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Anaesthesiologic management of a parturient with a known antiphospholipid syndrome
Pascal Vuilleumier
Universitätsklinik für Anästhesie und Schmerztherapie
Anaesthesthesiologic management of a parturient with a known antiphospholipid syndrome 2
Universitätsklinik für Anästhesiologie und Schmerztherapie
Case presentation: Mrs A. B. 1975
• Early monday morning, phone call from the midwives:
– Mrs A. B. needs a cesarean delivery in the next 30 minutes because of foetal distress
– Fraxiparine 0.6ml bid, last dose 12 h ago
– 100 mg Aspirin per day
Anaesthesthesiologic management of a parturient with a known antiphospholipid syndrome 3
Universitätsklinik für Anästhesiologie und Schmerztherapie
Case presentation: Mrs A. B. 1975
Anaesthesthesiologic management of a parturient with a known antiphospholipid syndrome 4
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Present history–2nd pregnancy, 33 5/7 weeks’ gestation–Intrauterine growth retardation with pathological FHR
monitoring–Antenatal corticosteroid therapy given–Mild excentric mitral insufficiency–Known antiphospholipid-antibody syndrome since 2005
with:• Left deep vein thrombosis and central pulmonary thromboembolism 11/2003
• Postpartal peripheric pulmonary thromboembolism with lung infarction despite treatment with Fraxiparin 0,6 ml 2*/day 13 month before
Anaesthesthesiologic management of a parturient with a known antiphospholipid syndrome 5
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Anaesthesiologic management
• Decision of general anaesthesia• Induction and intraoperative course uneventful• Uterotomy and delivery whithout problems, APGAR
9/10/10 and normal umbilical pH values• At the end of the cesarean delivery the uterine tonus is
good, there is no bleeding, extubation is smooth• Mother and baby are well
Anaesthesthesiologic management of a parturient with a known antiphospholipid syndrome 6
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Postoperative management
• Goal: Establishment of therapeutic anticoagulation as quick as possible
• Start Heparin 10‘000 IU/24 H immediately after umbilical ligature
• Uneventful adaptation of the newborn and no postpartal bleeding
• But…
Anaesthesthesiologic management of a parturient with a known antiphospholipid syndrome 7
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First postoperative day• 24H after C-section: acute retrosternal pain with irradiation
in the left arm, resistant to nitroglycerine
• The EKG shows ST-elevation in the inferior leads
• The initial Troponin is normal
• Angio-CT scan shows no lung embolism
• Transthoracic ECHO: – inferobasal hypokinesia, – right and left vertricules are normal in size–MI unchanged
Anaesthesthesiologic management of a parturient with a known antiphospholipid syndrome 8
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Further treatment and evaluation
• She is transferred to the ICU for rhythm monitoring
• 6 hours after the beginning of symptoms pain vanishes, but heart enzymes rise
• A coronarography is performed immediately
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Coronarography
• Subacute myocardial infarction with occlusion of a side-branch of the 1. Marginal, successful aspiration and PTCA
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48H later
• The clinical course in the ICU is favorable and she is transferred back to the women‘s ward on day 2
• Histology of the aspired thrombus reveals placental tissueSuspicion of patent foramen ovale, evaluation for a possible percutaneous closure after clinical stabilisation
Anaesthesthesiologic management of a parturient with a known antiphospholipid syndrome 11
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Evolution on day 2-4
–Ongoing haemoglobin fall with final Hb of 61g/l despite 4 blood concentrates
–Hemodynamics „just“ stable–The „FAST“-scan of the abdomen detects free liquid in
the peritoneum–The ongoing blood loss, the positive fast-scan and the
hemodynamics warrant an exploratory laparotomy
Anaesthesthesiologic management of a parturient with a known antiphospholipid syndrome 12
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Exploratory laparotomy
• Stable intraoperative progress, lavage, surgical hemostasis, need of 4 blood concentrates and 2 fresh frozen plasma,
• no clear bleeding source identified
Anaesthesthesiologic management of a parturient with a known antiphospholipid syndrome 13
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Day 5
• Back to the women‘s ward, there is negligable free liquid in the peritoneum and the Hb remains stable
• The problems at that time: –baby-blues–abdominal pain and
• problems finding the good words
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Cardiologic work-up
The search of a PFO is negative with TEE and a new coronarography
The differential diagnosis is another shunt or histological misdiagnosis
Anaesthesthesiologic management of a parturient with a known antiphospholipid syndrome 15
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Further investigations
• Because there are no therapeutic consequences in the absence of a PFO there are no further cardiologic investigations
• On day 5 appearance of psychomotor restlesness and on the following night one episode of hallucination
• The neurologist demands an MRI
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Day 5-6: MRI
• Multiple acute cerebral ischemic lesions
• Subacute and old corticallesions
Anaesthesthesiologic management of a parturient with a known antiphospholipid syndrome 17
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Day 11• New cerebral episode with a transitory motor
hemisyndrome on her right side and retrosternal pain–The CT-Scan shows no haemorrhage–The retrosternal pain is a new episode of cardiac ischemia
while being fully anticoagulated
• The treatment consists of maintenance of full anticoagulation and conservative support
• She leaves hospital on day 21 with Marcoumar and low-dose Aspirin
Anaesthesthesiologic management of a parturient with a known antiphospholipid syndrome 18
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Antiphospholipid syndrome
What did we do wrong?
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Antiphospholipid syndrome
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Clinical manifestationsRed flag symptoms
• Unexplained DVT or PE<50y• Stroke <50y• TIA<50y• Thrombosis at unusual site• Unexplained foetal loss >10 weeks• Severe or early pre-eclampsia• Severe intrauterine growth restriction• Pre-eclampsia with severe tc-penia• Cardiac valve disease
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Antiphospholipid syndrome, foetal effects
• Antiphospholipid antibodies can directly bind to trophoblast cells and cause:
–Direct cellular dammage–Defective invasiveness of trophoblastic cells–Activation of complement at the foetomaternal interface
• This results in an impared foetomaternal interface:– Early miscarriage– Pre-eclampsia– Placental infarction with
» Intrauterine growth restriction» Intrauterine foetal death
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Definition
• Systemic autoimmune disorder–Arterial and venous thromboses–Adverse outcome in pregnancy (mother and foetus)–Raised titres of antiphospholipid antibodies
• Primary antiphospholipid syndrome (APS) 50% of patients• Secondary syndrome associated with systemic lupus
erythematousus (20-35% of SLE patients develop antiphospholipid syndrome)
• Appears mainly in young women of fertile age
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Asherton’s syndrome• The catastrophic APS
–It consists of preciptous multisystem organ thrombosis and failure, typically
• Brain• Kidneys• Lungs• Skin
–Mortality is 50%
–It is triggered by pregnancy in 4% of cases
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Pathophysiology I
• The trigger for thrombosis may be pregnancy, infection and local endothelial dammage
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Pathophysiology II
• Antiphospholipid antibodies form a heterogenous group of antibodies directed at plasma proteins that bind to phospholipids
• In a process mediated by β2-glycoprotein I the antibodies bind to
–platelets–entothelial cells– activates complement,
finally resulting in activation of tissue factor, endothelial dammage and finally thrombosis
• In vivo: they are associated with recurrent thrombosis, • in vitro: they increase phospholipid dependent clotting
times, hence the misnomer lupus anticoagulant
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Pathophysiology III
• Interaction of antiphospholipids with–Prothrombin–Factor X–Protein C and–Plasmin
Which may impede fibrinolysis
• Interaction with annexin A5, a natural anticoagulant, may favor placental thrombosis and fetal loss
• Antiphospholipid antibody binding reduces secretion of human chorionic gonadotrophin (hCG).
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Universitätsklinik für Anästhesiologie und Schmerztherapie
Diagnostic criteria• The syndrome is present if at least one clinical and one
laboratory criteria is met:– Vascular thrombosis:
• One or more episodes of arterial, venous or small vessel thrombosis
– Morbidity in pregnancy:• One or more unexplained death of a morphologically normal
foetus beyond the 10th week of gestation– Laboratory
• Lupus anticoagulant on two occasions present• Medium or high titre of IgG or IgM anticardiolipin antibody on
two occasions• Medium or high titre of IgG or IgM anti-β2 glycoprotein I
antibody in two occasions
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Therapeutic management
• With proper management more than 70% of pregnant APS women will deliver a viable infant
• Pregnancy should be discouraged in all women with pulmonary hypertension or a recent thromotic event
• Close prenatal care with close observation for• Maternal hypertension• Proteinuria and other signs of pre-eclampsia• Close foetal monitoring to assess fetal growth and placental function
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Therapeutic management
Treatment of choice:• Preconceptional low dose aspirin• Heparin is usually started in the early first trimester
Evaluated:• Glucocorticoids offer no benefit• Intravenous immunoglobulins are associated with an
increased risk of pregnancy loss or premature birth
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Medical management
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Future therapies
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Summary
• Without antithrombotic treatment during pregnancy the symptomatic APS syndrome is a sword of damocles to mother and foetus
• During labour anticoagulation becomes a challenge to the obstetrician and the anaesthesiologist
• APS syndrome is related to all major causes of maternal deaths
–Hypertensive disorders of pregnancy–Embolic disorders and –Hemorrhage (because of anticoagulation)
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Thank you!
Questions?
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Universitätsklinik für Anästhesiologie und Schmerztherapie
Anaesthesthesiologic management of a parturient with a known antiphospholipid syndrome 35
Universitätsklinik für Anästhesiologie und Schmerztherapie
Anaesthesthesiologic management of a parturient with a known antiphospholipid syndrome 36
Universitätsklinik für Anästhesiologie und Schmerztherapie
Anaesthesthesiologic management of a parturient with a known antiphospholipid syndrome 37
Universitätsklinik für Anästhesiologie und Schmerztherapie
Anaesthesthesiologic management of a parturient with a known antiphospholipid syndrome 38
Universitätsklinik für Anästhesiologie und Schmerztherapie
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Universitätsklinik für Anästhesiologie und Schmerztherapie
Antiphospholipid syndrome, definition
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Antiphospholipid syndrome: Definition & Epidemiology
• The antiphospholipid syndrome is a prothrombotic disorder that results in both arterial and venous thrombosis and is characterized by the presence of two autoantibodies, lupus anticoagulant and anticardiolipin antibody.
• The prevalence of lupus anticoagulant and anticardiolipin antibody among patients with SLE are 34% and 44%, respectively
• The overall prevalence of antiphospholipid syndrome is unclear. In 2007, Hughes predicted that the prevalence of antiphospholipid syndrome will exceed that of SLE.
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