Severe hypertriglyceridaemia (HTG) is a rare condition in pregnancy, which can lead to life-threatening morbidities for mother and fetus. We present two cases of women with severe HTG in pregnancy and recurrent episodes of pancreatitis. Both women were of Indian descent and had diet-controlled gestational diabetes. Despite being prescribed a very-low fat diet (<20% of caloric intake) and omega 3 fatty acid supplementation, peak plasma triglyceride (TG) levels in the two cases were 39.1 mmol/L and 47.3 mmol/L, respectively. In both cases, we used intermittent insulin/dextrose infusions in combination with fasting, which was modestly effective in reducing TG levels although the effect was not sustained on discharge from hospital and resumption of dietary restriction. Post-partum both women have ongoing elevations in TG levels. Genetic testing showed the women to be compound heterozygous for loss-of-function variants in LPL and GPIHBP1, respectively, genes which encode for key proteins in the lipolytic pathway.
Complications of severe HTG in pregnancy include acute pancreatitis, hyperviscosity syndrome and preeclampsia. Various treatment strategies for severe HTG in pregnancy are described in limited published case reports. The mainstay of treatment is dietary modification, but many cases require additional treatment. Other options include omega 3 fatty acids, fibrates, niacin, intravenous heparin, intravenous insulin/dextrose and plasma exchange. There is insufficient evidence to recommend any single treatment modality over another; in these cases we chose to use intermittent insulin/dextrose infusions in combination with fasting in light of the perceived lower risk to mother and fetus associated with this treatment option. HTG is a rare but important condition in pregnancy to diagnose and increased awareness of this condition may lead to improved outcomes.