Pregnancy and vascular liver diseases · PDF file Coagulation changes in pregnancy. Outline...

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Transcript of Pregnancy and vascular liver diseases · PDF file Coagulation changes in pregnancy. Outline...

  • Pregnancy and vascular

    liver diseases

    J B Dilawari, Chandigarh, India

    Pierre-Emmanuel Rautou, Clichy, France

  • Outline

    • Introduction

    • Is pregnancy a risk factor for vascular liver

    disease?

    • What are the outcomes of pregnancy in women

    with vascular liver disorders ?

    • Management of pregnancy and delivery

  • Outline

    • Introduction

    • Is pregnancy a risk factor for vascular liver

    disease?

    • What are the outcomes of pregnancy in women

    with vascular liver disorders ?

    • Management of pregnancy and delivery

  • Pregnancy and liver disorders

    1. Pre-existing chronic liver disease:

    2. Unique to pregnancy:

    3. Developing during with pregnancy:

    Hyperemesis gravidarum

    Acute fatty liver of pregnancy

    HELLP, preeclampsia

    Intrahepatic cholestasis of pregnancy

    Viral hepatitis

    Drug induced liver injury

    Vascular liver disease

    Cirrhosis

    Viral hepatitis

    Cholestastic liver disease

    Benign liver nodules

    Wilson’s disease

    Vascular liver diseases

  • IVC

    PV HA

    HV HV

    • Affect either IVC, HV, PV

    • Frequent during pregnancy

    Pregnancy and vascular liver disorders

  • Hemodynamic changes in pregnancy

    - Vasodilatation, arterial BP

    - Cardiac out put , Blood Volume

    - Portal blood flow , P.V. diameter

    - Venous return in IVC due to Gravid uterus

    (blood flows from lower part to azygous system)

    Changes reminiscent of those of cirrhosis

    Munnell, JCI 1947. Mustafa, J Pregnancy 2012; Bissonnette, JCEH 2015

  • Procoagulant

    state

    Anticoagulant

    state

    Pregnancy

     Von Willebrand factor

     Fibrinogen

     Factors II, VII, VIII and X

     Protein S

    Resistance to activated protein C

     Plasminogen activator inhibitor-1

    Pregnancy

    -

    Battaglioli, Curr Opin Hematol 2007; Marik, NEJM 2008

    Coagulation changes in pregnancy

  • Outline

    • Introduction

    • Is pregnancy a risk factor for vascular liver

    disease?

    • What are the outcomes of pregnancy in women

    with vascular liver disorders ?

    • Management of pregnancy and delivery

  • • A 29 year old female

    • History of 2 miscarriages

    • Day 10 post-partum (baby born at 32 weeks):

    – Acute onset of right upper quadrant abdo. pain

    – Ascites and jaundice

    – T Bili: 5.6 mg/dL; AST/ALT: 205/418 IU/L; INR: 2.6

    – Doppler U/S: localized thrombosis right HV, Coma

    shaped collaterals

    CASE 1

    Is that a fortuitous association?

  • Is pregnancy a risk factor for Budd-Chiari

    syndrome?

    Mohanti, Hepatology 2001; Dilawari, Medicine 1994; Rautou, Gut 2009

    • Temporal association between the 2 conditions

    • Prevalence of pregnancy/postpartum in women

    aged 15-45 in France between 1995 and 2005:

    Yes (likely, and other risk factors frequently associated)

    Among women

    presenting with BCS:

    16% (7/43)

    In the general French

    population:

    7–8%

  • Pregnancy

    Budd-Chiari syndrome Yes (likely)

    Portal vein thrombosis Rare reports

    Sinusoidal obstruction syndrome No report

    Liver hemangioma Exceptional

    Obliterative portal veinopathy No report

    Is pregnancy a risk factor for vascular liver

    diseases?

    Bissonnette, JCEH 2015

  • Outline

    • Introduction

    • Is pregnancy a risk factor for vascular liver

    disease?

    • What are the outcomes of pregnancy in women

    with vascular liver disorders ?

    • Management of pregnancy and delivery

  • CASE 2

    • A 29 year old woman with BCS due to

    myeloproliferative disease desires a pregnancy

    • Treatment: vitamin K antagonists; hydroxyurea

    • Physical examination: no symptom

    • Blood test: INR 1,1; serum bilirubin normal

    - Is pregnancy contraindicated?

    - What is the expected risk for the baby, for her?

  • Pregnancy and BCS: Maternal outcome

    0

    10

    20

    30

    40

    50

    60

    % o

    f m

    a te

    rn a l d e

    a th

    Khuroo

    1963-78

    Dilawari

    1967-91

    Rautou

    1985-2005

    N=16 N=38

    Khan,

    2001-15

    N=16N=24

    Shukla

    2012-15

    N=15Number of pregnancies

    No treatment

    0% 0% 0%

    Anticoagulation  angioplasty/TIPS

  • 56 pregnancies

    GI Bleeding 0

    Genital, parietal bleeding 33 pts with anticoagulation

    7

    Thrombotic event 0

    Liver related ascites, pulmonary artery hypertension

    4

    Related to pregnancy cholestasis, placenta praevia, preeclampsia

    13

    Rautou, J Hepatol 2009; Khan, World J Hepatol 2017;

    Shukla, Liv Int 2017; Aggarwal Arch Gynecol Obstet 2013

    Pregnancy and BCS: Maternal outcome

  • Pregnancy and BCS: Fetal outcome

    Rautou, J Hepatol 2009; Khan, World J Hepatol 2017

    N u m

    b e

    r o

    f p

    re g

    n a

    n c ie

    s

    13

    3

    16

    8

    0

    2

    4

    6

    8

    10

    12

    14

    16

    18

    < 20 20-31 32-36 > 36

    Weeks of gestation

    40 pregnancies

    26 alive

    No sequelae

    1 stillbirth

    Miscarriage/

    ectopic

    pregnancy

    Early

    preterm

    Preterm

    Term

  • CASE 3

    • 34 year old female

    • INCPH revealed 1 year ago:

    • Splenomegaly, no varices

    • Liver blood tests abnormalities

    • Liver biopsy: no cirrhosis, nodular regenerative hyperplasia

    • Sicca syndrome

    • Desires pregnancy (1 daughter, 5 year-old)

    - Is fertility preserved?

    - Is pregnancy contraindicated?

    - What is the expected risk for the baby, for her?

  • 0,00%

    0,05%

    0,10%

    0,15%

    0,20%

    0,25%

    Kochhar R Dig Dis Sci. 1999

    INCPH and fertility

    Controls

    (N=44)

    Before diagnosis After diagnosis

    F e

    rt ili

    ty ra

    te

    of noncirrhotic portal hypertension (n=55)

    No difference

  • INCPH and pregnancy 24 pregnancies (16 women)

    Diagnosis 1st pregnancy

    controlled

    Anticoagulation (n=6)

    Antiplatelet (n=1)

    No treatment (n=19)

    • 7 w/o prophylaxis of GI bleeding

    • 5 with portal vein thrombosis

    • 4 TIPS

    Median: 38 months

    Andrade, revised

  • 24 pregnancies

    Maternal death 0

    Esophageal varices 1 w/o appropriate prophylaxis

    2

    Ascites 3

    Porto-pulmonary hypertension 1

    Genital-parietal bleeding 2

    Thrombotic event 1

    INCPH: maternal outcome

    Andrade, revised

  • 24 pregnancies in 16 women with INCPH

    N u m

    b e r

    o f p re

    g n a n c ie

    s

    5

    2

    8

    9

    0

    2

    4

    6

    8

    10

    < 20 20-31 32-36 > 36

    Weeks of gestation

    Miscarriage/

    ectopic

    pregnancy

    18 alive

    No sequelae

    2 death

    Andrade, revised; similar or better results in: Kochhar R Dig Dis Sci. 1999

    INCPH: fetal outcome

  • • 27 yr-old female

    • Acute portal vein thrombosis in 2009 – Superior mesenteric and splenic veins

    – Mesenteric intestinal ischemia

    • Associated conditions – Pernicious anemia (vit B12 supplementation)

    – Hyperhomocysteinemia

    CASE 3

  • • Anticoagulation therapy – By 3 months: portal cavernoma development

    – Maintained long term

    • Esophageal varices → Nonselective β-block.

    • 2015: Routine work-up – Normal liver blood tests

    – Unchanged esophageal varices

    – Desire for pregnancy

    CASE 3

  • What is your answer to patient’s

    concern about pregnancy outcome?

    1.The probability of a term birth of a healthy

    child is below 50%.

    2.The rate of miscarriage is increased.

    3.The probability of preterm birth is

    increased.

    4.The probability of congenital malformation

    is increased.

    5.Perinatal mortality rate is about 25 %.

  • 1.The probability of a term birth of a healthy

    child is below 50%.

    2.The rate of miscarriage is increased.

    3.The probability of preterm birth is

    increased.

    4.The probability of congenital malformation

    is increased.

    5.Perinatal mortality rate is about 25 %.

    What is your answer to patient’s

    concern about pregnancy outcome?

  • PVT: fetal outcome

    Mandal, Singapore Med J 2012. Hoekstra, J Hepatol 2012.

    Aggarwal, J Obstet G