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

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.

Perioperative Management and Clinical Outcomes of Liver Transplantation for Children with Homozygous Familial Hypercholesterolemia

“…The complexity of these challenging patients and the specific risks of LT require the collaboration of a multidisciplinary team that consists of LT surgeons, anesthesiologists, hepatologists, cardiologists, cardiac surgeons, neurological physicians, pediatricians, intensivists, and radiologists. Generally, patients with HoFH are reported to have severe cardiovascular comorbidities, such as CAD and aortic valve disease, which sometimes require surgical and nonsurgical interventions [ 38 , 39 , 40 , 49 ]. Therefore, caution must be taken in assessing cardiovascular risk to ensure patient safety since preoperative ASCVDs will inevitably increase the risk of postoperative cardiovascular complications [ 32 , 33 , 37 ].…”

“…Perioperative management of patients with HoFH is always challenging, primarily due to premature ASCVDs. However, there are only a few case reports regarding perioperative management of HoFH patients undergoing either dental, endovascular interventional, or open-heart surgery [ 38 , 39 , 40 ]. LT is generally accompanied by severe intraoperative hemodynamic disturbances, making it one of the highest-risk noncardiac surgeries.…”