#4772 DOUBLE FILTRATION PLASMAPHERESIS DURING HETEROLOGOUS FERTILIZATION PREGNANCY FOR THE TREATMENT OF HETEROZYGOUS FAMILIAL HYPERCHOLESTEROLEMIA

Abstract

Abstract Background and Aims Pregnancy in women affected by familial hypercholesterolemia is rare. Estrogen and progesterone fluctuations, featuring pregnancy, adversely influence lipid metabolism, causing considerable increase of LDL-C, triglyceride and lipoprotein A levels. These changes in fatty metabolism, essential for embryonic development, might be critical for placental circulation and for mother's health. Indeed, high lipoprotein amount can raise uteroplacental vascular resistance and it is associated with intrauterine growth restriction because of oxidative stress, whereas hypertriglyceridemia and hypercholesterolemia might cause an increased risk of cardiovascular accidents and pancreatitis. Lipid-lowering drugs, such as statins, ezetimibe or PCSK9 inhibitors are discontinued due to possible teratogen effects. Thus, LDL apheresis is the only available therapy for severe hypercholesterolemia during pregnancy. Method A 46 years old woman affected by heterozygous familial hypercholesterolemia in treatment with PCSK9 inhibitors and statins, decided to undergo an heterologous in vitro fertilization with oocyte donation. Hence, pharmacological therapy was stopped to avoid teratogen effects. We started a 10-months apheresis program with double filtration plasmapheresis (DFPP). The treatment was performed by a temporary double lumen central venous catheter. Initially, she underwent apheresis twice a month with an average exchange volume of 1.2, low blood flow (≤ 90 ml/min) and incremental plasma flow (from 5 to 30 ml/min every 10 minutes of treatment), to ensure a physiological procedure. We gradually modulated the exchange volume monitoring blood levels of LDL-C, Lp(a) and triglycerides before and after each procedure. We aimed to keep mean LDL-C level < 200 mg/dl. We did not use anti-coagulation during the procedure, monitoring TMP values and arterial-venous pressure. Results LDL-C levels remained about 227.2 ± SD 91 mg/dl, with a median of 186.8 mg/dl, complying with the target range (Figure 1). Mean triglycerides and Lp(a) values were, respectively, 180.3±94 mg/dl (median 146 mg/dl) and 77.3±36.3 mg/dl (median 55.7 mg/dl) (Figure 1). Our patient did not face any complications related to pregnancy or to the procedure, and gave birth at 38th gestational week to an healthy full-term male baby of 52 centimeters and 3.34 kilograms. Conclusion Although there is a widespread hesitation in performing extracorporeal blood purification treatment in pregnancy, this case underlines the essential role of LDL apheresis in in this setting, giving spark to standardize the procedure and to encourage its use also in pregnancy.