J Dent Anesth Pain Med.  2020 Feb;20(1):39-44. 10.17245/jdapm.2020.20.1.39.

Minimizing the risk of perioperative cardiovascular complications in homozygous familial hypercholesterolemia: a case report

• Khan S
• Min S
• Willard G
• Lo I
• D'Souza R
• Park A

• KMID: 2471172
• DOI: http://doi.org/10.17245/jdapm.2020.20.1.39

Abstract

Homozygous familial hypercholesterolemia (HoFH) is a rare inherited disorder that presents as abnormally elevated levels of low-density lipoprotein cholesterol and premature heart disease, requiring frequent intervention through lipid apheresis for management. The risk of perioperative cardiac events is higher in patients with HoFH because of its pathophysiological manifestations in the vascular system. Careful cardiac precautions and anesthetic assessments are necessary to ensure patient safety. In the following case report, we discuss the clinical course and anesthetic considerations for a 14-year-old girl with HoFH undergoing sedation for dental extractions and mandibular molar uprighting in an outpatient oral surgery clinic. Considerations included the use of heparin in the patient's weekly plasma lipid apheresis treatment. In order to reduce the risks of peri- and postoperative bleeding and perioperative cardiac events, the operation was scheduled for 4 days after apheresis. This allowed for adequate heparin clearance, while also reducing the likelihood of possible cardiac events. A literature review revealed no results for the outpatient management of patients with HoFH undergoing sedation for noncardiac procedures. Our reported case serves as a clinical example for physicians to be utilized in the future.

Keyword

Homozygous Familial Hypercholesterolemia; Dental Anesthesia; Oral Surgery

MeSH Terms

Adolescent
Anesthesia, Dental
Blood Component Removal
Cholesterol
Female
Heart Diseases
Hemorrhage
Heparin
Humans
Hyperlipoproteinemia Type II*
Lipoproteins
Molar
Outpatients
Patient Safety
Plasma
Surgery, Oral
Cholesterol
Heparin
Lipoproteins

Figure

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  Fig. 1 The panoramic radiograph shows impacted but not clinically erupted third molars, clinically erupted primary teeth close to exfoliation, and palpable mandibular second molars that are not entirely erupted.

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  Fig. 2 The figure shows the planned timeline for the patient before the start of the procedure.

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