Severe Hypertriglyceridemia in Pregnancy

Context: Pregnancy-related hypertriglyceridemia is rare, but it can be life threatening in some patients with genetic susceptibility. Complications can include acute pancreatitis, hyperviscosity syndrome, and possibly preeclampsia. We present a case of successful management of recurrent gestational chylomicronemia due to compound heterozygous mutations in the LPL gene. Evidence Acquisition: To outline advances in clinical management of this condition, we searched English language publications in PubMed, EMBASE, and ISI Web of Science (search terms: pregnancy, pregnancy complications, pregnan(star), hyperlipoproteinemia, hypertriglyceridemia, chylomicrons, chylomicronemia) and reference lists of relevant published articles from 2002 to 2011. We identified eight case reports. Evidence Synthesis: Interventions reported in those cases are reviewed including: 1) low-fat diet; 2) nutritional supplements; 3) oral prescription medications; 4) parenteral heparin; 5) insulin infusion in the context of hyperglycemia; and 6) therapeutic plasma exchange. Conclusions: Overall, our recommendations are to monitor for pregnancy-related hypertriglyceridemia in those with prepregnancy fasting triglyceride level greater than 4 mmol/liter and to institute therapy when triglyceride level increases to more than 10 mmol/liter. Therapy should include a multidisciplinary team to address dietary fat restriction, appropriate supplements, and possible medications when needed. Admission to hospital is recommended in severe cases. We conclude that complications are preventable with appropriate and timely intervention. (J Clin Endocrinol Metab 97: 2589-2596, 2012)