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Lung Transplantation for Pulmonary Vascular Disease in Children: A United Network for Organ Sharing Analysis

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Abstract

Pulmonary vascular disease (PVD) represents an important clinical indication for lung transplant (LTx) in infants, children, and adolescents. There is limited information on LTx outcomes in these patients. We explored LTx volumes and post-LTx survival in children with PVD compared to other diagnoses. The UNOS Registry was queried from 1989 to 2020 to identify first-time pediatric LTx recipients (< 18 yo). PVD was categorized as idiopathic pulmonary arterial hypertension (IPAH) and non-idiopathic arterial hypertension (non-IPAH) and compared to all other patients as other diagnoses. Univariate and multivariate regression models were performed. 984 pediatric LTx patients (593 before 2010 and 391 during/after 2010) were identified, of which 145 (14.7%) had PVD. There has been no significant change in annual rate of all LTxs over comparative eras. However, there has been a decrease in rate of LTxs for PVD patients. Children with PVD had similar survival to other LTx groups in the early era (p = 0.2) and the latter era (p = 0.9). Univariate Cox models, showed that LTx in patients with PVD was associated with a significantly less risk of mortality for children aged 6–11 years compared to younger and older cohorts (HR = 0.4 [0.17–0.98]; p = 0.045), whereas multivariate analysis showed a trend toward higher mortality in 11–17-year-olds (HR = 1.54 [0.97–2.45]; p = 0.06). For PVD patients, oxygen supplementation and ventilator support at LTx were associated with worse post-transplant survival (p = 0.029 and p = 0.01). There has been a decrease in LTx volume for pediatric patients with PVD in the modern era. Post-LTx outcomes for children with PVD are similar to those of other diagnoses in both eras, with children aged 6–11 years having the best survival. Given these findings, LTx should be considered for this patient population.

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Acknowledgements

This work is supported by the National Heart Lung and Blood Institute (1R01HL147957). Additionally, this work was supported in part (UNOS STAR dataset) by Health Resources and Services Administration contract 234-2005-37011C. The content is the responsibility of the authors alone and does not necessarily reflect the views or policies of the Department of Health and Human Services, nor does mention of trade names, commercial products, or organizations imply endorsement by the U.S. Government.

Funding

NIH 1R01HL147957-01 (HFA, AGG, AD,, FZ, DLSM).

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Authors and Affiliations

  1. Division of Congenital Heart Surgery, Heart Institute, Cincinnati Children’s Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, OH, USA

    Hosam F. Ahmed, Amalia Guzman-Gomez, Malika Desai, Alia Dani, David L. S. Morales & Farhan Zafar

  2. Division of Cardiology, Heart Institute, Cincinnati Children’s Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, OH, USA

    Paul J. Critser

  3. Division of Pulmonary Medicine, Heart Institute, Cincinnati Children’s Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, OH, 45229, USA

    Don Hayes Jr.

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  1. Hosam F. Ahmed
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  2. Amalia Guzman-Gomez
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  3. Malika Desai
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  4. Alia Dani
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  5. David L. S. Morales
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  6. Paul J. Critser
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  7. Farhan Zafar
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  8. Don Hayes Jr.
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Contributions

HFA: Conception and design, statistical analysis, drafting the manuscript AGG: Statistical analysis, reviewing and editing the manuscript MD: Statistical analysis, reviewing and editing the manuscript AD: Statistical analysis, reviewing and editing the manuscript DLSM: Data interpretation, reviewing and editing the manuscript PC: Data interpretation, reviewing and editing the manuscript FZ: Statistical analysis, data interpretation, reviewing and editing the manuscript DH: Conception and design, data interpretation, drafting the manuscript.

Corresponding author

Correspondence to Don Hayes Jr..

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Competing interests

Dr. Morales is a consultant for Abbott, Inc., Azyio, Inc., Berlin Heart, Inc., CorMatrix, Inc., Peca, Inc., Syncardia, Inc., and Xeltis, Inc., and serves as a principal investigator for FDA trials sponsored by Peca, Inc. and Xeltis, Inc. Dr Zafar is a procurement surgeon for Transmedics Inc. The rest of authors report no conflicts of interest and have no relevant disclosures regarding this manuscript.

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Ahmed, H.F., Guzman-Gomez, A., Desai, M. et al. Lung Transplantation for Pulmonary Vascular Disease in Children: A United Network for Organ Sharing Analysis. Pediatr Cardiol 45, 385–393 (2024). https://doi.org/10.1007/s00246-023-03356-x

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  • DOI: https://doi.org/10.1007/s00246-023-03356-x

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