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Heart Transplantation in Children with Mitochondrial Disease - 22/01/20

Doi : 10.1016/j.jpeds.2019.10.016 
Jeffrey G. Weiner, MD, MSCI 1, , Andrea N. Lambert, MD 1, Cary Thurm, PhD 2, Matt Hall, PhD 2, Jonathan H. Soslow, MD, MSCI 1, Tyler E. Reimschisel, MD, MHPE 3, David W. Bearl, MD, MA 1, Debra A. Dodd, MD 1, Brian Feingold, MD, MS 4, Justin Godown, MD 1
1 Department of Pediatric Cardiology, Monroe Carell Jr. Children's Hospital, Nashville, TN 
2 Children's Hospital Association, Lenexa, KS 
3 Department of Pediatrics, Monroe Carell Jr. Children's Hospital, Nashville, TN 
4 Department of Pediatrics and Clinical and Translational Science, University of Pittsburgh School of Medicine, Pittsburgh, PA 

Reprint requests: Jeffrey G. Weiner, MD, MSCI, Monroe Carell Jr. Children's Hospital at Vanderbilt University Medical Center, Department of Pediatric Cardiology, 2200 Children's Way, Suite 5230 DOT, Nashville, TN 37232-9119.Monroe Carell Jr. Children's Hospital at Vanderbilt University Medical CenterDepartment of Pediatric Cardiology2200 Children's WaySuite 5230 DOTNashvilleTN37232-9119

Abstract

Objectives

To compare the outcomes and comorbidities of children with mitochondrial disease undergoing heart transplantation with children without mitochondrial disease.

Study design

Using a unique linkage between the Pediatric Health Information System and Scientific Registry of Transplant Recipients databases, pediatric heart transplantation recipients from 2002 to 2016 with a diagnosis of cardiomyopathy were included. Post heart transplantation survival and morbidities were compared between patients with and without mitochondrial disease.

Results

A total of 1330 patients were included, including 47 (3.5%) with mitochondrial disease. Survival after heart transplantation was similar between patients with and without mitochondrial disease over a median follow-up of 4 years. Patients with mitochondrial disease were more likely to have a stroke after heart transplantation (11% vs 3%; P = .009), require a longer duration of mechanical ventilation after heart transplantation (3 days vs 1 day; P < .001), and have a longer intensive care unit stay after heart transplantation (10 vs 6 days; P = .007). The absence of a hospital readmission within the first post-transplant year was similar among patients with and without mitochondrial disease (61.7% vs 51%; P = .14). However, patients with mitochondrial disease who were readmitted demonstrated a longer length of stay compared with those without (median, 14 days vs 8 days; P = .03).

Conclusions

Patients with mitochondrial disease can successfully undergo heart transplantation with survival comparable with patients without mitochondrial disease. Patients with mitochondrial disease have greater risk for post-heart transplantation morbidities including stroke, prolonged mechanical ventilation, and longer intensive care unit and readmission length of stay. These results suggest that the presence of mitochondrial disease should not be an absolute contraindication to heart transplantation in the appropriate clinical setting.

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Keywords : ▪▪▪

Abbreviations : ECMO, ICU, ICD, PHIS, SRTR


Plan


 Supported through internal funding from the Katherine Dodd Faculty Scholar Program at Vanderbilt University (to J.G.). Research reported in this publication was supported by the National Heart, Lung, and Blood Institute of the National Institute of Health (6T32HL105334-06 [to J.W.] and K23HL123938 [to J.S.] [Bethesda, Maryland]. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health. The authors declare no conflicts of interest.


© 2019  Elsevier Inc. Tous droits réservés.
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Vol 217

P. 46 - février 2020 Retour au numéro
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