LUNG TRANSPLANTATION Pediatric Recipients ISHLT 2008 J Heart Lung Transplant 2008;27:

RECIPIENT AGE DISTRIBUTION FOR PEDIATRIC LUNG RECIPIENTS - NUMBER (Transplants: January June 2006) ISHLT 2008 Last updated based on data as of December 2006J Heart Lung Transplant 2008;27:

RECIPIENT AGE DISTRIBUTION FOR PEDIATRIC LUNG RECIPIENTS - PERCENTAGE (Transplants: January June 2006) ISHLT 2008 Last updated based on data as of December 2006J Heart Lung Transplant 2008;27:

DONOR TYPE DISTRIBUTION BY YEAR OF TRANSPLANT FOR PEDIATRIC LUNG RECIPIENTS (Transplants: ) ISHLT 2008 NOTE: This figure includes only the lung transplants that are reported to the ISHLT Transplant Registry. As such, this should not be construed as evidence that the number of lung transplants worldwide has declined in recent years. J Heart Lung Transplant 2008;27:

DONOR TYPE DISTRIBUTION BY RECIPIENT AGE GROUP WITHIN ERA FOR PEDIATRIC LUNG RECIPIENTS (Transplants: January June 2006) ISHLT 2008 Last updated based on data as of December 2006 J Heart Lung Transplant 2008;27:

AGE DISTRIBUTION FOR DONORS OF PEDIATRIC LUNG RECIPIENTS (Transplants: January June 2006) ISHLT 2008 Last updated based on data as of December 2006 J Heart Lung Transplant 2008;27:

AGE DISTRIBUTION OF PEDIATRIC LUNG RECIPIENTS By Year of Transplant Number of Transplants ISHLT J Heart Lung Transplant 2008;27:

NUMBER OF CENTERS REPORTING PEDIATRIC LUNG TRANSPLANTS ISHLT 2008 J Heart Lung Transplant 2008;27:

NUMBER OF CENTERS REPORTING PEDIATRIC LUNG TRANSPLANTS BY CENTER VOLUME ISHLT 2008 J Heart Lung Transplant 2008;27:

PEDIATRIC LUNG TRANSPLANTATION: Indications (Transplants: January 1990 – June 2006) DIAGNOSIS AGE: < 1 Year AGE: 1-5 Years AGE: 6-11 Years AGE: Years Cystic Fibrosis 33.7% %44169.% Primary Pulmonary Hypertension %1822.2%2311.8%538.3% Re-Transplant: Obliterative Bronchiolitis 67.4%84.1%223.4% Congenital Heart Disease %89.9%21.0%50.8% Idiopathic Pulmonary Fibrosis 78.6%63.1%233.6% Obliterative Bronchiolitis (Not Re-TX) 56.2%94.6%213.3% Re-Transplant: Not OB 34.8%11.2%73.6%162.5% Interstitial Pneumonitis 69.7%1113.6%10.5% 50.8% Pulmonary Vascular Disease 711.3%44.9%63.1%10.2% Eisenmengers Syndrome 11.6%56.2%52.6%60.9% Pulmonary Fibrosis, Other 11.6%11.2%42.1%111.7% Surfactant Protein B Deficiency 914.5%22.5% COPD/Emphysema 11.2%21.0%50.8% Bronchopulmonary Dysplasia 11.6%22.5%63.1% Bronchiectasis 31.5%40.6% Other 58.1%78.6%63.1%264.1% ISHLT 2008 Last updated based on data as of December 2006 J Heart Lung Transplant 2008;27:

PEDIATRIC LUNG TRANSPLANTATION: Other Indications (Transplants: January 1990 – June 2006) DIAGNOSIS AGE: < 1 Year AGE: 1-5 Years AGE: 6-11 Years AGE: Years Alpha Antitrypsin Deficiency1 0.2% ARDS/Pneumonia1 1.2%10.5% BOOP1 0.2% Dilated Myopathy: Adriamycin1 0.2% Graft-Vs-Host Disease (GVHD)1 1.2% 2 0.3% Idiopathic Pulmonary Hemosiderosis2 0.3% Inhalation Burns/Trauma1 0.2% Portopulmonary Hypertension1 1.2% Pulmonary Veno-Occlusive Disease1 0.2% Restrictive Lung Disease1 1.2% Rheumatoid Disease1 1.2% Scleroderma1 0.2% Secondary Pulmonary Hypertension 23.2% Other Lung Disease4 0.6% ISHLT 2008 Last updated based on data as of December 2006 J Heart Lung Transplant 2008;27:

PEDIATRIC LUNG TRANSPLANTATION: Other Indications (Transplants: January 1990 – June 2006) DIAGNOSIS (continued) AGE: < 1 Year AGE: 1-5 Years AGE: 6-11 Years AGE: Years Other – Specify: 34.8% 2 2.5%21.0% 3 0.5% Chronic Interstitial Pneumonia1 Chronic Lung Disease Unknown Etiology 1 Diaphragmatic Hernia 1 End Stage Interstitial Lung Disease1 Pulmonary1 Pulmonary Interstitial Emphysema 1 Respiratory Failure 1 TALC/Pneumoconiosis/Berylliososis1 Unknown 11 ISHLT 2008 Last updated based on data as of December 2006 J Heart Lung Transplant 2008;27:

PEDIATRIC LUNG TRANSPLANTATION: Other Indications (Transplants: January 1990 – June 2006) DIAGNOSIS (continued) AGE: < 1 Year AGE: 1-5 Years AGE: 6-11 Years AGE: Years Lung Disease: Other Specify 21.0% 3 0.5% Miscellaneous Lung: Not Specified2 Pulmonary Lymphangectasia 1 Not Reported 1 1 Other 10.5% 6 0.9% Congenital Anomaly1 Langerhans -Histiocytosis1 Not Reported 1 4 ISHLT 2008 Last updated based on data as of December 2006 J Heart Lung Transplant 2008;27:

DIAGNOSIS IN PEDIATRIC LUNG RECIPIENTS BY YEAR OF TRANSPLANT Age: Years ISHLT 2008 J Heart Lung Transplant 2008;27:

PEDIATRIC LUNG TRANSPLANTS: AGE DISTRIBUTION BY LOCATION Transplants between January 2000 and June 2006 ISHLT 2008 Last updated based on data as of December 2006J Heart Lung Transplant 2008;27:

PEDIATRIC LUNG TRANSPLANTS: DIAGNOSIS DISTRIBUTION BY LOCATION Transplants between January 2000 and June 2006 ISHLT 2008 NOTE: Unknown diagnoses were excluded from this tabulation. Total number of transplants reported: Europe = 82 North America = 338 Other = 24 Last updated based on data as of December 2006J Heart Lung Transplant 2008;27:

PEDIATRIC LUNG TRANSPLANTS: DONOR AGE DISTRIBUTION BY LOCATION Transplants between January 2000 and June 2006 ISHLT 2008 NOTE: Transplants with unknown donor age were excluded from this tabulation. Total number of transplants reported: Europe = 82 North America = 338 Other = 24 Last updated based on data as of December 2006J Heart Lung Transplant 2008;27:

LUNG TRANSPLANTATION Kaplan-Meier Survival by Age Group (Transplants: January June 2006) P = ISHLT 2008 J Heart Lung Transplant 2008;27:

PEDIATRIC LUNG TRANSPLANTATION Kaplan-Meier Survival by Procedure Type (Transplants: January June 2005) P <.0001 ISHLT 2008 Last updated based on data as of December 2006 J Heart Lung Transplant 2008;27:

PEDIATRIC LUNG TRANSPLANTATION Kaplan-Meier Survival for Congenital Diagnoses (Transplants: January 1990 – June 2005) ISHLT 2008 Eisenmengers vs. Other: p = 0.19 Last updated based on data as of December 2006J Heart Lung Transplant 2008;27:

PEDIATRIC LUNG TRANSPLANTATION Kaplan-Meier Survival by Age Group (Transplants: January June 2006) ISHLT 2008 J Heart Lung Transplant 2008;27:

PEDIATRIC LUNG TRANSPLANTATION Conditional Kaplan-Meier Survival by Age Group (Transplants: January June 2006) CONDITIONAL HALF-LIFE <1 Year: 12.1 Years 1-11 Years: 10.5 Years Years: 6.1 Years ISHLT 2008 J Heart Lung Transplant 2008;27:

PEDIATRIC LUNG TRANSPLANTATION Kaplan-Meier Survival by Era (Transplants: January June 2006) ISHLT 2008 J Heart Lung Transplant 2008;27:

PEDIATRIC LUNG TRANSPLANTATION Kaplan-Meier Survival by Donor Type for Recipients Age Years (Transplants: January June 2005) HALF-LIFE Deceased: 4.0 Years Living: 3.8 Years ISHLT 2008 Last updated based on data as of December 2006 J Heart Lung Transplant 2008;27:

PEDIATRIC LUNG RE-TRANSPLANTS Between January 1994 and June 2006 ISHLT 2008 Time Between Previous and Current Transplant Last updated based on data as of December 2006 J Heart Lung Transplant 2008;27:

PEDIATRIC LUNG RETRANSPLANTS Survival for Transplants Performed Between January 1994 and June 2006 ISHLT 2008 J Heart Lung Transplant 2008;27:

PEDIATRIC LUNG RECIPIENTS Functional Status of Surviving Recipients (Follow-ups: April 1994-June 2006) ISHLT 2008 Last updated based on data as of December 2006J Heart Lung Transplant 2008;27:

PEDIATRIC LUNG RECIPIENTS Rehospitalization Post-transplant of Surviving Recipients (Follow-ups: April June 2006) ISHLT 2008 Last updated based on data as of December 2006 J Heart Lung Transplant 2008;27:

PEDIATRIC LUNG RECIPIENTS Rehospitalization Post-transplant of Surviving Recipients (Follow-ups: April June 2006) ISHLT 2008 Last updated based on data as of December 2006 J Heart Lung Transplant 2008;27:

PEDIATRIC LUNG RECIPIENTS Induction Immunosuppression For transplants between January 2001 and June 2007 ISHLT 2008 Analysis is limited to patients who were alive at the time of the follow-up J Heart Lung Transplant 2008;27:

PEDIATRIC LUNG RECIPIENTS Induction Immunosuppression (Transplants: January June 2007) ISHLT 2008 Analysis is limited to patients who were alive at the time of the follow-up J Heart Lung Transplant 2008;27:

PEDIATRIC LUNG TRANSPLANTATION Kaplan-Meier Survival Stratified by Induction Use (Transplants: January June 2006) ISHLT 2008 J Heart Lung Transplant 2008;27:

PEDIATRIC LUNG RECIPIENTS Maintenance Immunosuppression at Time of Follow-up (F ollow-ups: January 2001 and June 2007) NOTE: Different patients are analyzed in Year 1 and Year 5 ISHLT 2008 Analysis is limited to patients who were alive at the time of the follow-up J Heart Lung Transplant 2008;27:

PEDIATRIC LUNG RECIPIENTS Maintenance Immunosuppression at Time of Follow-up (Follow-ups: January 2001 and June 2007) 1 Year Follow-up (N = 244)5 Year Follow-up (N = 116) NOTE: Different patients are analyzed in Year 1 and Year 5 ISHLT 2008 NOTE: 3% of patients were on both calcineurin inhibitors at different point during the year; these patients are not counted in either group. And 1% (2 patients) were on neither drugs during the year. In the 5-year tabulations, 16% were reported to be on both drugs during the year and 1% (1 patient) were reported to be on neither drugs. Analysis is limited to patients who were alive at the time of the follow-up J Heart Lung Transplant 2008;27:

PEDIATRIC LUNG RECIPIENTS Maintenance Immunosuppression Drug Combinations at Time of Follow-up Report (Follow-ups: January 2001 and June 2007) NOTE: Different patients are analyzed in Year 1 and Year 5 ISHLT 2008 Analysis is limited to patients who were alive at the time of the follow-up J Heart Lung Transplant 2008;27:

POST-LUNG TRANSPLANT MORBIDITY FOR PEDIATRICS Cumulative Prevalence in Survivors within 1 Year Post-Transplant (Follow-ups: April June 2006) ISHLT 2008 Last updated based on data as of December 2006J Heart Lung Transplant 2008;27:

POST-LUNG TRANSPLANT MORBIDITY FOR PEDIATRICS Cumulative Prevalence in Survivors within 5 Years Post-Transplant (Follow-ups: April June 2006) ISHLT 2008 Last updated based on data as of December 2006 J Heart Lung Transplant 2008;27:

POST-LUNG TRANSPLANT MORBIDITY FOR PEDIATRICS Cumulative Prevalence in Survivors within 7 Years Post-Transplant (Follow-ups: April June 2006) ISHLT 2008 Last updated based on data as of December 2006 J Heart Lung Transplant 2008;27:

Freedom from Bronchiolitis Obliterans For Pediatric Lung Recipients (Follow-ups: April June 2007) ISHLT 2008 J Heart Lung Transplant 2008;27:

FREEDOM FROM BRONCHIOLITIS OBLITERANS For Pediatric Lung Recipients by Induction Use (Follow-ups: April June 2007) ISHLT 2008 J Heart Lung Transplant 2008;27:

Freedom from Severe Renal Dysfunction* For Pediatric Lung Recipients (Follow-ups: April June 2006) * Severe renal dysfunction = Creatinine > 2.5 mg/dl (221 μmol/L), dialysis or renal transplant ISHLT 2008 Last updated based on data as of December 2006 J Heart Lung Transplant 2008;27:

MALIGNANCY POST-LUNG TRANSPLANTATION FOR PEDIATRICS Cumulative Incidence for Survivors (Follow-ups: April June 2006) Malignancy/Type1-Year Survivors 5-Year Survivors 7-Year Survivors No Malignancy469 (94.6%)111 (88.1%)51 (87.9%) Malignancy (all types combined) 27 (5.4%)15 (11.9%)7 (12.1%) Malignancy Type Lymph24146 Other311 Other includes Liver and primitive neuroectodermal tumor. ISHLT 2008 Last updated based on data as of December 2006 J Heart Lung Transplant 2008;27:

Freedom from Malignancy For Pediatric Lung Recipients (Follow-ups: April June 2006) ISHLT 2008 Last updated based on data as of December 2006J Heart Lung Transplant 2008;27:

PEDIATRIC LUNG TRANSPLANT RECIPIENTS: Cause Of Death (Deaths: January June 2006) CAUSE OF DEATH 0-30 Days (N =72 ) 31 Days - 1 Year (N = 107) >1 Year - 3 Years (N = 111) >3 Years - 5 Years (N = 56) >5 Years (N = 38) BRONCHIOLITIS 9 (8.4%)43 (38.7%)24 (42.9%)16 (42.1%) ACUTE REJECTION 1 (1.4%)3 (2.8%)1 (1.8%) LYMPHOMA 3 (2.8%)4 (3.6%)2 (3.6%)4 (10.5%) MALIGNANCY, NON- LYMPHOMA 1 (1.8%) CMV 6 (5.6%) INFECTION, NON-CMV 9 (12.5%)44 (41.1%)23 (20.7%)13 (23.2%)3 (7.9%) GRAFT FAILURE 24 (33.3%)20 (18.7%)21 (18.9%)7 (12.5%)8 (21.1%) CARDIOVASCULAR 9 (12.5%)4 (3.7%)2 (1.8%) TECHNICAL 10 (13.9%)1 (0.9%) MULTIPLE ORGAN FAILURE 6 (8.3%)9 (8.4%)9 (8.1%)3 (5.4%)3 (7.9%) OTHER 13 (18.1%)8 (7.5%)9 (8.1%)5 (8.9%)4 (10.5%) ISHLT 2008 Last updated based on data as of December 2006 J Heart Lung Transplant 2008;27:

PEDIATRIC LUNG TRANSPLANT RECIPIENTS: Other Cause Of Death (Deaths: January June 2006) CAUSE OF DEATH 0-30 Days (N =72 ) 31 Days - 1 Year (N = 107) >1 Year - 3 Years (N = 111) >3 Years - 5 Years (N = 56) >5 Years (N = 38) Graft Failure: Graft Infection4 (5.6%)3 2.8%)4 (3.6%)2 (5.3%) Graft Failure: Recurrent Disease1 (0.9%)1 (2.6%) Pulm: Pulmonary Embolism1 (1.4%)1 (1.8%) Cerebrovascular: Hemorrhage (Non-Stroke)3 4.2%)3 (2.8%) Cerebrovascular: Brain Anoxia1 (1.4%)1 (0.9%) Cerebrovascular: Other Specify3 4.2%) Hemorrhage: Gastrointestinal1 (0.9%) Hemorrhage: Respiratory1 (0.9%)1 (1.8%) Renal Failure1 (2.6%) Non-Compliance1 (0.9%)1 (1.8%) Primary Organ Failure1 (1.4%) Thromboembolic Disease1 (0.9%) Hus Hemolytic Uremic Syndrome1 (1.8%) Respiratory Failure1 (1.8%) ISHLT 2008 Last updated based on data as of December 2006 J Heart Lung Transplant 2008;27: