Computed tomography perfusion in differentiating portal hypertension: A correlation study with hepatic venous pressure gradient.

BACKGROUND: Hepatic venous pressure gradient (HVPG) is the gold standard for diagnosis of portal hypertension (PH), invasiveness and potential risks in the process of measurement limited its widespread use. AIM: To investigate the correlation of computed tomography (CT) perfusion parameters with HVPG in PH, and quantitatively assess the blood supply changes in liver and spleen parenchyma before and after transjugular intrahepatic portosystemic shunt (TIPS). METHODS: Twenty-four PH related gastrointestinal bleeding patients were recruited in this study, and all patients were performed perfusion CT before and after TIPS surgery within 2 wk. Quantitative parameters of CT perfusion, including liver blood volume (LBV), liver blood flow (LBF), hepatic arterial fraction (HAF), spleen blood volume (SBV) and spleen blood flow (SBF), were measured and compared before and after TIPS, and the quantitative parameters between clinically significant PH (CSPH) and non-CSPH (NCSPH) group were also compared. Then the correlation of CT perfusion parameters with HVPG were analyzed, with statistical significance as P < 0.05. RESULTS: For all 24 PH patients after TIPS, CT perfusion parameters demonstrated decreased LBV, increased HAF, SBV and SBF, with no statistical difference in LBF. Compared with NCSPH, CSPH showed higher HAF, with no difference in other CT perfusion parameters. HAF before TIPS showed positive correlation with HVPG (r = 0.530, P = 0.008), while no correlation was found in other CT perfusion parameters with HVPG and Child-Pugh scores. CONCLUSION: HAF, an index of CT perfusion, was positive correlation with HVPG, and higher in CSPH than NCSPH before TIPS. While increased HAF, SBF and SBV, and decreased LBV, were found after TIPS, which accommodates a potential non-invasive imaging tool for evaluation of PH.

Topographic Distribution Pattern in Hepatic Amyloidosis Presenting with Portal Hypertension.

Background/aims: The liver is often involved in both primary and secondary forms of amyloidosis. Significant clinical evidence of portal hypertension is relatively uncommon and seems to be related to the reduced sinusoidal lumen and increased resistance to blood flow due to massive perisinusoidal amyloid deposits. The relationships between the pattern and extent of amyloid deposition in patients presenting with portal hypertension have not yet been clearly demonstrated. This study is focusing on the topographic distribution of amyloidosis in patients presenting with portal hypertension. Methods: The study included biopsy-proven cases of hepatic amyloidosis. The clinical, biochemical, and serological data, involvement of the extrahepatic organs, and HVPG values were recorded. Tissue sections were re-evaluated for the distribution patterns of amyloid deposits. Results: We had 41 patients with hepatic amyloidosis, of which, 32 were male. A mixed pattern (sinusoidal and vascular) was the most common (32/41; 78%). Hepatic venous pressure gradient was available in 21 cases. Portal hypertension was found in 14 patients (14/21; 67%). Cases of portal hypertension were found to have a sinusoidal pattern (3/14; 21.4%), vascular pattern (1/14; 7.1%), or a mixed sinusoidal and vascular pattern (10/14; 71.4%). Those not having portal hypertension showed hepatic artery (HA) involvement in 6/7 (85.7%) cases. A comparative analysis between portal hypertension (PTH) and non-PTH groups showed that HA amyloid deposition was dominant in the non-PTH group (6/7; 85.7%) and sinusoidal deposition in the PTH group (13/14; 92.8%). The difference was found to be significant (P < 0.05). Conclusion: We found that portal hypertension was noted in cases with diffuse sinusoidal deposition or mixed sinusoidal with portal vein deposition. In the non-PHT group, the deposition was mainly in HA alone.

Technique innovation of endoscopic ultrasound portal pressure gradient measurement using standard manometer set for portal hypertension assessment.

Portal hypertension (PH) is still a major challenge to be managed in clinical practice. However, hepatic vein pressure gradient (HVPG) measurement is not always reliable for portal hypertension (PH) diagnosis. This study showed the impact of endoscopic ultrasound (EUS) as a promising tool for diagnosis and management PH condition.

The role of microRNAs on the pathogenesis, diagnosis and management of portal hypertension in patients with chronic liver disease.

INTRODUCTION: Portal hypertension (PH) is the elevated pressure in the portal vein, which results in poor functioning of the liver and is influenced by various factors like liver cirrhosis, nonalcoholic fatty liver disease, schistosomiasis, thrombosis, and angiogenesis. Though the diagnosis and treatment have been advanced, early diagnosis of the disease remains a challenge, and the diagnosis methods are often invasive. Hence, the clear understanding of the molecular mechanisms of PH can give rise to the development of novel biomarkers which can pave way for early diagnosis in noninvasive methods, and also the identification of target genes can elucidate an efficient therapeutic target. AREAS COVERED: PubMed and Embase database was used to search articles with search terms 'Portal Hypertension' or 'pathophysiology' and 'diagnosis' and 'treatment' or "role of miRNAs in portal hypertension. EXPERT OPINION: Interestingly, biomarkers like microRNAs (miRNAs) have been studied for their potential role in various diseases including hypertension. In recent years, miRNAs have been proved to be an efficient biomarker and therapeutic target and few studies have assessed the roles of miRNAs in PH. The present paper highlights the potential roles of miRNAs in PH.

Correlation between hepatic venous pressure gradient and portal venous pressure gradient in hepatitis B cirrhosis with different hepatic veins anatomy.

PURPOSE: The hepatic venous pressure gradient (HVPG) has been employed as the gold standard for indicating the portal venous pressure gradient (PPG) in the diagnosis of portal hypertension (PHT). However, little has been reported on whether the HVPG can accurately estimate the PPG in patients with hepatic vein collateral shunts. We aimed to explore the correlation between the HVPG and the PPG in hepatitis B cirrhosis patients with different hepatic vein anatomies. METHODS: A total of 461 hepatitis B cirrhosis patients with portal hypertension (PHT) who were treated with a transjugular intrahepatic portosystemic shunt (TIPS) between January 2016 and June 2020 were included. All patients underwent various venous pressure measurements and balloon-occluded compression hepatic venography during the TIPS operation. Agreements were evaluated by Pearson's correlation and the Bland-Altman method. Disagreements were assessed by paired t tests. RESULTS: The correlation coefficient (r) values (P < 0.001) between the HVPG and the PPG of the early (151 patients, 32.8 %), middle (73 patients, 15.8 %), late (46 patients, 10.0 %), portal vein (151 patients, 32.8 %), and no lateral branch development groups (40 patients, 8.7 %) were 0.373, 0.487, 0.569, 0.690, and 0.575, respectively; the determination coefficient (R2) values were 0.139, 0.238, 0.323, 0.475, and 0.330, respectively. According to the Bland-Altman method, agreement was the greatest in the portal vein development group, with the 95 % limits of agreement (95 % LoA, mean differences ± 1.96 SD) being the smallest. The differences were statistically significant (P < 0.05). CONCLUSION: The correlation between the HVPG and the PPG is the worst in early lateral branch development, followed by middle development, and the influence of lateral branches becomes significantly reduced in late development. Hepatic venous collateral formation is a vital factor for underestimation of the HVPG, which is the most accurate predictor of PPG in patients with portal vein development. Patients with no collateral channel development in the hepatic vein have a higher HVPG than PPG, which is an important reason for overestimation of the HVPG.

Overview of Complications in Cirrhosis.

Background: Cirrhosis is the outcome of chronic liver disease of any etiology due to progressive liver injury and fibrosis. Consequently, cirrhosis leads to portal hypertension and liver dysfunction, progressing to complications like ascites, variceal bleeding, hepatic encephalopathy, hepatorenal syndrome, hepatopulmonary syndrome, cirrhotic cardiomyopathy, sarcopenia, hepatocellular carcinoma, and coagulation disorders. End-stage liver disease leads to an impaired quality of life, loss of social and economic productivity, and reduced survival. Methods: This narrative review explains the pathophysiology of complications of cirrhosis, the diagnostic approach and innovative management, with focus on data from India. A comprehensive literature search of the published data was performed in regard with the spectrum, diagnosis, and management of cirrhosis and its complications. Results: There is a change in the epidemiology of metabolic syndrome, lifestyle diseases, alcohol consumption and the spectrum of etiological diagnosis in patients with cirrhosis. With the advent of universal vaccination and efficacious long-term viral suppression agents for chronic hepatitis B, availability of direct-acting antiviral agents for chronic hepatitis C, and a booming liver transplantation programme across the country, the management of complications is essential. There are several updates in the standard of care in the management of complications of cirrhosis, such as hepatorenal syndrome, hepatocellular carcinoma, and hepatic encephalopathy, and new therapies that address supportive and palliative care in advanced cirrhosis. Conclusion: Prevention, early diagnosis, appropriate management of complications, timely transplantation are cornerstones in the management protocol of cirrhosis and portal hypertension. India needs improved access to care, outreach of public health programmes for viral hepatitis care, health infrastructure, and disease registries for improved healthcare outcomes. Low-cost initiatives like immunization, alcohol cessation, awareness about liver diseases, viral hepatitis elimination, and patient focused decision-making algorithms are essential to manage liver disease in India.

Overlooked subclinical portal hypertension in non-cirrhotic NAFLD: Is it real and how to measure it?

Clinical and experimental advances related to the detection, magnitude and pathobiology of subclinical portal hypertension in non-alcoholic fatty liver disease (NAFLD), primarily observed in the presence of non-alcoholic steatohepatitis (NASH), prompt us to revisit current disease paradigms. Hepatic venous pressure gradient (HVPG) has been reported to underestimate portal pressure in NASH-related cirrhosis, while inaccuracy is more likely in non-cirrhotic livers, indicating a potential need for new and preferably non-invasive methods of measurement. Although clinically significant portal hypertension (HVPG ≥10 mmHg) retains its prognostic significance in NASH, subclinical portal hypertension (HVPG 6.0-9.5 mmHg) has been repeatedly detected in patients with NAFLD in the absence of cirrhosis or even significant fibrosis whereas the impact of these findings on disease outcomes remains unclear. Mechanocrine signalling pathways in various types of liver cell reveal a molecular basis for the adverse effects of subclinical portal hypertension and suggest a bidirectional relationship between portal pressure and fibrosis. These findings may guide efforts to improve risk assessment and identify novel therapeutic targets in NAFLD.

Transjugular Liver Biopsy with Hemodynamic Evaluation: Correlation between Hepatic Venous Pressure Gradient and Histologic Diagnosis of Cirrhosis.

OBJECTIVES: Measurement of hepatic vein pressures is the accepted gold standard for the evaluation of portal hypertension. This study was conducted to evaluate the correlation between hepatic vein pressure measurements and histologic findings from transjugular liver biopsies. The hypothesis was that higher hepatic venous pressure gradients would correlate with a histologic diagnosis of cirrhosis. MATERIAL AND METHODS: We identified all patients who underwent transjugular liver biopsies at our institution between January 2015 and December 2019. Of these, 178 patients who had undergone hemodynamic evaluations during the biopsy procedure were included in the study. Demographic information and laboratory data were extracted from the patients' electronic medical records. The hepatic vein pressure gradient (HVPG) was determined by subtracting the free hepatic venous pressure from the wedged hepatic venous pressure (WHVP), and the portosystemic gradient (PSG) was determined by subtracting the right atrial pressure from the WHVP. HVPG and PSG were compared by linear regression analysis and by calculating their receiver operating characteristics (ROC). RESULTS: HVPG and PSG measurements were significantly associated with cirrhosis, with area under the ROC curve of 0.79 and 0.78, respectively. At the optimal cutoff of 9 mmHg, sensitivity and specificity for HVPG were 71% and 83% for HVPG and 67 % and 81% for PSG, respectively. No statistical difference was observed between the two measurements. CONCLUSION: A transhepatic venous pressure gradient above a cutoff of 9 mmHg is predictive of histologic cirrhosis, regardless of whether it is expressed as HVPG or PSG, with acceptable to excellent performance characteristics.

Accurate ultrasonography-based portal pressure assessment in patients with hepatocellular carcinoma.

BACKGROUND: Portal pressure is of great significance in the treatment of hepatocellular carcinoma (HCC), but direct measurement is complicated and costly; thus, non-invasive measurement methods are urgently needed. AIM: To investigate whether ultrasonography (US)-based portal pressure assessment could replace invasive transjugular measurement. METHODS: A cohort of 102 patients with HCC was selected (mean age: 54 ± 13 years, male/female: 65/37). Pre-operative US parameters were assessed by two independent investigators, and multivariate logistic analysis and linear regression analysis were conducted to develop a predictive formula for the portal pressure gradient (PPG). The estimated PPG predictors were compared with the transjugular PPG measurements. Validation was conducted on another cohort of 20 non-surgical patients. RESULTS: The mean PPG was 17.32 ± 1.97 mmHg. Univariate analysis identified the association of the following four parameters with PPG: Spleen volume, portal vein diameter, portal vein velocity (PVV), and portal blood flow (PBF). Multiple linear regression analysis was performed, and the predictive formula using the PVV and PBF was as follows: PPG score = 19.336 - 0.312 × PVV (cm/s) + 0.001 × PBF (mL/min). The PPG score was confirmed to have good accuracy with an area under the curve (AUC) of 0.75 (0.68-0.81) in training patients. The formula was also accurate in the validation patients with an AUC of 0.820 (0.53-0.83). CONCLUSION: The formula based on ultrasonographic Doppler flow parameters shows a significant correlation with invasive PPG and, if further confirmed by prospective validation, may replace the invasive transjugular assessment.

Intrahepatic veno-venous collateralization and misrepresentative hepatic venous pressure gradients in children.

BACKGROUND: Accurate and reproducible means of measuring the portosystemic gradient are essential for risk stratification and treatment of portal hypertension. OBJECTIVE: To report the reliability of hepatic venous pressure gradients in children with intrahepatic veno-venous collateralization. MATERIALS AND METHODS: Between January 2012 and December 2019 (96 months), 39 patients with native livers underwent wedge hepatic venography and hepatic venous pressure gradient measurements at a tertiary pediatric center. All archived images were reviewed for balloon isolation of the hepatic vein and hepatic vein-to-hepatic vein (HV-HV) collaterals. HV-HV collaterals were categorized as present on the basis of non-catheterized segmental venous opacification despite appropriate balloon isolation. Hepatic venous pressure gradient was defined as the difference of wedge and free hepatic venous pressures. Wedge portosystemic gradient was defined as the difference between wedge hepatic venous pressure and right atrial (RA) pressures. For patients subsequently undergoing portal venous catheterization, portosystemic gradient was defined as the difference between main portal vein and RA pressures. RESULTS: Thirteen of 39 (33.3%) patients demonstrated HV-HV collaterals on wedge hepatic venography. The mean hepatic venous pressure gradient was 5.2±3.8 mmHg (range: 0-15 mmHg). The mean hepatic venous pressure gradient was 3.6±2.6 mmHg (range: 0-9 mmHg) in the presence of HV-HV collaterals and 5.9±4.2 mmHg (range: 1-15 mmHg) in the absence of HV-HV collaterals (P=0.043). Twelve (30.8%) patients were found to have varices: 10 gastroesophageal, 1 rectal and 1 stomal. The mean hepatic venous pressure gradient in patients with varices was 5.4±47 mmHg (range: 0-15 mmHg). For patients with varices, mean hepatic venous pressure gradient was 3.0±2.7 mmHg (range: 0-9 mmHg) in the presence of HV-HV collaterals and 10.3±4.1 mmHg (range: 5-15 mmHg) in the absence of HV-HV collaterals (P=0.004). Four (10.3%) patients had extrahepatic portal vein occlusion: 3 with cavernous transformation and 1 with type Ib Abernethy malformation. All patients with extrahepatic portal vein occlusion demonstrated HV-HV collaterals compared with 8 of 35 (22.9%) patients without extrahepatic portal vein occlusion (P=0.002). Four of 39 (10.3%) patients underwent direct portal pressure measurements: 3 via transhepatic and 1 via trans-splenic portal access. All had demonstrated HV-HV collaterals on wedged imaging. One had extrahepatic portal vein occlusion. The mean time between wedge portosystemic gradient and portosystemic gradient measurement was 3.75 days (range: 0-8 days). The mean wedge portosystemic gradient was 4.5±3.1 mmHg (range: 2-9 mmHg) and the mean portosystemic gradient was 14.5±3.7 mmHg (range: 12-20 mmHg) (P=0.006). CONCLUSION: HV-HV collateralization is frequently observed in children undergoing wedged portal venography and leads to misrepresentative hepatic venous pressure gradients. All patients undergoing hepatic venous pressure gradient measurement should have wedged venography to identify HV-HV collaterals and to qualify measured pressures. Additional techniques to obtain representative pressures in the presence of HV-HV collaterals warrant further investigation.

Portal hypertension in hepatitis B-related cirrhosis: Diagnostic accuracy of liver and spleen stiffness by 2-D shear-wave elastography.

AIM: To assess the diagnostic accuracy of liver and spleen stiffness measured by 2-D shear-wave elastography (SWE) in evaluation of clinically significant and severe portal hypertension (CSPH and SPH, respectively). METHODS: Clinical data of 155 hepatitis B-related cirrhosis patients and their liver and spleen stiffness (L-SWE and S-SWE, respectively) were collected. The diagnostic performances of L-SWE, S-SWE, the liver stiffness-spleen diameter to platelet ratio score (LSPS) and portal hypertension risk score were evaluated. RESULTS: One hundred and four patients were eligible for analysis. Clinically significant and severe PH were detected in 84 and 74 patients, respectively. Liver and spleen stiffness were significantly correlated with hepatic venous pressure gradient in overall, CSPH, and SPH groups (rL = 0.607, 0.554, and 0.412; rS = 0.665, 0.566, and 0.467, respectively; all P < 0.05). The area under the receiver operating characteristic curves of L-SWE, S-SWE, LSPS, and PH risk score were 0.72 (95% confidence interval [CI], 0.49-0.95), 0.81 (95% CI, 0.55-0.97), 0.76 (95% CI, 0.51-0.96), and 0.73 (95% CI, 0.55-0.88) for CSPH, and 0.77 (95% CI, 0.51-0.93), 0.85 (95% CI, 0.59-0.96), 0.80 (95% CI, 0.58-0.98), and 0.80 (95% CI, 0.59-0.93) for SPH. The best cut-off of L-SWE for determining CSPH and SPH were 16.1 kPa (sensitivity, 78%; specificity, 72%) and 23.5 kPa (sensitivity, 81%; specificity, 79%). For S-SWE, the best cut-offs were 25.3 kPa (sensitivity, 85%; specificity, 79%) and 33.4 kPa (sensitivity, 74%; specificity, 70%). A cut-off of L-SWE <13.2 kPa or S-SWE <23.2 kPa was able to rule out CSPH, whereas a cut-off of L-SWE >24.9 kPa or S-SWE >34.2 kPa was able to rule in CSPH. CONCLUSIONS: Liver and spleen stiffness measured by 2-D SWE are reliable and promising non-invasive parameters to assess CSPH and SPH.

Emerging therapies for portal hypertension in cirrhosis.

INTRODUCTION: Counteracting splanchnic vasodilatation and increased portal-collateral blood flow has been the mainstay for the treatment of portal hypertension (PH) over the past three decades. However, there is still large room for improvement in the treatment of PH. AREAS COVERED: The basic mechanism leading to portal hypertension is the increased hepatic vascular resistance to portal blood flow caused by liver structural abnormalities inherent to cirrhosis and increased hepatic vascular tone. Molecules modulating microvascular dysfunction which have undergone preclinical and clinical trials are summarized, potential drug development issues are addressed, and situations relevant to design of clinical trials are considered. EXPERT OPINION: Experimental and clinical evidence indicates that molecules modulating liver microvascular dysfunction may allow for 30-40% reduction in portal pressure. Several agents could be utilized in the earlier stages of cirrhosis (antifibrotics, antiangiogenics, etiological therapies) may allow reduction of fibrosis and halt progression of PH. This 'nip at the bud' policy, by combining therapies with existing agents used in advanced phase of cirrhosis and novel agents which could be used in early phase of cirrhotic spectrum, which are likely to hit the market soon would be the future strategy for PH therapy.

Hepatic venous pressure gradient is a useful predictor in guiding treatment on prevention of variceal rebleeding in cirrhosis.

BACKGROUND: The best therapy to prevent esophageal variceal (EV) rebleeding in cirrhotic patients who are non-responsive to pharmacological therapy have not been determined. AIMS: To evaluate efficacy of a strategy to assign different treatments according to hepatic vein pressure gradient (HVPG) values to prevent EV rebleeding in non-responders. METHODS: This study is a non-randomized controlled prospective study. 109 cirrhotic patients with EV bleeding who were non-responders based on two HVPG measurements were enrolled and divided two groups: 55 patients (EVL+ß-blocker group) were treated with endoscopic variceal ligation (EVL) and nonselective ß-blocker; 54 patients (HVPG-guided group) were treated with EVL and nonselective ß-blocker if HVPG ≤ 16 mmHg (low-HVPG), with percutaneous transhepatic variceal embolization (PTVE) if HVPG > 16 mmHg and ≤ 20 mmHg (medium-HVPG), or with transjugular intrahepatic portosystemic shunt (TIPS) if HVPG > 20 mmHg (high-HVPG). Patients were followed up for rebleeding and mortality. RESULTS: The mean follow-up period was 17.0 months; rebleeding was higher in the EVL+ß-blocker group than HVPG-guided group (25.5%, 9.3%, P = 0.026); 3-year probability of rebleeding in the EVL+Beta-blocker group increased with elevated levels of HVPG (12.5% vs 46.4% vs 64.9%, χ(2) = 11.551, P = 0.003), and 3-year probability of survival was no difference (96.6% vs 85.7% vs 90.9%, χ(2) = 2.638, P = 0.267). Rebleeding rate in PTVE group (7.7%) was lower than that in EVL+ß-blockergroup with medium-HVPG (35.7%), but there was no difference. Rebleeding rate in TIPS group (7.7%) was lower than that in EVL+ß-blockergroup with high-HVPG (45.5%), but there was no difference. CONCLUSIONS: HVPG measurement was useful for making decisions to select EVL and Beta-blocker, PTVE or TIPS in secondary prophylaxis. HVPG-guided treatment is feasible and effective in preventing esophageal varices rebleeding.

Risk factors for early rebleeding and mortality in acute variceal hemorrhage.

AIM: To investigate the risk factors for 6-wk rebleeding and mortality in acute variceal hemorrhage (AVH) patients treated by percutaneous transhepatic variceal embolization (PTVE). METHODS: A retrospective cohort study of AVH patients who had undergone PTVE treatment was conducted between January 2010 and December 2012. Demographic information, medical histories, physical examination findings, and laboratory test results were collected. The PTVE procedure was performed as a rescue therapy for patients who failed endoscopic and pharmacologic treatment. Survival analysis was estimated using the Kaplan-Meier method and compared using the log-rank test. The multivariate analysis was performed using the Cox regression test to identify independent risk factors for rebleeding and mortality. RESULTS: One hundred and one patients were included; 71 were males and the average age was 51 years. Twenty-one patients rebled within 6 wk. Patients with high-risk stigmata, PTVE with trunk obliteration, and a hepatic vein pressure gradient (HVPG) ≥ 20 mmHg were at increased risk for rebleeding (OR = 5.279, 95%CI: 2.782-38.454, P = 0.003; OR = 4.309, 95%CI: = 2.144-11.793, P < 0.001; and OR = 1.534, 95%CI: 1.062-2.216, P = 0.022, respectively). Thirteen patients died within 6 wk. A model for end-stage liver disease (MELD) score ≥ 18 and an HVPG ≥ 20 mmHg were associated with 6-wk mortality (OR = 2.162, 95%CI: 1.145-4.084, P = 0.017 and OR = 1.423, 95%CI: 1.222-1.657, P < 0.001, respectively). CONCLUSION: MELD score and HVPG in combination allow for early identification of patients with AVH who are at substantially increased risk of death over the short term.

Invasive and noninvasive methods to diagnose portal hypertension and esophageal varices.

Assessing the presence of clinically significant portal hypertension and esophageal varices is clinically important in cirrhosis. The reference standard techniques to assess the presence of portal hypertension and varices are the measurement of the hepatic vein pressure gradient and esophagogastroduodenoscopy, respectively. Some newer methods have shown a good performance, but none has been proven precise enough to replace hepatic vein pressure gradient measurement or esophagogastroduodenoscopy for the diagnosis of portal hypertension or the presence and grade of esophageal varices.

Pregnancy with portal hypertension.

Even though pregnancy is rare with cirrhosis and advanced liver disease, but it may co-exist in the setting of non-cirrhotic portal hypertension as liver function is preserved but whenever encountered together is a complex clinical dilemma. Pregnancy in a patient with portal hypertension presents a special challenge to the obstetrician as so-called physiological hemodynamic changes associated with pregnancy, needed for meeting demands of the growing fetus, worsen the portal hypertension thereby putting mother at risk of potentially life-threatening complications like variceal hemorrhage. Risks of variceal bleed and hepatic decompensation increase many fold during pregnancy. Optimal management revolves round managing the portal hypertension and its complications. Thus management of such cases requires multi-speciality approach involving obstetricians experienced in dealing with high risk cases, hepatologists, anesthetists and neonatologists. With advancement in medical field, pregnancy is not contra-indicated in these women, as was previously believed. This article focuses on the different aspects of pregnancy with portal hypertension with special emphasis on specific cause wise treatment options to decrease the variceal bleed and hepatic decompensation. Based on extensive review of literature, management from pre-conceptional period to postpartum is outlined in order to have optimal maternal and perinatal outcomes.

Isolated arterioportal fistula presenting with variceal hemorrhage.

We report a case of life-threatening hematemesis due to portal hypertension caused by an isolated arterioportal fistula (APF). Intrahepatic APFs are extremely rare and are a cause of presinusoidal portal hypertension. Etiologies for APFs are comprised of precipitating trauma, malignancy, and hereditary hemorrhagic telangiectasia, but these were not the case in our patient. Idiopathic APFs are usually due to congenital vascular abnormalities and thus usually present in the pediatric setting. This is one of the first cases of adult-onset isolated APF who presented with portal hypertension and was successfully managed through endoscopic hemostasis and subsequent interventional radiological embolization.