"Acquired" Brugada syndrome in a cardiac allograft.

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.

When is enough, enough? Exploring ethical and team considerations in paediatric cardiac care dilemmas.

PURPOSE OF REVIEW: Therapies for paediatric congenital and acquired heart disease continue to evolve and the appropriateness of pursuing life sustaining interventions at margins of standard therapy is ethically challenging. RECENT FINDINGS: With ongoing emphasis on shared decision making, recent literature explored physician and parental perspectives on communication with families and offering interventions for complex congenital heart disease and advanced heart failure. The inclusion of parental values and views in this process is now widely accepted. Identified outstanding challenges include difficulty with prognostication from the outset, adjusting long-term goals of care to changes in clinical parameters, need for consistency in communication including regular review meetings with family or surrogate decision-makers. Bioethics consultation and multidisciplinary team reviews may be helpful supports. Palliative care involvement in this population improves quality of life and alleviates parental distress but this collaboration is not optimized. SUMMARY: Decision to offer, forgo, or discontinue life-sustaining therapies for children with heart disease has nuanced and context-specific considerations, and must integrate burdens of interventions with patient and family values. Thus, decision making remains complex and demands thoughtful review of not only risks and benefits, but views and values, clearly communicated to team and family.