Pregnancy and Vascular Liver Disease
2015; Elsevier BV; Volume: 5; Issue: 1 Linguagem: Inglês
10.1016/j.jceh.2014.12.007
ISSN2213-3453
AutoresJulien Bissonnette, François Durand, Emmanuelle de Raucourt, Pierre‐François Ceccaldi, Aurélie Plessier, Dominique Valla, Pierre‐Emmanuel Rautou,
Tópico(s)Abdominal vascular conditions and treatments
ResumoVascular disorders of the liver frequently affect women of childbearing age. Pregnancy and the postpartum are prothrombotic states. Pregnancy seems to be a trigger for Budd–Chiari syndrome in patients with an underlying prothrombotic disorder. Whether pregnancy is a risk factor for other vascular liver disorders is unknown.In women with a known vascular liver disorder and a desire for pregnancy, stabilisation of the liver disease, including the use of a portal decompressive procedure when indicated, should be reached prior to conception. The presence of esophageal varices should be screened and adequate prophylaxis of bleeding applied in a manner similar to what is recommended for patients with cirrhosis. Most women likely benefit from anticoagulation during pregnancy and the postpartum. Labor and delivery are best managed by a multidisciplinary team with experience in this situation. Assisted vaginal delivery is the preferred mode of delivery. Although the risk of miscarriage and premature birth is heightened, current management of these diseases makes it very likely to see the birth of a live baby when pregnancy reaches 20 weeks of gestation. Vascular disorders of the liver frequently affect women of childbearing age. Pregnancy and the postpartum are prothrombotic states. Pregnancy seems to be a trigger for Budd–Chiari syndrome in patients with an underlying prothrombotic disorder. Whether pregnancy is a risk factor for other vascular liver disorders is unknown. In women with a known vascular liver disorder and a desire for pregnancy, stabilisation of the liver disease, including the use of a portal decompressive procedure when indicated, should be reached prior to conception. The presence of esophageal varices should be screened and adequate prophylaxis of bleeding applied in a manner similar to what is recommended for patients with cirrhosis. Most women likely benefit from anticoagulation during pregnancy and the postpartum. Labor and delivery are best managed by a multidisciplinary team with experience in this situation. Assisted vaginal delivery is the preferred mode of delivery. Although the risk of miscarriage and premature birth is heightened, current management of these diseases makes it very likely to see the birth of a live baby when pregnancy reaches 20 weeks of gestation. Vascular disorders of the liver include a spectrum of conditions involving the portal venous system, the intrahepatic vessels, the hepatic veins and the terminal portion of the inferior vena cava. Although rare, they frequently affect women of childbearing age.1DeLeve L.D. Valla D.-C. Garcia-Tsao G. American association for the study liver diseases. Vascular disorders of the liver.Hepatol. 2009; 49: 1729-1764https://doi.org/10.1002/hep.22772Crossref PubMed Scopus (673) Google Scholar Fertility rates have been reported to be unchanged in women with non-cirrhotic portal hypertension of various etiologies before and after onset of liver disease when compared with healthy controls.2Kochhar R. Kumar S. Goel R.C. Sriram P.V. Goenka M.K. Singh K. Pregnancy and its outcome in patients with noncirrhotic portal hypertension.Dig Dis Sci. 1999; 44: 1356-1361Crossref PubMed Scopus (42) Google Scholar This contrasts with cirrhosis, known to be associated with decreased fertility.3Joshi D. James A. Quaglia A. Westbrook R.H. Heneghan M.A. Liver disease in pregnancy.Lancet. 2010; 375: 594-605https://doi.org/10.1016/S0140-6736(09)61495-1Abstract Full Text Full Text PDF PubMed Scopus (251) Google Scholar Issues raised by pregnancy in patients with vascular liver disease include the following: (a) is pregnancy a risk factor for vascular liver disease? (b) what are the outcomes of pregnancy in women with established vascular diseases of the liver? and (c) how to manage pregnancy and delivery? The objective of this review article is to address these issues. Pregnancy is associated with several systemic hemodynamic changes. A rise in blood volume and cardiac output by 30–50% occurs during the second and third trimesters. Arterial blood pressure decreases by 10% during mid-pregnancy and returns to pre-pregnancy levels at term.4Mustafa R. Ahmed S. Gupta A. Venuto R.C. A comprehensive review of hypertension in pregnancy.J Pregnancy. 2012; 2012: 105918https://doi.org/10.1155/2012/105918Crossref PubMed Scopus (156) Google Scholar These features are due to a drop in systemic vascular resistance. Red blood cell total mass increases, but to a lower extent than total blood volume which results in a slight decrease in hematocrit levels. The gravid uterus obstructs the venous return in the inferior vena cava so that, at term, much of the blood flow from the lower part of the body is redirected to the azygos system.5Cheng Y.S. Pregnancy in liver cirrhosis and/or portal hypertension.Am J Obstet Gynecol. 1977; 128: 812-822Abstract Full Text PDF PubMed Scopus (99) Google Scholar, 6Kerr M.G. Scott D.B. Samuel E. Studies of the inferior vena cava in late pregnancy.Br Med J. 1964; 1: 522.4-533Google Scholar A limited number of studies have evaluated the changes in splanchnic blood flow during normal pregnancy. Studies dating back to the seventies and using bromsulfalein clearance rate have yielded inconsistent results, suggesting increased or unchanged splanchnic blood flow.7Munnell E.W. Taylor H.C. Liver blood flow in pregnancy-hepatic vein catheterization.J Clin Invest. 1947; 26: 952-956https://doi.org/10.1172/JCI101890Crossref PubMed Scopus (43) Google Scholar, 8Tindall V. The liver in pregnancy.Clin Obstet Gynecol. 1975; 2: 441-462Google Scholar Subsequent estimates of hepatic blood flow in normal pregnancy using ultrasonography with Doppler have demonstrated a significant rise in portal blood flow as compared to pre-pregnancy values during recumbency and standing rest.9Clapp 3rd, J.F. Stepanchak W. Tomaselli J. Kortan M. Faneslow S. Portal vein blood flow-effects of pregnancy, gravity, and exercise.Am J Obstet Gynecol. 2000; 183: 167-172https://doi.org/10.1067/mob.2000.105902Abstract Full Text Full Text PDF PubMed Scopus (69) Google Scholar This occurs via an increase in the diameter of the intra- and extra-hepatic branches of the portal vein, despite a reduction in mean blood velocity.10Mayo M.A. López-Cano A. Méndez C. Muñoz A. Pacheco J.M. Vico F.J. Hemodynamic modifications in splenic circulation studied by echo-Doppler during pregnancy.Gastroenterol Hepatol. 2002; 25: 148-152Crossref PubMed Scopus (3) Google Scholar, 11Nakai A. Sekiya I. Oya A. Koshino T. Araki T. Assessment of the hepatic arterial and portal venous blood flows during pregnancy with Doppler ultrasonography.Arch Gynecol Obstet. 2002; 266: 25-29Crossref PubMed Scopus (74) Google Scholar Blood flow in the hepatic artery is unchanged even though resistance indexes are reduced.10Mayo M.A. López-Cano A. Méndez C. Muñoz A. Pacheco J.M. Vico F.J. Hemodynamic modifications in splenic circulation studied by echo-Doppler during pregnancy.Gastroenterol Hepatol. 2002; 25: 148-152Crossref PubMed Scopus (3) Google Scholar, 11Nakai A. Sekiya I. Oya A. Koshino T. Araki T. Assessment of the hepatic arterial and portal venous blood flows during pregnancy with Doppler ultrasonography.Arch Gynecol Obstet. 2002; 266: 25-29Crossref PubMed Scopus (74) Google Scholar There are no data on hemodynamic changes occurring during pregnancy in women with chronic liver disease. Yet, if the above-mentioned changes also occur in patients with vascular liver disease, pregnancy may exacerbate portal hypertension. Indeed, the hypervolemic and systemic hyperdynamic state associated with pregnancy is reminiscent of the circulatory changes classically associated with portal hypertension and may thus further augment them.12García-Pagán J.-C. Gracia-Sancho J. Bosch J. Functional aspects on the pathophysiology of portal hypertension in cirrhosis.J Hepatol. 2012; 57: 458-461https://doi.org/10.1016/j.jhep.2012.03.007Abstract Full Text Full Text PDF PubMed Scopus (169) Google Scholar Presumed to take place for the hemostatic challenge of delivery, various coagulation changes occur during pregnancy, as reviewed elsewhere in detail.13Thornton P. Douglas J. Coagulation in pregnancy.Best Pract Res Clin Obstet Gynaecol. 2010; 24: 339-352https://doi.org/10.1016/j.bpobgyn.2009.11.010Abstract Full Text Full Text PDF PubMed Scopus (104) Google Scholar These changes are summarised in Figure 1. Briefly, there is an increased activity of procoagulant factors, a decrease in certain natural anticoagulant factors and in fibrinolysis. For many of these changes, close correlations have been established with the hormonal changes of pregnancy, and in particular with increased serum estradiol levels.14Sattar N. Greer I.A. Rumley A. et al.A longitudinal study of the relationships between haemostatic, lipid, and oestradiol changes during normal human pregnancy.Thromb Haemost. 1999; 81: 71-75PubMed Google Scholar This procoagulant state is illustrated by the elevated levels of fibrin degradation products, including D-dimers, during pregnancy.13Thornton P. Douglas J. Coagulation in pregnancy.Best Pract Res Clin Obstet Gynaecol. 2010; 24: 339-352https://doi.org/10.1016/j.bpobgyn.2009.11.010Abstract Full Text Full Text PDF PubMed Scopus (104) Google Scholar Accordingly, a decrease in commonly used coagulation tests, such as prothrombin time (PT), thrombin time (TT) and the activated partial thromboplastin time (aPTT) has been described in studies comparing pregnant patients to healthy controls.15Uchikova E.H. Ledjev I.I. Changes in haemostasis during normal pregnancy.Eur J Obstet Gynecol Reprod Biol. 2005; 119: 185-188https://doi.org/10.1016/j.ejogrb.2004.06.038Abstract Full Text Full Text PDF PubMed Scopus (91) Google Scholar, 16Hui C. Lili M. Libin C. et al.Changes in coagulation and hemodynamics during pregnancy: a prospective longitudinal study of 58 cases.Arch Gynecol Obstet. 2012; 285: 1231-1236https://doi.org/10.1007/s00404-011-2137-xCrossref PubMed Scopus (47) Google Scholar A practical consequence of that feature is that levels of coagulation parameters classically used to evaluate liver function, such as INR, should be interpreted with caution in pregnant patients with underlying liver disease. It must also be kept in mind that the antiphospholipid syndrome, present in some vascular liver disease patients, can cause a prolonged aPTT despite a procoagulant state. Mild thrombocytopenia related to increased plasma volume is another common feature of pregnancy.17Burrows R.F. Platelet disorders in pregnancy.Curr Opin Obstet Gynecol. 2001; 13: 115-119Crossref PubMed Scopus (21) Google Scholar Therefore, in pregnant women with vascular liver disease, decreasing platelet counts may not reflect increasing portal hypertension. In Indian studies including patients in the seventies and eighties, up to 47% of reported Budd–Chiari syndrome (BCS) cases were encountered in women presenting in pregnancy or postpartum.18Khuroo M.S. Datta D.V. Budd-Chiari syndrome following pregnancy. Report of 16 cases, with roentgenologic, hemodynamic and histologic studies of the hepatic outflow tract.Am J Med. 1980; 68: 113-121Abstract Full Text PDF PubMed Scopus (159) Google Scholar, 19Dilawari J.B. Bambery P. Chawla Y. et al.Hepatic outflow obstruction (Budd-Chiari syndrome). Experience with 177 patients and a review of the literature.Medicine (Baltimore). 1994; 73: 21-36Crossref PubMed Scopus (348) Google Scholar More recently, pregnancy was present within 3 months before BCS diagnosis in about 6% of female patients in an Indian and a large European multicentric study.20Darwish Murad S. Plessier A. Hernandez-Guerra M. et al.Etiology, management, and outcome of the Budd-Chiari syndrome.Ann Intern Med. 2009; 151: 167-175Crossref PubMed Scopus (371) Google Scholar, 21Mohanty D. Shetty S. Ghosh K. Pawar A. Abraham P. Hereditary thrombophilia as a cause of Budd-Chiari syndrome: a study from Western India.Hepatol Balt Md. 2001; 34: 666-670https://doi.org/10.1053/jhep.2001.27948Crossref PubMed Scopus (124) Google Scholar To clarify the relationship between pregnancy and BCS development, the prevalence of pregnancy or post-partum BCS was analysed in women of childbearing age seen consecutively in a reference center over a ten-year period. The proportion of women with a diagnosis of BCS made during pregnancy or postpartum was 16%, i.e. twice higher than the corresponding point prevalence of pregnancy or postpartum among women aged 15–45 years in the general French population.22Rautou P.-E. Plessier A. Bernuau J. Denninger M.-H. Moucari R. Valla D. Pregnancy: a risk factor for Budd-Chiari syndrome?.Gut. 2009; 58: 606-608https://doi.org/10.1136/gut.2008.167577Crossref PubMed Scopus (38) Google Scholar Most BCS cases occurred in patients with other risk factors for thrombosis than pregnancy.23Hiroe S. Itoh H. Matsumoto H. et al.Case of Budd-Chiari syndrome 3 months after vaginal delivery.J Obstet Gynaecol Res. 2008; 34: 605-608https://doi.org/10.1111/j.1447-0756.2008.00893.xCrossref PubMed Scopus (6) Google Scholar, 24Anbazhagan A. Harper A. 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Pregnancy: a risk factor for Budd-Chiari syndrome?.Gut. 2009; 58: 606-608https://doi.org/10.1136/gut.2008.167577Crossref PubMed Scopus (38) Google Scholar This may be due to the previously described decrease in functional levels of this protein during pregnancy.30Lefkowitz J.B. Clarke S.H. Barbour L.A. Comparison of protein S functional and antigenic assays in normal pregnancy.Am J Obstet Gynecol. 1996; 175: 657-660Abstract Full Text Full Text PDF PubMed Scopus (38) Google Scholar, 31Comp P.C. Thurnau G.R. Welsh J. Esmon C.T. Functional and immunologic protein S levels are decreased during pregnancy.Blood. 1986; 68: 881-885Crossref PubMed Google Scholar Indirect evidence suggests that myeloproliferative disorders (MPD) may also synergise with pregnancy to induce splanchnic vein thrombosis. Work-up for thrombosis risk factors has been incomplete in most studies, in particular regarding the JAK2V617F status. Five percent of 237 pregnancies in women with essential thrombocythemia, were associated with the development of splanchnic vein thrombosis in the weeks or months after delivery.32Randi M.L. Bertozzi I. Rumi E. et al.Pregnancy complications predict thrombotic events in young women with essential thrombocythemia.Am J Hematol. 2014; 89: 306-309https://doi.org/10.1002/ajh.23635Crossref PubMed Scopus (42) Google Scholar This incidence is higher than expected in essential thrombocythemia patients.33Casini A. Fontana P. Lecompte T.P. Thrombotic complications of myeloproliferative neoplasms: risk assessment and risk-guided management.J Thromb Haemost. 2013; 11: 1215-1227https://doi.org/10.1111/jth.12265Crossref PubMed Scopus (58) Google Scholar Thrombotic events happened more commonly after a complicated (28%) than after an uneventful pregnancy (3%).32Randi M.L. Bertozzi I. 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Garcia-Pagan J.-C. et al.Pregnancy in women with known and treated Budd-Chiari syndrome: maternal and fetal outcomes.J Hepatol. 2009; 51: 47-54https://doi.org/10.1016/j.jhep.2009.02.028Abstract Full Text Full Text PDF PubMed Scopus (62) Google Scholar All patients were in a stable condition at the time of conception, 9 of them having been previously treated with a portal decompressive procedure. One or several risk factors for thrombosis had been identified in 14 of these 16 women. Anticoagulation therapy was used during 17 of the pregnancies. As shown in Table 1 and Figure 2, miscarriage, defined as a spontaneous termination of pregnancy before 20 weeks of gestation, occurred in 29% of the pregnancies. After gestation week 20, one stillbirth occurred, but all other infants did well despite a high incidence of preterm birth.Table 1Outcomes of Pregnancies in Women with Previously Diagnosed Budd–Chiari Syndrome or Portal Vein Thrombosis.Budd–Chiari syndrome (24 pregnancies)34Rautou P.-E. Angermayr B. Garcia-Pagan J.-C. et al.Pregnancy in women with known and treated Budd-Chiari syndrome: maternal and fetal outcomes.J Hepatol. 2009; 51: 47-54https://doi.org/10.1016/j.jhep.2009.02.028Abstract Full Text Full Text PDF PubMed Scopus (62) Google ScholarPortal vein thrombosis (104 pregnancies)50Hoekstra J. Seijo S. Rautou P.E. et al.Pregnancy in women with portal vein thrombosis: results of a multicentric European study on maternal and fetal management and outcome.J Hepatol. 2012; 57: 1214-1219https://doi.org/10.1016/j.jhep.2012.07.034Abstract Full Text Full Text PDF PubMed Scopus (28) Google Scholar, 51Aggarwal N. Chopra S. Raveendran A. Suri V. Dhiman R.K. Chawla Y.K. Extra hepatic portal vein obstruction and pregnancy outcome: largest reported experience.J Obstet Gynaecol Res. 2011; 37: 575-580https://doi.org/10.1111/j.1447-0756.2010.01407.xCrossref PubMed Scopus (22) Google Scholar, 52Mandal D. Dattaray C. Sarkar R. Mandal S. Choudhary A. Maity T.K. Is pregnancy safe with extrahepatic portal vein obstruction? An analysis.Singapore Med J. 2012; 53: 676-680PubMed Google ScholarMiscarriages29%14%Stillbirths6%2%Premature births76%14%Cesarean section47%37%aData obtained from references 50,52.Variceal hemorrhages0%5%Non-variceal hemorrhages35%6%Thrombotic events15%3%aData obtained from references 50,52.a Data obtained from references 50Hoekstra J. Seijo S. Rautou P.E. et al.Pregnancy in women with portal vein thrombosis: results of a multicentric European study on maternal and fetal management and outcome.J Hepatol. 2012; 57: 1214-1219https://doi.org/10.1016/j.jhep.2012.07.034Abstract Full Text Full Text PDF PubMed Scopus (28) Google Scholar, 52Mandal D. Dattaray C. Sarkar R. Mandal S. Choudhary A. Maity T.K. Is pregnancy safe with extrahepatic portal vein obstruction? An analysis.Singapore Med J. 2012; 53: 676-680PubMed Google Scholar. Open table in a new tab Maternal outcome was good. No death occurred during pregnancy. Yet, three thrombotic events happened, including 2 related to shunt obstruction. Six bleeding events were recorded, including four in the early postpartum period. Prothrombin gene mutation was associated with a poorer outcome of pregnancies. Although great caution is required when comparing European patients seen after 1985 with Indian patients managed between 1960 and the early nineties, it seems that the current overall management of BCS has allowed an increasing number of young female patients to achieve an improved condition allowing them to consider pregnancy and go through this pregnancy without jeopardising their lives.18Khuroo M.S. Datta D.V. Budd-Chiari syndrome following pregnancy. Report of 16 cases, with roentgenologic, hemodynamic and histologic studies of the hepatic outflow tract.Am J Med. 1980; 68: 113-121Abstract Full Text PDF PubMed Scopus (159) Google Scholar, 19Dilawari J.B. Bambery P. Chawla Y. et al.Hepatic outflow obstruction (Budd-Chiari syndrome). Experience with 177 patients and a review of the literature.Medicine (Baltimore). 1994; 73: 21-36Crossref PubMed Scopus (348) Google Scholar Recent Indian data support this view although most women suffered BCS revealed by pregnancy in that report.35Aggarwal N. Suri V. Chopra S. Sikka P. Dhiman R.K. Chawla Y.K. Pregnancy outcome in Budd Chiari syndrome–a tertiary care centre experience.Arch Gynecol Obstet. 2013; 288: 949-952https://doi.org/10.1007/s00404-013-2834-8Crossref PubMed Scopus (15) Google Scholar Portal vein thrombosis (PVT) diagnosis during pregnancy or post-partum is uncommon. In the largest recent series, 0–4% of PVT cases were time-related to pregnancy.36Primignani M. Barosi G. Bergamaschi G. et al.Role of the JAK2 mutation in the diagnosis of chronic myeloproliferative disorders in splanchnic vein thrombosis.Hepatol Balt Md. 2006; 44: 1528-1534https://doi.org/10.1002/hep.21435Crossref PubMed Scopus (216) Google Scholar, 37Plessier A. Darwish-Murad S. 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Despite differences in the incidence of documented prothrombotic states and in the use of anticoagulation, the best information stems from three series of patients, two Indian and one European, including a total of 104 pregnancies (Table 1 and Figure 2).50Hoekstra J. Seijo S. Rautou P.E. et al.Pregnancy in women with portal vein thrombosis: results of a multicentric European study on maternal and fetal management and outcome.J Hepatol. 2012; 57: 1214-1219https://doi.org/10.1016/j.jhep.2012.07.034Abstract Full Text Full Text PDF PubMed Scopus (28) Google Scholar, 51Aggarwal N. Chopra S. Raveendran A. Suri V. Dhiman R.K. Chawla Y.K. Extra hepatic portal vein obstruction and pregnancy outcome: largest reported experience.J Obstet Gynaecol Res. 2011; 37: 575-580https://doi.org/10.1111/j.1447-0756.2010.01407.xCrossref PubMed Scopus (22) Google Scholar, 52Mandal D. Dattaray C. Sarkar R. Mandal S. Choudhary A. Maity T.K. Is pregnancy safe with extrahepatic portal vein obstruction? An analysis.Singapore Med J. 2012; 53: 676-680PubMed Google Scholar Maternal and fetal outcomes are summarised in Table 1 and Figure 2. Fetal outcomes were good with low numbers of stillbirths and perinatal deaths despite a slightly higher rate of prematurity than expected in the general population.53Blencowe H. Cousens S. Oestergaard M.Z. et al.National, regional, and worldwide estimates of preterm birth rates in the year 2010 with time trends since 1990 for selected countries: a systematic analysis and implications.Lancet. 2012; 379: 2162-2172https://doi.org/10.1016/S0140-6736(12)60820-4Abstract Full Text Full Text PDF PubMed Scopus (2912) Google Scholar Results for the mothers also described increased morbidity, but no death. Only five episodes of variceal hemorrhage happened, including three in patients without adequate prophylaxis for portal hypertension-related bleeding. Other hemorrhagic events were genital or parietal bleedings and occurred in most cases in the peri-partum period and in patients not on anticoagulation. There were two thrombotic events, one transient ischemic attack and one splenic infarction. No case of mesenteric ischemia or deep-vein thrombosis was reported. The only statistically significant risk factor for unfavorable outcome identified in the European study was a higher platelet count at diagnosis. Similar to BCS, this association points to chronic underlying prothrombotic conditions, and particularly MPD, as a possible cause for an unfavorable pregnancy outcome, due to thrombotic occlusion in the placental circulation. A previous Indian study dating back to 2001 described the outcome of 23 pregnancies in 12 women with PVT.54Aggarwal N. Sawhney H. Vasishta K. Dhiman R.K. Chawla Y. Non-cirrhotic portal hypertension in pregnancy.Int J Gynaecol Obstet. 2001; 72: 1-7Abstract Full Text Full Text PDF PubMed Scopus (56) Google Scholar However, the interpretation of this study is limited by the fact that PVT was diagnosed during the index pregnancy in 6 out of the 12 patients. This may explain the higher frequency of fetal complications than in the two more recent series mentioned above: there were 4 miscarriages (17%), 3 stillbirths (16%), and only 2 preterm births (11%). Hereditary hemorrhagic telangiectasia is a rare genetically transmitted vascular disease affecting the brain, heart, lungs and liver circulation through the formation of vascular malformations. When symptomatic, liver involvement can manifest as high-output cardiac failure, ischemic cholangiopathy, porto-systemic encephalopathy or portal hypertension.55Khalid S.K. Garcia-Tsao G. 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