Managing lipid disorders in pregnant women with lysosomal acid lipase deficiency (LAL-D), who are at increased risk of dyslipidemia and early atherosclerosis, can be challenging, albeit essential to preventing adverse pregnancy outcomes.

“There are inherent challenges in the treatment of dyslipidemias during pregnancy and the postpartum period given the lack of adequate data in this population and the contraindication of traditional therapeutic agents. However, it remains of utmost importance to optimize screening and identification of patients at high-risk for atherosclerotic cardiovascular disease so that proper counseling can be provided and the risk for pregnancy complications and downstream cardiovascular complications can be addressed,” wrote Gurleen Kaur, MD, and Martha Gulati, MD, MS, in a review article published in Progress in Cardiovascular Diseases.

Prior to the advent of enzyme replacement therapy, lipid-lowering drugs, such as statins, were frequently prescribed to LAL-D patients, particularly those with milder cholesteryl ester storage disease phenotypes. However, the use of statins during pregnancy is not recommended by several institutions, although there is no clear evidence that statins are teratogenic. Therefore, the use of statins during pregnancy should be carefully considered by patients and clinicians. Moreover, certain statins, such as pravastatin, are being investigated as potential therapeutic agents for preeclampsia.


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Data on the use of nonstatin lipid-lowering drugs such as ezetimibe, a cholesterol absorption inhibitor that has been tested as monotherapy or in combination with statins in treatment of LAL-D, in pregnant women is limited. However, studies in animal models have demonstrated a risk to the fetus. Hence, treatment with ezetimibe should be discontinued at preconception and during lactation under appropriate clinical guidance.

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Bile acid sequestrants, another treatment strategy for LAL-D patients, are safer for pregnant women than other lipid-lowering agents as they do not pass into systemic circulation. These include, for example, cholestyramine.

Lipid metabolism suffers considerable physiologic alterations during a normal pregnancy. There is an increase in lipid concentrations as gestational age increases as consequence of hormonal alterations that promote lipogenesis over lipolysis. However, maternal dyslipidemia beyond physiologic levels is associated with adverse pregnancy outcomes, including preterm birth, preeclampsia, and large-for-gestational-age infants. Screening for and adequately managing atherogenic dyslipidemia during pregnancy by optimizing lifestyle and using adequate pharmacological approaches when needed is of the utmost importance.

Reference

Kaur G, Gulati M. Considerations for treatment of lipid disorders during pregnancy and breastfeeding. Prog Cardiovasc Dis. Published online November 16, 2022. doi:https://doi.org/10.1016/j.pcad.2022.11.001