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J Cardiothorac Vasc Anesth. 2014 Oct;28(5):1243-50. doi: 10.1053/j.jvca.2013.09.015. Epub 2014 Jan 23.

Anesthesia and LEOPARD syndrome: a review of forty-nine anesthetic exposures.

Author information

1
Department of Anesthesiology, Mayo Clinic, Rochester, MN; Department of Anaesthesia, National University Hospital, National University Health System, Republic of Singapore.
2
Department of Anesthesiology, Mayo Clinic, Rochester, MN.
3
Department of Anesthesiology, Mayo Clinic, Rochester, MN. Electronic address: sprung.juraj@mayo.edu.

Abstract

OBJECTIVES:

LEOPARD syndrome is a rare congenital disease that can manifest with cardiac anomalies, multiple lentigines, ocular hypertelorism, growth retardation, and deafness. The purpose of this case series was to review the most prominent comorbidities associated with LEOPARD syndrome, and describe perioperative outcomes in a series of patients undergoing anesthesia.

DESIGN:

Retrospective case series review

SETTING:

Tertiary care institution

PARTICIPANTS:

Patients diagnosed with LEOPARD syndrome who underwent surgical procedures requiring anesthesia at this institution.

INTERVENTION:

The medical and anesthesia records of patients with LEOPARD syndrome were reviewed. Demographic information, clinical features of LEOPARD syndrome, comorbidities, intraoperative and postoperative events and complications were recorded. A systematic literature review also was conducted.

MEASUREMENTS AND MAIN RESULTS:

Nine patients with LEOPARD syndrome underwent 49 procedures under general anesthesia (n = 40) or monitored anesthesia care (n = 9). The majority of operations were related to correction of cardiac anomalies (n = 20). The most common cardiac malformations were ventricular septal hypertrophy and pulmonary (or subpulmonary) stenosis, and major perioperative complications were related to severe arrhythmias and/or cardiac decompensation.

CONCLUSIONS:

Dominant pathology associated with perioperative complications in patients with LEOPARD syndrome is related to cardiac disease. A large proportion of patients with this condition have ventricular septal hypertrophy, which tends to progress with age; therefore, these patients undergoing anesthesia should have recent cardiologist evaluation.

KEYWORDS:

LEOPARD syndrome; anesthesia; arrhythmia; ventricular septal hypertrophy

PMID:
24461361
DOI:
10.1053/j.jvca.2013.09.015
[Indexed for MEDLINE]
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