CLINICAL OBSTETRICS AND GYNECOLOGY

Volume 66, Number 1, 176–185

Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.

The Spectrum of
Hepatic Critical Care
During Pregnancy:
A Clinical Review
LUCÍA RIVERA MATOS, MD,
and NANCY S. REAU, MD, FAASLD
Rush University Medical Center, Section of Hepatology, Chicago,
Illinois

Abstract: Hepatic disease during pregnancy can Management remains a challenge, given
result in the development of critical illness requiring the need to consider both the mother and
special attention from a multidisciplinary team with
a low threshold for tertiary care transfer to provide the fetus. Although liver test abnormal-
access to liver transplantation. Management of this ities impact 3 to 5 percent of pregnancies,
population requires taking into consideration the they rarely herald progression to liver
benefit and risks of both mother and fetus. failure.1 Still, it can be difficult to separate
A myriad of diseases has been recognized, some early signs of significant liver pathology
being unique to pregnancy while others are common
to the general population. We present a review of from less serious diseases. This is partly
the literature on the diagnosis, management, and from the broad differential of possible
prognosis of these diseases to aid in the optimization explanations. Liver injury can be attrib-
of care in this special population. uted to exacerbations of pre-existing liver
Key words: liver disease, pregnancy, acute liver failure, disease, pregnancy-specific hepatic dis-
critical care, cirrhosis, liver transplant
ease, and acquired liver injury. The degree
of injury ranges from asymptomatic
abnormal liver enzymes to acute liver
LIVER DISEASE DURING failure. In addition, the prevalence of
PREGNANCY pregnancy in women with cirrhosis has
Liver-related obstetric emergencies are increased steadily both due to improve-
uncommon but can be associated ment in the treatment modalities of liver
with significant maternal and fetal risk. disease and the increase in the incidence
of cirrhosis at a younger age. Reproduc-
Correspondence: Nancy S. Reau, MD, FAASLD, Rush tive care in patients with liver diseases
University Medical Center–Section of Hepatology, requires collaboration between obstetri-
1725 West Harrison St. | Suite 319 | Chicago, Illinois
60612. E-mail: Nancy_reau@rush.edu cians, hepatologists, and specialists in
The authors declare no conflicts of interest. maternal-fetal medicine to provide the

CLINICAL OBSTETRICS AND GYNECOLOGY / VOLUME 66 / NUMBER 1 / MARCH 2023

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 Hepatic Critical Care During Pregnancy 177

best outcome. This article will address to increasing pressure of the gravis uterus
pregnancy in women at high risk for liver- onto the vena cava. Maternal mortality
related complications and the manage- may be as high as 20 to 50% with variceal
ment of gravid women with significant bleeding.1
liver injury. Understanding risk before pregnancy is
vital as it allows both interventions to
decrease the risk of complications and a
proactive plan to be in place. The Model
Pregnancy in Women With for End-Stage Liver Disease (MELD)
Established Liver Disease scoring system, which is used for organ
Pregnancy is rare in patients with allocation, is also commonly used to
end-stage liver disease as rates of infertility predict outcomes in patients with liver
are high in decompensated cirrhosis. Dis- cirrhosis for pregnancy. Patients with a
turbances in menstrual periods are seen in MELD > 10 are advised against preg-
up to 25% of patients with advanced liver nancy as they are at very high risk for
disease. This is related to altered estrogen liver-related complications. In contrast, a
metabolism and disruption of the hypo- MELD <6 confers lower risk for severe
thalamic-pituitary axis.1,2 In contrast, pa- liver-related complications.1,5
tients with well-compensated cirrhosis Given the catastrophic consequences of
(cirrhosis without symptoms of liver dis- a variceal bleeding event, guidelines rec-
ease or clinically significant portal hyper- ommended an esophagogastroduodeno-
tension) can be expected to have fertility scopy (EGD) to screen for GEV in all
rates similar to the general population.2 women with cirrhosis within 12 months of
Traditionally, cirrhosis was felt to be a conception (Fig. 1).1,6 Nonselective beta-
disease of advanced age. However, the blockers are recommended for patients
prevalence of cirrhosis secondary to meta- with GEV without high-risk stigmata.
bolic-associated fatty liver disease and The presence of high-risk stigmata should
alcohol-related liver disease has been in- trigger a band ligation protocol to erad-
creasing in reproductive-aged women.1–3 icate GEV before conception.1,6
The peripartum course may be impacted If EGD was not performed before
by the underlying liver disease, and up to conception, guidelines recommend that pa-
24% of patients with advanced liver dis- tients with cirrhosis should have an EGD in
ease develop hepatic decompensation dur- the second trimester (Fig. 2).1,6 Although
ing pregnancy.4 Physiological changes in algorithms using noninvasive testing and
pregnancy can worsen the hemodynamic platelet counts can estimate the risk of
consequences of portal hypertension, thus clinically significant portal hypertension in
increasing the risk of variceal bleeding and patients with compensated cirrhosis, these
other liver-related complications.2 Preg- tests have not been validated in pregnancy.
nancy itself leads to a hyperdynamic state Performing EGD in pregnancy is safe, and
with an increase in cardiac output that the American College of Gastroenterology
when combined with splanchnic vasodila- advises to use meperidine and propofol as
tion in cirrhosis, ends up in overall impair- sedative agents, while benzodiazepines
ment of circulatory function. The most should be avoided.6
catastrophic sequelae of portal hyperten- For gravid women that present with
sion during pregnancy is gastroesophageal gastrointestinal bleeding, management is
variceal (GEV) bleeding, which most com- similar to the nonpregnant population.
monly occurs in the second trimester and Initial management is focused on fluid
during delivery, specifically during the resuscitation and hemodynamical stabili-
second stage of labor. This is attributed zation of the mother. Infection prophylaxis

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178 Matos and Reau

FIGURE 1. Algorithm for variceal screening within 1 year of preconception.1,6

with antibiotics should be started, and considered.1 This procedure is performed

cephalosporins are the antibiotic class of by interventional radiologists and consists
choice. The use of octreotide infusion of creating a connection between the portal
(category B) is also recommended to de- and hepatic vein, leading to a bypass of the
crease portal blood flow and pressures.6 It portal circulation with the goal of relieving
is important to note that over-transfusion portal hypertension. The procedure carries
has been associated with poorer outcomes the risk of radiation which should be
due to an increase in portal pressures, thus discussed with the mother; however,
a transfusion goal to hemoglobin level of cases of TIPS in pregnancy with successful
7.0 g/dL is recommended.7 The primary deliveries have been described.8
therapy for variceal bleeding in pregnancy Limited data is available regarding
is endoscopic variceal band ligation mother and infant outcomes in patients
(EVBL) and in cases of refractory hemor- with known liver cirrhosis; however recent
rhage, Transjugular Intrahepatic Portosys- retrospective cohorts have shown a higher
temic Shunt (TIPS) procedure should be frequency of adverse events in pregnancy,

FIGURE 2. Algorithm for variceal screening during pregnancy.1,6

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 Hepatic Critical Care During Pregnancy 179

including liver-related complications,9 an-  Acute variceal bleeding during preg-

tepartum and postpartum hemorrhage,9,10 nancy requires coordinated care, pref-
intrahepatic cholestasis of pregnancy erably in a center able to offer access to
(ICP),9,10 metabolic complications,10 pre- liver transplantation
term birth,9–11 and puerperal infections.8
Cirrhotic women were more likely to
require induction of labor and cesarean
birth.9–11 Infants born to women with Acute Liver Injury (ALI) and
compensated cirrhosis were more likely Acute Liver Failure (ALF)
to be large for gestational age or have Acute onset of hepatic disease in preg-
stillbirth, respiratory distress, or death nancy can be secondary to a myriad of
within 1 year of birth.10 However, infants causes but most commonly include viral,
born to women with decompensated drug-induced, vascular, autoimmune, and
cirrhosis were small-for-gestational-age pregnancy-specific complications.
compared with their matched control Severe acute liver injury (ALI) from
individuals.10 any etiology can progress to acute liver
Most importantly, maternal mortality failure (ALF), defined as ALI [jaundice,
rates are very low, and rates of stillbirth, abrupt increase in serum aminotransfer-
gestational diabetes, and congenital mal- ases (2-3 fold), and coagulopathy (INR >
formations were not increased when com- 1.5)] followed by hepatic encephalopathy
pared with the general population.11 (HE) in a patient without known liver
Improvement in rates of mortality likely disease.12 The presence of HE is required
reflects better multidisciplinary care. for ALF, which can be further subdivided
 Women with established liver disease based on the timeframe of HE onset
should have an assessment for cirrhosis (Table 1).12 Subacute liver failure carries
before conception. the lowest chance for spontaneous recov-
 Individuals with cirrhosis and MELD ery, making a delay in onset a poor
> 10 should be advised that pregnancy prognostic sign and a reason for extra
carries a significant risk for hepatic vigilance.
decompensation. Presentation, evaluation, and man-
 Individuals with cirrhosis should have agement of hepatic failure in pregnancy
an EGD in the 12 months before are similar to the general population.
conception to screen for gastroesopha- Though, the decision to try to maintain
geal varices (GEV) and, if present, the pregnancy versus terminating the
begin therapy based on their size and pregnancy will be dependent on whether
stigmata. the hepatic compromise is induced by
 If an EGD was not done before con- pregnancy as well as the viability of the
ception, pregnant women with cirrhosis infant. All women with ALI should be
should have GEV screening in the admitted and monitored closely for signs
second trimester of pregnancy. of HE, and all women with signs of

TABLE 1. Classifications of Acute Liver Failure Based on Symptom Timeline12*

Classification Definition
Hyperacute liver failure Development of HE within 7 d of jaundice onset
Acute liver failure Development of HE within 8 to 28 d of jaundice onset
Subacute liver failure Development of HE within 5 to 12 wk of jaundice onset

*Considering jaundice as the first symptom.

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180 Matos and Reau

hepatic failure should be immediately Stabilize and Monitor Appropriately

moved to an institution where liver Management of ALF is multidisciplinary.
transplantation (LT) can be considered. Support should be provided with inten-
sive care unit (ICU) monitoring along
INITIAL EVALUATION with N-acetylcysteine (NAC) infusion
Establishing prognosis, stabilization, and regardless of etiology. Liberal use of
a search for an etiology should begin NAC is now encouraged as trials have
immediately (Table 2). shown improvement for early coma grade
as well as transplant-free survival inde-
pendent of acetaminophen toxicity.12
Prognosis The most feared complication of
Determining the disease severity is acute liver failure is cerebral edema
imperative and dynamic. INR, fibrino- and intracranial hypertension (ICH),
gen, factor V level, liver blood tests, renal which can rapidly progress to herniation
function, arterial ammonia level and and brain death. Close neurological
blood gas, and serum lactic acid should monitoring should be untaken, and if
all be done on admission. Although there there is a concern for worsening ence-
are several scoring systems, the King’s phalopathy to Grade III-IV, intubation
College criteria (Table 3) are most fre- is advised with a low threshold to
quently used to assess prognosis, priori- discuss intracranial pressure monitor
tization for referral, and the need for liver placement.12
transplantation. It is important to keep in Risk for ICH is the highest in those
mind that the patient’s clinical condition with hyperacute or acute ALF, younger
can deteriorate rapidly. Thus, frequent age, concomitant renal failure, the need
serial monitoring of coagulation, liver for inotropic support and persistent ele-
chemistry, glucose level, lactic acid, and vation in arterial ammonia.12
hemodynamic status is warranted.12 Hy- Multiple organs are impacted by ALF
perammonemia also carries prognostic other than just the brain and will influ-
value, with the risk for cerebral edema ence management. Additional pathology
being low if serum ammonia level is less may also be specific to the etiology.12
than 150 μmol/L, whereas levels  Volume resuscitation is important as
> 200 μmol/L, that do not drop with most patients are volume depleted,
interventions are at high risk for cerebral however, volume overload is important
edema.12 to avoid.
 Hypotension should be managed with
vasopressors; noradrenaline is the vas-
opressor of choice.
 Avoid oral and nasogastric feeding in
TABLE 2. Immediate Measures on those with hepatic encephalopathy.
Evaluation of Patients With  Hypoglycemia is common, and glucose
ALI/ALF12
infusion should be standard.
Exclusion of liver cirrhosis, alcohol-induced liver  Renal Replacement Therapy (RRT)
disease, and malignant infiltration should be used to correct the acidosis
Intensive screening for signs of HE
Initiate early discussion with a transplant center. and metabolic derangements, including
Workup etiology of injury hyperammonemia.
Transfer to Transplant Center if INR > 1.5 and  Changes in PT are important for prog-
shows the presence of hepatic encephalopathy nosis. Correction of coagulation is only
Contraindications to liver transplant should not recommended for active bleeding and
exclude transfer to the tertiary care center
invasive procedures.

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 Hepatic Critical Care During Pregnancy 181

TABLE 3. King’s College Criteria

King’s College Criteria for Acute Liver Failure (ALF)

Acetaminophen-induced All Other Causes of ALF

Arterial pH <7.3 irrespective of HE PT > 100 s/INR > 6.5
OR all of the below OR
HE > Grade III or IV HE AND Any 3 of the following:
Serum creatinine > 3.4 mg/dL (300 µmol/L) AND Age <10 y or > 40 y
Etiology: indeterminant, drug-induced hepatitis
Interval jaundice encephalopathy > 7 d
Serum Bilirubin > 18 mg/dL (300 µmol/L)
INR > 3.5 (PT > 50 s)
INR > 6.5 or PT > 100 s

 Sedatives should be avoided, if possible, Baseline chest radiograph, electrocardio-

but when needed, propofol is preferred gram (ECG), and hepatic ultrasound with
for sedation. dopplers to exclude vascular thrombosis
 Patients with ALF are at risk for should also be obtained.
bacterial infection, and surveillance
for infection should be considered. ACUTE VIRAL HEPATITIS
Pregnancy has little impact on the inci-
Search for an Etiology dence or severity of acute viral hepatitis
Causes for acute hepatitis and ALI apart from hepatitis E virus (HEV) and
include viral, vascular, genetic (ie, herpes simplex virus (HSV). Management
Wilson’s disease), autoimmune, ischemic, of acute viral hepatitis in pregnancy is
and pregnancy-specific etiologies (pre- primarily supportive, similar to the non-
eclamptic liver rupture, HELLP, and pregnant population.
fatty liver of pregnancy).1,12 Exacerba-
tions of existing chronic liver disease HEPATITIS E VIRUS (HEV)
must also be considered. Worldwide viral HEV is transmitted from the fecal-oral
hepatitis (HEV, HBV) accounts for the route. Pregnant women, especially in the
majority of ALF, however, in the United second and third trimester, are at risk for
States and Europe, acetaminophen is the severe outcomes, including fulminant
primary explanation.12 In the pregnant hepatitis and high rates of neonatal fatal-
population, pregnancy-specific liver inju- ity (up to 33%).1 Infection is most com-
ries are also a common explanation for mon in countries with poor sanitation but
ALF, especially in the late second and has also been seen in developed countries.
third trimester. Onset can also occur in A high index of suspicion is important.
the early postpartum period. Though ribavirin and interferon may
On admission, serology to assess for offer antiviral effects, both are contra-
acute or reactivated viral hepatitis indicated in pregnancy, making treatment
(HBsAg, anti-HBc IgM, anti-HAV IgM, primarily supportive. Given the high rates
anti-HEV IgM, anti-HSV IgM, anti-VZV of fulminant hepatitis, liver transplanta-
IgM, CMV, HSV, EBV, and VZV PCR) tion should be considered in those
should be sent. Autoimmune markers, progressing to acute liver failure.13,14
toxicology screen, and acetaminophen Rates of maternal to child transmission
level should be assessed. Labs to assess (MTCT) have been reported between 33
criteria for acute fatty liver of pregnancy to 100% and may impact peripartum
(AFLP) should be drawn. management.1

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182 Matos and Reau

HERPES SIMPLEX VIRUS (HSV) patients with unrecognized advanced

Pregnancy increases the risk of dissemi- fibrosis.17 Rates of reactivation are delayed
nated HSV infection. HSV hepatitis should but unchanged by the use of antiviral
be considered with a presentation of fever therapy. AASLD recommends discontinu-
and high aminotransferase levels, leukope- ation of the antiviral therapy started to
nia, thrombocytopenia, and renal compro- prevent MTCT at delivery or in the first 12
mise. Rash is uncommon. As the disease weeks postpartum1 with careful monitor-
can rapidly worsen, empiric therapy with ing for 6 months postpartum or postanti-
acyclovir is justified. There are several case viral cessation. Patients with cirrhosis or
reports of successful LT in patients that who meet traditional indications for ther-
progress to ALF.1 apy should not stop the treatment.

HEPATITIS B VIRUS (HBV) AUTOIMMUNE HEPATITIS (AIH)

Acute HB infection (AHB) in pregnancy AIH does not always impact fertility,
is usually mild and does not have adverse making this an important condition to
effects on the fetus. Clinical presentation consider in the differential between in-
consists of fever, fatigue, jaundice, and creased liver enzymes and ALI during
loss of appetite. A prospective study pregnancy. Though liver enzymes in
found that fever was less common in women with AIH tend to improve during
pregnant patients with AHB when com- pregnancy and spontaneous remission has
pared with the general population.15 been reported, severe flares can occur at
Clinical recovery from the infection is any time.1 A flare during pregnancy can
similar in pregnant and nonpregnant pa- lead to maternal-fetal complications such
tients. However, it has been suggested as fetal loss and stillbirth. ALF leading to
that pregnancy might be a risk factor for liver transplantation has also been re-
the development of chronic infection. ported.
Like the nonpregnant population, ALF
from AHB can occur. Liver transplant for DE NOVO AIH
ALF related to acute HBV infection De novo presentation of AIH during
during pregnancy has been described with pregnancy can vary from asymptomatic
favorable outcomes for both mother and liver enzyme elevations to ALF or even
fetus.16 complications from portal hypertension,
Chronic hepatitis B virus (cHBV) with 25% experiencing acute severe AIH.
infection is generally well tolerated during Coexisting autoimmune disease should
pregnancy though severe symptomatic raise concern for diagnosis, and diagnostic
flares during pregnancy or postpartum have workup is the same in pregnant and
been described. Antiviral therapy is recom- nonpregnant patients apart from a liver
mended for women with high risk for biopsy, which is reserved for cases of
MTCT despite active/passive immuniza- diagnostic uncertainty in pregnancy. Lab-
tion. Although a symptomatic flare can oratory testing should include quantitative
occur at any time during pregnancy, they immunoglobulins and immune markers:
are most common in the first 12 weeks anti-nuclear antibody, anti-smooth muscle
postpartum or after discontinuation of antibody, anti-mitochondrial antibody
antiviral therapy.1 and anti-liver/kidney microsome anti-
Even when they occur, HBV flares body. Antibody-negative AIH is also well
commonly resolve spontaneously, but the described making clinical suspicion imper-
development of ALF has been described, ative. Liver biopsy should be considered in
and severe HBV reactivation leading to atypical cases and in the absence of
fulminant liver failure is more common in serological markers when suspicion is

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 Hepatic Critical Care During Pregnancy 183

high. Biopsy is generally deferred until hypercoagulable states, which can be

after the second trimester if possible. exacerbated by pregnancy. Clinical pre-
Upon diagnosis of AIH, evaluation for sentation commonly includes acute ab-
cirrhosis is of high importance as one- dominal pain, ascites, hepatomegaly, and
third will have established cirrhosis at the lower extremity edema. Distinction of
time of presentation.1,18 Treatment is also ascites and hepatomegaly can be chal-
similar to steroid induction and the addi- lenging on the physical exam during
tion of azathioprine for steroid-sparing pregnancy, with a low threshold for assis-
effects after 2 weeks. Those that present tance from ultrasonography. Diagnosis of
with acute severe AIH should be assessed thrombosis can be made by ultrasonog-
for liver transplantation, especially if they raphy with dopplers of the abdomen with
do not respond rapidly to steroids. CT venography if ultrasound is nondiag-
nostic, but suspicion is high. Initial man-
CHRONIC AIH agement is with low-molecular weight
Approximately 20% of patients with AIH heparin (vitamin K antagonists are con-
will flare in pregnancy, and this is asso- traindicated in pregnancy). Thromboly-
ciated with both maternal and fetal sis, thrombectomy, TIPS, and portocaval
complications.18 Flares of AIH can lead shunt can be considered in those with
to hepatic compromise, which compli- progression despite heparin. While TIPS
cates up to 11% of pregnancies in women results in a bypass of the obstruction, it
with known AIH.1 The risk of decom- does involve radiation exposure to both
pensation leading to LT or death is the mother and fetus. However, TIPS safety
highest in women with cirrhosis (21% vs has been recognized in recent case series.8
4% without cirrhosis).1 Assessment for Warfarin and vitamin K antagonists
biochemical remission should be done in should be avoided during pregnancy due
each trimester. In addition, flares are as to teratogenic effects, however, if alter-
twice as common in the postpartum peri- native agents are not feasible, it should at
od after immune system reconstitution least be avoided during the first trimester
making close monitoring imperative for and 2 to 3 weeks before anticipated
the first 12 weeks. The risk of flare is delivery.21
much lower with continued use of immu- Incidence of BCS during pregnancy has
nosuppression which is recommended been reported as 6.8%.22 The disease can
during pregnancy. progress rapidly to acute liver failure
It is also important to recognize an without appropriate management. A trial
increased incidence of pregnancy-associated of anticoagulation should not delay LT
liver disease in women with AIH, including evaluation in those that present with
gestational diabetes and hypertensive com- ALF. Successful LT for acute BCS during
plications in pregnancy (gestational hyper- pregnancy has been described in case
tension, eclampsia, pre-eclampsia, and reports.23
HELLP syndrome).18–20 Rates of pre- Pregnancy outcomes in patients with
eclampsia are as high as 30%.20 Low-dose known and treated BCS are favorable,
aspirin is recommended from 12 to 16 though higher rates of fetal loss are
weeks gestation in groups at high risk for reported, especially before 20 weeks.24
pre-eclampsia and should be offered to
women with known AIH.20 LIVER DISEASE UNIQUE TO
PREGNANCY
BUDD-CHIARI SYNDROME Liver disease unique to pregnancy is a
Budd-Chiari Syndrome (BCS) or hepatic spectrum of injuries, including hyperem-
vein thrombosis is associated with esis gravidarum, pre-eclampsia/eclampsia,

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184 Matos and Reau

intrahepatic cholestasis of pregnancy, show fatty deposition, and the Swansea

HELLP syndrome, and acute fatty liver Criteria can assist in diagnosis with 100%
of pregnancy (AFLP). However, severe sensitivity and 57% specificity. Emergent
hepatic dysfunction is rarely seen with fetal delivery is the backbone of treatment,
disorders other than HELLP, AFLP, with vaginal birth preferred. If liver failure
and pre-eclampsia/eclampsia. Diagnosis progresses after delivery, LT will be re-
and management of these diseases are quired. A retrospective review on LT from
reviewed elsewhere.1 The management of 1991 to 2015 found that patients under-
ALF secondary to these injuries is no going LT for AFLP had outcomes similar
different than any other etiology apart patients with ALF from other causes.25
from expedient delivery as this often In conclusion, there are multiple etiol-
begins to improve the disease process. It ogies that can result in severe damage to
is also important to recognize associated the liver during pregnancy. Close mon-
conditions. In severe pre-eclampsia/ itoring with a low threshold for transfer to
eclampsia, vasospasms involving the liver a center capable of liver transplantation is
can result in the formation of hepatic of high importance as some of the etiol-
hematoma or even hepatic rupture.1 Both ogies have high rates of mortality and
hepatic hematoma and rupture may war- complications to both mother and fetus.
rant interventional radiology or surgical Liver transplantation remains an impor-
intervention, and either can result in hep- tant last resort when the standard of care
atic failure requiring transplant. Clinical fails. A multidisciplinary approach, in-
symptoms include nausea/vomiting, right cluding obstetricians, critical care, and
upper quadrant or right shoulder pain, hepatologists, is advised when taking care
and hypotension and should trigger emer- of this population. In addition, the prev-
gent cross-sectional imaging. Conserva- alence of pregnancy in women with
tive management has been suggested for underlying liver disease has increased
subcapsular hematomas, however, given over the past decade due to improved
concurrent HELLP syndrome and risk of therapeutic options and carries unique
disseminated intravascular coagulation risks that need to be prioritized by pro-
(DIC), surgical intervention is recom- viders taking care of this population,
mended in this population and multidisci- preferably before conception.
plinary surgical care is vital. Successful
cases of liver transplantation for HELLP
syndrome have been reported.
Acute fatty liver of pregnancy (AFLP) is References
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 Hepatic Critical Care During Pregnancy 185

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