Successful management of a pregnancy complicated with familial hypertriglyceridemia: A case report

Case Report

HJOG 2026, 25 (1), 72-75 | doi: 10.33574/hjog.0617

Nithesh Prabhu, G. Shyamala

Department of Obstetrics and Gynecology, Kasturba Medical College, Manipal Academy of Higher Education,
Manipal, India.

Correspondence: Dr. Nithesh Prabhu, Assistant Professor, Department of Obstetrics and Gynecology, Kasturba Medical College, Manipal Academy of Higher Education, Manipal, India, Tel.: +919008897493, e-mail: nitheshprabhu92@gmail.com


Abstract

Background: Pregnancy complicated with familial hypertriglyceridemia is a high risk pregnancy, and if improperly managed, can lead to life threatening complications like acute pancreatitis. We present a case of successful management of a severe hypertriglyceridemia in pregnancy in a patient with Type 4 dyslipidemia in a tertiary care center of South India.
Case: A 32 year old Rh negative primigravida was a known case of familial hypertriglyceridemia. She had a progressive increase in triglyceride levels during the pregnancy which required multiple hospital admissions with a multidisciplinary approach for stringent lipid control with dietary modifications, lipid lowering drugs, insulin infusion along with periodic monitoring of the fetus. The pregnancy was uneventful and she delivered a healthy baby without any postnatal complications.

Keywords: Familial hypertriglyceridemia, pregnancy, high-risk pregnancy, multidisciplinary approach

Case

A 32 year old primigravida, a known case of familial hypertriglyceridemia since 2 years, being managed by a physician presented at 29+weeks for antenatal care. Initially, she was on statins, niacin and saroglitazar [dual Peroxisome Proliferator-Activated Receptor (PPAR) agonist]. She had stopped statins and PPAR agonist after 1.5 years of treatment due to migraine, and had continued on low fat diet, exercise and niacin. Her sister also is a known patient of hypertriglyceridemia, indicating a familial tendency. She had no other comorbidy.
She had conceived spontaneously 4 months after discontinuation of the lipid lowering agents. Confirmation scan was done at 6 weeks, showed an intrauterine gestational sac with cardiac activity. She was started on omega3 fatty acids and folic acid supplementation (500mcg/day). All her routine antenatal investigations were normal. However, triglyceride level at 8 weeks was 1950mg/dL.
Combined aneuploidy screening at 11-12 weeks showed low risk for trisomies as well as pre-eclampsia. At 12 weeks gestation, she was started on gemfibrozil, 300mg twice a day as a lipid lowering agent and aspirin 75mg once a day prophylactically. Detailed fetal structural study at 19weeks of gestation was normal.
Triglyceride levels were monitored periodically, which showed an increasing trend through the second trimester, and reached a peak of 7960mg/dL at 27 weeks. At that point, she received insulin infusion and dextrose, with strict blood glucose monitoring. After the treatment, triglyceride levels came down slightly to 5100mg/dL. She remained asymptomatic throughout pregnancy. Her Glucose Tolerance test was normal. Indirect Coomb’s test done in view of Rh negative status remained negative. At 28 weeks, she received prophylactic Anti D (300mcg, intramuscular). Close maternal and fetal surveillance was done; fetal growth remained appropriate for gestation, with reassuring fetal well being tests.
At 39weeks of gestation, she had a spontaneous vaginal delivery of a healthy male baby, weighing 3kg. Postnatally, triglycerides were 865mg/dL. Lipid profile of the neonate was normal. gemfibrozil was stopped and omega 3 fatty acids were continued post delivery. During her postnatal checkup 6 weeks later, her triglyceride level was near normal.

Figure 1. Patient’s Triglyceride levels in the antepartum and postpartum period with lipid lowering agents used.

Discussion

There are alterations in the lipid homeostasis in pregnancy due to the hormonal milieu. Almost all the lipid components, including the total cholesterol and triglycerides rise in the first 8 weeks; but there is a disproportionate increase in the triglycerides as compared to the other lipid components in a normal pregnancy, causing a profound rise, which is 2-4 times the pre-pregnancy levels by the third trimester.[1] The mean triglyceride levels are 78.8mg/dL, 151.5mg/dL and 239.1mg/dL in the first, second and third trimesters, respectively.[2,3]
“Gestational hypertriglyceridemia is defined as fasting plasma triglyceride level above the age-adjusted 95th percentile for the nonpregnant population.’’ “Severe gestational hypertriglyceridemia is arbitrarily defined as a plasma triglyceride level greater than 1009.7mg/dL’’[4,5] and is associated with complications like acute pancreatitis, hyperviscosity syndrome and preeclampsia. [6,7,8] With preexisting primary or secondary dyslipidemia or insulin resistance, this rise in triglycerides will be enormous.
In familial hypertriglyceridemia, a type IV hyperlipidemia, there are heterozygous inactivating mutations of the lipoprotein lipase gene.[9,10] There is a moderate increase in the triglyceride levels (200-500mg/dL).[11] These patients exhibit impaired catabolism of triglyceride-rich lipoproteins, hepatic overproduction of very low-density lipoproteins (VLDL), increased apo C-III expression, and low serum levels of HDL-C (hypoalphalipoproteinemia)[6,9] .The diagnosis is confirmed when the triglycerides are elevated (>90th percentile) with normal or mildly increased cholesterol (<90th centile).[12]
Wong et al reported a case of severe gestational hypertriglyceridemia induced acute pancreatitis in a primigravida with overt diabetes at 22 weeks who presented with abdominal pain and nausea. Blood work up revealed a lipemic plasma with triglyceride level of >8000mg/dL and elevated lipase. She was treated with intravenous insulin with dextrose infusion followed by supervised fasting which drastically reduced the TG levels to <900mg/dL.[5]
A case of asymptomatic severe gestational hypertriglyceridemia in a primigravida at 21 weeks with hypertensive crisis was reported by Kleess et al. She was treated with antihypertensives, intravenous insulin with intensive glucose monitoring and dietary advice. [4]
Goldberg et al reported a similar case in a multigravida with known deficiency of lipoprotein lipase enzyme. She had 3 successful pregnancies with recurrent chylomicronemia which was treated with a combination of gemfibrozil, low fat diet and omega-3 supplementation. [13]
Cao et al reported two cases of pregnancies complicated with familial hypertriglyceridemia. The importance of compliance with the medications to prevent poor outcomes was demonstrated. Non compliance with gemfibrozil required multiple admissions for treatment of hypertriglyceridemia induced acute pancreatitis while the other patient who was compliant with the lipid lowering medications had no such episode. [11]
Sivakumaran et al reported the successful use of 13 therapeutic plasma exchanges from 19 to 36weeks of gestation in the management of hypertriglyceridemia during pregnancy in a patient with familial hypertriglyceridemia. The patient did not develop pancreatitis and she had an uneventful delivery at term. [14]
In patients with known familial hyperlipidemias, pre-conceptional management is of utmost importance. Lipid lowering agents like statins (gemfibrozil) and niacin (lipid-lowering dose of 1500-3000mg/day) are used. Due to their controversial teratogenicity, [13,15] they are discontinued while planning pregnancy and can be restarted in the second trimester if necessary. Early institution of a low fat diet (<20% of calories from fat/day) and exercise is necessary. During pregnancy, a multidisciplinary team collaboration of a high risk obstetrician, endocrinologist, physician, dietician, physiotherapist and a neonatologist is imperative. Omega 3 fatty acids, 3-4g/day supplementation reduces hepatic triglyceride synthesis and enhances lipoprotein lipase insulin infusion can be used to acutely lower the TG levels. Not only does it reverse hyperglycemia and thereby hyperglycemia-induced inhibition of LPL, insulin is also a rapid and potent LPL activator. [16] Plasmapheresis can be considered if hypertriglyceridemia is severe and refractory to all other therapies. [5]
The above case was reported since pregnancy with severe hypertriglyceridemia is extremely rare. The importance of a strict lipid control to avoid life threatening complications was highlighted. Such a high risk pregnancy should be managed at a tertiary center. The combined efforts by a multidisciplinary team led to a successful uncomplicated term pregnancy with an uneventful postnatal period.

Conflicts of Interest

No competing interests .

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