1 lung
waiting list ...................... deceased donation ......... transplant ....................... donor-recipient matching outcomes ........................ pediatric transplant ......... transplant center maps....
6 10 11 14 15 18 25
M. Valapour1,2 , M. A. Skeans1 , B. M. Heubner1 , J. M. Smith1,3 , M. I. Hertz1,4 , L. B. Edwards5,6 , W. S. Cherikh5,6 , E. R. Callahan5,6 , J. J. Snyder1,7 , A. K. Israni1,7,8 , B. L. Kasiske1,8 1
Scientific Registry of Transplant Recipients, Minneapolis Medical Research Foundation, Minneapolis, MN
2
Department of Pulmonary Medicine, Respiratory Institute, Cleveland Clinic, Cleveland, OH
3
Department of Pediatrics, University of Washington, Seattle, WA
4
Department of Medicine, Division of Pulmonary, Allergy, Critical Care and Sleep Medicine, University of Minnesota, Minneapolis, MN
5
Organ Procurement and Transplantation Network, Richmond, VA
6
United Network for Organ Sharing, Richmond, VA
7
Department of Epidemiology and Community Health, University of Minnesota, Minneapolis, MN
8
Department of Medicine, Hennepin County Medical Center, University of Minnesota, Minneapolis, MN
OPTN/SRTR 2013 Annual Data Report:
lung
ABSTRACT Lungs are allocated to adult and adolescent transplant candidates (aged ě 12 years) on the basis of age, geography, blood type compatibility, and the lung allocation score (LAS), which reflects risk of waitlist mortality and probability of posttransplant survival. In 2013, the most adult candidates, 2394, of any year were added to the list. Overall median waiting time for candidates listed in 2013 was 4.0 months. The preferred procedure remained bilateral lung transplant, representing approximately 70% of lung transplants in 2013. Measures of short-term and longterm survival have plateaued since the implementation of the LAS in 2005. The number of new child candidates (aged 0-11 years) added to the lung transplant waiting list increased to 39 in 2013. A total of 28 lung transplants were performed in child recipients, 3 for ages younger than 1 year, 9 for ages 1 to 5 years, and 16 for ages 6 to 11 years. The diagnosis of pulmonary hypertension was associated with higher survival rates than cystic fibrosis or other diagnosis (pulmonary fibrosis, bronchiolitis obliterans, bronchopulmonary dysplasia). For child candidates, infection was the leading cause of death in year 1 posttransplant and graft failure in years 2 to 5.
KEY WORDS End-stage lung disease, lung allocation score, lung transplant, organ allocation, transplant outcomes.
I still catch myself on the street, or at a cafe, or wherever, and I'll think about how wonderful it feels to breathe —and I'll start welling up... I've had beautiful (donated) lungs for over 13 years now. I still think about them every single day, and about my donor. Steve, lung/kidney recipient
2
OPTN/SRTR 2013 Annual Data Report
INTRODUCTION As of June 30, 2013, nearly 11,000 recipients were living with a lung transplant in the US (Figure LU 5.3). Lung transplant is increasingly used as a treatment for patients who have end-stage lung diseases or are critically ill (Figures LU 3.1 and LU 3.5). Lungs are allocated to US transplant candidates aged 12 years or older primarily on the basis of age, geography, blood type (ABO) compatibility, and the lung allocation score (LAS). Implemented in 2005, the LAS calculation reflects the risk of waitlist mortality while avoiding transplants in candidates whose likelihood of survival is poor. The LAS applies to adolescent (aged 12-17 years) and adult (aged 18 years or older) candidates. As of June 10, 2013, pediatric candidates (aged younger than 12 years) can ask for an exception from the national Lung Review Board and be assigned a LAS to give primary access to lung offers from adult and adolescent donors. Therefore, the adult section below includes data for candidates and recipients aged 12 years or older and for 10 pediatric candidates who received an exception LAS. As part of the development of the LAS system, pulmonary diagnoses for candidates aged 12 years or older were categorized into four main groups based on survival probability and pathophysiology of the underlying disease. The four groups are: group A, obstructive lung disease (e.g., chronic obstructive lung disease/emphysema); group B, pulmonary vascular disease (e.g., idiopathic pulmonary arterial hypertension); group C, cystic fibrosis and immunodeficiency disorders; and group D, restrictive lung disease (e.g., idiopathic pulmonary fibrosis). The Organ Procurement and Transplantation Network (OPTN) Board of Directors approved the first comprehensive revision of the LAS calculation in November 2012;
the revised LAS will be implemented in February 2015. This revision includes modifications to the variables used in the LAS calculation and to the relative weight of the variables used to predict risk of death in the next year without transplant and in the first year after transplant. The revised LAS will further improve the survival prediction for all diagnostic groups; these effects will likely be most impactful for candidates in diagnosis group B.
ADULT LUNG TRANSPLANT WAITING LIST
After an initial decline immediately following implementation of the LAS system in May 2005, the number of new patients added to the waiting list has grown steadily, reaching the maximum of 2327 active new patients aged 12 years or older in 2013 (Figure LU 1.1). During the same time, the number of inactive candidates continued to decline every year and reached an all-time low of 302 on December 31, 2013 (Figure LU 1.1). Because the LAS system prioritizes the sickest patients for transplant, it changed listing practices in the lung transplant community and thus the characteristics of waitlisted candidates. Candidates on the waiting list are increasingly older, more likely to be from diagnosis group D, and more likely to have a higher LAS (Figure LU 1.2). Candidates aged 65 years or older make up an increasing proportion of the waiting list every year, to an all-time high of 23.9% of lung transplant candidates in 2013; proportions of all other adult age groups have declined. The proportion of candidates aged 18 to 34 years decreased from 16.0% of the waiting list in 2004 to 10.9% in 2013, the proportion aged 35 to 49 years from 25.0% to just
3 lung
13.7%, and the proportion aged 50 to 64 years from 52.0% to 49.5%. Adolescents aged 12 to 17 years make up a small proportion of the waiting list, at 2%. The proportion of candidates in diagnosis group D continues to increase every year, to an all-time high of 50.2% of the waiting list in 2013. All other diagnostic groups represented a smaller proportion of the waiting list in 2013 than in 2004; group A showed the most dramatic decline, from 42.7% in 2004 to 33.8% in 2013. Group A candidates also had the lowest median LAS at transplant (Figure LU 3.3). The trend in LAS distribution on the waiting list is toward higher scores; for example, the LAS was 50 to 100 for 13.1% of candidates in 2006 (the first full year after implementation) and for 28.0% in 2013 (Figure LU 1.2). Women made up approximately 60% of the waiting list in 2013, as before LAS implementation (Figure LU 1.3). Retransplant remains relatively uncommon. On December 31, 2013, 3.2% of candidates on the waiting list were listed for retransplant (Figure LU 1.3). Since the LAS was implemented, transplant rates have increased for all lung transplant candidates (Figure LU 1.4). Over the past 4 years, by height category, transplant rates have been consistently highest for candidates who are taller than 183 cm and lowest for candidates who are shorter than 160 cm. In 2013, the most candidates, 2394, were added to the list (Figure LU 1.1), and the most lung transplants among candidates aged 12 and older, 1918, were performed (Figure LU 3.5). Overall median waiting time for candidates first listed in 2013 was 4.0 months, varying from 2.6 months for group D candidates to 9.7 months for group B candidates (Figure LU 1.9). Despite this level of transplant activity, the overall waitlist mortality rate was 15.1 per 100 waitlist years for candidates awaiting transplant in 2012-2013, representing a plateau after a trend of increasing waitlist mortality rates since the LAS was implemented (Figure LU 1.10). Waitlist mortality rates in 2012-2013 remained highest for candidates aged 12 to 17 years, at 25.4 deaths per 100 waitlist years, an increase from 19.0 in 2010-2011 (Figure LU 1.10). Candidates aged 18 to 34 years followed at 21.0 deaths per 100 waitlist years. Diagnosis group D composed the largest proportion of the waiting list and had the highest waitlist morality rates. As expected, the waitlist mortality rate was nearly 10 times greater for candidates with LAS 50 or higher than for candidates with LAS less than 50 (Figure LU 1.10). Candidates with LAS less than 40 had nearly the same waitlist mortality rate, ranging from 3.6 to 6.2 deaths per 100 waitlist years (Figure LU 1.10). Since implementation of the LAS, the waitlist mortality rate has declined within every LAS group of 30 or higher. Because the distribution of LAS on the waiting list has shifted upwards, the overall waitlist mortality rate has increased, but rates are improving within every LAS grouping except less than 30 (< 1% of candidates).
TRANSPLANT
In 2013, 1946 total lung transplants were performed, including adult and pediatric recipients, the most ever in a single year (Figure LU 3.1). The preferred procedure remained bilateral lung transplant, representing approximately 70% of lung transplants in 2013 (Figure 3.5). Lung transplant recipients have been undergoing transplant with higher LAS (Figures LU 3.3). When the LAS system was implemented, the median LAS at transplant was 36.6; it increased to its highest median value of 43.0 in 2013. The distribution of the LAS at the time of transplant also shifted, with an increase in the seventy-fifth percentile scores to 58.7 in 2013. Median LAS at transplant was highest for candidates in diagnosis groups D and B, at 48.9 and 47.7, respectively, and lowest for candidates in group A, at 34.1. An important trend is the rising relative percentage of transplant recipients who are hospitalized in the intensive care unit at transplant (Figure LU 3.5): 14.1% in 2013 compared with 3.7% a decade earlier. In 2013, 4.8% of recipients were on extracorporeal membrane oxygenation (ECMO) at transplant. Since implementation of the LAS, men, candidates aged 65 years or older, and candidates in diagnosis group D have composed increasingly larger proportions of transplant recipients each year (Figure LU 3.2). In 2013, 28.7% of all US lung recipients were aged 65 years or older, compared with 7.2% in 2003, before implementation of the LAS (Figure LU 3.5). This shift may reflect both the LAS policy, which increases transplant access for patients at high risk of mortality, and a change in the views of the lung transplant community regarding age and transplant candidacy. The proportion of female lung transplant recipients markedly decreased since implementation of the LAS. In 2003, female candidates underwent 47.1% of all lung transplants, but only 39.5% by 2013 (Figure LU 3.5). This trend is largely explained by female candidates' lower LAS. Waitlist mortality rates were slightly higher for black candidates than for white candidates, 15.2 deaths per 100 waitlist years versus 14.4; however, for the first time this rate was substantially higher for Hispanics at 24.9 (Figure LU 1.10). Time will tell whether this trend is stable. OUTCOMES
Measures of short-term survival (30-day and 1-year) and long-term survival (3-year and 5-year) have plateaued since implementation of the LAS (Figure LU 5.2). Overall 5-year unadjusted patient survival was 53.6%, and varied by age (P < 0.0001), LAS at transplant (P = 0.0145), procedure type (P < 0.0001), and diagnosis group (P = 0.0030) (Figure LU 5.1). Survival was consistently lowest among recipients aged 65 years or older, those with LAS greater than 60, and those in diagnosis group B (Figure LU 5.1). Procedure choice also appeared to affect survival. Sur-
data behind the figures can be downloaded from our website, at srtr.transplant.hrsa.gov
4
OPTN/SRTR 2013 Annual Data Report
vival was worse for patients undergoing single lung transplant than for those undergoing bilateral lung transplant. However, importantly, these registry data regarding single and bilateral lung transplants were not adjusted for age, LAS, or diagnoses, variables that may mediate the observed survival differences. Patients undergoing transplant due to diagnoses that do not require bilateral transplant (e.g., alpha-1 antitrypsin deficiency, chronic obstructive pulmonary disease, idiopathic pulmonary fibrosis), and who therefore may undergo either type of transplant, also have improved survival after bilateral lung transplant compared with single lung transplant. Several complications can adversely affect the health of transplant recipients and likely contribute to mortality (Figure LU 5.5). In 2013, infection was the primary cause of mortality in lung transplant recipients in year 1 posttransplant (Figure LU 5.8). Incidence of graft failure and infection were the same at 5 years. Other respiratory causes (e.g., respiratory failure, acute respiratory distress, pulmonary embolism), cardiovascular/cerebrovascular disease, and malignancy were the other frequently reported causes of death in this population.
PEDIATRIC LUNG TRANSPLANT WAITING LIST
In 2013, the number of new child candidates (aged 0-11 years) added to the lung transplant waiting list increased to 39, and all but 3 were listed as active when they were added to the waiting list (Figure LU 6.1). The number of prevalent pediatric candidates (i.e., on the waiting list on December 31 of a given year) steadily decreased from a peak of 127 in 2004 to a total of 36 in 2013, when almost twice as many were inactive as were active. The largest age group of child candidates waiting during 2013 was aged 6 to 11 years; these candidates made up 65.6% of the waiting list (Figure LU 6.2). The proportion of candidates aged younger than 1 year had steadily increased to a peak of 24.5% in 2012, but decreased to 13.1% in 2013 (Figure LU 6.2). Of child lung candidates in 2013, 63.9% were on the waiting list for less than 1 year, 19.7% for 1 to less than 2 years, 6.6% for 2 to less than 4 years, and 9.8% for 4 or more years (Figure LU 6.2). Nearly 40% of candidates on the list on December 31, 2013, were aged 11 years or older, compared with 54.5% in 2003 (Figure LU 6.3). Compared with 2003, almost twice as many candidates were on the waiting list for less than 12 months in 2013. Of patients removed from the waiting list in 2013, 31 (66.0%) were removed due to undergoing transplant, 5 (10.6%) due to death, 3 (6.4%) due to improved condition, and 3 (6.4%) due to being too sick to undergo transplant (Figure LU 6.4). Regarding 3-year outcomes for pediatric lung transplant candidates listed in 2010, 62.9% underwent trans-
plant, 22.9% died waiting, 8.6% were still waiting, and 5.7% were removed from the list for reasons other than transplant or death (Figure LU 6.5). The overall child lung transplant rate peaked in 2013 at 175 per 100 waitlist years; 234 transplants per 100 waitlist years were performed in candidates aged younger than 6 years and 148 transplants per 100 waitlist years in candidates aged 6 to 11 years (Figure LU 6.6). Waitlist mortality in 2012-2013 was 17.5 per 100 waitlist years (Figure LU 6.7). Rates were higher for patients aged younger than 6 years than for patients aged 6 to 11 years, 24.7 versus 13.9 per 100 waitlist years TRANSPLANT
In 2013, a total of 28 lung transplants were performed in recipients aged 0 to 11 years, 3 for ages younger than 1 year, 9 for ages 1 to 5 years, and 16 for ages 6 to 11 years (Figure LU 6.8). Comparing 2001-2003 with 2011-2013 shows a larger proportion of pediatric transplant recipients aged 1 to 5 years, more pulmonary fibrosis diagnoses, and fewer pulmonary hypertension diagnoses (LU 6.9). Half of child lung transplant recipients in 2011-2013 were on the waiting list for 90 days or less. More recipients in 2011-2013 were on a ventilator, ECMO, or both at the time of transplant (40.7%), compared recipients in 2001-2003 (25.5%). The procedure of choice was bilateral transplant, performed in all patients. Medicaid coverage for pediatric lung transplant increased, with a corresponding decrease in private insurance coverage. Among pediatric lung transplant recipients from 2009 to 2013, 64.8% were cytomegalovirus (CMV) negative and 60.0% were Epstein-Barr virus (EBV) negative (Figure LU 6.10). The combination of a CMV-positive donor and a CMV-negative recipient occurred in 28.6% of transplants; this combination for EBV occurred in 35.2% of transplants. OUTCOMES
Patients survival was 100% at 30 days and 93.9% at 1 year for transplants in 2011-2012, 63.3% at 3 years for transplants in 2009-2010, 57.0% at 5 years for transplants in 2007-2008, and 38.2% at 10 years for transplants in 2001-2002 (Figure LU 6.14). For children who underwent transplant in 2001 to 2008, 1-, 3-, and 5-year patient survival was 80.6%, 65.0%, and 56.2%, respectively (Figure LU 6.16). By age, 5-year patient survival was poorest for recipients aged younger than 1 year. The diagnosis of pulmonary hypertension was associated with higher survival rates than cystic fibrosis or other diagnoses, including pulmonary fibrosis, bronchiolitis obliterans, and bronchopulmonary dysplasia. Infection was the leading cause of death in year 1 posttransplant and graft failure in years 2 to 5 (Figure LU 6.17). The incidence of first acute rejection increased over time posttransplant. The incidence within 1 year in recipients aged
5 lung
6 to 11 years was double that in younger recipients (Figure LU 6.15). The incidence of posttransplant lymphoproliferative disorder among EBV-negative recipients was 10.9% at 5 years posttransplant, compared with 2.6% among EBV-positive recipients (Figure LU 6.11). Five-year posttransplant complication rates in child lung transplant recipients were similar to complication rates in
adult recipients: hypertension (63.4%), bronchiolitis obliterans syndrome (26.8%), renal dysfunction (17.1%), hyperlipidemia (17.1%), diabetes (12.2%), and malignancy (7.3%) (Figure LU 6.12). For most child lung transplant recipients (90.2%), functional status was reported as ``no assistance needed'' at 5 years posttransplant (LU 6.12).
data behind the figures can be downloaded from our website, at srtr.transplant.hrsa.gov
6
OPTN/SRTR 2013 Annual Data Report
waiting list 4000
New patients Active Inactive All
Patients on list on Dec 31 each year Active Inactive All
3000 Patients
3000 Patients
4000
2000 1000
2000 1000
0
0 03
05
07 Year
09
11
13
03
05
07 Year
09
11
13
LU 1.1 Candidates aged 12 years or older waiting for lung transplant A new patient is one who first joined the list during the given year, without having been listed in a previous year. Previously listed candidates who underwent transplant and subsequently relisted are considered new. Candidates concurrently listed at multiple centers are counted once. Concurrently listed candidates who are active at any program are considered active; those who are inactive at all programs are considered inactive.
LU 1.2 Distribution of candidates aged 12 years or older actively waiting for lung transplant Candidates waiting for transplant any time in the given year. Candidates listed concurrently at multiple centers are counted once. Age is determined at the later of listing date or January 1 of the given year. Time on the waiting list is determined at the earlier of December 31 or removal from the waiting list. Medical urgency status is the most severe during the year. Only candidates who were active for at least 1 day are included.
7 lung
waiting list Age
Sex Race
Citizenship
Diagnosis group
Lung allocation score
Blood type
Height (cm)
Waiting time
Medical urgency Lung tx history Multi-organ
All candidates
12-17 18-34 35-49 50-64 65+ Female Male White Black Hispanic Asian Other/unknown US citizen Non-citizen resident Non-citizen non-resident Other/unknown A B C D Other/unknown < 30 30-< 35 35-< 40 40-< 50 50+ Unknown A B AB O < 150 150-
waiting list ...................... deceased donation ......... transplant ....................... donor-recipient matching outcomes ........................ pediatric transplant ......... transplant center maps....
6 10 11 14 15 18 25
M. Valapour1,2 , M. A. Skeans1 , B. M. Heubner1 , J. M. Smith1,3 , M. I. Hertz1,4 , L. B. Edwards5,6 , W. S. Cherikh5,6 , E. R. Callahan5,6 , J. J. Snyder1,7 , A. K. Israni1,7,8 , B. L. Kasiske1,8 1
Scientific Registry of Transplant Recipients, Minneapolis Medical Research Foundation, Minneapolis, MN
2
Department of Pulmonary Medicine, Respiratory Institute, Cleveland Clinic, Cleveland, OH
3
Department of Pediatrics, University of Washington, Seattle, WA
4
Department of Medicine, Division of Pulmonary, Allergy, Critical Care and Sleep Medicine, University of Minnesota, Minneapolis, MN
5
Organ Procurement and Transplantation Network, Richmond, VA
6
United Network for Organ Sharing, Richmond, VA
7
Department of Epidemiology and Community Health, University of Minnesota, Minneapolis, MN
8
Department of Medicine, Hennepin County Medical Center, University of Minnesota, Minneapolis, MN
OPTN/SRTR 2013 Annual Data Report:
lung
ABSTRACT Lungs are allocated to adult and adolescent transplant candidates (aged ě 12 years) on the basis of age, geography, blood type compatibility, and the lung allocation score (LAS), which reflects risk of waitlist mortality and probability of posttransplant survival. In 2013, the most adult candidates, 2394, of any year were added to the list. Overall median waiting time for candidates listed in 2013 was 4.0 months. The preferred procedure remained bilateral lung transplant, representing approximately 70% of lung transplants in 2013. Measures of short-term and longterm survival have plateaued since the implementation of the LAS in 2005. The number of new child candidates (aged 0-11 years) added to the lung transplant waiting list increased to 39 in 2013. A total of 28 lung transplants were performed in child recipients, 3 for ages younger than 1 year, 9 for ages 1 to 5 years, and 16 for ages 6 to 11 years. The diagnosis of pulmonary hypertension was associated with higher survival rates than cystic fibrosis or other diagnosis (pulmonary fibrosis, bronchiolitis obliterans, bronchopulmonary dysplasia). For child candidates, infection was the leading cause of death in year 1 posttransplant and graft failure in years 2 to 5.
KEY WORDS End-stage lung disease, lung allocation score, lung transplant, organ allocation, transplant outcomes.
I still catch myself on the street, or at a cafe, or wherever, and I'll think about how wonderful it feels to breathe —and I'll start welling up... I've had beautiful (donated) lungs for over 13 years now. I still think about them every single day, and about my donor. Steve, lung/kidney recipient
2
OPTN/SRTR 2013 Annual Data Report
INTRODUCTION As of June 30, 2013, nearly 11,000 recipients were living with a lung transplant in the US (Figure LU 5.3). Lung transplant is increasingly used as a treatment for patients who have end-stage lung diseases or are critically ill (Figures LU 3.1 and LU 3.5). Lungs are allocated to US transplant candidates aged 12 years or older primarily on the basis of age, geography, blood type (ABO) compatibility, and the lung allocation score (LAS). Implemented in 2005, the LAS calculation reflects the risk of waitlist mortality while avoiding transplants in candidates whose likelihood of survival is poor. The LAS applies to adolescent (aged 12-17 years) and adult (aged 18 years or older) candidates. As of June 10, 2013, pediatric candidates (aged younger than 12 years) can ask for an exception from the national Lung Review Board and be assigned a LAS to give primary access to lung offers from adult and adolescent donors. Therefore, the adult section below includes data for candidates and recipients aged 12 years or older and for 10 pediatric candidates who received an exception LAS. As part of the development of the LAS system, pulmonary diagnoses for candidates aged 12 years or older were categorized into four main groups based on survival probability and pathophysiology of the underlying disease. The four groups are: group A, obstructive lung disease (e.g., chronic obstructive lung disease/emphysema); group B, pulmonary vascular disease (e.g., idiopathic pulmonary arterial hypertension); group C, cystic fibrosis and immunodeficiency disorders; and group D, restrictive lung disease (e.g., idiopathic pulmonary fibrosis). The Organ Procurement and Transplantation Network (OPTN) Board of Directors approved the first comprehensive revision of the LAS calculation in November 2012;
the revised LAS will be implemented in February 2015. This revision includes modifications to the variables used in the LAS calculation and to the relative weight of the variables used to predict risk of death in the next year without transplant and in the first year after transplant. The revised LAS will further improve the survival prediction for all diagnostic groups; these effects will likely be most impactful for candidates in diagnosis group B.
ADULT LUNG TRANSPLANT WAITING LIST
After an initial decline immediately following implementation of the LAS system in May 2005, the number of new patients added to the waiting list has grown steadily, reaching the maximum of 2327 active new patients aged 12 years or older in 2013 (Figure LU 1.1). During the same time, the number of inactive candidates continued to decline every year and reached an all-time low of 302 on December 31, 2013 (Figure LU 1.1). Because the LAS system prioritizes the sickest patients for transplant, it changed listing practices in the lung transplant community and thus the characteristics of waitlisted candidates. Candidates on the waiting list are increasingly older, more likely to be from diagnosis group D, and more likely to have a higher LAS (Figure LU 1.2). Candidates aged 65 years or older make up an increasing proportion of the waiting list every year, to an all-time high of 23.9% of lung transplant candidates in 2013; proportions of all other adult age groups have declined. The proportion of candidates aged 18 to 34 years decreased from 16.0% of the waiting list in 2004 to 10.9% in 2013, the proportion aged 35 to 49 years from 25.0% to just
3 lung
13.7%, and the proportion aged 50 to 64 years from 52.0% to 49.5%. Adolescents aged 12 to 17 years make up a small proportion of the waiting list, at 2%. The proportion of candidates in diagnosis group D continues to increase every year, to an all-time high of 50.2% of the waiting list in 2013. All other diagnostic groups represented a smaller proportion of the waiting list in 2013 than in 2004; group A showed the most dramatic decline, from 42.7% in 2004 to 33.8% in 2013. Group A candidates also had the lowest median LAS at transplant (Figure LU 3.3). The trend in LAS distribution on the waiting list is toward higher scores; for example, the LAS was 50 to 100 for 13.1% of candidates in 2006 (the first full year after implementation) and for 28.0% in 2013 (Figure LU 1.2). Women made up approximately 60% of the waiting list in 2013, as before LAS implementation (Figure LU 1.3). Retransplant remains relatively uncommon. On December 31, 2013, 3.2% of candidates on the waiting list were listed for retransplant (Figure LU 1.3). Since the LAS was implemented, transplant rates have increased for all lung transplant candidates (Figure LU 1.4). Over the past 4 years, by height category, transplant rates have been consistently highest for candidates who are taller than 183 cm and lowest for candidates who are shorter than 160 cm. In 2013, the most candidates, 2394, were added to the list (Figure LU 1.1), and the most lung transplants among candidates aged 12 and older, 1918, were performed (Figure LU 3.5). Overall median waiting time for candidates first listed in 2013 was 4.0 months, varying from 2.6 months for group D candidates to 9.7 months for group B candidates (Figure LU 1.9). Despite this level of transplant activity, the overall waitlist mortality rate was 15.1 per 100 waitlist years for candidates awaiting transplant in 2012-2013, representing a plateau after a trend of increasing waitlist mortality rates since the LAS was implemented (Figure LU 1.10). Waitlist mortality rates in 2012-2013 remained highest for candidates aged 12 to 17 years, at 25.4 deaths per 100 waitlist years, an increase from 19.0 in 2010-2011 (Figure LU 1.10). Candidates aged 18 to 34 years followed at 21.0 deaths per 100 waitlist years. Diagnosis group D composed the largest proportion of the waiting list and had the highest waitlist morality rates. As expected, the waitlist mortality rate was nearly 10 times greater for candidates with LAS 50 or higher than for candidates with LAS less than 50 (Figure LU 1.10). Candidates with LAS less than 40 had nearly the same waitlist mortality rate, ranging from 3.6 to 6.2 deaths per 100 waitlist years (Figure LU 1.10). Since implementation of the LAS, the waitlist mortality rate has declined within every LAS group of 30 or higher. Because the distribution of LAS on the waiting list has shifted upwards, the overall waitlist mortality rate has increased, but rates are improving within every LAS grouping except less than 30 (< 1% of candidates).
TRANSPLANT
In 2013, 1946 total lung transplants were performed, including adult and pediatric recipients, the most ever in a single year (Figure LU 3.1). The preferred procedure remained bilateral lung transplant, representing approximately 70% of lung transplants in 2013 (Figure 3.5). Lung transplant recipients have been undergoing transplant with higher LAS (Figures LU 3.3). When the LAS system was implemented, the median LAS at transplant was 36.6; it increased to its highest median value of 43.0 in 2013. The distribution of the LAS at the time of transplant also shifted, with an increase in the seventy-fifth percentile scores to 58.7 in 2013. Median LAS at transplant was highest for candidates in diagnosis groups D and B, at 48.9 and 47.7, respectively, and lowest for candidates in group A, at 34.1. An important trend is the rising relative percentage of transplant recipients who are hospitalized in the intensive care unit at transplant (Figure LU 3.5): 14.1% in 2013 compared with 3.7% a decade earlier. In 2013, 4.8% of recipients were on extracorporeal membrane oxygenation (ECMO) at transplant. Since implementation of the LAS, men, candidates aged 65 years or older, and candidates in diagnosis group D have composed increasingly larger proportions of transplant recipients each year (Figure LU 3.2). In 2013, 28.7% of all US lung recipients were aged 65 years or older, compared with 7.2% in 2003, before implementation of the LAS (Figure LU 3.5). This shift may reflect both the LAS policy, which increases transplant access for patients at high risk of mortality, and a change in the views of the lung transplant community regarding age and transplant candidacy. The proportion of female lung transplant recipients markedly decreased since implementation of the LAS. In 2003, female candidates underwent 47.1% of all lung transplants, but only 39.5% by 2013 (Figure LU 3.5). This trend is largely explained by female candidates' lower LAS. Waitlist mortality rates were slightly higher for black candidates than for white candidates, 15.2 deaths per 100 waitlist years versus 14.4; however, for the first time this rate was substantially higher for Hispanics at 24.9 (Figure LU 1.10). Time will tell whether this trend is stable. OUTCOMES
Measures of short-term survival (30-day and 1-year) and long-term survival (3-year and 5-year) have plateaued since implementation of the LAS (Figure LU 5.2). Overall 5-year unadjusted patient survival was 53.6%, and varied by age (P < 0.0001), LAS at transplant (P = 0.0145), procedure type (P < 0.0001), and diagnosis group (P = 0.0030) (Figure LU 5.1). Survival was consistently lowest among recipients aged 65 years or older, those with LAS greater than 60, and those in diagnosis group B (Figure LU 5.1). Procedure choice also appeared to affect survival. Sur-
data behind the figures can be downloaded from our website, at srtr.transplant.hrsa.gov
4
OPTN/SRTR 2013 Annual Data Report
vival was worse for patients undergoing single lung transplant than for those undergoing bilateral lung transplant. However, importantly, these registry data regarding single and bilateral lung transplants were not adjusted for age, LAS, or diagnoses, variables that may mediate the observed survival differences. Patients undergoing transplant due to diagnoses that do not require bilateral transplant (e.g., alpha-1 antitrypsin deficiency, chronic obstructive pulmonary disease, idiopathic pulmonary fibrosis), and who therefore may undergo either type of transplant, also have improved survival after bilateral lung transplant compared with single lung transplant. Several complications can adversely affect the health of transplant recipients and likely contribute to mortality (Figure LU 5.5). In 2013, infection was the primary cause of mortality in lung transplant recipients in year 1 posttransplant (Figure LU 5.8). Incidence of graft failure and infection were the same at 5 years. Other respiratory causes (e.g., respiratory failure, acute respiratory distress, pulmonary embolism), cardiovascular/cerebrovascular disease, and malignancy were the other frequently reported causes of death in this population.
PEDIATRIC LUNG TRANSPLANT WAITING LIST
In 2013, the number of new child candidates (aged 0-11 years) added to the lung transplant waiting list increased to 39, and all but 3 were listed as active when they were added to the waiting list (Figure LU 6.1). The number of prevalent pediatric candidates (i.e., on the waiting list on December 31 of a given year) steadily decreased from a peak of 127 in 2004 to a total of 36 in 2013, when almost twice as many were inactive as were active. The largest age group of child candidates waiting during 2013 was aged 6 to 11 years; these candidates made up 65.6% of the waiting list (Figure LU 6.2). The proportion of candidates aged younger than 1 year had steadily increased to a peak of 24.5% in 2012, but decreased to 13.1% in 2013 (Figure LU 6.2). Of child lung candidates in 2013, 63.9% were on the waiting list for less than 1 year, 19.7% for 1 to less than 2 years, 6.6% for 2 to less than 4 years, and 9.8% for 4 or more years (Figure LU 6.2). Nearly 40% of candidates on the list on December 31, 2013, were aged 11 years or older, compared with 54.5% in 2003 (Figure LU 6.3). Compared with 2003, almost twice as many candidates were on the waiting list for less than 12 months in 2013. Of patients removed from the waiting list in 2013, 31 (66.0%) were removed due to undergoing transplant, 5 (10.6%) due to death, 3 (6.4%) due to improved condition, and 3 (6.4%) due to being too sick to undergo transplant (Figure LU 6.4). Regarding 3-year outcomes for pediatric lung transplant candidates listed in 2010, 62.9% underwent trans-
plant, 22.9% died waiting, 8.6% were still waiting, and 5.7% were removed from the list for reasons other than transplant or death (Figure LU 6.5). The overall child lung transplant rate peaked in 2013 at 175 per 100 waitlist years; 234 transplants per 100 waitlist years were performed in candidates aged younger than 6 years and 148 transplants per 100 waitlist years in candidates aged 6 to 11 years (Figure LU 6.6). Waitlist mortality in 2012-2013 was 17.5 per 100 waitlist years (Figure LU 6.7). Rates were higher for patients aged younger than 6 years than for patients aged 6 to 11 years, 24.7 versus 13.9 per 100 waitlist years TRANSPLANT
In 2013, a total of 28 lung transplants were performed in recipients aged 0 to 11 years, 3 for ages younger than 1 year, 9 for ages 1 to 5 years, and 16 for ages 6 to 11 years (Figure LU 6.8). Comparing 2001-2003 with 2011-2013 shows a larger proportion of pediatric transplant recipients aged 1 to 5 years, more pulmonary fibrosis diagnoses, and fewer pulmonary hypertension diagnoses (LU 6.9). Half of child lung transplant recipients in 2011-2013 were on the waiting list for 90 days or less. More recipients in 2011-2013 were on a ventilator, ECMO, or both at the time of transplant (40.7%), compared recipients in 2001-2003 (25.5%). The procedure of choice was bilateral transplant, performed in all patients. Medicaid coverage for pediatric lung transplant increased, with a corresponding decrease in private insurance coverage. Among pediatric lung transplant recipients from 2009 to 2013, 64.8% were cytomegalovirus (CMV) negative and 60.0% were Epstein-Barr virus (EBV) negative (Figure LU 6.10). The combination of a CMV-positive donor and a CMV-negative recipient occurred in 28.6% of transplants; this combination for EBV occurred in 35.2% of transplants. OUTCOMES
Patients survival was 100% at 30 days and 93.9% at 1 year for transplants in 2011-2012, 63.3% at 3 years for transplants in 2009-2010, 57.0% at 5 years for transplants in 2007-2008, and 38.2% at 10 years for transplants in 2001-2002 (Figure LU 6.14). For children who underwent transplant in 2001 to 2008, 1-, 3-, and 5-year patient survival was 80.6%, 65.0%, and 56.2%, respectively (Figure LU 6.16). By age, 5-year patient survival was poorest for recipients aged younger than 1 year. The diagnosis of pulmonary hypertension was associated with higher survival rates than cystic fibrosis or other diagnoses, including pulmonary fibrosis, bronchiolitis obliterans, and bronchopulmonary dysplasia. Infection was the leading cause of death in year 1 posttransplant and graft failure in years 2 to 5 (Figure LU 6.17). The incidence of first acute rejection increased over time posttransplant. The incidence within 1 year in recipients aged
5 lung
6 to 11 years was double that in younger recipients (Figure LU 6.15). The incidence of posttransplant lymphoproliferative disorder among EBV-negative recipients was 10.9% at 5 years posttransplant, compared with 2.6% among EBV-positive recipients (Figure LU 6.11). Five-year posttransplant complication rates in child lung transplant recipients were similar to complication rates in
adult recipients: hypertension (63.4%), bronchiolitis obliterans syndrome (26.8%), renal dysfunction (17.1%), hyperlipidemia (17.1%), diabetes (12.2%), and malignancy (7.3%) (Figure LU 6.12). For most child lung transplant recipients (90.2%), functional status was reported as ``no assistance needed'' at 5 years posttransplant (LU 6.12).
data behind the figures can be downloaded from our website, at srtr.transplant.hrsa.gov
6
OPTN/SRTR 2013 Annual Data Report
waiting list 4000
New patients Active Inactive All
Patients on list on Dec 31 each year Active Inactive All
3000 Patients
3000 Patients
4000
2000 1000
2000 1000
0
0 03
05
07 Year
09
11
13
03
05
07 Year
09
11
13
LU 1.1 Candidates aged 12 years or older waiting for lung transplant A new patient is one who first joined the list during the given year, without having been listed in a previous year. Previously listed candidates who underwent transplant and subsequently relisted are considered new. Candidates concurrently listed at multiple centers are counted once. Concurrently listed candidates who are active at any program are considered active; those who are inactive at all programs are considered inactive.
LU 1.2 Distribution of candidates aged 12 years or older actively waiting for lung transplant Candidates waiting for transplant any time in the given year. Candidates listed concurrently at multiple centers are counted once. Age is determined at the later of listing date or January 1 of the given year. Time on the waiting list is determined at the earlier of December 31 or removal from the waiting list. Medical urgency status is the most severe during the year. Only candidates who were active for at least 1 day are included.
7 lung
waiting list Age
Sex Race
Citizenship
Diagnosis group
Lung allocation score
Blood type
Height (cm)
Waiting time
Medical urgency Lung tx history Multi-organ
All candidates
12-17 18-34 35-49 50-64 65+ Female Male White Black Hispanic Asian Other/unknown US citizen Non-citizen resident Non-citizen non-resident Other/unknown A B C D Other/unknown < 30 30-< 35 35-< 40 40-< 50 50+ Unknown A B AB O < 150 150-
