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Cardiac arrhythmias following anesthesia induction in infantile-onset Pompe disease: a case series.

Publication ,  Journal Article
Wang, LY-J; Ross, AK; Li, JS; Dearmey, SM; Mackey, JF; Worden, M; Corzo, D; Morgan, C; Kishnani, PS
Published in: Paediatr Anaesth
August 2007

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.

Duke Scholars

Published In

Paediatr Anaesth

DOI

ISSN

1155-5645

Publication Date

August 2007

Volume

17

Issue

8

Start / End Page

738 / 748

Location

France

Related Subject Headings

  • alpha-Glucosidases
  • Sevoflurane
  • Retrospective Studies
  • Propofol
  • Methyl Ethers
  • Male
  • Infant, Newborn
  • Infant
  • Humans
  • Heart Arrest
 

Citation

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Wang, L.-J., Ross, A. K., Li, J. S., Dearmey, S. M., Mackey, J. F., Worden, M., … Kishnani, P. S. (2007). Cardiac arrhythmias following anesthesia induction in infantile-onset Pompe disease: a case series. Paediatr Anaesth, 17(8), 738–748. https://doi.org/10.1111/j.1460-9592.2007.02215.x
Wang, Luke Y-J, Allison K. Ross, Jennifer S. Li, Stephanie M. Dearmey, Joanne F. Mackey, Maryalice Worden, Deyanira Corzo, Claire Morgan, and Priya S. Kishnani. “Cardiac arrhythmias following anesthesia induction in infantile-onset Pompe disease: a case series.Paediatr Anaesth 17, no. 8 (August 2007): 738–48. https://doi.org/10.1111/j.1460-9592.2007.02215.x.
Wang LY-J, Ross AK, Li JS, Dearmey SM, Mackey JF, Worden M, et al. Cardiac arrhythmias following anesthesia induction in infantile-onset Pompe disease: a case series. Paediatr Anaesth. 2007 Aug;17(8):738–48.
Wang, Luke Y. J., et al. “Cardiac arrhythmias following anesthesia induction in infantile-onset Pompe disease: a case series.Paediatr Anaesth, vol. 17, no. 8, Aug. 2007, pp. 738–48. Pubmed, doi:10.1111/j.1460-9592.2007.02215.x.
Wang LY-J, Ross AK, Li JS, Dearmey SM, Mackey JF, Worden M, Corzo D, Morgan C, Kishnani PS. Cardiac arrhythmias following anesthesia induction in infantile-onset Pompe disease: a case series. Paediatr Anaesth. 2007 Aug;17(8):738–748.
Journal cover image

Published In

Paediatr Anaesth

DOI

ISSN

1155-5645

Publication Date

August 2007

Volume

17

Issue

8

Start / End Page

738 / 748

Location

France

Related Subject Headings

  • alpha-Glucosidases
  • Sevoflurane
  • Retrospective Studies
  • Propofol
  • Methyl Ethers
  • Male
  • Infant, Newborn
  • Infant
  • Humans
  • Heart Arrest