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Lung Transplantation as An Intervention for Pediatric Pulmonary Hypertension
  • Ernestina Melicoff,
  • Don Hayes,
  • Christian Benden
Ernestina Melicoff
Baylor College of Medicine
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Don Hayes
University of Cincinnati
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Christian Benden
University of Zurich
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Abstract

Lung transplantation is recognized as a treatment option for selected patients with end-stage lung disease and pulmonary vascular disorders. Overall, pulmonary hypertension (PH) is the second most frequent indication for children requiring lung transplantation. Pediatric lung transplantation can be complex; in PH patients timing for listing and the perioperative management can be particularly challenging making interdisciplinary collaboration with the referring PH team essential. Because pediatric patients typically have preserved cardiac index and exercise tolerance even with advanced disease, they should be referred early even if they do not meet the proposed criteria for listing by ISHLT published in 2015: NYHA functional class III or IV without improvement, cardiac index < 2 liters/min/m^2, mean right atrial pressure of > 15 mmHg. Bridging strategies with extracorporeal support should be determined at time of listing prior to transplantation anticipating the possibility of clinical deterioration. Bilateral lung transplantation using cardiopulmonary bypass to provide hemodynamics stability is the standard practice in pediatric centers. The immediate post-transplant period is characterized by dramatic normalization of PVR as well as changes in the RV and LV physiology and function which can be life-threatening. Induction, immunosuppression, prophylaxis and surveillance are not different from patients without PH. Overall, outcomes in pediatric lung and heart-lung transplant patients for PH are not different from those children undergoing lung or heart-lung transplant for other indications. In fact, long-term survival is superior in children with idiopathic PH compared to other diseases, there is also a dramatic improvement in quality of life in most recipients

Peer review status:UNDER REVIEW

07 May 2020Submitted to Pediatric Pulmonology
07 May 2020Submission Checks Completed
07 May 2020Assigned to Editor
11 May 2020Reviewer(s) Assigned
02 Jun 2020Review(s) Completed, Editorial Evaluation Pending
03 Jun 2020Editorial Decision: Revise Major
10 Sep 20201st Revision Received
11 Sep 2020Submission Checks Completed
11 Sep 2020Assigned to Editor
11 Sep 2020Reviewer(s) Assigned
16 Sep 2020Review(s) Completed, Editorial Evaluation Pending