Some of the complications of acute fatty liver of pregnancy include diabetes insipidus, disseminated intravascular coagulopathy, spontaneous labor, and postpartum hemorrhage.
The disease has been associated with a deficiency of long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD). The fetus is usually homozygous for the mutation while the mother is heterozygous. Long-chain fatty acids accumulate on the fetal side and then transfer to the mother's circulation, Dr. Koteish said.
He advised performing genetic tests on the mother, father, and infant for the various mutations that can occur to the LCHAD gene, because the infant is at risk of sudden death, cardiomyopathy, and neuromyopathy.
Diseases coincidental with pregnancy
Patients with hepatitis B or C virus infections that occur coincidentally with pregnancy do not need therapy, Dr. Koteish advised. HBV infection is transmitted to the fetus 90% of the time when the mother is positive for hepatitis B e antigen. However, hepatitis B vaccine given at birth provides protection against transmission.
HCV transmission to the fetus occurs 6%–10% or more of the time, depending on the mother's viremic load.
Once the disease activity of women with autoimmune hepatitis or Wilson's disease becomes stable, fertility returns. These patients may conceive but should continue therapy.
Herpes simplex virus hepatitis represents a threat to the fetus. Visceral dissemination of HSV, though normally rare, occurs more commonly in pregnancy, typically in the second or third trimester. The symptoms and signs of HSV hepatitis include a flulike prodrome, vesicles in about half of patients, and high ALT and AST levels. A biopsy and serology or culture are enough to make the diagnosis. Once the infection is under control with acyclovir, delivery can occur with less risk of transmission to the infant.
Hepatitis E virus infection is possible on trips to endemic areas, such as tropical and subtropical countries including India and Mexico. The virus normally has a fecal-oral route of transmission, but vertical transmission has been reported. A self-limited illness develops in about 80% of women, but up to 20% progress to liver failure; the disease can lead to high fetal complication rates as well as an increasing risk of fatality as term approaches.
Fertility is reduced in cirrhosis, making pregnancy rare, but pregnancy is more common in women with noncirrhotic portal hypertension. Little data exist on the risks involved in pregnancy with noncirrhotic portal hypertension, but reports have documented rates of fetal wastage in 8%–20%, spontaneous abortion in 15%–20%, and perinatal mortality in 11%–18%. Maternal complications develop in 30%–50% of women, but these tend to be less common and less severe if the woman is diagnosed and undergoes decompression before conception.
If a woman has received a liver transplant, pregnancy does not seem to affect the functioning of the graft. However, conception needs to be delayed for at least 6 months after the transplant surgery because of the risk of cytomegalovirus infection. Immunosuppressive drugs should be continued throughout the pregnancy.