The annual Reports of the International Society for Heart and Lung Transplantation (ISHLT) International Thoracic Organ Transplant Registry traditionally detail analyses using a common theme across all thoracic organ transplants, using the most current data reported to the Registry. Due to a changing regulatory environment, with the Registry undergoing an update in data acquisition, the 2020 and 2021 Registry Reports were derived using the data examined in the 2019 annual Reports.1–4 The 2020 and 2021 Reports on pediatric lung transplantation presented trends in the characteristics of donors and recipients, and associated post-transplant outcomes, respectively.5,6 Due to recent developments in pediatric lung transplantation that will be discussed in this report, pulmonary vascular diseases (PVDs) is the focus chosen for this year. This 25th annual Pediatric Lung and Heart-Lung Transplant Report used data submitted to the Registry on 2,323 pediatric recipients of deceased donor lung transplants and 454 pediatric recipients of deceased donor heart-lung transplants between January 1, 1992, and June 30, 2018.
Rather than exploring a common theme across Registry Reports, the Reports for this year focus on key topics relative to each respective adult and pediatric thoracic transplant group. Due to recent literature demonstrating a rising number of lung transplants in children with idiopathic pulmonary arterial hypertension (IPAH) and pulmonary hypertension (PH)-not IPAH,7 PVDs were chosen as the focus for the 25th annual Pediatric Lung and Heart-Lung Transplant Report. We refer the reader to the 2019 Report on pediatric lung transplantation for a detailed description of the full cohort and additional core analyses not directly related to the focus explored in this year’s Report. The Registry slide set available online (https://ishlt.org/research-data/registries/ttx-registry/ttx-registry-slides) provides more detail, additional analyses, and other information not included in this printed Report.
Statistical methods
Data collection, conventions, and statistical methods
Since the Registry’s inception, 481 heart transplant centers, 260 lung transplant centers, and 184 heart-lung transplant centers have reported data to the Registry. The results reported herein aim to provide as granular detail as possible, with data retained in the ISHLT Registry for pediatric lung and heart-lung transplants through June 30, 2018. With the current report examining the same patient cohort as the 2019 Report,1 an overview of children with PVDs and post-transplant outcomes is performed. Historically, cystic fibrosis (CF) has been the most common indication for a lung transplant in children, followed by PVDs.1 However, a paradigm shift occurred in CF with the release of elexacaftor/teza-caftor/ivacaftor therapy in November 2019, with a significant reduction in the number of pediatric lung transplants performed for CF in the United States in both 2020 and 2021.7 In this same study, the authors detailed an upward trend in the number of lung transplants in children with PVDs including IPAH and PH-not IPAH as well as ILD.7 This increase in lung transplants performed in children with PH is occurring despite the availability of an alternative surgical option with the placement of a Potts shunt.8
There is a paucity of data on post-transplant outcomes for children with PVDs, especially PH-not IPAH. In one study, pediatric lung and heart-lung transplant recipients with IPAH were found to have similar outcomes to children transplanted with other diagnoses.9 The authors found that only 65 children under 18 years of age underwent a lung or heart-lung transplant between May 2005 and December 2015 in the US and concluded this surgical treatment option is underutilized. The goal of this year’s focused report is to describe trends in the characteristics of pediatric lung and heart-lung transplant recipients with PVDs (IPAH and PH-not IPAH) and to identify important recipient and transplant characteristics that are associated with post-transplant survival at 1 year, post-transplant survival at 5 years conditional upon surviving 1 year, and freedom from bronchiolitis obliterans syndrome (BOS)/chronic lung allograft dysfunction (CLAD) conditional upon surviving to discharge after transplant. This year’s pediatric lung transplant report refers to specific online e-slides when particular data are discussed but not shown due to space limitations; eSlide L(p) refers to the online pediatric lung transplant slides.
The ISHLT TTX Registry website (https://ishlt.org/research-data/registries/ttx-registry#data-fields-look-up-tables-forms) provides detailed spreadsheets of the data elements collected in the Registry before 2019. The Registry requires submission of core donor, recipient, and transplant procedure variables around the time of transplantation and at yearly follow-up. Therefore, these variables have low rates of missing data. Nevertheless, data quality depends on the accuracy and completeness of reporting. Rates of missingness may significantly increase for Registry variables that rely on voluntary reporting. However, the Registry uses various quality control measures to ensure acceptable data quality and completeness before including data for analyses.
Analytical conventions
For this year’s report, analyses of pediatric lung and heart-lung transplants do not include data for combined lung with other organ transplants. In addition, the Registry does not capture the exact occurrence date for most secondary outcomes (e.g., BOS/CLAD), but it does capture the event within a period of time (i.e., between the first and the second-year annual follow-up visits). For the report’s analyses, we use the mid-point between the annual follow-ups as a surrogate for the event date. On follow-up where a death is reported, some under-reporting of secondary outcomes and other information is highly probable. Thus, we restrict some analyses to include only surviving recipients to reduce the potential of underestimating event rates or other outcomes. For time-to-event analyses, we censor the follow-up of recipients who do not experience the event of interest at the last time the recipient was reported not to have had the event, which would either be the most recent annual follow-up or the time of re-transplantation. We truncate time-to-event graphs (e.g., survival graphs) when the number of individuals at risk becomes <10. Finally, the locations of transplant centers were divided into Europe, North America, and Other regions (e.g., South America, Asia, the Middle East, Australia, and others).
Recipient characteristics
With an objective to assess changes in characteristics of pediatric recipients with PVDs over time, we examined the associations of these changes with outcomes. Table 1 (eSlide L(p) 4) outlines the diagnosis distribution of pediatric lung and heart-lung transplant recipients across diagnoses between January 1992 and June 2018 by era. For our analysis, we defined the eras as January 1992 to December 2000 (N = 855), January 2001 to December 2009 (N = 952), and January 2010 to June 2018 (N = 970). have Over time, there been variations by era regarding diagnoses for transplant, with the most recent trends comparing January 2001-December 2009 to January 2010-June 2018 showing increases in patients with a diagnosis of PVD, especially IPAH, as well as ILD and those classified as Other (e.g., non-CF bronchiectasis, obliterative bronchiolitis, bronchopulmonary dysplasia, etc.). In contrast, there has been a reduction in the number of transplants for patients with CF during the January 2010 to June 2018 era. These findings support a recent publication that described an increasing number of lung transplants in children with PVDs and ILD and decreasing numbers in children with CF in the United States.7
Table 1.
Diagnosis Distribution by Era (Transplants: January 1992-June 2018)
| Jan 1992-Dec 2000 (N = 855) | Jan 2001-Dec 2009 (N = 952) | Jan 2010-Jun 2018 (N = 970) | p-value | |
|---|---|---|---|---|
| Diagnosis | <0.0001 | |||
| • Pulmonary vascular disease (PVD) | 247 (30.8%) | 162 (17.9%) | 194 (20.5%) | |
| • Idiopathic pulmonary arterial hypertension (IPAH) | 107 (13.3%) | 105 (11.6%) | 135 (14.3%) | |
| Pulmonary hypertension (PH)-notIPAH | 140 (17.5%) | 57 (6.3%) | 59 (6.2%) | |
| Cystic Fibrosis (CF) | 391 (48.8%) | 504 (55.6%) | 482 (51.0%) | |
| Obstructive bronchiolitis (OB non-Retransplant) | 14 (1.7%) | 51 (5.6%) | 49 (5.2%) | |
| Interstitial lung disease (ILD not IIP) | 19 (2.4%) | 44 (4.9%) | 54 (5.7%) | |
| Retransplant | 51 (6.4%) | 56 (6.2%) | 49 (5.2%) | |
| Other | 80 (10.0%) | 89 (9.8%) | 118 (12.5%) |
Continuous factors are expressed as median (5th–95th percentiles).
Summary statistics included transplants with non-missing data.
Diagnosis categories were compared across eras using the chi-square statistic.
Table 2 (eSlides L(p) 5–8) outlines recipient characteristics of children with PVDs (IPAH and PH-not IPAH) by era. Over time, more lung and heart-lung transplants are being performed in children with PVDs in Europe and Other regions, whereas fewer are being performed in North America. Contemporary practice has shifted toward lung transplantation over heart-lung transplantation for children with PVDs. Current trends demonstrate an increase in female donors for both male and female pediatric recipients. Notably, more children with PVDs are undergoing transplantation while on extracorporeal membrane oxygenation (ECMO) in the modern era, suggesting sicker patients are being transplanted today than before. More children on ECMO may explain the observation that more recipients are being allosensitized with elevated panel reactive antibody (PRA) levels. Although children with PVDs in the modern era are on ECMO at the time of transplant, there is less renal and hepatic dysfunction.
Table 2.
Recipient Characteristics by Era (PVD Transplants: January 1992-June 2018)
| Jan 1992-Dec 2000 (N = 247) | Jan 2001-Dec 2009 (N = 162) | Jan 2010-Jun 2018 (N = 194) | p-value | |
|---|---|---|---|---|
| Geographic location | ||||
| - Europe | 28.7% | 29.0% | 46.4% | <0.0001 |
| - North America | 67.6% | 63.6% | 43.8% | |
| - Other | 3.6% | 7.4% | 9.8% | |
| Age (years) (continuous) | 11.0 (0.0–17.0) | 13.0 (0.0–17.0) | 13.0 (0.0–17.0) | 0.071 |
| Age (years) (categorical) | ||||
| - <1 | 10.9% | 9.9% | 5.7% | 0.206 |
| - 1–5 | 23.1% | 16.7% | 22.7% | |
| - 6–10 | 14.6% | 11.7% | 12.9% | |
| - 11–17 | 51.4% | 61.7% | 58.8% | |
| Male | 45.7% | 41.4% | 43.8% | 0.681 |
| Weight (kg) | 53.0 (41.0–78.0) | 54.7 (40.9–82.0) | 53.0 (41.0–78.6) | 0.664 |
| Height (cm) | 163.0 (148.0–180.0) | 162.0 (149.9–181.3) | 162.0 (146.0–180.0) | 0.836 |
| BMI (kg/m2) | 19.7 (15.6–27.7) | 19.9 (15.9–27.8) | 20.1 (15.9–31.2) | 0.444 |
| ABO blood type | ||||
| - A | 44.5% | 46.9% | 35.6% | 0.368 |
| - AB | 4.1% | 5.6% | 6.7% | |
| - B | 9.8% | 9.3% | 11.9% | |
| - O | 41.6% | 38.3% | 45.9% | |
| Donor-recipient sex match | ||||
| - Female-female | 27.5% | 28.6% | 33.0% | 0.022 |
| - Female-male | 15.4% | 16.8% | 25.3% | |
| - Male-female | 26.7% | 29.8% | 23.2% | |
| - Male-male | 30.4% | 24.8% | 18.6% | |
| Transplant procedure | ||||
| - Lung | 40.1% | 57.4% | 80.9% | <0.0001 |
| - Heart-Lung | 59.9% | 42.6% | 19.1% | |
| PRA ≥ 20% | 7.1% | 5.2% | 16.4% | 0.0375 |
| PRA ≥ 80% | 2.1% | 3.9% | 0.0% | 0.2684 |
| Previous cardiothoracic surgery | 34.9%a | 35.8% | 33.3% | 0.9348 |
| Hospitalized | 41.4% | 45.5% | 47.7% | 0.599 |
| Ventilator use | 19.5% | 19.6% | 19.3% | 0.9985 |
| ECMO use | 4.3% | 5.6% | 13.3% | 0.0349 |
| CMV antibody positive | 17.8% | 30.5% | 26.8% | 0.0588 |
| EBV antibody positive | 50.0%b | 52.1% | 45.6% | 0.6695 |
| Hepatitis B antibody positive | 2.0%a | 5.0% | 2.6% | 0.493 |
| Hepatitis C antibody positive | 2.0%a | 2.2% | 0.0% | 0.3778 |
| Bilirubin (mg/dl) | 0.7 (0.3–2.0)a | 0.5 (0.2–1.7) | 0.4 (0.2–1.2) | 0.0007 |
| Creatinine(mg/dl) | 0.5 (0.2–1.1)a | 0.6 (0.2–1.1) | 0.4 (0.1–1.0) | 0.0014 |
| GFR (ml/min/1.73 m2)c | 88.8 (57.1–119.1)a | 89.3 (55.9–156.4) | 96.9 (59.4–163.1) | 0.082 |
| PCW mean (mmHg) | 13.5 (4.0–35.0)a | 12.0 (4.0–32.0) | 11.0 (6.0–21.0) | 0.278 |
| PA mean (mmHg) | 58.0 (19.5–79.5)a | 60.5 (16.0–80.0) | 59.0 (13.0–80.0) | 0.6798 |
| PVR (woods unit) | 17.2 (3.6–37.3)a | 13.3 (0.8–27.7) | 8.9 (0.4–31.9) | 0.4378 |
| FEV1% predicted | 47.0 (26.0–69.0) | 72.0 (47.0–99.0) | 76.0 (42.0–99.0) | 0.0047 |
| FVC% predicted | 53.0 (30.0–86.0) | 78.0 (50.0–104.0) | 80.0 (50.0–102.0) | 0.0286 |
Continuous factors are expressed as median (5th–95th percentiles).
Summary statistics included transplants with known/non-missing data.
Based on Apr 1994-Dec 2000 transplants.
Based on Oct 1999-Dec 2000 transplants.
GFR was estimated using the modified Schwartz formula.Abbreviations: BMI, body mass index; CMV, cytomegalovirus; EBV, Epstein Barr virus; ECMO, extracorporeal membrane oxygenation; FEV1, forced expiratory volume in one second; FVC, forced vital capacity; GFR, Glomerular Filtration Rate; PA, pulmonary artery pressure; PCW, pulmonary capillary wedge; PRA, panel reactive antibody; PVR, pulmonary vascular resistance.
Interestingly, our analysis found that the traditional measures of PH severity by heart catheterization, which include mean pulmonary artery pressure (PAP) and pulmonary vascular resistance (PVR), have not changed over time despite sicker children due to PVDs underwent a transplant. This observation is supported by previous literature that identified that the traditional measure of mean PAP in IPAH disease severity does not accurately reflect the likely-hood of longer-term survival for these children after lung or heart-lung transplant.9 Spirometric data were collected and analyzed in children with PVDs. Both forced vital capacity (FVC) percent predicted and forced expiratory volume in one second (FEV1) percent predicted to have increased over time, which suggests a changing phenotype in the pediatric PVD population.
Figure 1 (eSlide L(p) 9) shows recipient diagnosis distribution by location and era. In the most recent era, developing trends include a decreasing number of transplants for CF in all 3 regions, but less so in North America, and increasing numbers of transplants in children for IPAH in Europe and Other Regions and for other diagnoses in North America and Other Regions. Notably, the higher variation in Other Regions may reflect the initiation of transplant programs in new parts of the world. Figure 2A (eSlide L(p) 10) shows rise and fall in the median recipient age over time for children with PVDs who underwent lung or heart-lung transplantation, while the median recipient body mass index (BMI) appears to have an upward trajectory (Figure 2B, eSlide L(p)10). Additional recipient and donor characteristics for children with PVDs who underwent a lung or a heart-lung transplant that are not discussed in the report are provided in the Registry slide set available online, including the recipient sex distribution by location and era (eSlide L(p) 11), donor-recipient sex distribution by location and era (eSlide L(p) 12), and recipient ABO blood type distribution by location and era (eSlide L(p) 13).
Figure 1.
Diagnosis distribution by location and era (transplants: January 1992-June 2018).
Figure 2.
Median recipient (A) age (PVD transplant: January 1992-June 2018), (B) body mass index (transplant: January 1992-June 2018), by year.
Table 3 (eSlide L(p) 14) outlines the causes of death of pediatric lung and heart-lung transplant recipients with PVDs at key time periods after transplant (≤1 year, 1–5 years, and >5 years). During the first post-transplant year, graft failure was the primary cause of death, followed by infection and cardiovascular complications. Beyond this first year, BOS/CLAD or the pathologic surrogate obliterative bronchiolitis becomes the primary cause of death, followed by graft failure and infection. Re-transplantation after lung transplant is becoming more commonplace in both adult and pediatric lung transplant recipients.1,5–7 Figure 3 (eSlide L(p) 15) shows the rising cumulative incidence of re-transplant for children with PVDs.
Table 3.
Recipient Causes of Death (PVD Transplants: January 1992-June 2018).
| Cause of Death | ≤1 Year (N = 130) | 1 Year-5 Years (N = 101) | >5 Years (N = 51) |
|---|---|---|---|
| OB/BOS | 5 (3.8%) | 45 (44.6%) | 21 (41.2%) |
| Acute rejection | 7 (5.4%) | 2 (2.0%) | 1 (2.0%) |
| Malignancy | 2 (1.5%) | 3 (3.0%) | 1 (2.0%) |
| Infection | 21 (16.2%) | 11 (10.9%) | 3 (5.9%) |
| Graft failure | 35 (26.9%) | 24 (23.8%) | 15 (29.4%) |
| Cardiovascular | 19 (14.6%) | 3 (3.0%) | 4 (7.8%) |
| Multiple organ failure | 15 (11.5%) | 5 (5.0%) | 2 (3.9%) |
| Other | 26 (20.0%) | 8 (7.9%) | 4 (7.8%) |
Abbreviation: OB/BOS, obliterative bronchiolitis/bronchiolitis obliteran syndrome.
Figure 3.
Cumulative incidence of retransplant (primary PVD transplants: January 1995-June 2017).
Survival
Survival within 12 months of transplantation
To determine how recipient characteristics were associated with shorter-term post-lung transplant survival in pediatric lung transplant recipients, we used univariate analysis with Kaplan-Meier curves to assess survival within 12 months of lung transplantation in children between January 2000 and June 2017. The cohorts were divided into 2 eras: 2000–2008 and 2009–2017. Survival for pediatric lung and heart-lung transplant recipients with PVDs within 12 months had a trend toward higher survival in the more recent era that did not reach statistical significance (Figure 4, eSlide L(p) 17). Expanding our analysis to include location, significant differences in 12-month survival in Europe and North America by era (eSlide L(p) 18) were not detected. Next, we stratified recipients by age (0–5 years, 6–10 years, and 11–17 years) and did not identify survival differences within 12 months of transplant across these age groups (Figure 5, eSlide L(p) 19).
Figure 4.
Kaplan-Meier survival within 12 months by era (PVD transplants: January 2000-June 2017).
Figure 5.
Kaplan-Meier survival within 12 months by recipient age (PVD transplants: January 2000-June 2017).
Comparing transplant recipients across diagnoses, we found that children with CF had better survival within 12 months of a transplant than children with PVDs (Figure 6, eSlide L(p) 20). We further examined this survival difference between CF and PVDs by differentiating PVDs into IPAH and PH-not IPAH and determined that the PH-not IPAH cohort of patients had the lowest survival at 12 months (Figure 7, eSlide L(p) 21).
Figure 6.
Kaplan-Meier survival within 12 months by diagnosis (transplants: January 2000-June 2017).
Figure 7.
Kaplan-Meier survival within 12 months by diagnosis (transplants: January 2000-June 2017).
The median estimated glomerular filtration rate (eGFR) was higher in the more recently transplanted pediatric lung recipients,1,5,6 so we explored the effect of recipient eGFR on the 12-month survival after lung or heart-lung transplant in children with PVDs. Using eGFR of 90 ml/min/1.73 m2 as a reference point, our analysis did not detect an effect of eGFR on shorter-term survival for these pediatric recipients (Figure 8, eSlide L(p) 22). Additionally, we found no difference in survival within 12 months of transplant, comparing lung transplant to heart-lung transplant in children with PVDs (Figure 9, eSlide L(p) 23).
Figure 8.
Kaplan-Meier Survival within 12 months by recipient estimated GFR (PVD transplants: January 2000-June 2017). GFR was estimated using the modified Schwartz formula.
Figure 9.
Kaplan-Meier survival within 12 months by procedure type (PVD transplants: January 2000-June 2017).
Survival within 5 years of transplantation conditional on survival to 1 year
To explore the effect of recipient characteristics on longer-term survival after lung and heart-lung transplant in children with PVDs, we used univariate analysis with Kaplan-Meier survival curves to determine survival within 5 years of transplantation conditional on survival to 1 year. The cohorts were divided into 2 eras: 1996–2004 and 2005–2013. As seen with our shorter-term survival analysis, there was a non-statistically significant difference in longer-term survival in the modern era for pediatric lung and heart-lung transplant recipients with PVDs (Figure 10 (eSlide L(p) 25). Stratifying recipients by age (0–5 years, 6–10 years, and 11–17 years), no difference in survival within 5 years of transplantation conditional on survival to 1 year was detected across these age groups (Figure 11, eSlide L(p) 26).
Figure 10.
Kaplan-Meier survival within 5 years conditional on survival to 1 year by era (PVD transplants: January 1996-June 2013).
Figure 11.
Kaplan-Meier survival within 5 years conditional on survival to 1 year by recipient age (PVD transplants: January 1996-June 2013).
Contrasting our findings to shorter-term outcomes, our longer-term transplant survival analysis found no differences across diagnoses, including PVDs, CF, and Other (e.g., non-CF bronchiectasis, obliterative bronchiolitis, bronchopulmonary dysplasia, etc.) (Figure 12, eSlide L(p) 27). We further differentiated the diagnosis of PVDs into IPAH and PH-not IPAH in our analysis, and did not observe a significant difference in survival within 5 years of transplantation conditional on survival to 1 year (Figure 13, eSlide L(p) 28). Moreover, there was no effect between recipient eGFR and longer-term survival (Figure 14, eSlide L(p) 29). In comparison to survival within 12 months of transplant, our longer-term analysis in this patient cohort found that lung transplant had a trend toward higher survival versus heart-lung transplant that did not reach statistical significance (Figure 15, eSlide L(p) 30).
Figure 12.
Kaplan-Meier survival within 5 years conditional on survival to 1 year by diagnosis (transplants: January 1996-June 2013).
Figure 13.
Kaplan-Meier survival within 5 years conditional on survival to 1 year by diagnosis (transplants: January 1996-June 2013).
Figure 14.
Kaplan-Meier survival within 5 years conditional on survival to 1 year by recipient estimated GFR (PVD transplants: January 1996-June 2013). GFR was estimated using the modified Schwartz formula.
Figure 15.
Kaplan-Meier survival within 5 years conditional on survival to 1 year by procedure type (PVD transplants: January 1996-June 2013).
Freedom from bronchiolitis obliterans syndrome conditional on survival to discharge
Due to the effect of BOS/CLAD on longer-term survival after lung transplantation,10,11 we examined freedom from BOS/CLAD conditional on survival to discharge by era and recipient age for transplants between January 1996 and June 2013 with the cohorts divided into 2 eras: 1996–2004 and 2005–2013. Figure 16 (eSlide L(p) 32) shows no difference in freedom from BOS/CLAD conditional on survival to discharge across these 2 eras. Stratifying recipients by age (0–5 years, 6–10 years, and 11–17 years at transplant), no difference in freedom from BOS/CLAD conditional on survival to discharge was identified (Figure 17, eSlide L(p) 33).
Figure 16.
Kaplan-Meier freedom from bronchiolitis obliterans syndrome/chronic lung allograft dysfunction (BOS/CLAD) conditional on survival to discharge by era (PVD transplants: January 1996-June 2013).
Figure 17.
Kaplan-Meier freedom from bronchiolitis obliterans syndrome/chronic lung allograft dysfunction (BOS/CLAD) conditional on survival to discharge by recipient age (PVD transplants: January 1996-June 2013).
Multivariable Cox analyses
We next performed multivariable Cox proportional hazards regression analyses to identify independent risk factors associated with mortality within 12 months of transplantation and within 5 years of transplantation conditional on survival to 1 year for pediatric lung and heart-lung transplant recipients with PVDs. Covariates included in the multivariable models are listed in Supplemental Table 1. These analyses establish independent associations between risk factors and outcomes but cannot establish causality. It is important to acknowledge that risk factors for post-transplant mortality differ by time since transplant, and longer-term data reflect patients transplanted in earlier eras, and the findings may not apply to current conditions. Therefore, specific associations observed among these data should be interpreted with caution.
Assessing children with PVDs who underwent lung and heart-lung transplantation between January 1990 and June 2017, significant categorical risk factors for 1-year mortality were a diagnosis of PH-not IPAH versus IPAH and ventilator use at transplant (Figure 18, eSlide L(p) 35). Therefore, clinicians need to remain aware of the risk of lower survival for patients with PH-not IPAH or patients on mechanical ventilation at the time of lung or heart-lung transplant among children with PVDs. A continuous risk factor significantly associated with higher 1-year mortality was lower recipient eGFR (eSlide L(p) 37).
Figure 18.
Statistically significant categorical risk factors for 5-year mortality conditional on survival to 1 year with 95% confidence limits (PVD transplants: January 1987-June 2013, N = 411).
When we examined 5-year survival conditional on surviving 1 year among children who underwent a transplant between 1987 and June 2013, we found no recipient or donor factors significantly associated with survival. However, recent transplant eras, 2005–6/2013 and 1996–2004, were both associated with lower risk of mortality compared to the earliest era, 1987–1995 (eSlide L(p) 39), a reassuring finding showing that outcomes have improved over time.
Conclusions
The 2022 ISHLT Registry Report on pediatric lung and heart-lung transplantation provides an update on key factors related to children who underwent a transplant for PVDs. As we witness changes in the demographics of patients undergoing lung or heart-lung transplantation, it is essential to examine post-transplant outcomes for children with PVDs as the need for a transplant in this patient population is increasing.7 Therefore, children with PVDs will be vital to facilitating further advancements in pediatric lung and heart-lung transplantation. Due to the relatively small sample size, these results should be interpreted cautiously, even if we found statistical significance. Despite this limitation, this year’s report provides insight into important variables regarding pediatric lung and heart-lung recipients that can help clinicians with limited available evidence in children with PVDs.
Supplementary Material
Acknowledgment
The authors wish to thank Ms. Lyna Cherikh, United Network of Organ Sharing Research Department Intern, for her assistance with preparing the figures/table for the manuscript and reviewing the manuscript.
Disclosure statement
Michael Perch received an institutional research grant from Roche, Ambu, Zambon, consultant fees from Takeda, Zambon, PulmonX, Mallinkrodt, GSK, Novartis, Boeringer-Ingelheim. Michael O. Harhay received consulting fees from Trinity life sciences. Luciano Potena received consulting fees from Biotest, Novartis, Takeda, Sandoz and CareDx. Andreas Zuckermann served on the speakers bureau of Paragonix, Mallinckrodt, Medtronik and Franz Kohler Chemie, and received research grants from Biotest and Xvivo. Josef Stehlik received consulting fees for Medtronic, Natera, Sanofi-Aventis, Transmedics and research support from Natera; Wida S. Cherikh, Aparna Sadavarte, Kelsi Lindblad and Anne Zehner received funding from ISHLT; Don Hayes, Jr., Eileen Hsich and Tajinder P. Singh do not have any relevant financial disclosures.
Footnotes
Supplementary materials
Supplementary material associated with this article can be found in the online version at https://doi.org/10.1016/j.healun.2022.07.020.
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