Year : 2011 | Volume
: 14 | Issue : 3 | Page : 235--236
Double valve replacement in a patient with antiphospholipid antibody syndrome
Rajinder S Rawat, Yatin Mehta, Pravin Saxena, Rajiv Juneja, Anil Bhan
Medanta Institute of Critical Care and Anesthesia, Medanta the Medicity, Gurgaon, Haryana, India
Rajinder S Rawat
Consultant of Cardiac Anesthesiology, K-303, Sarita Vihar, New Delhi - 110 076
|How to cite this article:|
Rawat RS, Mehta Y, Saxena P, Juneja R, Bhan A. Double valve replacement in a patient with antiphospholipid antibody syndrome.Ann Card Anaesth 2011;14:235-236
|How to cite this URL:|
Rawat RS, Mehta Y, Saxena P, Juneja R, Bhan A. Double valve replacement in a patient with antiphospholipid antibody syndrome. Ann Card Anaesth [serial online] 2011 [cited 2020 Apr 9 ];14:235-236
Available from: http://www.annals.in/text.asp?2011/14/3/235/84035
Antiphospholipid antibody syndrome (APLS) is an autoimmune disorder characterized by recurrent systemic arterial and venous thrombosis, recurrent abortion, thrombocytopenia and neurological disorders.  Despite aggressive anticoagulation and lack of significant preoperative co-morbidities, patients with APLS undergoing cardiothoracic surgery appear to have high rate of thromboembolic events and perioperative mortality.  Report of valve replacement in APLS patients seems to be rare, and it`s management during perioperative period is challenging in theses patient.
A 27 year old female was admitted with complaint of breathlessness on exertion for two months. She was a known to suffer from APLS and rheumatic valvular heart disease. A detailed history revealed two spontaneous missed abortions 5 and 7 years ago, following which she delivered a baby by emergency caesarean section one year ago.Echocardiography performed on her confirmed the requirement of double valve replacement. Antinuclear antibody (ANA) was negative, but cardiolipin antibody IgG (20.12 IgG phospholipid unit (GPL) U/ml) and IgM (24.68 IgM phospholipid unit (MPL) U/ml) were raised. Serum Beta-2 microglobulin (4996 micro gram/ml) was also high.
The standard cardiac anesthesia protocol using opioid based induction and maintenance was followed. Monitoring consisted of measuring direct arterial blood pressure, central venous pressure, pulmonary artery pressure, cardiac output (using thermodilution pulmonary artery catheter), transesophageal echocardiography, urine output and core temperature. Patient was mechanically ventilated with tidal volume of 8 ml/kg with respiratory rate adjusted to obtain end expiratory carbon dioxide tension of 35-40 mm Hg. Anesthesia was maintained with isoflurane (0.6--1.0%) in oxygen-air mixture with inspired oxygen fraction of 0.5, and intermittent doses of midazolam, fentanyl and vecuronium.
Porcine heparin 400 units/kg was used to keep activated clotting time (ACT) above 500 seconds throughout the surgery. Conventional cardiopulmonary bypass roller pumps (Sarns HLM 8000, USA) and a membrane oxygenator (Capiox sx 18, Michigan, USA) were used. perfusion pressure was increased if needed using nor adrenaline bolus. The patient was cooled to 30°C and ante grade cardioplegic solution was used into aortic root after cross clamping. The lowest hematocrit accepted was 18% and one unit of packed red blood cells was used. We completed double valve replacement using 31 mm Perimount, prosthetic mitral tissue valve and 23 mm Perimount, prosthetic aortic tissue valve (Edwards Lifesciences, USA). Patient was weaned off from bypass with epinephrine 0.03 μg/kg/min infusion. After termination of bypass, blood remaining in the CPB circuit was retransfused. Half dose of protamine means 0.5 mg per 100 units of Heparin used. Commonly 1 mg per 100 units of heparin is recommended to reverse heparin effects. The ACT of 178 seconds was accepted as there was minimal chest tube drainage. Patient was shifted to intensive care unit. After confirming a decreasing trend in chest tube drainage, low dose porcine heparin infusion (500 units /hr) was started to patient within 4 hours. Trachea was extubated after 10 hour of admission to the ICU, following which aspirin 75 mg per oral once a day was started and ceased after commencing oral warfarin therapy. The post-operative period was uneventful. The patient was discharged on postoperative day 9.
APLS is called primary when symptoms appear in absence of other related disease and secondary when it occurs in conjunction with other autoimmune disease i.e. systemic lupus erythematosus. The APLS diagnosis is established by the Sapporo criteria,  which are both clinical and laboratory based. Clinical criteria consist of vascular thrombosis or pregnancy morbidity and the laboratory criteria are positive tests on two or more occasions at least 12 weeks apart of (1) Lupus anticoagulant (LAC) (2) high titre (> 40 GPL or MPL ) anticardiolipin, (3) anti-beta-2 glycoprotein antibodies. There are only few reported cases in literature which mentions cardiac surgery in APLS patient and these reports high incidence of thromboembolic phenomenon.  Berkun et al published 10 cases of valve replacement in a period of 13 years and reported considerably higher incidence of morbidity and mortality in APLS patients.  Considering thrombophilic tendency in APLS patients, strict management of anticoagulant therapy is required. A withdrawal of anticoagulant therapy is reported to be precipitating factors for the occurrence of catastrophic events.  Therefore the authors continued aspirin till two days prior to surgery. There is no consensus in the literature as to the optimal method for assuring adequate perioperative anticoagulation undergoing cardiac surgery in patients with APLS. Sheikh et al elected to empirically double the ACT to more than 999 seconds.  Moreover obtaining factor Xa or plasma heparin concentrations were considered impractical. ACT may be markedly affected in these patients so it may not be suitable as a guide to the heparinization although anecdotal successes have been reported with empirical heparin management during CPB.  We administered heparin 400 units/kg in our case to achieve ACT above 500 seconds. A restrictive strategy of protamine use is advised in cases with APS.  We gave half dose of protamine means 0.5 mg per 100 units of Heparin used. Commonly we give 1 mg per 100 units of heparin to reverse heparin effects. There was abnormal bleeding in our case. we started low dose heparin infusion (500 unit /hr) within 4 hours of arrival in ICU to prevent thrombosis. After extubation, we started early aspirin 75 mg orally in order to prevent antiplatelet aggregation and thus thromboembolic event. Long term treatment with warfarin has been shown to be more effective than antiplatelet therapy for the secondary prevention of thromboembolic events in patients with APLS. Heparin was replaced with warfarin to maintain International Normalized Ratio 2.0-3.0 in our case.
In case of catastrophic event venous thromboembolism a combination of anticoagulants and steroids plus either plasmapheresis or intravenous immunoglobulin has been reported to lead to acceptable recovery. 
Considering thrombophilic tendency in APLS patients, strict management of anticoagulant therapy, reduced protamine dose, early institution of anticoagulant, and early administration of antiplatelets, use of tissue valve and long term use of warfarin seems to be a good choice in patient with APLS, in order to prevent thromboembolic and catastrophic events.
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