The Pediatric Pulmonologist and the Infant or Child Before Lung Transplantation

Pretransplant Evaluation and Care of the Potential Pediatric Lung Recipient
  • Anjani K. Ravindra
  • Jonathan E. Spahr
  • Geoffrey Kurland
Reference work entry
Part of the Organ and Tissue Transplantation book series (OTT)

Abstract

While pediatric lung transplantation began in 1987, survival rates have remained stagnant. Therefore, the role of pretransplant evaluation and care has become more crucial in identifying appropriate transplantation candidates and affording their best chance of success. There are a variety of indications for pediatric lung transplantation that vary in prevalence based on age; specific indications can at times direct pretransplant care. Factors to consider when evaluating a candidate include a clear diagnosis that warrants transplantation, a support system for posttransplantation care, and access to a lung transplantation center. A number of previously absolute contraindications have now become relative contraindications due to medical advances; however, significantly advanced organ dysfunction remains an absolute contraindication. Referral for transplantation is based on a “transplantation window” or a time where survival benefit of transplantation is high. This “window” is dynamic, as changes in a patient’s clinical status or new infections can rapidly alter a patient’s acceptability as a recipient. To better assess timing of referral, the evaluation process includes blood work, cultures, lung function testing, as well as assessment of non-pulmonary systems by specialists as well as psychosocial evaluation. The lung allocation score (LAS) was developed in 2005 to help improve waiting time for organs and weighs both waitlist mortality and survival probability posttransplantation. Reevaluation thus must be recurrent to adjust a patient’s LAS score accordingly. Overall, the quality of life pretransplantation has to be weighed against the predicted outcome posttransplantation.

Keywords

Children Transplantation Immunosuppression Evaluation Lung Cystic fibrosis Allocation Recipient Referral Survival benefit Waiting period 

References

  1. Aris RM, Routh JC, LiPuma JJ, Heath DG, Gilligan PH (2001) Lung transplantation for cystic fibrosis patients with Burkholderia cepacia complex. Survival linked to genomovar type. Am J Respir Crit Care Med 164(11):2102–2106CrossRefPubMedGoogle Scholar
  2. Benden C, Ridout DA, Edwards LB, Boehler A, Christie JD, Sweet SC (2013) Body mass index and its effect on outcome in children after lung transplantation. J Heart Lung Transplant 32(2):196–201CrossRefPubMedGoogle Scholar
  3. Bright-Thomas RJ, Johnson SC (2014) What is the role of noninvasive ventilation in cystic fibrosis? Curr Opin Pulm Med 20(6):618–622CrossRefPubMedGoogle Scholar
  4. Clabby ML, Canter CE, Moller JH, Bridges ND (1997) Hemodynamic data and survival in children with pulmonary hypertension. J Am Coll Cardiol 30(2):554–560CrossRefPubMedGoogle Scholar
  5. de Sante J, Caplan A, Hippen B, Testa G, Lantos JD (2014) Was Sarah Murnaghan treated justly? Pediatrics 134:155–162CrossRefGoogle Scholar
  6. Dobbels F, Vanhaecke J, Dupont L, Nevens F, Verleden G, Pirenne J, De Geest S (2009) Pretransplant predictors of posttransplant adherence and clinical outcome: an evidence base for pretransplant psychosocial screening. Transplantation 87(10):1497–1504CrossRefPubMedGoogle Scholar
  7. Egan TM, Murray S, Bustami RT, Shearon TH, McCullough KP, Edwards LB, Coke MA, Garrity ER, Sweet SC, Heiney DA, Grover FL (2006) Development of the new lung allocation system in the United States. Am J Transplant 6:1212–1227CrossRefPubMedGoogle Scholar
  8. Englum BR et al (2016) Impact of donor and recipient hepatitis C status in lung transplantation. J Heart Lung Transplant 35(2):228–235CrossRefPubMedGoogle Scholar
  9. Faro A, Visner G, Mallory GB (2007) Lung and heart-lung transplantation. In: Fine RN, Webber SA et al (eds) Pediatric solid organ transplantation, 2nd edn. Blackwell, OxfordGoogle Scholar
  10. Fong TL, Cho YW, Hou L, Hutchinson IV, Barbers RG, Herrington CS (2011) Outcomes after lung transplantation and practices of lung transplant programs in the United States regarding hepatitis C seropositive recipients. Transplantation 91(11):1293–1296CrossRefPubMedGoogle Scholar
  11. Goldfarb SB et al (2016) The registry of the international society for Heart and Lung Transplantation: nineteenth pediatric lung and heart-lung transplantation report-2016; Focus theme: primary diagnostic indications for transplant. J Heart Lung Transplant 35 (10): 1196–1205Google Scholar
  12. Gomberg-Maitland M, Glassner-Kolmin C, Watson S, Frantz R, Park M, Frost A, Benza RL, Torres F (2013) Survival in pulmonary arterial hypertension patients awaiting lung transplantation. J Heart Lung Transplant 32(12):1179–1186CrossRefPubMedGoogle Scholar
  13. Hadjiliadis D et al (2007) Survival of lung transplant patients with cystic fibrosis harboring panresistant bacteria other than Burkholderia cepacia, compared with patients harboring sensitive bacteria. J Heart Lung Transplant 26(8):834–838CrossRefPubMedGoogle Scholar
  14. Hayes D Jr, McConnell PI, Preston TJ, Yates AR, Kirkby S, Galantowicz M (2013) Active rehabilitation with venovenous extracorporeal membrane oxygenation as a bridge to lung transplantation in a pediatric patient. World J Pediatr 9(4):373–374CrossRefPubMedGoogle Scholar
  15. Hayes D Jr, Higgins RS, Kirkby S, McCoy KS, Wehr AM, Lehman AM, Whitson BA (2014) Impact of pulmonary hypertension on survival in patients with cystic fibrosis undergoing lung transplantation: an analysis of the UNOS registry. J Cyst Fibros 13(4):416–423Google Scholar
  16. Humpl T, Berger RM, Austin ED, Fasnacht Boillat MS, Bonnet D, Ivy DD, Zuk M, Beghetti M, Schulze-Neick I (2016) Treatment initiation in paediatric pulmonary hypertension: insights from a multinational registry. Cardiol Young 20:1–10Google Scholar
  17. Kallet RH (2011) Patient-ventilator interaction during acute lung injury, and the role of spontaneous breathing: Part 1: respiratory muscle function during critical illness. Respir Care 56(2):181–189CrossRefPubMedGoogle Scholar
  18. Kerem E, Reisman J, Corey M, Canny GJ, Levison H (1992) Prediction of mortality in patients with cystic fibrosis. N Engl J Med 326(18):1187–1191CrossRefPubMedGoogle Scholar
  19. Kurland G, Orenstein DM (2001) Lung transplantation and cystic fibrosis: the psychosocial toll. Pediatrics 107(6):1419–1420CrossRefPubMedGoogle Scholar
  20. Lancaster TS, Miller JR, Epstein DJ, DuPont NC, Sweet SC, Eghtesady P (2016) Improved waitlist and transplant outcomes for pediatric lung transplantation after implementation of the lung allocation score. J Heart Lung Transplant 16:30365–30365Google Scholar
  21. Liou TG, Adler FR, Cahill BC, FitzSimmons SC, Huang D, Hibbs JR, Marshall BC (2001a) Survival effect of lung transplantation among patients with cystic fibrosis. JAMA 286(21):2683–2689CrossRefPubMedPubMedCentralGoogle Scholar
  22. Liou TG, Adler FR, Fitzsimmons SC et al (2001b) Predictive 5-year survivorship model of cystic fibrosis. Am J Epidemiol 153:345–352CrossRefPubMedPubMedCentralGoogle Scholar
  23. Liou TG, Adler FR, Cox DR, Cahill BC (2007) Lung transplantation and survival in children with cystic fibrosis. N Engl J Med 357(21):2143–2152CrossRefPubMedPubMedCentralGoogle Scholar
  24. Malamud AL, Ricard PE (2016) Feasibility of the six-minute walk test for patients who have cystic fibrosis, are ambulatory, and require mechanical ventilation before lung transplantation. Phys Ther 96(9):1468–1476CrossRefPubMedGoogle Scholar
  25. Mallory GB, Elidemir O (2006) Pediatric lung transplantation. In: Lynch JP, Ross DJ (eds) Lung and heart-lung transplantation, 1st edn. CRC Press, New YorkGoogle Scholar
  26. Marshall SE, Kramer MR, Lewiston NJ, Starnes VA, Theodore J (1990) Selection and evaluation of recipients for heart-lung and lung transplantation. Chest 98(6):1488–1494CrossRefPubMedGoogle Scholar
  27. Olland A, Falcoz PE, Canuet M, Massard G (2013) Should we perform bilateral-lung or heart–lung transplantation for patients with pulmonary hypertension? Interact Cardiovasc Thorac Surg 17(1):166–170CrossRefPubMedPubMedCentralGoogle Scholar
  28. Orens et al (2006) International guidelines for the selection of lung transplant candidates: 2006 update--a consensus report from the Pulmonary Scientific Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant 25(7):745–755CrossRefPubMedGoogle Scholar
  29. Ploegstra MJ, Zijlstra WM, Douwes JM, Hillege HL, Berger RM (2015) Prognostic factors in pediatric pulmonary arterial hypertension: a systematic review and meta-analysis. Int J Cardiol 184:198–207CrossRefPubMedGoogle Scholar
  30. Radley-Smith R, Aurora P (2006) Transplantation as a treatment for end-stage pulmonary hypertension in childhood. Paediatr Respir Rev 7(2):117–122CrossRefPubMedGoogle Scholar
  31. Sandoval J, Bauerle O, Gomez A, Palomar A, Martínez Guerra ML, Furuya ME (1995) Primary pulmonary hypertension in children: clinical characterization and survival. J Am Coll Cardiol 25(2):466–474CrossRefPubMedGoogle Scholar
  32. Schaellibaum G et al (2011) Bilateral lung transplantation for pediatric idiopathic pulmonary arterial hypertension: a multi-center experience. Pediatr Pulmonol 46(11):1121–1127CrossRefPubMedGoogle Scholar
  33. Spahr JE, Meyer KC (2011) Lung transplantation. In: Hricik D (ed) Primer on transplantation, 3rd edn. Wiley-Blackwell, OxfordGoogle Scholar
  34. Spahr JE, West SC (2014) Heart-lung transplantation: pediatric indications and outcomes. J Thorac Dis 6(8):1129–1137PubMedPubMedCentralGoogle Scholar
  35. Stephenson AL, Sykes J, Berthiaume Y, Singer LG, Aaron SD, Whitmore GA, Stanojevic S (2015) Clinical and demographic factors associated with post-lung transplantation survival in individuals with cystic fibrosis. J Heart Lung Transplant 34(9):1139–1145CrossRefPubMedGoogle Scholar
  36. Toprak D, Midyat L, Freiberger D et al (2017) Outcomes of mechanical support in a pediatric lung transplant center. Pediatr Pulmonol 52(3):360–366CrossRefPubMedGoogle Scholar
  37. Upala S, Panichsillapakit T, Wijarnpreecha K, Jaruvongvanich V, Sanguankeo A (2016) Underweight and obesity increase the risk of mortality after lung transplantation: a systematic review and meta-analysis. Transpl Int 29(3):285–296CrossRefPubMedGoogle Scholar
  38. Weill et al (2015) A consensus document for the selection of lung transplant candidates: 2014--an update from the Pulmonary Transplantation Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant 34(1):1–15CrossRefPubMedGoogle Scholar
  39. Wray J, Radley-Smith R (2004) Depression in pediatric patients before and 1 year after heart or heart-lung transplantation. J Heart Lung Transplant 23(9):1103–1110CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Anjani K. Ravindra
    • 1
  • Jonathan E. Spahr
    • 2
  • Geoffrey Kurland
    • 1
  1. 1.Division of Pediatric Pulmonology, Allergy, and Immunology, Department of PediatricsChildren’s Hospital of PittsburghPittsburghUSA
  2. 2.Division of Pediatric PulmonologyGeisinger Medical CenterDanvilleUSA

Section editors and affiliations

  • J Jeffrey Malatack
    • 1
  1. 1.Diagnostic Referral DivisionNemours/Alfred I. duPont Hospital for ChildrenWilmingtonUSA

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