title: Late profound muscle weakness following heart transplantation due to Danon disease.
abstract: BACKGROUND
  Postoperative muscle weakness is a serious complication in surgical intensive care patients. It is mostly described as critical illness polyneuromyopathy. Risk factors include intensive care length of stay, sepsis, poor glycemic control, and combined use of corticosteroids and neuromuscular blocking agents, malnutrition, and electrolyte imbalance.
  
  
  METHODS
  We report a case of late-progressive, profound weakness after heart transplantation for noncompaction cardiomyopathy which required prolonged mechanical ventilation. The patient's muscle strength recovered completely after prolonged rehabilitation.
  
  
  RESULTS
  Electromyographic assessment showed myopathy. Muscle biopsy revealed Danon disease, a genetic disorder affecting the lysosomal-associated membrane protein 2 gene (LAMP2).
  
  
  CONCLUSIONS
  The finding of this genetic disorder was unexpected, because the preoperative echocardiographic diagnosis of noncompaction cardiomyopathy has not been reported in Danon disease. This report underlines the need for early availability of pathology results from the explanted heart, which showed the same disorder.
authors.affiliation: Department of Anesthesia, Stanford University School of Medicine, 300 Pasteur Drive, Stanford, California 94305, USA. pieterva@stanford.edu
authors.forename: Pieter
authors.initials: P
authors.lastname: Van Der Starre
authors.forename: Tobias
authors.initials: T
authors.lastname: Deuse
authors.forename: Chad
authors.initials: C
authors.lastname: Pritts
authors.forename: Carlos
authors.initials: C
authors.lastname: Brun
authors.forename: Hannes
authors.initials: H
authors.lastname: Vogel
authors.forename: Philip
authors.initials: P
authors.lastname: Oyer
mesh_terms: D052120:Glycogen Storage Disease Type IIb / Q000150:complications / Q000601:surgery*; D016027:Heart Transplantation / Q000009:adverse effects*; D006801:Humans; D008297:Male; D018908:Muscle Weakness / Q000209:etiology*; D055815:Young Adult