FCS in Pregnancy and Optimizing Specialist Referral for High-Risk Patients

Pregnancy in a patient with FCS represents one of the highest-acuity scenarios in lipid medicine, with estrogen-driven triglyceride rises compounding an already unmanageable baseline and creating meaningful risk for both mother and fetus.

Alan Brown, MD, MHA, describes managing a patient who had experienced 30 episodes of pancreatitis since age 3, first encountered at age 28 and newly pregnant. With the published literature limited to scattered case reports, and the patient already highly adherent to her diet yet still experiencing recurrent pancreatitis, observation alone was not viable.

Brown describes 2 evidence-supported options beyond strict dietary management: serial plasma exchanges timed to keep triglycerides in check between sessions, and total parenteral nutrition with complete fat restriction for the full duration of pregnancy, requiring prolonged inpatient admission. His patient declined the latter. Plasma exchanges were initiated on a weekly schedule, with triglycerides measured before each exchange to guide the interval. She had no pancreatitis during the pregnancy and delivered a healthy infant.

The case continued after delivery. Enrolled in an early APOC3 inhibitor study, she arrived for her first on-study visit with a positive pregnancy test, this time carrying twins. The plasma exchange strategy was repeated throughout. She delivered 2 healthy babies and has remained pancreatitis-free for more than 5 years on an APOC3 inhibitor. Brown notes that data from centers managing multiple FCS pregnancies show meaningful maternal and fetal mortality, and describes monitoring triglycerides every 2 weeks with a low threshold for intervention as the appropriate approach. No safety data currently exist for newer FCS therapies in pregnancy.

Stephan P. Babirak, MD, PhD, addresses the clinical signals that should prompt any non-specialist to refer. Both experts describe a consistent set of flags: persistent severe hypertriglyceridemia unresponsive to therapy, recurrent pancreatitis, absence of the usual secondary causes, a lean body habitus particularly in younger patients, and a history of unexplained abdominal pain. Babirak notes that dermatologists flagging eruptive xanthomas and ophthalmologists identifying lipemia retinalis have been among his most diagnostically productive referral sources. Brown highlights the role of dietitians as a frontline touchpoint in FCS diagnosis.

Both close by noting that APOC3 inhibitors have changed the management landscape for eligible patients, with Brown emphasizing that getting patients to a specialist who can evaluate them for these agents is the priority.

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Our Panelists:

Alan Brown, MD, MHA, is a professor of cardiovascular medicine at Wake Forest University and a past president of the National Lipid Association.

Stephan P. Babirak, MD, PhD, is the founder and medical director of Metabolic Leader, a metabolic and endocrine care center in Scarborough, Maine, where he specializes in lipoprotein disorders.

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Disclosures for Brown include New Amsterdam, Merck, Novartis, Arrowhead, Amgen, Regeneron, and Ionis. Babirak has no relevant disclosures to report.

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References

1. Javed F, et al. Familial chylomicronemia syndrome: an expert clinical review from the National Lipid Association. J Clin Lipidol. 2025;19(3):382-403. doi:10.1016/j.jacl.2025.03.013

2. US Food and Drug Administration. FDA approves drug to reduce triglycerides in adults with familial chylomicronemia syndrome. FDA. November 18, 2025. Accessed June 12, 2026. https://www.fda.gov/drugs/news-events-human-drugs/fda-approves-drug-reduce-triglycerides-adults-familial-chylomicronemia-syndrome

3. US Food and Drug Administration. FDA approves drug to reduce triglycerides in adult patients with familial chylomicronemia syndrome. FDA. December 19, 2024. Accessed June 12, 2026. https://www.fda.gov/drugs/news-events-human-drugs/fda-approves-drug-reduce-triglycerides-adult-patients-familial-chylomicronemia-syndrome