppression (Transplants: January 2001 – June 2014) 90 80 %

Pediatric Lung Transplants Induction Immunosuppression (Transplants: January 2001 – June 2014) 90 80 % of patients 70 60 50 40 30 20 10 0 01 02 03 04 05 06 07 08 09 10 11 12 13 /14 001002003004005006007008009010011012013 /14 20 20 20 204 -6 2 2 2 2 2 2 2 4 -6 1 1 1 / / 1 1/ 1 Any Induction Polyclonal ALG/ATG 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 IL-2 R Antagonist Analysis is limited to patients who were alive at the time of the discharge.

Pediatric Lung Transplants Kaplan-Meier Survival Stratified by Induction Use (Transplants: January 2008 – June

Pediatric Lung Transplants Kaplan-Meier Survival Stratified by Induction Use (Transplants: January 2008 – June 2013) 100 Survival (%) 75 p=0. 9444 50 25 Induction (N = 209) No Induction (N = 92) 0 0 1 2 3 Years 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 4 5

Pediatric Lung Transplants Maintenance Immunosuppression at Time of Follow-up (Follow-ups: January 2008 – June

Pediatric Lung Transplants Maintenance Immunosuppression at Time of Follow-up (Follow-ups: January 2008 – June 2014) 100 Year 1 (N = 277) Year 5 (N = 142) % of patients 80 60 40 20 0 Cyclosporine Tacrolimus Sirolimus/ Everolimus MMF/MPA Azathioprine Prednisone NOTE: Different patients are analyzed in Year 1 and Year 5. 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 Analysis is limited to patients who were alive at the time of the follow-up.

Pediatric Lung Transplants Maintenance Immunosuppression at Time of Follow-up (Follow-ups: January 2008 – June

Pediatric Lung Transplants Maintenance Immunosuppression at Time of Follow-up (Follow-ups: January 2008 – June 2014) 100 % of Patients 80 60 Tac MMF/ MPA Cy. A AZA 40 20 0 Calcineurin Cell. Cycle Prednisone Inhibitor 1 Year Follow-up (N = 277) NOTE: Different patients are analyzed in Year 1 and Year 5. Calcineurin Cell. Cycle Prednisone Inhibitor 5 Year Follow-up (N = 142) NOTE: No patients were on both calcineurin inhibitors at different points during the 1 -year, and two patients were on neither drug during the 1 -year. In the 5 -year tabulations, 1 patient was reported to be on both drugs during the year and none were reported to be on neither drug. 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 Analysis is limited to patients who were alive at the time of the follow-up.

Pediatric Lung Transplants Maintenance Immunosuppression Drug Combinations at Time of Follow-up (Follow-ups: January 2008

Pediatric Lung Transplants Maintenance Immunosuppression Drug Combinations at Time of Follow-up (Follow-ups: January 2008 – June 2014) 100% Other % of Patients 80% Tacrolimus + Sirolimus/Everolimus Tacrolimus 60% Tacrolimus + MMF/MPA 40% Tacrolimus + AZA Cyclosporine + MMF/MPA 20% Cyclosporine + AZA 0% Year 1 (N = 277) Year 5 (N = 142) NOTE: Different patients are analyzed in Year 1 and Year 5. 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 Analysis is limited to patients who were alive at the time of the follow-up.

Post Transplant Morbidities 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033

Post Transplant Morbidities 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033

Pediatric Lung Transplants Cumulative Morbidity Rates in Survivors within 1, 5 and 7 Years

Pediatric Lung Transplants Cumulative Morbidity Rates in Survivors within 1, 5 and 7 Years Post Transplant (Follow-ups: April 1994 – June 2014) Within 1 Year Total N with known response Within 5 Years Total N with known response Within 7 Years Hypertension* 41. 4% (N = 765) 67. 7% (N = 229) – Renal Dysfunction 9. 4% (N = 795) 29. 6% (N = 247) 42. 8% Outcome Abnormal Creatinine ≤ 2. 5 mg/dl 6. 5% 23. 1% 32. 6% Creatinine > 2. 5 mg/dl 1. 9% 4. 0% 6. 5% Chronic Dialysis 0. 8% 1. 6% 0. 7% Renal Transplant 0. 3% 0. 8% 2. 9% Hyperlipidemia* 5. 1% (N = 781) 17. 7% (N = 231) – Diabetes* 21. 3% (N = 797) 35. 2% (N = 250) – Bronchiolitis Obliterans Syndrome 12. 2% (N = 739) 35. 9% (N = 192) 41. 9% 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 996 -1008 Total N with known response (N = 138) (N = 93) * Data are not available 7 years post-transplant.

Pediatric Lung Transplants Cumulative Morbidity Rates in Survivors within 1 Year Post. Transplant by

Pediatric Lung Transplants Cumulative Morbidity Rates in Survivors within 1 Year Post. Transplant by Induction Use (Transplants: January 2000 – June 2013) Induction No Induction Within 1 Year Total number with known response Hypertension 44. 5% (N = 344) 43. 3% (N = 194) Renal Dysfunction 11. 2% (N = 357) 10. 0% (N = 210) Outcome Abnormal Creatinine ≤ 2. 5 mg/dl 8. 7% 6. 7% Creatinine > 2. 5 mg/dl 2. 0% 1. 9% Chronic Dialysis 0. 6% 1. 0% Renal Transplant 0% 0. 5% Hyperlipidemia 5. 1% (N = 350) 10. 2% (N = 196) Diabetes 20. 7% (N = 358) 25. 6% (N = 211) Bronchiolitis Obliterans Syndrome 11. 5% (N = 338) 9. 2% (N = 195) 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033

Pediatric Lung Transplants Cumulative Morbidity Rates in Survivors within 5 Years Post. Transplant by

Pediatric Lung Transplants Cumulative Morbidity Rates in Survivors within 5 Years Post. Transplant by Induction Use (Transplants: January 2000 – June 2009) Induction No Induction Within 5 Years Total number with known response Hypertension 73. 4% (N = 79) 60. 3% (N = 68) Renal Dysfunction 29. 5% (N = 88) 32. 0% (N = 75) Outcome Abnormal Creatinine ≤ 2. 5 mg/dl 28. 4% 26. 7% 1. 1% 1. 3% Chronic Dialysis 0% 4. 0% Renal Transplant 0% 0% Creatinine > 2. 5 mg/dl Hyperlipidemia 28. 8% (N = 80) 21. 2% (N = 66) Diabetes 37. 8% (N = 90) 38. 2% (N = 76) Bronchiolitis Obliterans Syndrome 29. 7% (N = 74) 47. 5% (N = 59) 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033

Pediatric Lung Transplants Freedom from Bronchiolitis Obliterans Syndrome (Follow-ups: April 1994 – June 2014)

Pediatric Lung Transplants Freedom from Bronchiolitis Obliterans Syndrome (Follow-ups: April 1994 – June 2014) % Free from Bronchiolitis Obliterans Syndrome 100 80 60 40 20 0 0 1 2 3 4 5 Years 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 6 7 8 9 10

Pediatric Lung Transplants Freedom from Bronchiolitis Obliterans Syndrome by Era (Transplants: April 1994 –

Pediatric Lung Transplants Freedom from Bronchiolitis Obliterans Syndrome by Era (Transplants: April 1994 – June 2013) % Free from Bronchiolitis Obliterans Syndrome 100 p = 0. 0537 80 60 40 20 4/1994 -2003 (N=306) 2004 -6/2013 (N=411) 0 0 1 2 3 4 Years 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 5 6 7 8

Pediatric Lung Transplants % Free from Bronchiolitis Obliterans Syndrome Freedom from Bronchiolitis Obliterans Syndrome

Pediatric Lung Transplants % Free from Bronchiolitis Obliterans Syndrome Freedom from Bronchiolitis Obliterans Syndrome by Age Group (Follow-ups: April 1994 – June 2014) 100 No pair-wise comparisons were significant at p<0. 05. 80 60 40 20 <1 year (N=60) 1 -5 Years (N=79) 6 -10 Years (N=128) 11 -17 years (N=476) 0 0 1 2 3 4 Years 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 5 6 7 8

Pediatric Lung Transplants % Free from Bronchiolitis Obliterans Syndrome Freedom from Bronchiolitis Obliterans Syndrome

Pediatric Lung Transplants % Free from Bronchiolitis Obliterans Syndrome Freedom from Bronchiolitis Obliterans Syndrome by Diagnosis (Follow-ups: April 1994 – June 2014) 100 80 p = 0. 0428 60 40 Cystic Fibrosis (N=398) 20 IPAH (N=68) 0 0 1 2 3 4 Years 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 5 6 7 8

Pediatric Lung Transplants % Free from Bronchiolitis Obliterans Syndrome Freedom from Bronchiolitis Obliterans Syndrome

Pediatric Lung Transplants % Free from Bronchiolitis Obliterans Syndrome Freedom from Bronchiolitis Obliterans Syndrome by Induction Use (Follow-ups: April 1994 – June 2014) 100 80 p=0. 1733 60 40 20 Induction (N = 355) No Induction (N = 378) 0 0 1 2 3 4 Years 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 5 6 7 8

Pediatric Lung Transplants Freedom from Severe Renal Dysfunction* % Free from Severe Renal Dysfunction

Pediatric Lung Transplants Freedom from Severe Renal Dysfunction* % Free from Severe Renal Dysfunction (Follow-ups: April 1994 – June 2014) 100 80 60 *Severe renal dysfunction = Creatinine > 2. 5 mg/dl (221 μmol/L), dialysis or renal transplant 40 20 0 0 1 2 3 4 5 Years 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 6 7 8 9 10

Pediatric Lung Transplants Freedom from Severe Renal Dysfunction* by Era % Free from Severe

Pediatric Lung Transplants Freedom from Severe Renal Dysfunction* by Era % Free from Severe Renal Dysfunction (Transplants: April 1994 – June 2013) 100 80 p = 0. 0176 60 *Severe renal dysfunction = Creatinine > 2. 5 mg/dl (221 μmol/L), dialysis or renal transplant 40 4/1994 -2003 (N=331) 20 2004 -6/2013 (N=439) 0 0 1 2 3 4 Years 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 5 6 7 8

Pediatric Lung Transplants Cumulative Post-Transplant Malignancy Rates in Survivors (Follow-ups: April 1994 – June

Pediatric Lung Transplants Cumulative Post-Transplant Malignancy Rates in Survivors (Follow-ups: April 1994 – June 2014) Malignancy/Type No Malignancy (all types combined) 1 -Year Survivors 5 -Year Survivors 770 (95. 1%) 231 (89. 5%) 132 (91%) 40 (4. 9%) 27 (10. 5%) 13 (9%) Malignancy Lymphoma Type* 38 26 13 Other 2 1 0 Type Not Reported 0 1 0 “Other” includes liver and primitive neuroectodermal tumor. * Recipients may have experienced more than one type of malignancy so the sum of individual malignancy types may be greater than the total number with malignancy. 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033

Pediatric Lung Transplants Freedom from Malignancy (Follow-ups: April 1994 – June 2014) % Free

Pediatric Lung Transplants Freedom from Malignancy (Follow-ups: April 1994 – June 2014) % Free from Malignancy 100 90 80 70 All malignancy Lymphoma 60 Skin Other 50 0 1 2 3 4 5 Years 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 6 7 8 9 10

Pediatric Lung Transplants Freedom from Malignancy by Age Group (Follow-ups: April 1994 – June

Pediatric Lung Transplants Freedom from Malignancy by Age Group (Follow-ups: April 1994 – June 2014) % Free from Malignancy 100 90 80 No pair-wise comparisons were significant at p<0. 05. 70 <1 Year 1 -5 years 60 6 -10 years 11 -17 years 50 0 1 2 3 4 5 Years 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 6 7 8 9

Pediatric Lung Transplants Freedom from Malignancy by Induction Use (Transplants: January 2000 – June

Pediatric Lung Transplants Freedom from Malignancy by Induction Use (Transplants: January 2000 – June 2013) % Free from Malignancy 100 90 p = 0. 9405 80 70 Induction No Induction 60 50 0 1 2 3 4 5 Years 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 6 7 8 9

Pediatric Lung Transplants Cause of Death (Deaths: January 1990 – June 2014) CAUSE OF

Pediatric Lung Transplants Cause of Death (Deaths: January 1990 – June 2014) CAUSE OF DEATH 0 -30 Days (N=142) 31 Days - 1 Year (N=196) 0 24 (12. 2%) 91 (37. 0%) 47 (39. 5%) 59 (46. 1%) 3 (2. 1%) 4 (2. 0%) 5 (2. 0%) 3 (2. 5%) 0 LYMPHOMA 0 9 (4. 6%) 7 (2. 8%) 4 (3. 4%) 5 (3. 9%) MALIGNANCY, NON-LYMPHOMA 0 2 (1. 0%) 1 (0. 4%) 0 6 (4. 7%) CMV 0 5 (2. 6%) 0 0 0 INFECTION, NON-CMV 22 (15. 5%) 63 (32. 1%) 37 (15. 0%) 21 (17. 6%) 9 (7. 0%) GRAFT FAILURE 41 (28. 9%) 36 (18. 4%) 62 (25. 2%) 27 (22. 7%) 29 (22. 7%) CARDIOVASCULAR 21 (14. 8%) 8 (4. 1%) 4 (1. 6%) 1 (0. 8%) TECHNICAL 17 (12. 0%) 5 (2. 6%) 6 (2. 4%) 3 (2. 5%) 2 (1. 6%) MULTIPLE ORGAN FAILURE 15 (10. 6%) 23 (11. 7%) 13 (5. 3%) 4 (3. 4%) 8 (6. 3%) OTHER 23 (16. 2%) 17 (8. 7%) 20 (8. 1%) 9 (7. 6%) 9 (7. 0%) BRONCHIOLITIS ACUTE REJECTION 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 >1 Year - 3 Years >3 Years - 5 Years (N=246) (N=119) >5 Years (N=128)

Pediatric Lung Transplants Cause of Death (Deaths: January 2000 – June 2014) CAUSE OF

Pediatric Lung Transplants Cause of Death (Deaths: January 2000 – June 2014) CAUSE OF DEATH 0 -30 Days (N=76) 31 Days - 1 Year (N=120) 0 14 (11. 7%) 65 (35. 7%) 39 (39. 0%) 55 (45. 5%) 3 (3. 9%) 2 (1. 7%) 5 (2. 7%) 2 (2. 0%) 0 LYMPHOMA 0 3 (2. 5%) 3 (1. 6%) 4 (4. 0%) 5 (4. 1%) MALIGNANCY, NON-LYMPHOMA 0 2 (1. 7%) 1 (0. 5%) 0 6 (5. 0%) CMV 0 2 (1. 7%) 0 0 0 INFECTION, NON-CMV 13 (17. 1%) 34 (28. 3%) 25 (13. 7%) 16 (16. 0%) 8 (6. 6%) GRAFT FAILURE 10 (13. 2%) 25 (20. 8%) 51 (28. 0%) 24 (24. 0%) 28 (23. 1%) CARDIOVASCULAR 13 (17. 1%) 6 (5. 0%) 3 (1. 6%) 1 (1. 0%) 1 (0. 8%) TECHNICAL 10 (13. 2%) 3 (2. 5%) 3 (1. 6%) 3 (3. 0%) 2 (1. 7%) MULTIPLE ORGAN FAILURE 13 (17. 1%) 19 (15. 8%) 11 (6. 0%) 4 (4. 0%) 7 (5. 8%) OTHER 14 (18. 4%) 10 (8. 3%) 15 (8. 2%) 7 (7. 0%) 9 (7. 4%) BRONCHIOLITIS ACUTE REJECTION 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 >1 Year - 3 Years >3 Years - 5 Years (N=182) (N=100) >5 Years (N=121)

Pediatric Lung Transplants Relative Incidence of Leading Causes of Death (Deaths: January 2000 –

Pediatric Lung Transplants Relative Incidence of Leading Causes of Death (Deaths: January 2000 – June 2014) 50 % of Deaths 40 Bronchiolitis Infection (non-CMV) Graft Failure Cardiovascular Multiple Organ Failure 30 20 10 0 0 -30 days (N=76) 31 days– 1 year (N=120) >1 year– 3 years >3 years– 5 years (N=182) (N=100) 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 >5 years (N=121)

Multivariable Analyses 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033

Multivariable Analyses 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033

Pediatric Lung Transplants (January 1996 – June 2013) Risk Factors For 1 Year Mortality/Graft

Pediatric Lung Transplants (January 1996 – June 2013) Risk Factors For 1 Year Mortality/Graft Failure 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 (N = 841) * Retransplant includes those with a retransplant diagnosis or a previous transplant was reported.

Pediatric Lung Transplants (January 1996 – June 2013) Risk Factors For 1 Year Mortality/Graft

Pediatric Lung Transplants (January 1996 – June 2013) Risk Factors For 1 Year Mortality/Graft Failure Continuous Factors (see figure) Recipient age 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 (N = 841)

Pediatric Lung Transplants (January 1996 – June 2013) Risk Factors For 1 Year Mortality/Graft

Pediatric Lung Transplants (January 1996 – June 2013) Risk Factors For 1 Year Mortality/Graft Failure Recipient Age Hazard Ratio of 1 Year Mortality/Graft Failure 2, 5 p = 0. 0409 2, 0 1, 5 1, 0 0, 5 0, 0 0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 Recipient Age 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 (N = 841) 17

Pediatric Lung Transplants (1996 -6/2009) Risk Factors For 5 Year Mortality/Graft Failure with 95%

Pediatric Lung Transplants (1996 -6/2009) Risk Factors For 5 Year Mortality/Graft Failure with 95% Confidence Limits (N = 636) 2015 JHLT. 2015 Oct; 34(10): 1255 -1263 * Other = All causes of death other than anoxia, stroke and head trauma ** Retransplant includes those with a retransplant diagnosis or a previous transplant was reported.

Pediatric Lung Transplants (1996 -6/2009) Risk Factors For 5 Year Mortality/Graft Failure Continuous Factors

Pediatric Lung Transplants (1996 -6/2009) Risk Factors For 5 Year Mortality/Graft Failure Continuous Factors (see figures) Recipient age Donor BMI/recipient BMI 2015 JHLT. 2015 Oct; 34(10): 1255 -1263

Hazard Ratio of 5 Year Mortality/Graft Failure Pediatric Lung Transplants (1996 -6/2009) Risk Factors

Hazard Ratio of 5 Year Mortality/Graft Failure Pediatric Lung Transplants (1996 -6/2009) Risk Factors For 5 Year Mortality/Graft Failure with 95% Confidence Limits Recipient age 2, 5 p = 0. 0090 2, 0 1, 5 1, 0 0, 5 0, 0 0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 Recipient age 2015 JHLT. 2015 Oct; 34(10): 1255 -1263 (N = 636) 17

Hazard Ratio of 5 Year Mortality/Graft Failure Pediatric Lung Transplants (1996 -6/2009) Risk Factors

Hazard Ratio of 5 Year Mortality/Graft Failure Pediatric Lung Transplants (1996 -6/2009) Risk Factors For 5 Year Mortality/Graft Failure with 95% Confidence Limits Donor BMI/recipient BMI 2, 5 p = 0. 0366 2, 0 1, 5 1, 0 0, 5 0, 0 0, 8 0, 9 1, 0 1, 1 Donor BMI/recipient BMI 2015 JHLT. 2015 Oct; 34(10): 1255 -1263 (N = 636) 1, 2

Pediatric Lung Transplants (1994 -6/2004) Risk Factors For 10 Year Mortality/Graft Failure 2015 JHLT.

Pediatric Lung Transplants (1994 -6/2004) Risk Factors For 10 Year Mortality/Graft Failure 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1025 -1033 (N = 449)

Pediatric Lung Transplants (1994 -6/2004) Risk Factors For 10 Year Mortality/Graft Failure Continuous Factors

Pediatric Lung Transplants (1994 -6/2004) Risk Factors For 10 Year Mortality/Graft Failure Continuous Factors (see figures) Recipient age 2015 JHLT. 2015 Oct; 34(10): 1255 -1263

Hazard Ratio of 10 Year Mortality/Graft Failure Pediatric Lung Transplants (1994 -6/2004) Risk Factors

Hazard Ratio of 10 Year Mortality/Graft Failure Pediatric Lung Transplants (1994 -6/2004) Risk Factors For 5 Year Mortality/Graft Failure with 95% Confidence Limits Recipient age 2, 5 p = 0. 0363 2, 0 1, 5 1, 0 0, 5 0, 0 0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 Recipient age 2015 JHLT. 2015 Oct; 34(10): 1255 -1263 (N = 449)

Early Graft Failure (EGF) 2015 JHLT. 2015 Oct; 34(10): 1255 -1263

Early Graft Failure (EGF) 2015 JHLT. 2015 Oct; 34(10): 1255 -1263

Pediatric Lung Transplants Cumulative Incidence of EGF by Transplant Type (Transplants: January 2005 –

Pediatric Lung Transplants Cumulative Incidence of EGF by Transplant Type (Transplants: January 2005 – December 2013) 10% Incidence of EGF (%) 9% Primary (N=930) 8% First Retransplant (N=45) 7% p = 0. 0010 6% 5% 4% 3% 2% 1% 0% 0 2 4 6 8 10 12 14 16 Days 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1009 -1024 18 20 22 24 26 28 30

Pediatric Lung Transplants % with EGF by EGF Type and Year for Primary Transplants

Pediatric Lung Transplants % with EGF by EGF Type and Year for Primary Transplants N with retransplant % with EGF 10 10% 9 9% 8 8% 7 7% 6 6% 5 5% 4 4% 3 3% 2 2% 1 1% 0 2005 2006 2007 2008 2009 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1009 -1024 2010 2011 2012 2013 0% Overall % with EGF N with death

Pediatric Lung Transplants Cumulative Incidence of EGF by Era (Transplants: January 2005 – December

Pediatric Lung Transplants Cumulative Incidence of EGF by Era (Transplants: January 2005 – December 2013) Incidence of EGF (%) 10% 9% 2005 -2007 (N=301) 8% 2008 -2013 (N=675) 7% p = 0. 6921 6% 5% 4% 3% 2% 1% 0% 0 2 4 6 8 10 12 14 16 Days 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1009 -1024 18 20 22 24 26 28 30

Pediatric Lung Transplants Cumulative Incidence of EGF by Diagnosis (Primary Transplants: January 2005 –

Pediatric Lung Transplants Cumulative Incidence of EGF by Diagnosis (Primary Transplants: January 2005 – December 2013) Incidence of EGF (%) 10% 9% Cystic Fibrosis (N=551) 8% Non-Cystic Fibrosis (N=357) 7% p = 0. 1641 6% 5% 4% 3% 2% 1% 0% 0 2 4 6 8 10 12 14 16 Days 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1009 -1024 18 20 22 24 26 28 30

Pediatric Lung Transplants Cumulative Incidence of EGF by Diagnosis and Era (Primary Transplants: January

Pediatric Lung Transplants Cumulative Incidence of EGF by Diagnosis and Era (Primary Transplants: January 2005 – December 2013) 10% 9% Incidence of EGF (%) 8% CF/2005 -2007 (N=171) CF/2008 -2013 (N=380) Non-CF/2005 -2007 (N=107) Non-CF/2008 -2013 (N=250) 7% No pair-wise comparisons between eras within each diagnosis and between diagnoses within each era were significant at p < 0. 05. 6% 5% 4% 3% 2% 1% 0% 0 2 4 6 8 10 12 14 16 Days 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1009 -1024 18 20 22 24 26 28 30

Pediatric Lung Transplants % with EGF by EGF Type and Age Group (Primary Transplants:

Pediatric Lung Transplants % with EGF by EGF Type and Age Group (Primary Transplants: January 2005 – December 2013) Death Retransplant 10% % of transplants 9% 8% No pair-wise comparisons of % with EGF were significant at p < 0. 05. 7% 6% 5% 4% 3% 2% 1% 0% 0 -5 years (N=126) 6 -10 years (N=702) 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1009 -1024 11 -17 years (N=102)

Pediatric Lung Transplants % with EGF by EGF Type, Gender and Diagnosis (Primary Transplants:

Pediatric Lung Transplants % with EGF by EGF Type, Gender and Diagnosis (Primary Transplants: January 2005 – December 2013) Death Retransplant 10% 9% % of transplants 8% No comparisons of % with EGF within each diagnosis were significant at p < 0. 05. 7% 6% 5% 4% 3% 2% 1% 0% Female (N=338) Male (N=213) Cystic Fibrosis 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1009 -1024 Female (N=200) Male (N=157) Non-Cystic Fibrosis

Pediatric Lung Transplants Cumulative Incidence of EGF by Donor Gender (Primary Transplants: January 2005

Pediatric Lung Transplants Cumulative Incidence of EGF by Donor Gender (Primary Transplants: January 2005 – December 2013) 10% 9% Female (N=526) Male (N=404) Incidence of EGF (%) 8% 7% p = 0. 9782 6% 5% 4% 3% 2% 1% 0% 0 2 4 6 8 10 12 14 16 Days 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1009 -1024 18 20 22 24 26 28 30

Pediatric Lung Transplants Cumulative Incidence of EGF by Donor Age Group (Transplants: January 2005

Pediatric Lung Transplants Cumulative Incidence of EGF by Donor Age Group (Transplants: January 2005 – December 2013) 10% 9% 0 -10 (N=305) 11 -17 (N=276) 18 -34 (N=151) 35 -49 (N=157) Incidence of EGF (%) 8% 7% 6% No pair-wise comparisons of % with EGF were significant at p < 0. 05. 5% 4% 3% 2% 1% 0% 0 2 4 6 8 10 12 14 16 Days 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1009 -1024 18 20 22 24 26 28 30

Pediatric Lung Transplants % with EGF by Location and Era for Primary Transplants 10%

Pediatric Lung Transplants % with EGF by Location and Era for Primary Transplants 10% 9% No pair-wise comparisons between eras within each location and between locations within each era were significant at p < 0. 05. % of transplants 8% 7% 6% 5% 4% 3% 2% 1% 0% 2005 -2007 2008 -2013 (N=111) (N=295) Europe 2005 -2007 2008 -2013 (N=160) (N=304) 2005 -2007 2008 -2013 (N=15) (N=45) North America Other* 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1009 -1024

Pediatric Lung Transplants % with EGF and non-EGF Early Failures by Location and Era

Pediatric Lung Transplants % with EGF and non-EGF Early Failures by Location and Era for Primary Transplants 14% EGF % of transplants 12% 10% non-EGF early failures* No pair-wise comparisons of % with EGF or comparisons of % with non-EGF early failures between eras within each location and between locations within each era were significant at p < 0. 05. 8% 6% 4% 2% 0% 2005 -2007 2008 -2013 (N=111) (N=295) 2005 -2007 2008 -2013 (N=160) (N=304) Europe North America 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1009 -1024 2005 -2007 2008 -2013 (N=15) (N=45) Other * non-EGF early failures include non-EGF retransplants and deaths within 30 days

Pediatric Lung Transplants % with EGF by EGF Type and Center Volume (Primary Transplants:

Pediatric Lung Transplants % with EGF by EGF Type and Center Volume (Primary Transplants: January 2005 – December 2013) Death Retransplant 10% 9% % of transplants 8% No pair-wise comparisons of % with EGF were significant at p < 0. 05. 7% 6% 5% 4% 3% 2% 1% 0% 1 -4 (N=536) 5 -9 (N=194) 10 -19 (N=200) Average number of pediatric lung transplants per year 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1009 -1024

Pediatric Lung Transplants Cumulative Incidence of EGF by Ischemic Time (Transplants: January 2005 –

Pediatric Lung Transplants Cumulative Incidence of EGF by Ischemic Time (Transplants: January 2005 – December 2013) 10% 9% 4 -<6 (N=256) Incidence of EGF (%) 8% 6+ (N=215) 7% p = 0. 7189 6% 5% 4% 3% 2% 1% 0% 0 2 4 6 8 10 12 14 16 18 20 22 24 26 28 30 Days 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1009 -1024 None of the patients in 2 -<4 hours group (N=56) had EGF

Pediatric Lung Transplants Cumulative Incidence of EGF by Donor/Recipient BMI Ratio (Transplants: January 2005

Pediatric Lung Transplants Cumulative Incidence of EGF by Donor/Recipient BMI Ratio (Transplants: January 2005 – December 2013) 10% <0. 85 (N=71) 9% 0. 85 -<1. 15 (N=213) 1. 15+ (N=351) Incidence of EGF (%) 8% 7% No pair-wise comparisons of % with EGF were significant at p < 0. 05. 6% 5% 4% 3% 2% 1% 0% 0 2 4 6 8 10 12 14 16 Days 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1009 -1024 18 20 22 24 26 28 30

Pediatric Lung Transplants Cumulative Incidence of EGF by Donor Cause of Death (Transplants: January

Pediatric Lung Transplants Cumulative Incidence of EGF by Donor Cause of Death (Transplants: January 2005 – December 2013) 10% Head trauma (N=283) 9% Stroke (N=160) Other (N=295) Incidence of EGF (%) 8% 7% No pair-wise comparisons of % with EGF were significant at p < 0. 05. 6% 5% 4% 3% 2% 1% 0% 0 2 4 6 8 10 12 14 16 Days 2015 JHLT. 2015 2014 Oct; 34(10): 33(10): 1255 -1263 1009 -1024 18 20 22 24 26 28 30

Pediatric Lung Transplants % with EGF by Donor and Recipient Characteristics (Primary Transplants: January

Pediatric Lung Transplants % with EGF by Donor and Recipient Characteristics (Primary Transplants: January 2005 – December 2013) 2015 JHLT. 2015 Oct; 34(10): 1255 -1263