{"paper_id":"d8d8a667-da9a-40a5-ab53-858371730184","body_text":"https://doi.org/10.1007/s12664-023-01341-z\nEDITORIAL\nPelvic congestion needs attention in infertile women with Budd‑Chiari \nsyndrome\nAnand Sharma1 · C. E. Eapen2 \n© Indian Society of Gastroenterology 2023\nThe advances in our understanding of the interplay between \nBudd-Chiari syndrome (BCS) and pregnancy have trans -\nlated into improved survival. Earlier reports documented \nBCS occurring in pregnant women as a sinister combination \nresulting in deaths in up to 50% of the mothers [ 1, 2]. It is \nindeed remarkable to note 100% maternal survival in recent \nreports of BCS in pregnancy (Table  1).\nMany of the reports of BCS in pregnancy are from \nIndia. The largest series of patients with BCS in pregnancy \nreported to date features in this issue of this journal [ 6]. \nThe authors of this study document remarkable improve -\nment in maternal survival with no complications related \nto pregnancy or liver disease in any of the study patients. \nThe authors also did not find significant delay in diagnosis \nof BCS in their cohort, contrary to older studies of BCS \npatients [8]. It is likely that increasing awareness of this con-\ndition among clinicians, improved diagnostic modalities and \nappropriate treatments all have contributed to the excellent \nmaternal survival now reported in these patients.\nBCS presenting as acute liver failure or acute-on-chronic \nliver failure is uncommon and has poorer outcomes. In a \nprevious report from the same center, clinical presentation \nas acute-on-chronic liver failure was noted in 5% of BCS \npatients [9]. The current paper on BCS in pregnancy reports \nacute presentation of BCS in five out of the 121 (4%) study \npatients [6]. Endovascular therapies may improve survival \nin these uncommon presentations of BCS [ 9].\nIn contrast to the success achieved in maternal outcomes \nin pregnant women with BCS, fetal outcomes still remain \nsub-optimal (Table  1). In addition, infertility continues to \nbe a problem in women with BCS.\nPrimary infertility rates of 19.8% 6–25% 4 have been \nreported in women with BCS. Metabolic and hormonal \nalterations associated with liver dysfunction may contribute \nto infertility in women with chronic liver diseases.\nAnother proposed mechanism of infertility or fetal \nlosses in BCS may be pelvic congestion. Obstruction of \ninferior vena cava (IVC) resulting in congestion of pel -\nvic organs, evidenced by dilated pelvic veins, has been \ndescribed [ 10]. An analogy to this hypothesis may be \ndrawn from the link between IVC obstruction and vari -\ncocele, which is associated with male infertility. Surgery \nto correct varicocele improves male fertility by improving \nsperm count and quality [ 11].\nOne study reported 12 women in whom pelvic congestion \nsyndrome was considered the only cause for infertility. Ovar-\nian varices noted on transvaginal ultrasound or pelvic veno-\ngram were treated by endovascular means. Subsequently, \neight out of the 12 women went on to become pregnant. All \npatients experienced complete or partial relief of pelvic pain. \nThe authors concluded that embolization of ovarian varices \nis a safe and effective treatment in women with infertility \nlinked to ovarian varices, who are trying to conceive [12].\nA recent study has highlighted increased risk of new-\nonset heart failure with reduced left ventricular ejection \nfraction in infertile women [13]. However, congestive heart \nfailure causing pelvic congestion, in turn contributing to \ninfertility, has not been well studied.\nChronic pelvic pain and presence of atypical varicose \nveins are features of pelvic congestion syndrome. Endovas-\ncular treatment is being considered for this condition [ 14]. \nFuture studies need to focus on symptoms of pelvic con -\ngestion and imaging for varices in ovaries and other pelvic \norgans in patients with BCS who are infertile or have had \nprior pregnancy losses. Infertility caused by pelvic con -\ngestion may be expected to be seen more often in patients \nwith BCS caused by obstruction at the level of IVC than \nof hepatic veins. Studies are needed to see if treatments to \nrelieve pelvic congestion, if present, will help women with \nBCS who are infertile or have had prior fetal loss.\n * C. E. Eapen \n eapen@cmcvellore.ac.in\n1 Department of Gastroenterology, All India Institute \nof Medical Sciences, Rishikesh 249 203, India\n2 Department of Hepatology, Christian Medical College, \nVellore, Tamil Nadu 632 004, India\nIndian Journal of Gastroenterology (January–February 2023) 42(1):14–16\nPublished online: 10 February 2023\n1 3\n\nIndian Journal of Gastroenterology (January–February 2023) 42(1):14–16 \nHormonal pills are used to treat female infertility and \ntheir thrombogenic potential can also lead to exacerbation \nof underlying hepatic venous thrombosis in BCS [ 15].\nThe thrombophilic tendency associated with pregnancy \nmay predispose to venous thromboembolism. In a study of \n72 pregnant women, venous thromboembolism developed \nduring the first trimester of pregnancy in 29 (40%) women, \nin the second trimester in 13 women (18%), and in the \nthird trimester in 30 women (42%) [ 16]. A population-\nbased study conducted over 30 years identified postpartum \nperiod as the time with maximal risk for venous throm -\nboembolism and pulmonary embolism during pregnancy \n[17]. Acute presentation of BCS during pregnancy is \nuncommon. Analysis of the clinical features (for exam -\nple, is this more common in any particular trimester of \npregnancy or in postpartum period) may improve our \nunderstanding of this uncommon presentation and lead to \nimproved treatment outcomes. Being uncommon, multi-\ncenter studies are needed to address this issue.\nThe use of oral anticoagulants in the first trimester of \npregnancy raises the concern of fetal neural tube defects. \nHence, low molecular weight heparin is recommended dur-\ning the first trimester in women who need anticoagulation \nduring pregnancy. As low molecular weight heparin is an \nexpensive parenteral drug, many patients in resource-con -\nstrained settings opt for oral anticoagulants to treat BCS \nduring pregnancy. However, teratogenic effects of oral anti-\ncoagulants are not predictable [ 18].\nIn conclusion, while the exciting advances achieved in \ntreating BCS during pregnancy are laudable, there is scope \nfor further improvement, especially of fetal outcomes in this \nscenario.\nDeclarations \nConflict of interest AS, and CEE declare no competing interests.\nDisclaimer The authors are solely responsible for the data and the con-\ntents of the paper. In no way, the Honorary Editor-in-Chief, Editorial \nBoard Members, the Indian Society of Gastroenterology or the printer/\npublishers are responsible for the results/findings and content of this \narticle.\nReferences\n 1. Khuroo MS, Datta DV. Budd-Chiari syndrome following pregnancy. \nReport of 16 cases, with roentgenologic, hemodynamic and histologic \nstudies of the hepatic outflow tract. Am J Med. 1980;68:113–21.\n 2. Dilawari JB, Bambery P, Chawla Y, et al. Hepatic outflow obstruc-\ntion (Budd-Chiari syndrome) experience with 177 patients and a \nreview of the literature. Medicine (Baltimore). 1994;73:21–36.\n 3. Rautou PE, Angermayr B, Garcia-Pagan JC, et al. Pregnancy in \nwomen with known and treated Budd-Chiari syndrome: maternal \nand fetal outcomes. J Hepatol. 2009;51:47–54.\n 4. Shukla A, Sadalage A, Gupta D, et al. Pregnancy outcomes in \nwomen with Budd Chiari Syndrome before onset of symptoms \nand after treatment. Liver Int. 2018:754–9.\n 5. Khan F, Rowe I, Martin B, et al. Outcomes of pregnancy in \npatients with known Budd-Chiari syndrome. World J Hepatol. \n2017;9:945–52.\n 6. Biswas S, Sheikh S, Vaishnav M, et al. Pregnancy outcomes \nin patients with Budd Chiari syndrome: A tertiary care experi -\nence. Indian J Gastroenterol. 2023;42.  https:// doi. org/ 10. 1007/ \ns12664- 022- 01307-7.\n 7. Wiegers H, Hamulyák E, Damhuis S, et al. Pregnancy outcomes in \nwomen with Budd-Chiari syndrome or portal vein thrombosis – a \nmulticentre retrospective cohort study. BJOG. 2022;129:608–17.\n 8. Sharma A, Goel A, Moses V, et al. Anticoagulating Budd-Chiari \nsyndrome patients presenting with variceal bleed: a retrospective \nstudy. J Gastroenterol Hepatol. 2020;35:1397–1403.\n 9. Shalimar, Sharma S, Gamanagatti SR, et al. Acute on chronic \nliver failure in Budd Chiari syndrome: profile and predictors of \noutcome. Dig Dis Sci. 2020;65:2719–29.\n 10. Priyanka S, Parveen M, Unmesh S. Budd Chiari syndrome pre -\nsenting as pelvic congestion syndrome : a rare case report. IOSR \nJ Dent Med Sci. 2016;15:76–7.\n 11. Jensen CFS, Østergren P, Dupree JM, Ohl DA, Sønksen J, Fode \nM. Varicocele and male infertility. Nat Rev Urol. 2017;14:523–33.\n 12. Liu J, Han L, Han X. The effect of a subsequent pregnancy after \novarian vein embolization in patients with infertility caused by \npelvic congestion syndrome. Acad Radiol. 2019;26:1373–7.\nTable 1  Some of the reported series of Budd-Chiari syndrome and pregnancy\nMTP medical termination of pregnancy\nYears of study Number of pregnant \nwomen enrolled\nMaternal survival Fetal outcome Reference no\n1963–1978   16   50% Intrauterine death: 5 (including MTP: 3) [1]\n1967–1991   38   48% Data not available [2]\n1985–2005   16 100%  < 20 weeks gestation: 7, stillbirth: 1 [3]\n2012–2015   60 100%  < 20 weeks: 59 (including MTP: 5), stillbirth: 10 [4]\n2001–2015     7 100%  < 20 weeks: 6 [5]\n2017–2020 121 100%  < 24 weeks: 16, stillbirth: 1, preterm: 5 [6]\n2008–2021   12 100%  < 24 weeks: 8 [7]\n15\n1 3\n\nIndian Journal of Gastroenterology (January–February 2023) 42(1):14–16\n 13. Lau ES, Wang D, Roberts M, et al. Infertility and risk of heart failure in \nthe Women’s Health Initiative. J Am Coll Cardiol. 2022;79:1594–603.\n 14. Bałabuszek K, Toborek M, Pietura R. Comprehensive overview \nof the venous disorder known as pelvic congestion syndrome. Ann \nMed. 2022;54:22–36.\n 15. Sharma A, Keshava SN, Eapen A, Elias E, Eapen CE. An \nupdate on the management of Budd-Chiari syndrome. Dig Dis \nSci. 2021;66:1780–90.\n 16. Blanco-Molina A, Trujillo-Santos J, Criado J, et al. Venous throm-\nboembolism during pregnancy or postpartum: findings from the \nRIETE Registry. Thromb Haemost. 2007;97:186–90.\n 17. Heit JA, Kobbervig CE, James AH, Petterson TM, Bailey KR, \nMelton LJ 3rd. Trends in the incidence of venous thromboembo-\nlism during pregnancy or postpartum: a 30-year population-based \nstudy. Ann Intern Med. 2005;143:697–706.\n 18. Dhillon SK, Edwards J, Wilkie J, Bungard TJ. High-versus low-\ndose warfarin-related teratogenicity: a case report and systematic \nreview. J Obstet Gynaecol Can. 2018;40:1348–57\nPublisher's note  Springer Nature remains neutral with regard to \njurisdictional claims in published maps and institutional affiliations.\n16\n1 3","source_license":"CC0","license_restricted":false}