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pubmed-article:17596219pubmed:abstractTextPatients 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).lld:pubmed
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pubmed-article:17596219pubmed:articleTitleCardiac arrhythmias following anesthesia induction in infantile-onset Pompe disease: a case series.lld:pubmed
pubmed-article:17596219pubmed:affiliationDivision of Pediatric Anesthesiology, Duke Uinvesity Medical Center, Durham, NC 27710, USA.lld:pubmed
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