Homozygous familial hypercholesterolemia is a rare metabolic disorder with characteristic clinical presentations, such as tendon xanthomas, hypercholesterolemia, and significant cardiovascular disease including premature coronary artery disease. We describe a case of a 56-year-old woman with homozygous familial hypercholesterolemia. She had been treated with low-density lipoprotein apheresis for 23 years. Preoperative echocardiography and coronary angiography showed severe aortic valve stenosis and right coronary artery stenosis. Aortic valve replacement with patch enlargement of the aortic valve annulus, and coronary artery bypass grafting were successfully performed. She was discharged uneventfully.

The purpose of this study was to investigate the pharmacokinetics of teicoplanin (TEIC) in patients undergoing open heart surgery. We also attemped to define the optimum TEIC therapy protocol for prevention of perioperative infection and for treatment of staphylococcal endocarditis such as that caused by methicillin-resistant Staphylococcus aureus (MRSA). Serum TEIC concentrations were measured in 14 patients divided into two groups of 7 patients each undergoing elective open heart surgery. Patients in group I received 400mg of TEIC and patients in group II received 800mg, both administered as a slow intravenous infusion over 20min immediately after induction of anesthesia. The peak serum level (mean±standard error) of TEIC was respectively 57±11 and 139±39μg/ml at 2min after administration and then the TEIC level decreased gradually to 26± 7 and 55±10μg/ml at 60min after administration. The serum level of TEIC decreased rapidly to 17±5 and 31±7μg/ml, respectively, at the start of extracorporeal circulation (ECC), and was 11±2 and 27±6μg/ml after 60min of ECC, 8±2 and 23±7μg/ml at 2min after the termination of ECC, 8±3 and 23±6μg/ml at 60min after the termination of ECC, and 7±2 and 22±5μg/ml on admission to ICU. No side effects were seen during the study, such as red neck syndrome, renal dysfunction, hearing disorders, or postoperative infection. Our results suggested that the optimum dose of TEIC for prevention of perioperative infection was around 400mg, providing levels in excess of the MIC for most pathogens that have been found to cause infection following open heart surgery, including MRSA. In addition, a dose of 800mg was needed to keep trough levels above 20μg/ml for treatment of staphylococcal endocarditis. It was also suggested that half of the initial dose should be administered on admission to ICU and also at the start of ECC if the operation is going to last longer than 7h on the basis of the concentration-time curve.

A 61-year-old man was referred to our hospital for treatment of hemolytic anemia after ascending aortic replacement aortic dissection. Cine mode magnetic resonance imaging (MRI) showed stenosis at the proximal anastomostic site of a Teflon strip. We diagnosed hemolytic anemia induced by collision of red blood cells on the inverted felt strip. Conservative therapy with Sarpogrelate and β-blockers was effective to treat his hemolytic anemia. However, 7 years later he was re-admitted because of infective endocarditis at the aortic valve, and underwent aortic root replacement. Intraoperative findings showed a stiff and inverted Teflon felt strip causing stenosis of the proximal anastomosis. Hemolytic anemia should be considered a rare complication of using a Teflon felt strip to reinforce anastomosis for acute aortic dissection.