Cardiac arrest in Wilson's disease after curative liver transplantation: a life-threatening complication of myocardial copper excess?

ESC Heart Fail. 2019 Feb;6(1):228-231. doi: 10.1002/ehf2.12395. Epub 2019 Jan 8.

Abstract

We report the case of a 38-year-old man who presented with cardiac arrest 1 year after curative liver transplantation for Wilson's disease. Clinical work-up proofed myocardial copper and iron accumulation using mass spectrometry, which led most likely to myocardial fibrosis as visualized by cardiovascular magnetic resonance (unprecedented delayed enhancement pattern) and endomyocardial biopsy. Consequently, cardiac arrest due to ventricular fibrillation and subsequent episodes of sustained ventricular tachycardia were considered as primary cardiac manifestation of Wilson's disease. This can, as illustrated by our case, occur even late after curative liver transplantation, which is an important fact that treating physicians should be aware of during clinical follow-up of these patients.

Keywords: Cardiac arrest; Copper; Ventricular arrhythmia; Wilson disease.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Biopsy
  • Copper / metabolism*
  • Electrocardiography
  • Follow-Up Studies
  • Heart Arrest / diagnosis
  • Heart Arrest / etiology*
  • Hepatolenticular Degeneration / complications*
  • Hepatolenticular Degeneration / diagnosis
  • Hepatolenticular Degeneration / surgery
  • Humans
  • Liver Transplantation*
  • Magnetic Resonance Imaging, Cine
  • Male
  • Mass Spectrometry
  • Myocardium / metabolism*
  • Myocardium / pathology
  • Ventricular Fibrillation / complications*
  • Ventricular Fibrillation / diagnosis

Substances

  • Copper