The changing landscape of pediatric lung transplantation

Abstract

There has been a shift over decades in the diagnostic indications for lung transplantation in children; in particular, there has been a reduction in the proportion of pediatric cystic fibrosis (CF) patients undergoing lung transplantation early in life, and more transplants occurring in other diagnostic groups. Here, we examine trends in pediatric lung transplantation with regards to indications by analyzing data from the United Network of Organ Sharing, the International Society for Heart and Lung Transplantation Thoracic Transplant Registry, and other sources. Over the past two years, there has been a precipitous decline in both the number of transplants due to CF and the proportion of CF cases relative to the total number of transplants, likely not solely due to the COVID-19 pandemic. In 2020, primary pulmonary arterial hypertension for the firsttime surpassed CF as main indication for pediatric lung transplantation in the United States, a finding that is also reflected in international data. We discuss the effect of novel CFTR modulator therapies as a major factor leading to this shifting landscape.Based on our trending, pulmonary hypertension-related diagnoses and pediatric interstitial lung diseases are rising indications, for which we suggest adjustments of consensus guidelines around candidate selection criteria.

Monitoring practices of chronic lung allograft dysfunction in pediatric lung transplantation

“…There has been a shift in the diagnostic indications for lung transplantation (LTx) in children 1 . More specifically, there has been a relative reduction in the proportion of children with cystic fibrosis (CF) undergoing LTx, and pediatric LTx is occurring more frequently in other diagnostic groups like pulmonary arterial hypertension and childhood interstitial lung disease (ILD) 1,2 . This shift has brought about discussion on the need for more pediatric‐specific guidelines around candidate selection criteria 3–5 .…”

“…In addition to candidate selection, detection, and monitoring of chronic lung allograft dysfunction (CLAD) is also a burgeoning topic in pediatric LTx. This is true in particular with growing experience in transplanting infants with congenital end‐stage lung diseases 1,6 . This experience is essential since, according to a recent review examining the United Network of Organ Sharing (UNOS) database, the indication for ILD in pediatric LTx is gradually trending upward 1,7 .…”

“…This is true in particular with growing experience in transplanting infants with congenital end‐stage lung diseases 1,6 . This experience is essential since, according to a recent review examining the United Network of Organ Sharing (UNOS) database, the indication for ILD in pediatric LTx is gradually trending upward 1,7 . This is relevant to CLAD detection and monitoring following pediatric LTx since conventional methods for doing so—including spirometry to obtain forced expiratory volume in 1 s (FEV 1 )—often cannot be performed reliably in younger children, and are relatively insensitive to early changes in small airways 8 …”

“…1 More specifically, there has been a relative reduction in the proportion of children with cystic fibrosis (CF) undergoing LTx, and pediatric LTx is occurring more frequently in other diagnostic groups like pulmonary arterial hypertension and childhood interstitial lung disease (ILD). 1,2 This shift has brought about discussion on the need for more pediatric-specific guidelines around candidate selection criteria. [3][4][5] In addition to candidate selection, detection, and monitoring of chronic lung allograft dysfunction (CLAD) is also a burgeoning topic in pediatric LTx.…”

“…1,6 This experience is essential since, according to a recent review examining the United Network of Organ Sharing (UNOS) database, the indication for ILD in pediatric LTx is gradually trending upward. 1,7 This is relevant to CLAD detection and monitoring following pediatric LTx since conventional methods for doing so-including spirometry to obtain forced expiratory volume in 1 s (FEV 1 )-often cannot be performed reliably in younger children, and are relatively insensitive to early changes in small airways. 8 Recently, an adult-focused consensus document was developed by the International Society for Heart and Lung Transplantation (ISHLT) Pulmonary Council on the definition of CLAD and its pathogenesis, prevention, and treatment, including relevant investigations.…”