title: Cardiac arrhythmias following anesthesia induction in infantile-onset Pompe disease: a case series.
abstract: BACKGROUND
  Patients with infantile-onset Pompe disease suffer from marked hypertrophic cardiomyopathy and an increased risk of arrhythmia. A noncompliant left ventricle predisposes these infants to diastolic heart failure with elevated left ventricular enddiastolic pressure (LVEDP); these patients also commonly develop systolic heart failure. Given this baseline cardiac physiology, coronary perfusion pressure becomes highly sensitive to abrupt changes in diastolic blood pressure (DBP).
  
  
  METHODS
  We retrospectively reviewed the experiences of 139 patients enrolled in clinical trials investigating the treatment of infantile-onset Pompe disease with recombinant human acid alpha-glucosidase (rhGAA). Adverse events were screened for those involving anesthesia.
  
  
  RESULTS
  Nine patients (6%) with infantile-onset Pompe disease experienced an arrhythmia or cardiopulmonary arrest soon after the induction of general anesthesia. Of these events, propofol was involved in four arrhythmias; sevoflurane without propofol was associated with an additional two. Deaths resulting from arrhythmia appeared to correlate with left ventricular mass indices >350 g x m(-2).
  
  
  CONCLUSIONS
  With the advent of enzyme replacement therapy (ERT) using rhGAA, and increased survivability, more infantile Pompe patients will likely present for surgical procedures. Additional care in maximizing coronary perfusion pressure and minimizing arrhythmia risk must be given. For these reasons, it is recommended that anesthesia for infantile Pompe patients specifically avoid propofol or high concentrations of sevoflurane and, instead, use an agent such as ketamine as the cornerstone for induction in order to better support coronary perfusion pressure and to avoid decreasing DBP with vasodilatory agents.
authors.affiliation: Division of Pediatric Anesthesiology, Duke Uinvesity Medical Center, Durham, NC 27710, USA.
authors.forename: Luke Y-J
authors.initials: LY
authors.lastname: Wang
authors.forename: Allison K
authors.initials: AK
authors.lastname: Ross
authors.forename: Jennifer S
authors.initials: JS
authors.lastname: Li
authors.forename: Stephanie M
authors.initials: SM
authors.lastname: Dearmey
authors.forename: Joanne F
authors.initials: JF
authors.lastname: Mackey
authors.forename: Maryalice
authors.initials: M
authors.lastname: Worden
authors.forename: Deyanira
authors.initials: D
authors.lastname: Corzo
authors.forename: Claire
authors.initials: C
authors.lastname: Morgan
authors.forename: Priya S
authors.initials: PS
authors.lastname: Kishnani
mesh_terms: D000768:Anesthesia, General / Q000009:adverse effects*; D018686:Anesthetics, Intravenous / Q000009:adverse effects*; D001145:Arrhythmias, Cardiac / Q000209:etiology*; D002986:Clinical Trials as Topic; D000075202:Contraindications; D017809:Fatal Outcome; D005260:Female; D006009:Glycogen Storage Disease Type II / Q000150:complications* / Q000188:drug therapy; D006323:Heart Arrest / Q000209:etiology*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008738:Methyl Ethers / Q000009:adverse effects*; D015742:Propofol / Q000009:adverse effects*; D012189:Retrospective Studies; D000077149:Sevoflurane; D000520:alpha-Glucosidases / Q000627:therapeutic use
chemical_list: D018686:Anesthetics, Intravenous; D008738:Methyl Ethers; D000077149:Sevoflurane; C509951:GAA protein, human; D000520:alpha-Glucosidases; D015742:Propofol