Indications for pediatric lung transplantation in 2025: A new era

Abstract

The year 2025 marks an important landmark: almost 40 years since the first pediatric lung transplant (LTX), over 3-5 years since the availability of elexacaftor/tezacaftor/ivacaftor in several countries, and 5-10 years since striking shifts were reported in the diagnoses that accounted for pediatric LTX. We review historic indications for pediatric LTX, highlighting shifts in these over time, and analyze data from the ISHLT International Thoracic Organ Transplant Registry, United Network of Organ Sharing, Canadian Cystic Fibrosis (CF) Registry, and other databases up to the present day. Currently, pediatric CF-related LTX cases are at record lows in many countries. Non-retransplant bronchiolitis obliterans seems to be on the rise as a transplant indication in pediatrics, which is particularly true in the younger age group per ISHLT data. Childhood interstitial lung disease is increasing as an indication, especially in North America. Idiopathic pulmonary arterial hypertension (IPAH) and pulmonary hypertension as a whole now account for record highs as indications for pediatric LTX around the world, with IPAH alone now accounting for nearly 20% of pediatric LTX in the United States, for instance. This information will help guide future international pediatric thoracic transplant consensus guidelines around candidate selection and optimization, placing more emphasis on non-CF considerations.

Keywords: Cystic fibrosis; Indications; Interstitial lung disease; Lung transplant; Pediatric; Pulmonary hypertension.

Figure 1

ISHLT International Thoracic Organ Transplant Registry Report 2021. Pediatric lung transplant recipient diagnosis distribution by era and location, 1992-2018. CF, cystic fibrosis; COPD, chronic obstructive pulmonary disease; IPF, idiopathic pulmonary fibrosis; ISHLT, International Society for Heart and Lung Transplantation.

Figure 2

ISHLT International Thoracic Organ Transplant Registry Report 2022. Pediatric lung and heart-lung transplant recipient diagnosis distribution by era and location, 1992-2018. CF, cystic fibrosis; IIP, idiopathic interstitial pneumonia; ILD, interstitial lung disease; IPAH, idiopathic pulmonary arterial hypertension; ISHLT, International Society for Heart and Lung Transplantation; OB, obliterative bronchiolitis; PH, pulmonary hypertension; Retx, retransplant.

Figure 3

Number of pediatric lung transplants for cystic fibrosis over time from 2013 to 2024 inclusive, United Network of Organ Sharing data. CF, cystic fibrosis.

Figure 4

Percentage of total pediatric lung transplants (%) attributed to cystic fibrosis over time from 2003 to 2024 inclusive, United Network of Organ Sharing data. Linear regression trendline in black. Note y axis upper limit is 80%. CF, cystic fibrosis.

Figure 5

The Canadian Cystic Fibrosis Registry 2022 Annual Data Report. Number of solid organ transplants, almost exclusively for lung transplantation or combined lung with another organ, 2003-2022. Includes pediatric and adult transplants.

Figure 6

Number of pediatric lung transplants for interstitial lung disease over time from 1995 to 2024 inclusive, United Network of Organ Sharing data. ILD, interstitial lung disease.

Figure 7

Percentage of total pediatric lung transplants (%) attributed to interstitial lung disease over time from 1995 to 2024 inclusive, United Network of Organ Sharing data. Linear regression trendline in black. Note y axis upper limit is 40%. ILD, interstitial lung disease.

Figure 8

Percentage of total pediatric lung transplants (%) attributed to idiopathic pulmonary arterial hypertension over time from 1995 to 2024 inclusive, United Network of Organ Sharing data. Linear regression trendline in black. Note y axis upper limit is 25%. IPAH, idiopathic pulmonary arterial hypertension.

Figure 9

Percentage of total pediatric lung transplants (%) attributed to pulmonary hypertension (both primary (IPAH) and secondary) over time from 1995 to 2024 inclusive, United Network of Organ Sharing data. Linear regression trendline in black. Note y axis upper limit is 30%. IPAH, idiopathic pulmonary arterial hypertension; PH, pulmonary hypertension.