Dr. Anisha Ramniklal Gala is working in Fernandez Hospital since a decade, runs high-risk pregnancy clinic and prepregnancy counseling clinic. She has completed her fellowship in Maternal Medicine from NTR University of Heath Sciences. She has keen interest in teaching and training students. She has served as faculty for workshops conducted by Fernandez Hospital such as Obstetric Emergencies and Life Support Skills, and Fetal Monitoring. She has presented various papers and posters and won awards for posters such as ‘‘Twins—growth profile, HIV in Pregnancy’’. She has won a second prize at ‘‘World Obstetric Anaesthesia Congress’’ for poster—‘‘Disease Specific Maternal Mortality—Liver Disorders lead the list!’’ She was the Principal Investigator for International Observational E-Registry on the use of Dilapan-S.
Background: The course of pregnancy in a woman with
portal hypertension is a difficult one as it is associated with
complications like variceal bleeding, splenic artery rupture
and coagulopathy. All these pose a threat to a woman’s life.
Although this condition is rare, every obstetrician should
have a high index of suspicion when an antenatal mother
presents with splenomegaly, thrombocytopenia or
hematemesis. Hence, we aimed to review maternal and fetal
outcomes in pregnant women with portal hypertension.
Methods: In a retrospective observational study, 41 women
and 47 pregnancies were evaluated, from January 2000–
December 2015 at Fernandez Hospital, a tertiary referral
perinatal center. Maternal outcomes studied were variceal
bleed during pregnancy, surgical procedures, morbidity and mortality. Neonatal variables were gestational age at
delivery, birth weight and morbidities.
Results: Mean maternal age was 26.4 years. Average gestational
age at delivery was 36.5 weeks. Mean birth weight
was 2507.5 g. There were three maternal deaths out of 47
deliveries, the cause of death was massive variceal bleed in
one, the second one was due to cardiac arrest on MRI table,
and the third death was due to splenic hilar vessel bleed.
There was one stillbirth, and no neonatal deaths.
Conclusion A multidisciplinary approach is essential to
improve perinatal outcomes in pregnancy complicated by
portal hypertension. Surgical measures to reduce portal
venous pressure done before pregnancy or beta blockers
during pregnancy might help reduce sudden variceal
bleeds.
Keywords: Portal hypertension Variceal bleed
Pregnancy Outcomes
Portal hypertension in pregnancy is a rare and potentially lethal condition. Pregnancy is a possibility as long as liver function is well preserved although fertility is compromised in women with cirrhotic portal hypertension [1–3]. Pregnancy is associated with profound hemodynamic changes such as increase in blood volume, increased cardiac output and decrease in systemic vascular resistance [2, 3]. These hemodynamic changes may contribute to rapid deterioration of the underlying portal hypertension [2–5]. There is an increased risk of variceal hemorrhage in pregnancy due to increased portal pressure resulting from increased intra-abdominal pressure, vena caval compression and Valsalva maneuver in labor [3, 4].
Maternal mortality rates in women with portal hypertension range from 2 to 18% [1]. Uncontrolled variceal bleeding, often severe and unpredictable, is a major cause for mortality [1–8]. Other causes of maternal death include splenic artery aneurysm rupture, coagulopathy and liver failure [1–11]. Hematemesis is reported in 20–30% of women with cirrhosis [1]. A mortality rate of 50–60% has been reported in the presence of hematemesis [1]. Perinatal mortality rates may also be as high as 11–18% and are primarily due to preterm birth or fetal growth restriction [1–11].
The aim of the study was to review maternal and fetal outcomes in pregnant women with portal hypertension managed during a fifteen-year period ending in 2015, and to invite discussion on the development of formal evidence-based guidelines to standardize the safe management of these women in future.
A retrospective review of pregnancies with portal hypertension was carried out to determine maternal and perinatal outcomes during the fifteen-year period 2000–2015. These women were managed at a tertiary maternal and perinatal care center at Hyderabad, India which conducts approximately 8000 deliveries annually. All pregnant women who book for care at this hospital receive a preliminary assessment using a standardized antenatal care protocol that includes a detailed demographic history, current and past obstetric, medical and surgical history and gestational age, specific ultrasound examination for prenatal genetic screening, structural anomalies and fetal well-being. Any abnormality revealed by this basic assessment is followed up, using appropriate disease specific guidelines of the institute. Guidelines and protocols used at the institute are based on published international standards and have been adapted to suit the needs of the local population.
For review, the records of women with a diagnosis of portal hypertension were extracted from electronic database. Those who did not deliver at the hospital were excluded from the review.
Relevant maternal details such as age and parity were noted. A note was made of the causal factors where possible, and if there were any cirrhotic changes in the liver. Information related to the baseline evaluation of the disease with investigations such as ultrasound, endoscopy and liver function tests was recorded together with interventions to reduce variceal bleeds such as use of beta blockers, sclerotherapy, banding and shunt surgery. In cases where the women suffered complications as a result of portal hypertension or had other preexisting morbidities, these were also recorded. Pregnancy outcomes such as gestational age at delivery, mode of delivery, mean birth weight and whether the outcome was a stillbirth, were noted. Neonatal morbidities such as hypoglycemia, jaundice and bradycardia, were also recorded.
Table 1 summarizes our findings related to these women. There were 41 mothers with portal hypertension who delivered 47 babies during the review period. All the pregnancies were singleton. The total number of deliveries at the hospital for the same period was 76,923. All 41 mothers were referred to the hospital as known cases of portal hypertension or with signs and symptoms such as thrombocytopenia which warranted further investigation. The mean age of the women with portal hypertension was 26.48 (SD = 2.85) years (range 22.00–38.00 years). Twenty-two (46.80%) women were primigravida. Seventeen (36.17%) of the 47 pregnancies were complicated by cirrhotic portal hypertension. Splenomegaly was present in all 47 pregnancies, and hematemesis was present in three (6.38%) of the 47 pregnancies. Endoscopy was performed in 38 (80.85%) pregnancies where the women were booked in the antenatal period, the rest having presented in labor. Esophageal varices of grade 1 severity were present in nine pregnancies (19.14%), and of grade 2 and 3 in 16 pregnancies (34.04%). Interventions for decreasing the risk of variceal bleed are presented in Table 2. Women who did not have any intervention were found to have higher incidence of hematemesis. Preexisting medical conditions seen were Type 2 diabetes in one (2.12%), severe anemia in one (2.12%) and hypothyroidism in three (6.38%) of 47 pregnancies. Maternal complications of pregnancy which occurred were preeclampsia in three (6.38%), gestational diabetes in two (4.25%), acute renal failure in one (2.12%) and premature rupture of membranes in one pregnancy (2.12%).
There were three maternal deaths in this group. The first
of these deaths occurred in a woman who was referred late
at 36 weeks of gestation. It was at this stage that she was
diagnosed to have cirrhotic portal hypertension with
abnormal liver function tests, coagulopathy, anuria and
acute renal failure. She underwent a cesarean section and
required ventilatory support, but subsequently died on the
ninth postoperative day following a massive variceal bleed.
The second maternal death was of a woman who was
referred for evaluation at 20 weeks of gestation with a
diagnosis of portal hypertension secondary to portal vein
thrombus. She was undergoing an MRI when she suffered a
cardiac arrest and could not be resuscitated. The cause of
death remains unknown as consent for an autopsy was not
given. The third mortality was of a mother referred in labor
who suffered a splenic hilar vessel bleed. She had been
diagnosed with portal hypertension eight-year prior to her
death, but was not on regular follow-up.
Perinatal details of the 47 pregnancies are presented in
Table 3. There were no neonatal deaths. There was however
one stillbirth resulting in a perinatal mortality rate of
21.27/1000 (1/47 9 1000) births.
The diagnosis of portal hypertension in routine antenatal
women requires a high index of suspicion. This case series
had a good perinatal outcome but had a high maternal
mortality rate of 6.38%. Majority of these mothers had a
confirmed diagnosis before pregnancy. In this series, noncirrhotic
portal hypertension was more common than cirrhotic
disease, probably due to low fertility rates reported
in cirrhotic women.
The diagnostic markers which alerted to the high index
of suspicion for diagnosis of portal hypertension were splenomegaly (100%) and thrombocytopenia (61.7%). By
contrast, liver function tests were poor markers of portal
hypertension and were found to be abnormal in only four
out of 47 pregnancies. It is our view that the presence of
splenomegaly and low platelet count should trigger a
request for an abdominal ultrasound which is the key to
confirm the diagnosis of portal hypertension. The current
trend at many clinics in India of not including a basic
complete hematological profile through a full blood count
or a complete physical examination to make a note of any
splenomegaly as part of the first antenatal assessment may
be delaying confirmation of the diagnosis and impacting on
the pregnancy outcomes.
Pregnancy with portal hypertension has a very high maternal morbidity and mortality, and these women should be referred for delivery at a tertiary care center [1–11]. In this series, mortality was higher for those mothers with hematemesis (one death out of four) as has also been observed by other authors [12, 13]. Furthermore, the incidence of hematemesis was 8.5% in this series, a proportion which falls within the range of 6–33% reported in previous reviews [1, 12, 13]. The incidence of hematemesis was 16.66% (3/18) in women who had no intervention to reduce portal pressures, while hematemesis was seen in 4.34% in women with beta blockers, 14.28% in women who had variceal banding and it was not seen in women who had shunt surgery. The findings of this study suggest that it would be prudent to evaluate all women for the presence of esophageal varices and to offer beta blockers and surgical intervention to avoid variceal bleed. Emergency tamponade measures like a Sengstaken–Blakemore tube should be available along with blood and blood products facilities. Drugs such as octreotide (Pregnancy Category B) should be made available in the emergency crash cart to treat acute variceal bleeding [1, 12, 13].
One of the maternal deaths was due to ruptured splenic artery aneurysm. Splenic artery aneurysm rupture has been reported to occur in 2.6% of all pregnancies with cirrhosis with a high maternal mortality rate of 70% and fetal mortality rate of 90%. Considering the fact that this is associated with high mortality rates, clinicians must consider ruptured splenic artery aneurysm if a woman presents with acute abdomen or hypotensive shock that is unexplainable or there is a sudden maternal collapse [6, 7, 14–16].
There were no neonatal deaths or major neonatal morbidities suggesting that portal hypertension in pregnancy may not be associated with a substantially higher risk of adverse neonatal outcomes. One baby was stillborn and weighed 2.7 kg at term in a woman with non-cirrhotic portal hypertension. The cause of stillbirth was unexplained.
This review suggests a need for a registry to be launched for the purpose of gathering data and information on portal hypertension in pregnancy. The data may help in assessing the effectiveness of some interventions. Such evidence would form the basis of standardized evidence-based guidelines which would enhance the safe management of women with this rare condition and improve their chances of survival.
Conflict of Interest Anisha Ramniklal Gala, Tarakeswari Surapaneni, Nuzhat Aziz and Sailaja Devi Kallur declare that they have no conflict of interest. Human and Animal Rights This research does not involve animals. Informed Consent Informed consent regarding use of data for medical studies/research at the time of admission and antenatal booking is taken from all mothers at Fernandez Hospital. This is an observational study.
