LUNG TRANSPLANTATION Pediatric Recipients ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 RECIPIENT AGE DISTRIBUTION FOR PEDIATRIC LUNG RECIPIENTS - NUMBER (Transplants: January 1986 - June 2010) 1400 Number of Transplants 1200 <1 1-5 6-11 12-17 1000 800 600 400 200 0 1986-1995 (N=329) ISHLT 1996-6/2010 (N=1,335) 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 Analysis includes living donor transplants RECIPIENT AGE DISTRIBUTION FOR PEDIATRIC LUNG RECIPIENTS - PERCENTAGE (Transplants: January 1986 - June 2010) 100% 90% 80% % of Transplants 12-17 70% 60% 6-11 50% 1-5 40% 30% <1 20% 10% 0% 1986-1995 (N=329) ISHLT 1996-6/2010 (N=1,335) 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 Analysis includes living donor transplants DONOR TYPE DISTRIBUTION BY YEAR OF TRANSPLANT FOR PEDIATRIC LUNG RECIPIENTS (Transplants: 1986-2009) 130 120 100 90 80 70 60 50 40 30 20 10 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 20 02 20 03 20 04 20 05 20 06 20 07 20 08 20 09 Number of Transplants 110 Living Deceased Transplant Year ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 NOTE: This figure includes only the lung transplants that are reported to the ISHLT Transplant Registry. As such, the presented data may not mirror the changes in the number of lung transplants performed worldwide. Analysis includes living donor transplants DONOR TYPE DISTRIBUTION BY RECIPIENT AGE GROUP WITHIN ERA FOR PEDIATRIC LUNG RECIPIENTS (Transplants: January 1986 - June 2010) 1000 Number of Transplants 900 800 Living Deceased 700 600 500 400 300 200 100 0 0-5 years 6-11 years 12-17 years 0-5 years 1986-1995 6-11 years 12-17 years 1996-6/2010 Recipient Age (Years) ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 Analysis includes living donor transplants AGE DISTRIBUTION FOR DONORS OF PEDIATRIC LUNG RECIPIENTS (Transplants: January 1986 - June 2010) 100% 76 9 90% 189 % of Transplants 80% 268 70% 60+ 50-59 60% 325 50% 35-49 18-34 40% 12-17 30% 658 20% 10% 0% Donor Age (Years) ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 0-11 130 120 110 100 90 80 70 60 50 40 30 20 10 0 127 12-17 Years 1-11 Years 108 96 97 95 96 112 102 89 <1 Year 82 73 73 72 74 78 49 52 48 45 23 1 3 5 7 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 20 02 20 03 20 04 20 05 20 06 20 07 20 08 20 09 Number of Transplants AGE DISTRIBUTION OF PEDIATRIC LUNG RECIPIENTS: By Year of Transplant 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. ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 Analysis includes living donor transplants NUMBER OF CENTERS REPORTING PEDIATRIC LUNG TRANSPLANTS 40 30 20 10 0 19 86 19 87 19 8 19 8 89 19 90 19 91 19 9 19 2 93 19 94 19 9 19 5 96 19 97 19 98 19 9 20 9 00 20 01 20 02 20 03 20 04 20 05 20 0 20 6 07 20 08 20 09 Number of Centers Reporting Pediatric Transplants 50 Transplant Year ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 Analysis includes living donor transplants NUMBER OF CENTERS REPORTING PEDIATRIC LUNG TRANSPLANTS BY CENTER VOLUME 20+ transplants 10-19 transplants 40 5-9 transplants 1-4 transplants 30 20 10 0 19 86 19 8 19 7 88 19 89 19 90 19 91 19 9 19 2 93 19 9 19 4 95 19 96 19 97 19 9 19 8 99 20 0 20 0 01 20 0 20 2 03 20 04 20 0 20 5 06 20 0 20 7 08 20 09 Number of Centers Reporting Pediatric Transplants 50 Transplant Year ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 Analysis includes living donor transplants 130 120 110 100 90 80 70 20+ transplants 10-19 transplants 5-9 transplants 1-4 transplants 60 50 40 30 20 10 0 19 86 19 8 19 7 8 19 8 89 19 9 19 0 9 19 1 92 19 9 19 3 94 19 95 19 96 19 97 19 9 19 8 9 20 9 00 20 0 20 1 02 20 0 20 3 04 20 05 20 0 20 6 0 20 7 08 20 09 Number of Transplants NUMBER OF PEDIATRIC LUNG TRANSPLANTS BY CENTER VOLUME Transplant Year ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 Analysis includes living donor transplants PEDIATRIC LUNG TRANSPLANTATION: Indications (Transplants: January 1990 – June 2010) AGE: < 1 Year DIAGNOSIS AGE: 1-5 Years AGE: 6-11 Years AGE: 12-17 Years Cystic Fibrosis 1 1.2% 5 4.5% 167 56.0% 775 71.7% Idiopathic Pulmonary Arterial Hypertension 12 14.0% 25 22.5% 30 10.1% 75 6.9% 7 6.3% 9 3.0% 34 3.1% Re-Transplant: Obliterative Bronchiolitis Congenital Heart Disease 13 15.1% 8 7.2% 4 1.3% 9 0.8% Idiopathic Pulmonary Fibrosis 9 10.5% 20 18.0% 13 4.4% 38 3.5% 10 9.0% 19 6.4% 45 4.2% 27 2.5% Obliterative Bronchiolitis (Not Re-TX) Re-Transplant: Not OB 3 3.5% 4 3.6% 8 2.7% Interstitial Pneumonitis 1 1.2% 2 1.8% 2 0.7% Pulmonary Vascular Disease 8 9.3% 7 6.3% 3 1.0% Eisenmenger’s Syndrome 1 1.2% 5 4.5% 5 1.7% 7 0.6% Pulmonary Fibrosis, Other 5 5.8% 5 4.5% 12 4.0% 22 2.0% Surfactant Protein B Deficiency 15 17.4% 3 2.7% 1 COPD/Emphysema 4 4.7% 2 1.8% 3 1.0% 9 0.8% Bronchopulmonary Dysplasia 2 2.3% 2 1.8% 6 2.0% 1 0.1% Bronchiectasis 1 1.2% 5 1.7% 13 1.2% Other 11 12.8% 12 4.0% 26 2.4% ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 6 5.4% 0.4% 1 0.1% Analysis includes living donor transplants DIAGNOSIS IN PEDIATRIC LUNG RECIPIENTS BY YEAR OF TRANSPLANT Age: 12-17 Years 100 % of Transplants IPAH Cystic Fibrosis 75 50 25 0 1990 1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005 2006 2007 2008 2009 ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 Analysis includes living donor transplants PEDIATRIC LUNG TRANSPLANTS: AGE DISTRIBUTION BY LOCATION Transplants between January 2000 and June 2010 0-5 years 6-11 years 12-17 years % of Transplants 100% 80% 60% 40% 20% 0% Europe (N=359) ISHLT North America (N=576) 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 Other (N=54) Analysis includes living donor transplants PEDIATRIC LUNG TRANSPLANTS: DIAGNOSIS DISTRIBUTION BY LOCATION Transplants between January 2000 and June 2010 Cystic Fibrosis IPAH IPF OB Other Congenital heart disease Re-TX % of Transplants- 100% 80% 60% 40% 20% 0% Europe (N=331) North America (N=576) Other (N=47) NOTE: Unknown diagnoses were excluded from this tabulation. ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 Total number of transplants reported: Europe = 359 North America = 576 Other = 54 Analysis includes living donor transplants PEDIATRIC LUNG TRANSPLANTS: DONOR AGE DISTRIBUTION BY LOCATION Transplants between January 2000 and June 2010 0-5 6-11 12-17 18-34 35-49 50-59 60+ 100% % of Donors 80% 60% 40% 20% 0% Europe (N=354) North America (N=540) Other (N=53) NOTE: Transplants with unknown donor age and living donor transplants were excluded from this tabulation. ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 Total number of transplants reported: Europe = 359 North America = 576 Other = 54 LUNG TRANSPLANTATION Kaplan-Meier Survival by Recipient Age Group (Transplants: January 1990 - June 2009) 100 P = 0.0420 Adult (N=31,794) Pediatric (N=1,353) Survival (%) 75 50 25 HALF-LIFE Adult = 5.4 Years; Pediatric = 5.5 Years 0 0 1 2 3 4 5 6 7 8 9 Years ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 10 11 12 13 14 15 16 17 PEDIATRIC LUNG TRANSPLANTATION Kaplan-Meier Survival by Procedure Type (Transplants: January 1990 - June 2009) 100 Single Lung P < .0001 Bilateral/Double Lung (N=1,267) 75 Survival (%) (N=84) 50 25 HALF-LIFE Single Lung: 2.2 Years; Bilateral/double Lung: 5.7 Years 0 0 1 2 3 4 5 6 7 Years ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 8 9 10 11 12 13 14 PEDIATRIC LUNG TRANSPLANTATION Kaplan-Meier Survival for Congenital Diagnoses (Transplants: January 1990 – June 2009) 100 Eisenmenger’s vs. Other: p = 0.4357 Survival (%) 75 N at risk = 17 N at risk = 13 N at risk = 10 50 Eisenmenger's Syndrome (N=15) 25 Other Congenital Heart Disease (N=31) Eisenmenger's + Other Congenital Heart Disease (N=46) 0 0 1 2 Years ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 3 4 PEDIATRIC LUNG TRANSPLANTATION Kaplan-Meier Survival by Recipient Age Group (Transplants: January 1990 - June 2009) 100 <1 year vs. 1-11 years: p = 0.0987 <1 year vs. 12-17 years: p = 0.6665 1-11 years vs. 12-17 years: p = 0.0219 <1 Year 75 (N=83) Survival (%) 1-11 Years (N=365) 12-17 Years (N=905) 50 N at risk = 26 N at risk = 11 25 HALF-LIFE <1 Year: 6.4 Years 1-11 Years: 7.3 Years 12-17 Years: 4.7 Years N at risk = 42 0 0 1 2 3 4 5 6 7 Years ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 8 9 10 11 12 13 PEDIATRIC LUNG TRANSPLANTATION Conditional Kaplan-Meier Survival by Recipient Age Group (Transplants: January 1990 - June 2009) 100 <1 year vs. 1-11 year: p = 0.9598 <1 year vs. 12-17 years: p = 0.2727 1-11 years vs. 12-17 years: p =0.0254 Survival (%) 75 N at risk = 26 CONDITIONAL HALF-LIFE <1 Year: 8.8 Years 1-11 Years: 11.2 Years 12-17 Years: 7.7 Years 50 N at risk = 11 N at risk = 42 25 <1 Year (N=51) 1-11 Years (N=267) 12-17 Years (N=650) 0 0 1 2 ISHLT 3 4 5 6 7 Years 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 8 9 10 11 12 13 PEDIATRIC LUNG TRANSPLANTATION Kaplan-Meier Survival by Era (Transplants: January 1988 - June 2009) 100 HALF-LIFE Unconditional 1988-1994: 5.2 Years; 1995-2001: 4.7 Years; 2002-6/2009: 5.7 Conditional 1988-1994: 13.6 Years; 1995-2001: 7.8 Years; 2002-6/2009: na Survival (%) 80 1988-1994 vs. 1995-2001: p = 0.6966 1988-1994 vs. 2002-6/2009: p = 0.0631 1995-2001 vs. 2002-6/2009: p = 0.0552 60 N at risk = 30 N at risk = 42 40 1988-1994 (N=210) 20 N at risk = 20 1995-2001 (N=501) 2002-6/2009 (N=653) 0 0 1 2 3 4 5 6 7 Years ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 8 9 10 11 12 13 14 PEDIATRIC LUNG TRANSPLANTATION Kaplan-Meier Survival by Donor Type for Recipients Age 12-17 Years (Transplants: January 1990 - June 2009) 100 p = 0.0677 Survival (%) 75 HALF-LIFE Deceased: 4.7 Years Living: 3.7 Years 50 N at risk = 71 N at risk = 21 25 Deceased Donor (N = 905) Living Donor (N = 78) 0 0 1 ISHLT 2 3 4 5 Years 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 6 7 8 9 10 PEDIATRIC LUNG RE-TRANSPLANTS Between January 1994 and June 2010 Number of Re-Transplants 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 ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 Analysis includes living donor transplants PEDIATRIC LUNG RETRANSPLANTS Survival for Transplants Performed Between January 1994 and June 2009 100 90 Survival (%) 80 70 60 N at risk at 5 years = 17 50 40 30 20 10 0 0 1 2 3 Years ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 4 5 PEDIATRIC LUNG RETRANSPLANTS STRATIFIED BY INTER-TRANSPLANT INTERVAL Survival for Transplants Performed Between January 1988 and June 2009 100 90 p = 0.1510 Survival (%) 80 70 60 N at risk = 10 50 40 N at risk = 11 30 20 <1 Year (N = 30) 10 1+ Year (N = 59) 0 0 1 2 3 Years ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 4 5 Analysis includes living donor transplants. Only patients who were less than 18 years old at the time of re-transplant are included. PEDIATRIC LUNG RETRANSPLANTS STRATIFIED BY DIAGNOSIS Survival for Transplants Performed Between January 1988 and June 2009 100 90 p = 0.8536 Survival (%) 80 70 60 50 N at risk = 10 40 30 N at risk = 12 20 Obliterative Bronchiolitis (N = 51) 10 Non Obliterative Bronchiolitis (N = 57) 0 0 1 2 3 Years ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 4 Analysis includes living donor transplants. Only patients who were less than 18 years old at the time of re-transplant are included. 5 PEDIATRIC LUNG RECIPIENTS Cross-Sectional Analysis Functional Status of Surviving Recipients (Follow-ups: April 1994-June 2010) 100% 80% 60% 40% 20% No Activity Limitations Performs with Assistance Total Assistance 0% 1 Year (N = 328) ISHLT 3 Years (N = 206) 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 5 Years (N = 127) PEDIATRIC LUNG RECIPIENTS Functional Status of Surviving Recipients US Recipients Only (Follow-ups: March 2005 - June 2010) 100% 10% 80% 20% 30% 40% 60% 50% 60% 40% 70% 80% 20% 90% 100% 0% 1 Year (N = 237) ISHLT 2 Years (N = 194) 3 Years (N = 159) 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 PEDIATRIC LUNG RECIPIENTS Rehospitalization Post-transplant of Surviving Recipients (Follow-ups: April 1994 - June 2010) 100% 80% 60% 40% 20% No Hospitalization Hospitalized, Not Rejection/Not Infection Hospitalized, Rejection Hospitalized, Rejection + Infection Hospitalized, Infection Only 0% Up to 1 Year (N=626) ISHLT Between 2 and 3 Years (N=383) 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 Between 4 and 5 Years (N=226) PEDIATRIC LUNG RECIPIENTS Rehospitalization Post-transplant of Surviving Recipients (Follow-ups: April 1994 - June 2010) 100% 80% 60% 40% 20% No Hospitalization Hospitalized, Rejection Hospitalized, Rejection + Infection Hospitalized, Not Rejection/Not Infection Hospitalized, Infection Only 0% Up to 1 Year (N=626) ISHLT Between 1 and 3 Years (N=348) 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 Between 3 and 5 Years (N=207) PEDIATRIC LUNG RECIPIENTS Induction Immunosuppression For transplants between January 2001 and June 2010 70 60 % of patients 50 40 30 20 10 0 Any Induction (N = 282) ISHLT Polyclonal ALG/ATG (N = 65) 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 IL-2R Antagonist (N = 214) Analysis is limited to patients who were alive at the time of the discharge PEDIATRIC LUNG RECIPIENTS Induction Immunosuppression (Transplants: January 2001 - June 2010) 80 2001 2003 2005 2007 2009 70 % of patients 60 2002 2004 2006 2008 1/2010-6/2010 50 40 30 20 10 0 Any Induction ISHLT Polyclonal ALG/ATG 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 IL-2R Antagonist Analysis is limited to patients who were alive at the time of the discharge PEDIATRIC LUNG TRANSPLANTATION Kaplan-Meier Survival Stratified by Induction Use (Transplants: January 2001 - June 2009) 100 p = 0.8136 Survival (%) 75 50 HALF-LIFE Induction: 6.1 Years No Induction: 5.1 Years 25 Induction (N = 230) No Induction (N = 160) 0 0 1 2 3 Years ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 4 5 PEDIATRIC LUNG RECIPIENTS Maintenance Immunosuppression at Time of Follow-up (Follow-ups: January 2001 – June 2010) 100 Year 1 (N = 348) Year 5 (N = 153) % of Patients 80 60 40 20 0 Cyclosporine Tacrolimus Sirolimus MMF Azathioprine Prednisone NOTE: Different patients are analyzed in Year 1 and Year 5 ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 Analysis is limited to patients who were alive at the time of the follow-up PEDIATRIC LUNG RECIPIENTS Maintenance Immunosuppression at Time of Follow-up (Follow-ups: January 2001 – June 2010) 100 % of Patients 80 60 Tac Tac MMF MMF 40 20 0 CyA CyA Calcineurin Inhibitor AZA CellCycle Calcineurin Inhibitor Prednisone 1 Year Follow-up (N = 348) 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 ISHLT 2011 AZA CellCycle Prednisone 5 Year Follow-up (N = 153) NOTE: 5% of patients were on both calcineurin inhibitors at different point during the 1-year; these patients are not counted in either group. And 0.3% (1 patient) was on neither drug during the 1-year. In the 5-year tabulations, 13% were reported to be on both drugs during the year and 1% (2 patients) was reported to be on neither drugs. J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 PEDIATRIC LUNG RECIPIENTS Maintenance Immunosuppression Drug Combinations at Time of Follow-up Report (Follow-ups: January 2001 – June 2010) 100% Other % of Patients 80% Tacrolimus + MMF 60% Tacrolimus + AZA 40% Cyclosporine + MMF 20% Cyclosporine + AZA 0% Year 1 (N = 348) Year 5 (N = 153) NOTE: Different patients are analyzed in Year 1 and Year 5 ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 Analysis is limited to patients who were alive at the time of the follow-up POST-LUNG TRANSPLANT MORBIDITY FOR PEDIATRICS Cumulative Prevalence in Survivors within 1 Year Post-Transplant (Follow-ups: April 1994 - June 2010) Within 1 Year Total number with known response Hypertension 42.2% (N = 604) Renal Dysfunction Abnormal Creatinine < 2.5 mg/dl Creatinine > 2.5 mg/dl Chronic Dialysis Renal Transplant 10.2% 7.3% 1.9% 0.6% 0.3% (N = 618) Hyperlipidemia 4.8% (N = 622) Diabetes 24.7% (N = 619) Bronchiolitis Obliterans Syndrome 13.7% (N = 577) Outcome ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 POST-LUNG TRANSPLANT MORBIDITY FOR PEDIATRICS Cumulative Prevalence in Survivors within 5 Years Post-Transplant (Follow-ups: April 1994 - June 2010) Within 5 Years Total number with known response Hypertension 68.8% (N = 160) Renal Dysfunction Abnormal Creatinine < 2.5 mg/dl Creatinine > 2.5 mg/dl Chronic Dialysis Renal Transplant 20.1% 13.6% 3.0% 2.4% 1.2% (N = 169) Hyperlipidemia 14.5% (N = 165) Diabetes 34.3% (N = 166) Bronchiolitis Obliterans Syndrome 36.4% (N = 129) Outcome ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 POST-LUNG TRANSPLANT MORBIDITY FOR PEDIATRICS Cumulative Prevalence in Survivors within 7 Years Post-Transplant (Follow-ups: April 1994 - June 2010) Outcome Renal Dysfunction Within 7 Years Total number with known response 31.0% (N = 84) 21.4% 3.6% 0.0% 6.0% Abnormal Creatinine < 2.5 mg/dl Creatinine > 2.5 mg/dl Chronic Dialysis Renal Transplant Diabetes 33.9% (N = 62) Bronchiolitis Obliterans Syndrome 46.0% (N = 50) ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 Freedom from Bronchiolitis Obliterans Syndrome For Pediatric Lung Recipients (Follow-ups: April 1994 - June 2010) % Free from Bronchiolitis Obliterans Syndrome 100 90 80 70 60 50 40 30 20 10 0 0 1 2 3 4 Years ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 5 6 7 Freedom from Bronchiolitis Obliterans Syndrome Stratified by Age Group For Pediatric Lung Recipients (Follow-ups: April 1994 - June 2010) % Free from Bronchiolitis Obliterans Syndrome 100 90 p = 0.0434 80 70 60 50 40 <1 Year (N=50) 30 1-11 Years (N=187) 20 12-17 Years (N=344) 10 0 0 ISHLT 1 2 3 Years 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 4 5 6 Freedom from Bronchiolitis Obliterans Syndrome Stratified by Diagnosis For Pediatric Lung Recipients (Follow-ups: April 1994 - June 2010) % Free from Bronchiolitis Obliterans Syndrome 100 90 p = 0.1604 80 70 60 50 40 Cystic Fibrosis (N=315) 30 20 IPAH (N=58) 10 0 0 ISHLT 1 2 3 Years 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 4 5 Freedom from Bronchiolitis Obliterans Syndrome For Pediatric Lung Recipients by Induction Use (Follow-ups: April 1994 - June 2010) % Free from Bronchiolitis Obliterans Syndrome 100 p = 0.7123 75 50 25 Induction (N = 229) No Induction (N =323) 0 0 1 2 3 Years ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 4 5 6 Freedom from Severe Renal Dysfunction* % Free from Severe Renal Dysfunction For Pediatric Lung Recipients (Follow-ups: April 1994 - June 2010) 100 90 80 70 60 50 40 * Severe renal dysfunction = Creatinine > 2.5 mg/dl (221 μmol/L), dialysis or renal transplant 30 20 10 0 0 1 2 3 4 Years ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 5 6 7 8 MALIGNANCY POST-LUNG TRANSPLANTATION FOR PEDIATRICS Cumulative Incidence for Survivors (Follow-ups: April 1994 - June 2010) Malignancy/Type No Malignancy Malignancy (all types combined) Malignancy Type* 1-Year Survivors 5-Year Survivors 7-Year Survivors 596 (94.5%) 152 (88.4%) 77 (89.5%) 35 (5.5%) 20 (11.6%) 9 (10.5%) Lymph 32 20 9 Other 2 1 0 Type Not Reported 1 0 0 ”Other” includes Liver and primitive neuroectodermal tumor. * Recipients may have experienced more than one type of malignancy so sum of individual malignancy types may be greater than total number with malignancy. ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 Freedom from Malignancy For Pediatric Lung Recipients (Follow-ups: April 1994 - June 2010) % Free from Malignancy 100 90 80 70 60 All malignancy Lymph Skin Other 50 0 1 2 3 4 5 Years ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 6 7 8 9 PEDIATRIC LUNG TRANSPLANT RECIPIENTS: Cause Of Death (Deaths: January 1992- June 2010) 31 Days - 1 Year (N = 135) >1 Year - 3 Years (N = 176) >3 Years - 5 Years (N = 83) >5 Years (N = 82) 13 (9.6%) 70 (39.8%) 34 (41.0%) 34 (41.5%) 3 (2.2%) 1 (0.6%) 2 (2.4%) 3 (3.7%) LYMPHOMA 7 (5.2%) 5 (2.8%) 4 (4.8%) 5 (6.1%) MALIGNANCY, NONLYMPHOMA 1 (0.7%) 1 (0.6%) 2 (2.4%) 3 (3.7%) CMV 5 (3.7%) CAUSE OF DEATH 0-30 Days (N =97) BRONCHIOLITIS ACUTE REJECTION 3 (3.1%) INFECTION, NON-CMV 13 (13.4%) 46 (34.1%) 26 (14.8%) 17 (20.5%) 11 (13.4%) GRAFT FAILURE 30 (30.9%) 27 (20.0%) 45 (25.6%) 11 (13.3%) 14 (17.1%) CARDIOVASCULAR 15 (15.5%) 6 (4.4%) 4 (2.3%) 1 (1.2%) 1 (1.2%) TECHNICAL 11 (11.3%) 3 (2.2%) 1 (0.6%) 1 (1.2%) 1 (1.2%) 8 (8.2%) 13 (9.6%) 9 (5.1%) 3 (3.6%) 7 (8.5%) 17 (17.5%) 11 (8.1%) 14 (8.0%) 8 (9.6%) 6 (7.3%) MULTIPLE ORGAN FAILURE OTHER ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 PEDIATRIC LUNG TRANSPLANT RECIPIENTS: Relative Incidence of Leading Causes of Death (Deaths: January 1992 - June 2010) 50 Bronchiolitis Graft Failure Multiple Organ Failure Infection (non-CMV) Cardiovascular Percentage of Deaths 40 30 20 10 0 0-30 Days (N = 97) ISHLT 31 Days – 1 Year (N = 135) >1 Year – 3 Years (N =176) 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 >3 Years – 5 Years (N = 83) >5 Years (N = 82 ) PEDIATRIC LUNG TRANSPLANT RECIPIENTS (1/1992-6/2009) Risk Factors For 1 Year Mortality/Graft Failure VARIABLE N Relative Risk P-value Single lung transplant 15 3.15 0.0026 1.49 - 6.63 On ventilator 124 3.14 <.0001 2.08 - 4.75 Year of transplant: 1992-1999 vs. 2000-6/2009 329 2.08 <.0001 1.50 - 2.90 Donor CMV+/Recipient CMV- 252 1.31 0.0920 0.96 - 1.79 N=795 ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 95% Confidence Interval PEDIATRIC LUNG TRANSPLANT RECIPIENTS (1/1992-6/2009) Risk Factors For 1 Year Mortality/Graft Failure Continuous Factors (see figures) Pediatric transplant center volume Recipient age (borderline) N=795 ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 PEDIATRIC LUNG TRANSPLANT RECIPIENTS (1/1992-6/2009) Risk Factors For 1 Year Mortality/Graft Failure Relative Risk of 1 Year Mortality Center Volume Pediatric Transplants 2 p = 0.0143 1.5 1 0.5 0 0 5 10 Center Volume (cases per year) ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 15 20 PEDIATRIC LUNG TRANSPLANT RECIPIENTS (1/1992-6/2009) Risk Factors For 1 Year Mortality/Graft Failure Relative Risk of 1 Year Mortality Recipient Age 2 p = 0.0738 1.5 1 0.5 0 0 1 2 3 4 5 6 7 8 9 10 Recipient Age ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 11 12 13 14 15 16 17 PEDIATRIC LUNG TRANSPLANT RECIPIENTS (1/1992-6/2005) Risk Factors For 5 Year Mortality/Graft Failure VARIABLE N Relative Risk Single lung transplant 15 2.45 0.0032 1.35 - 4.45 On ventilator 88 2.27 <.0001 1.53 - 3.36 Year of transplant: 1992-1999 vs. 2000-6/2005 329 1.59 <.0001 1.26 - 2.01 N=583 ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 P-value 95% Confidence Interval PEDIATRIC LUNG TRANSPLANT RECIPIENTS (1/1992-6/2005) Risk Factors For 5 Year Mortality/Graft Failure Continuous Factors (see figures) Recipient age Height difference (borderline) N=583 ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 PEDIATRIC LUNG TRANSPLANT RECIPIENTS (1/1992-6/2005) Risk Factors For 5 Year Mortality/Graft Failure Relative Risk of 5 Year Mortality Recipient Age 2 p = 0.0179 1.5 1 0.5 0 0 1 2 3 4 5 6 7 8 9 10 Recipient Age ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 11 12 13 14 15 16 17 PEDIATRIC LUNG TRANSPLANT RECIPIENTS (1/1992-6/2005) Risk Factors For 5 Year Mortality/Graft Failure Relative Risk of 5 Year Mortality Height Difference 2 p = 0.0999 1.5 1 0.5 0 -15 -10 -5 0 5 10 Donor Height - Recipient Height (cm) ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 15 20 PEDIATRIC LUNG TRANSPLANT RECIPIENTS (1/1990-6/2000) Risk Factors For 10 Year Mortality/Graft Failure VARIABLE N Relative Risk P-value On ventilator 66 2.12 <.0001 1.48 - 3.03 Single lung transplant 25 1.97 0.0085 1.19 - 3.25 N=380 ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 95% Confidence Interval PEDIATRIC LUNG TRANSPLANT RECIPIENTS (1/1990-6/2000) Risk Factors For 10 Year Mortality/Graft Failure Continuous Factors (see figures) Recipient age N=380 ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 PEDIATRIC LUNG TRANSPLANT RECIPIENTS (1/1990-6/2000) Risk Factors For 10 Year Mortality/Graft Failure Relative Risk of 10 Year Mortality Recipient Age 2 p = 0.0195 1.5 1 0.5 0 0 1 2 3 4 5 6 7 8 9 10 Recipient Age ISHLT 2011 J Heart Lung Transplant. 2011 Oct; 30 (10): 1071-1132 11 12 13 14 15 16 17