Portal hypertension during pregnancy is a rare but challenging condition that requires careful management to ensure the well-being of both the mother and the fetus. It is characterized by increased pressure in the portal vein system, often associated with liver diseases such as cirrhosis. However, there are non-cirrhotic causes of portal hypertension that can pose unique clinical dilemmas in pregnant women [1-3]. This case report presents the clinical course of a 26-year-old pregnant woman with portal hypertension, which was found to be of a non-cirrhotic etiology.

Pregnancy, a physiological state characterized by numerous hemodynamic changes, can exacerbate the complications of portal hypertension. This case underscores the importance of recognizing and managing non-cirrhotic portal hypertension in pregnancy, which differs from the more common cirrhotic etiology. To the best of our knowledge, there is limited literature available on the management of such cases during pregnancy [4-7].

In this report, we describe the clinical presentation, diagnostic workup, and management strategies employed in a pregnant patient with portal hypertension due to a non-cirrhotic cause. The case highlights the importance of a multidisciplinary approach involving obstetricians, hepatologists, and hematologists to ensure a safe and successful pregnancy outcome in the presence of this challenging condition.

This case also emphasizes the need for vigilant monitoring of liver function, portal hemodynamics, and thrombocytopenia during pregnancy in patients with a history of portal hypertension. The decision to avoid invasive interventions like endoscopy in this patient was made to minimize the risk of triggering labor. Instead, a focus on routine antenatal care and close surveillance was employed to manage the condition.              

Through this case report, we aim to contribute to the existing literature on non-cirrhotic portal hypertension in pregnancy and highlight the importance of a tailored approach to care for these patients. Additionally, we underscore the significance of shared decision-making between the healthcare team and the patient, with a focus on optimizing maternal and fetal outcomes in this unique clinical scenario.

Case Report

The case involves a 26-year-old gravid 2, para 1 (G2P1001) woman at 16 weeks of gestation who presented to the Obstetrics and Gynecology outpatient department for routine antenatal care (ANC). Her medical history was notable for a previous episode of hematemesis one year ago, during which grade 3 esophageal varices were identified and successfully treated with band ligation. Her first pregnancy had been uneventful, culminating in a full-term vaginal delivery at a nearby district hospital.

Upon examination, the patient exhibited hepatomegaly, consistent with her 16-week gestational age. Additionally, a positive fetal pole presentation (FPP) was noted. Notably, she provided a history of variceal bleeding in the past but did not have cirrhotic liver disease.

Further investigation through abdominal ultrasonography (USG) revealed hepatomegaly without distortion of liver architecture, massive splenomegaly, and dilatation of portal collaterals. Subsequently, a spleno-portal axis imaging with portal venous gas (SLIOG with POG) confirmed the presence of portal hypertension. Given the complexity of her condition, a consultation with a hepatologist was sought. It was advised not to perform invasive interventions like endoscopy due to the risk of inducing premature labor. Instead, the patient was recommended to undergo monthly routine ANC with liver function tests (LFTs) and other pertinent investigations.

Throughout her pregnancy, the patient's LFTs remained within the normal range, and her coagulation profile was unremarkable. Infective hepatitis was ruled out, but thrombocytopenia was noted. At 33 weeks of gestation, the patient was admitted for antenatal steroid coverage, and her subsequent antenatal course was uneventful.

The patient presented to the labor room with the onset of labor, and she delivered a healthy female child weighing 2.8 kilograms at 37 weeks and 6 days of gestation. The second stage of labor was expedited with vacuum assistance. Careful attention was paid to preventing postpartum hemorrhage, and methergine was avoided. Adequate blood products, including platelets and fresh frozen plasma (FPP), were arranged in anticipation of high-risk complications related to variceal bleeding, thrombocytopenia, and potential blood product requirements.

The final diagnosis in this case was non-cirrhotic portal hypertension, a condition that posed unique challenges during the course of pregnancy.

Portal hypertension during pregnancy, especially of non-cirrhotic origin, presents a clinical challenge that requires careful management to ensure the well-being of both the mother and the fetus [8-9]. This case report describes the management of a pregnant woman with portal hypertension due to a non-cirrhotic cause, emphasizing the importance of a multidisciplinary approach and tailored care plans.

Non-cirrhotic portal hypertension is a less common but significant condition characterized by increased portal vein pressure in the absence of cirrhosis. The etiology can include disorders such as portal vein thrombosis, Budd-Chiari syndrome, and others [9-10]. In our case, the patient had a history of variceal bleeding related to portal hypertension but did not have cirrhotic liver disease, highlighting the need for a comprehensive diagnostic approach in such cases.

Pregnancy itself induces various hemodynamic changes, which can exacerbate the complications of portal hypertension. Management decisions must balance the risks associated with portal hypertension and potential interventions with the need to maintain a healthy pregnancy [8-9]. In our case, the decision to avoid invasive procedures like endoscopy was made to minimize the risk of initiating premature labor.

Our approach involved a multidisciplinary team comprising obstetricians, hepatologists, and hematologists. Such collaboration is crucial in managing complex cases of portal hypertension in pregnancy. It allows for comprehensive assessment, risk stratification, and tailored care plans that consider both maternal and fetal outcomes.

Vigilant monitoring during pregnancy is essential in patients with a history of portal hypertension. Regular assessments of liver function, portal hemodynamics, and thrombocytopenia are necessary to detect and manage complications promptly. In our case, monthly ANC with LFTs and other investigations played a critical role in monitoring the patient's condition.

This case aligns with the limited literature available on non-cirrhotic portal hypertension in pregnancy. Studies by Kumar et al. [8] and Wanless et al. [9] have highlighted the challenges and complexities of managing such cases during pregnancy. Kumar et al. reported a similar non-cirrhotic portal hypertension case in pregnancy, emphasizing the rarity of this condition. Wanless et al.'s work on the regression of cirrhosis sheds light on the differences in disease mechanisms between cirrhotic and non-cirrhotic portal hypertension.

Furthermore, Tandon et al. [10] have discussed the use of renin-angiotensin-aldosterone inhibitors in reducing portal pressure, which could be relevant when considering therapeutic options in non-cirrhotic portal hypertension during pregnancy. However, their application in pregnancy warrants further investigation and careful consideration of potential risks and benefits.

This case report highlights the complexity of managing non-cirrhotic portal hypertension during pregnancy and emphasizes the importance of a multidisciplinary approach and tailored care plans. Further research and collaboration within the medical community are needed to better understand and manage this rare condition, with a focus on optimizing maternal and fetal outcomes.

1. Pereira, S.P. et al. “Maternal and perinatal outcome in pregnancies complicated by severe liver disease.” Gut, vol. 45, no. 3, 1999, pp. 446–450.

2. Tripathi, D. et al. “Haemodynamic effects of common therapies for chronic liver disease: Effects on the portal circulation.” Expert Review of Gastroenterology & Hepatology, vol. 2, no. 4, 2008, pp. 537–548.

3. European Association for the Study of the Liver. “EASL clinical practice guidelines for the management of patients with decompensated cirrhosis.” Journal of Hepatology, vol. 69, no. 2, 2018, pp. 406–460.

4. Sarin, S.K. and Kumar, A. “Noncirrhotic portal hypertension.” Clinical Liver Disease, vol. 18, no. 2, 2014, pp. 451–476.

5. Kumar, S. et al. “Non-cirrhotic portal hypertension: A comprehensive review.” World Journal of Gastroenterology, vol. 21, no. 40, 2015, pp. 11393–11409.

6. Khurana, A. et al. “Etiology and outcome of acute renal failure in pregnancy.” Indian Journal of Medical Research, vol. 138, no. 5, 2013, pp. 83–89.

7. Northup, P.G. et al. “Hypercoagulation and thrombophilia in non-cirrhotic portal vein thrombosis prior to liver transplantation.” Clinical Gastroenterology and Hepatology, vol. 10, no. 1, 2012, pp. 72–78.

8. Kumar, A. et al. “Non-cirrhotic portal hypertension in pregnancy: a rare entity.” Cureus, vol. 13, no. 8, 2021, pp. e16767.

9. Wanless, I.R. et al. “Regression of human cirrhosis: morphologic features and the genesis of incomplete septal cirrhosis.” Archives of Pathology & Laboratory Medicine, vol. 124, no. 11, 2000, pp. 1599–1607.

10. Tandon, P. et al. “Renin-angiotensin-aldosterone inhibitors in the reduction of portal pressure: a systematic review and meta-analysis.” Journal of Hepatology, vol. 53, no. 2, 2010, pp. 273–282.