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: RESULTS: 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.
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Authors | Luke Y-J Wang, Allison K Ross, Jennifer S Li, Stephanie M Dearmey, Joanne F Mackey, Maryalice Worden, Deyanira Corzo, Claire Morgan, Priya S Kishnani |
Journal | Paediatric anaesthesia
(Paediatr Anaesth)
Vol. 17
Issue 8
Pg. 738-48
(Aug 2007)
ISSN: 1155-5645 [Print] France |
PMID | 17596219
(Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- Anesthetics, Intravenous
- Methyl Ethers
- Sevoflurane
- GAA protein, human
- alpha-Glucosidases
- Propofol
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Topics |
- Anesthesia, General
(adverse effects)
- Anesthetics, Intravenous
(adverse effects)
- Arrhythmias, Cardiac
(etiology)
- Clinical Trials as Topic
- Contraindications
- Fatal Outcome
- Female
- Glycogen Storage Disease Type II
(complications, drug therapy)
- Heart Arrest
(etiology)
- Humans
- Infant
- Infant, Newborn
- Male
- Methyl Ethers
(adverse effects)
- Propofol
(adverse effects)
- Retrospective Studies
- Sevoflurane
- alpha-Glucosidases
(therapeutic use)
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