Preeclampsia Anesthesia Management 2009 - DOC

							                              Jane Huffnagle, DO

                   Coagulation Disorders and Pregnancy



I.   Thombotic and Thrombolytic Pathways

     a. Primary Hemostasis

            i. Platelet adherence

           ii. Platelet activation

     b. Secondary Hemostasis

            i. Initiation of coagulation cascade1
       c. Clot Formation – limited by antithrombin III and proteins S and C

       d. Fibrinolytic System – plasmin breaks down fibrin

II.    Assessment of Coaguation

       a. Lab Tests – PT, aPTT, fibrinogen level, bleeding time2
       b.
            Thromboelastography 3-5

       c. PFA – 100

III.   Platelet Disorders - Thrombocytopenia
      a. Gestational Thrombocytopenia

              i. Usually mild – platelet count > 90K

             ii. No increased risk of bleeding – mom or baby

            iii. Diagnosis of exclusion

      b. Idiopathic/Immune Thrombocytopenic Purpura (ITP)

              i. Platelet-specific auto-antibodies (IgG) coat platelets, sensitized platelets
                 destroyed by RE system 6,7

             ii. Prednisone or IVIG tx

            iii. Infant may be thrombocytopenic 8,9

            iv. RA if platelet count > 75K

      c. Other Causes of Thrombocytopenia

              i. Spurious – clumped platelets

             ii. Viral – HIV, CMV, EB 10

            iii. Drug treatement – heparin, sulfonamides, PCN, rifampin, quinine 11

            iv. Associated with antiphospholipid antibodies – lupus anticoagulant,
                anticardiolipin antibodies, antibodies that cause + positive syphilis test

      d. Benard-Soulier Syndrome

              i. Deficiency of glycoproteins Ib, IX, and V

             ii. Intrapartum bleeding – avoid IM injections and RA 12,13

IV.   Platelet Function Disorders

      a. MYH9-Related Disease

              i. May-Hegglin, 14-16 Sebastian Syndrome, Fechtner Syndrome, Epstein
                 Syndrome

      b. Glanzmann Thrombasthenia (GT) 17-19

              i. Sever platelet dysfunction with failure of platelet plug formation
     c. Platelet Storage Pool Deficiency (SPD)

            i. Hermansky-Pudlak Syndrome, Grey Platelet Syndrome

            ii. Reports of pregnancy 20-22

     d. Preeclampsia and HELLP Syndrome

            i. HTN, proteinuria

            ii. Hemolysis, Elevated Liver enzymes, Low Platelets

           iii. Thrombocytopenia - < 20K

     e. Thrombotic Thrombocytopenic Purpura (TTP) and Hemolytic Uremic Syndrome
        (HUS)

            i. Diagnostic pentad – fever, thrombocytopenia, microantiopathic
               hemolytic anemia, neorologic signs (TTP), renal failure (HUS) 23

            ii. Deficiency of/or inhibitor to vW factor cleaving protease 24

           iii. IV IgG, prednisone, prostacyclin

     f. Miscellaneous Platelet Disorders – Sticky Platelet Syndrome, Neonatal
        Alloimmune Thombocytopenia (NAIT) 25

V.   Blood Vessel Wall Disorders

     a. Hereditary Hemorrhagic Telangiectasia (HHT, Osler-Weber-Rendu Syndrome)

            i. AV fistulae involving almost all organs

            ii. Epistaxis, GI hemorrhage, AVMs 26

     b. Pseudoxanthoma Elasticum (PXE)

            i. Abnormal collagen and elastin fibers

            ii. Hemorrhage and thrombosis

     c. Allergic Purpura (Henoch-Schönlein Purpura, HSP)

            i. IgA-mediated vasculitis

            ii. Purpura, hematuria, proteinuria, abdominal pain, GI bleeding, arthralgias
      d. Anesthetic Management

              i. If ongoing hemorrhage – RA contraindicated

             ii. Abnormal spinal and epidural vessels – chance of epidural bleeding

            iii. Risk of difficult intubation – calcification and aggregation of elastic fibers
                 in laryngeal ligaments and cartilage 27

VI.   Factor Defiencies

      a. Fibrinogen (Factor I) Deficiency

              i. Three Congenital Types 28 – afibrinogenemia, hypofibrinogenemia,
                 dysfibrinogenemia

             ii. RA contraindicated

      b. Prothrombin (Factor II) Deficiency

              i. Two types – hypoprothrombinemia, dysprothrombinemia

             ii. Spontaneous fetal loss and PPH 29

      c. Factor V Deficiency

              i. PPH, PT and PTT prolonged

             ii. Give FFP or solvent-detergent plasma once in labor

      d. Factor VII Deficiency

              i. Autosomal recessive 30 or aquired

             ii. Tx – recombinant factor VIIa 31,32

            iii. Case report

      e. von Willebrand Disease – types 33

              i. Type I – quantitative abnormality of Factor VIII:vWF

             ii. Type II – (subtypes IIa, IIb, IIM, IIN) qualitative abnormality of Factor
                 VIII:vWF

            iii. Type III – defective synthesis of Factor VIII
        iv. Tx : Type I and IIa – DDAVP 34, Type IIb and III – humate P 33,34

f. Factor VIII Deficiency (Hemophilia A)

         i. X-linked recessive – deficiency of Factor VIII 35
         ii.
               Rare in females, 10-30% of nonpregnant carriers have low levels 35

g. Factor IX Deficiency (Hemophilia B, Christmas Disease)

         i. rare in females, bleeding similar to hemophilia A

h. Factor X Deficiency

        i.     Heterozygous asymptomatic, homozygous severe bleeding

        ii. RA controversial

i.   Factor XI Deficiency

         i. Associated with Noonan syndrome, Gaucher syndrome, von Willebrand
            disease, Factor VII, VII deficiencies

        ii. Prolonged PTT – may need Factor XI concentrate if C/S 36

j.   Factor XII Deficiency

         i. May be associated with thrombosis rather than bleeding
         ii.
               PTT prolonged, recurrent abortion 37

k. Factor XIII Deficiency

         i. Risk of spontaneous bleeding, sever bruising, muscle hematomas,
            hemarthroses, intracranial hemorrhage, spontaneous pregnancy loss,
            PPH

        ii. Factor XIII concentrate needed throughout pregnancy 38

l.   Anesthetic Management of Congenital Coagulopathies

         i. Early consultation with hematologist and anesthesiologist

        ii. Assessment of specific factor levels

        iii. If question about coagulopathy – NO RA!!
VII.        Hypercoagulable States

            a. Protein C Deficiency

                    i. Inhibits activated Factors V and VIII

                   ii. Thrombosis occurs in 25% of pregnancies unless anticoagulated 39

            b. Protein S Deficiency

                    i. Acts as a cofactor for protein C

                   ii. Needs anticoagulation

            c. Antithrombin III Deficiency

                    i. Inactivates thrombin and Factors IXa, Xa, XIa and XIIa

                   ii. Deficiencies can be quantitative and qualitative

                  iii. Need anticoagulation 40,41

            d. Antiphospholipid Syndrome

                    i. Two antibodies – lupus anticoagulant and anticardiolipin antibody

                   ii. Associated with thrombotic events

                  iii. PTT is prolonged (phospholipid-dependent coagulation assays) but
                       bleeding is rare

                  iv. RA okay if no other Factor deficiencies and patient is not anticoagulated

            e. Factor V Leiden Deficiency

                    i. Factor V Leiden cannot be inactivated - leads to overproduction of
                       thrombin and excess clotting

                   ii. Risk of DVT, PE, preeclampsia, miscarriage, still birth, abruption

                  iii. Need anticoagulation in homozygous form, also if hx of DVT in
                       heterozygous form 42-44

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