Cardiac arrest related to anaesthesia in Williams-Beuren syndrome

Rev Esp Anestesiol Reanim (Engl Ed). 2018 Apr;65(4):234-237. doi: 10.1016/j.redar.2017.11.005. Epub 2017 Dec 13.
[Article in English, Spanish]

Abstract

Williams-Beuren syndrome is the clinical manifestation of a congenital genetic disorder in the elastin gene, among others. There is a history of cardiac arrest refractory to resuscitation manoeuvres in anaesthesia. The incidence of myocardial ischaemia is high during anaesthetic induction, but there are patients who do not have this condition yet also have had very serious cardiac events, and issues that are still to be resolved. Case descriptions will enable the common pathophysiological factors to be defined, and decrease morbidity and mortality. We report the case of a 3-year-old boy with cardiac arrest at induction, rescued with circulatory assistance with extracorporeal membrane oxygenation and hypothermia induced for cerebral protection.

Keywords: Anaesthesia; Anestesia; Cardiac arrest; Cardiopathy; Cardiopatía; Death sudden; Isquemia miocárdica; Muerte súbita; Myocardial ischaemia; Parada cardiaca; Síndrome Williams; Williams syndrome.

Publication types

  • Case Reports

MeSH terms

  • Anesthesia, Inhalation / adverse effects*
  • Anesthetics, Inhalation / adverse effects*
  • Aortic Stenosis, Supravalvular / etiology
  • Aortic Stenosis, Supravalvular / surgery
  • Arteries / pathology
  • Bradycardia / etiology
  • Child, Preschool
  • Combined Modality Therapy
  • Disease Susceptibility
  • Extracorporeal Membrane Oxygenation
  • Heart Arrest / chemically induced*
  • Heart Arrest / etiology
  • Heart Arrest / physiopathology
  • Heart Valves / pathology
  • Humans
  • Hypothermia, Induced
  • Hypoxia-Ischemia, Brain / etiology
  • Hypoxia-Ischemia, Brain / prevention & control
  • Intraoperative Complications / chemically induced*
  • Intraoperative Complications / etiology
  • Male
  • Muscle Hypotonia / etiology
  • Paresis / etiology
  • Postoperative Complications / etiology
  • Sevoflurane / adverse effects*
  • Williams Syndrome / complications*
  • Williams Syndrome / pathology

Substances

  • Anesthetics, Inhalation
  • Sevoflurane