Safety and efficacy of therapeutic plasma exchange in a pregnant patient with familial hypercholesterolemia

Abstract

Dear Sir, Pregnant patients with familial hypercholesterolemia might have an increased risk for premature cardiovascular disease. Treatment such as plasmapheresis appears to be a safe and effective procedure. Plasmapheresis is uncommonly used in pregnancy (1,2), although the technology is widely available. Using modern technology has reduced the complications and morbidity (3). Thus its value in pregnancy is underrated, especially because of the pharmacological limitations of treating many conditions in pregnancy. Because of such limitations, we decided to use plasmapheresis in a patient with familial hypercholesterolemia. To our knowledge, only two patients with this condition have been similarly treated (1,2). Familial hypercholesterolemia is an inherited disorder characterized by excessive amounts of cholesterol in the blood and increased levels of low-density lipoproteins, tendinous xanthomas and premature coronary artery disease. Approximately 1 in 500 patients inherit a single copy of the gene and present with cholesterol levels ranging from 300 to 500 mg/100 ml (4). We present a young woman who had a myocardial infarction of the anterior wall at age 26, significantly increased plasma lipid levels, and a history of angina pectoris. Coronary angiography showed stenosis of the left anterior descending coronary artery. At that time, the diagnosis of familial hypercholesterolemia was made by the presence of hypercholesterolemia in both parents and one sibling (father with three myocardial infarction episodes and a heart transplant, mother with myocardial infarction and bypass grafting, brother with two myocardial infarctions). Seven years later, at age 33, the patient was referred to our hospital at 28 weeks in her first pregnancy. The patient complained of pain in the thoracic cage (angina pectoris), neuralgia of the right facial nerve and the skin of the right lower limb. The electrocardiogram was normal. The serum lipid levels were: total cholesterol 510.6 mg/dl, high-density lipoprotein-C 74.8 mg/dl, low-density lipoprotein-C 383.2 mg/dl, and triglycerides 262.8 mg/dl. Due to the limitation of pharmacological therapy associated with pregnancy, the cardiologist recommended treatment with plasmapheresis with low-density lipoprotein apheresis. Plasmapheresis with a double filtration system using the Autopheresis C System (Fenwal, Lake Zurich –USA) was performed at 30 weeks of pregnancy. The plasma volume removed was 1,980 ml, and the patient received 1,400 ml of 0.9% NaCl, 200 ml of 20% albumins and 400 ml of acid-citrate-dextrose solution. Two days after the procedure, total cholesterol had decreased by 29%, low-density lipoprotein-C by 36%, and triglycerides increased by 14% and the patient's symptoms of angina pectoris subsided. The lipid profile was repeated again at 32 and 34 weeks of gestation and indicated only narrow change (TC-382.0 mg/dl and LDL −264.0 mg/dl). At 34 weeks of gestation the patient was again evaluated by the cardiologist who recommended cesarean section on the indication of active ischemic heart disease and increased risk of myocardial infarction. The patient was delivered at 35 weeks gestation by planned cesarean section (male, birthweight 2,800 g). The cesarean section was performed under spinal/epidural anesthesia at the department of cardiology. After delivery the cardiologist recommended treatment with oral statins, metoprolol, clopidognel bisulphate and acetylsalicylic acid. On postpartum day 5, coronary angiography revealed multiple atherosclerotic changes without significant stenosis. The main concern here was aggravation of coronary insufficiency in the mother, predominantly due to the hemodynamic changes that occur during pregnancy. Thompson et al. showed that if plasmapheresis was repeated every 2–3 weeks, the average cholesterol level would fall by 50% (5). Because of a cardiovascular indication for an early delivery (history and risk of infarction), we could not proceed with another plasmapheresis. However, we think that regular plasma exchange may significantly lower plasma lipid levels, when there is a risk that pregnancy may have deleterious effects on the maternal cardiac status. By temporarily decreasing the plasma lipid levels, the patient's cardiac condition remained stable through the pregnancy and we were able to deliver the patient without too much risk of preterm complications for the baby as well as minimize possible harmful effects for the mother. Plasmapheresis has been shown to cause regression or delay the onset of atherosclerosis in familial hypercholesterolemia and seems to be a safe and effective way to temporally lower the plasma lipid concentrations.