Perioperative management for liver transplant in a patient with familial amyloid polyneuropathy with heart involvement

Rev Esp Anestesiol Reanim. 2015 Jan;62(1):46-8. doi: 10.1016/j.redar.2014.03.003. Epub 2014 Apr 16.
[Article in English, Spanish]

Abstract

Familial amyloid polyneuropathy (FAP) is a systemic amyloidosis caused by mutated transthyretin. Cardiac amyloidosis, the major prognostic determinant in systemic amyloidosis, is characterized by infiltration of the myocardium, leading to cardiomyopathy and conduction disturbances. Liver transplantation is the only curative option for patients with FAP. The case is presented of a 36-year-old patient with type i FAP with cardiac involvement, proposed for liver transplant surgery, which was successfully performed without any preoperative event of interest.

Keywords: Amiloidosis; Amiloidosis cardíaca; Amyloidosis; Cardiac amyloidosis; Familial amyloid polyneuropathy; Liver transplantation; Polineuropatía amiloidótica familiar; Trasplante hepático.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Amyloid Neuropathies, Familial / complications*
  • Atrioventricular Block / diagnostic imaging
  • Atrioventricular Block / etiology
  • Cardiomyopathies / diagnostic imaging
  • Cardiomyopathies / etiology*
  • Humans
  • Intraoperative Complications / prevention & control
  • Liver Transplantation / methods*
  • Male
  • Monitoring, Intraoperative
  • Norepinephrine / therapeutic use
  • Perioperative Care / methods*
  • Postoperative Complications / prevention & control
  • Vascular Resistance / drug effects

Substances

  • Norepinephrine