"Acquired" Brugada syndrome in a cardiac allograft.

Autor: Power A; Department of Pediatrics, UT Southwestern Medical Center and Children's Medical Center, Dallas, Texas, USA., Lynch Á; Department of Pediatrics, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada., Zahavich L; Department of Genetic Counseling, Hospital for Sick Children, Toronto, Ontario, Canada., Lévesque SA; Centre Hospitalier Universitaire de Sherbrooke, Université de Sherbrooke, Sherbrooke, Quebec, Canada., Stephenson EA; Department of Pediatrics, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada., Jean-St-Michel E; Department of Pediatrics, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada., Dipchand AI; Department of Pediatrics, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada., Jeewa A; Department of Pediatrics, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
Jazyk: angličtina
Zdroj: Pediatric transplantation [Pediatr Transplant] 2022 Aug; Vol. 26 (5), pp. e14276. Date of Electronic Publication: 2022 Mar 27.
DOI: 10.1111/petr.14276
Abstrakt: Introduction: Brugada syndrome is an inherited channelopathy characterized by arrhythmia and an increased risk of sudden cardiac death (SCD). Implantation of a defibrillator for primary or secondary prevention is the only effective strategy to decrease the risk of SCD in Brugada syndrome. We present a case in which a cardiac donor had a pathogenic variant for Brugada syndrome, discovered on genetic testing after transplantation.
Case Report: A young child with dilated cardiomyopathy underwent orthotopic heart transplantation from a donor with in-hospital cardiac arrest in the context of fever and a normal ECG. Approximately 1 month after transplant, the donor's post mortem genetic testing revealed a pathogenic loss-of-function SCN5A variant associated with Brugada syndrome, which was confirmed on genetic testing on a post-transplant endomyocardial biopsy from the recipient. The recipient's post-transplant electrocardiographic monitoring revealed persistent right bundle branch block and progressive, asymptomatic sinus node dysfunction. The recipient was managed with precautionary measures including aggressive fever management, avoidance of drugs that increase arrhythmia risk in Brugada syndrome, and increased frequency of arrhythmia surveillance. The recipient remains asymptomatic at over 3 years post-transplant with preserved graft function and no documented ventricular arrhythmias.
Conclusion: We describe the clinical course of "acquired" Brugada syndrome in a cardiac allograft recipient, which has not been previously reported. The time-sensitive nature of donor organ selection, especially in critically ill recipients, combined with the growing use of molecular autopsies in patients with unexplained etiologies for death may increasingly result in important donor genetic information being made available after transplantation.
(© 2022 Wiley Periodicals LLC.)
Databáze: MEDLINE
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