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      Anesthetic management of antiphospholipid syndrome patients who underwent cardiac surgery: three cases report

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          Abstract

          Antiphospholipid syndrome (APS) is a rare disease in which patients display prolonged coagulation test results in vitro, but usually develop thrombotic symptoms in vivo. Patients with APS are at increased risk of valvular heart disease or coronary vascular disease, conditions that often necessitate cardiac surgery via bypass. The management of anticoagulation during cardiopulmonary bypass (CPB) is particularly challenging in these patients because of the unique features of APS. Patients with APS are constantly at risk of arterial and venous thrombotic events. Therefore it is very important to maintain proper anticoagulation perioperatively, especially during CPB. In this paper, we present three successful cases of APS patients who underwent cardiac surgery with CPB.

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          Antiphospholipid syndrome.

          Mark Crowther, Guillermo Ruiz-Irastorza, Ware Branch … (2010)
          The antiphospholipid syndrome causes venous, arterial, and small-vessel thrombosis; pregnancy loss; and preterm delivery for patients with severe pre-eclampsia or placental insufficiency. Other clinical manifestations are cardiac valvular disease, renal thrombotic microangiopathy, thrombocytopenia, haemolytic anaemia, and cognitive impairment. Antiphospholipid antibodies promote activation of endothelial cells, monocytes, and platelets; and overproduction of tissue factor and thromboxane A2. Complement activation might have a central pathogenetic role. Of the different antiphospholipid antibodies, lupus anticoagulant is the strongest predictor of features related to antiphospholipid syndrome. Therapy of thrombosis is based on long-term oral anticoagulation and patients with arterial events should be treated aggressively. Primary thromboprophylaxis is recommended in patients with systemic lupus erythematosus and probably in purely obstetric antiphospholipid syndrome. Obstetric care is based on combined medical-obstetric high-risk management and treatment with aspirin and heparin. Hydroxychloroquine is a potential additional treatment for this syndrome. Possible future therapies for non-pregnant patients with antiphospholipid syndrome are statins, rituximab, and new anticoagulant drugs. Copyright © 2010 Elsevier Ltd. All rights reserved.
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            Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of a series of 280 patients from the "CAPS Registry".

            Ricard Cervera, Silvia Bucciarelli, Miguel Plasín … (2009)
            To describe the clinical and laboratory features, as well as the precipitating factors, treatment and outcome of patients with catastrophic antiphospholipid syndrome (APS). We analyzed the 280 patients included until September 2008 in the website based international registry of patients with catastrophic APS ("CAPS Registry") (http://www.med.ub.es/MIMMUN/FORUM/CAPS.HTM). The entire series includes 201 (72%) female and 79 (28%) male patients with a mean age of 37 +/- 14 years (range, 11-60 years). A total of 129 (46%) patients suffered from primary APS, 112 (40%) from systemic lupus erythematosus, 14 (5%) from lupus-like disease, and 25 (9%) from other autoimmune diseases. The catastrophic episode was the first manifestation of the APS in 129 (46%) patients. A precipitating factor was reported in 53% of the patients. The first clinical manifestation at the time of the catastrophic episode was a pulmonary complication in 24% of the cases, a neurologic feature in 18% and a renal feature in 18%. During the catastrophic episode, intraabdominal involvement was identified in the majority of patients, mainly consisting of renal (71%), hepatic (33%), gastrointestinal (25%), splenic (19%), adrenal (13%), and pancreatic (8%) manifestations. 123 (44%) patients died at the time of the catastrophic APS event but the higher recovery rate was achieved by the combination of anticoagulants plus corticosteroids plus plasma exchange (PE) and/or intravenous immunoglobulins (IVIG) (69% versus 54%). The catastrophic APS is an uncommon but potentially life-threatening condition that needs high clinical awareness. The therapeutical connotation is that this may be corrected with the combination of anticoagulation plus steroids plus attempts at achieving a prompt reduction of antiphospholipid antibody titer (i.e. PE and/or IVIG).
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              Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines.

              J Piette, Y. Shoenfeld, Peter Paul De Deyn … (2002)
              The term 'catastrophic' antiphospholipid syndrome (APS) is used to define an accelerated form of APS resulting in multiorgan failure. Although catastrophic APS patients represent less than 1% of all patients with APS, they are usually in a life-threatening medical situation that requires high clinical awareness. The careful and open discussion of several proposals by all participants in the presymposium workshop on APS consensus, held in Taormina on occasion of the 10th International Congress on aPL and chaired by Munther A Khamashta and Yehuda Shoenfeld (29 September 2002), has allowed the acceptation of a preliminary set of classification criteria. On the other hand, the optimal management of catastrophic APS must have three clear aims: to treat any precipitating factors (prompt use of antibiotics if infection is suspected, amputation for any necrotic organ, high awareness in patients with APS who undergo an operation or an invasive procedure), to prevent and to treat the ongoing thrombotic events and to suppress the excessive cytokine 'storm'. Anticoagulation (usually intravenous heparin followed by oral anticoagulants), corticosteroids, plasma exchange, intravenous gammaglobulins and, if associated with lupus flare, cyclophosphamide, are the most commonly used treatments for catastrophic APS patients.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Korean J Anesthesiol
                Journal ID (iso-abbrev): Korean J Anesthesiol
                Journal ID (publisher-id): KJAE
                Title: Korean Journal of Anesthesiology
                Publisher: The Korean Society of Anesthesiologists
                ISSN (Print): 2005-6419
                ISSN (Electronic): 2005-7563
                Publication date (Print): February 2014
                Publication date (Electronic): 28 February 2014
                Volume: 66
                Issue: 2
                Pages: 164-168
                Affiliations
                Department of Anesthesiology and Pain Medicine, Seoul National University Hospital, Seoul, Korea.
                Author notes
                Corresponding author: Yunseok Jeon, M.D., Ph.D., Department of Anesthesiology and Pain Medicine, Seoul National University Hospital, 101, Daehak-ro, Jongno-gu, Seoul 110-744, Korea. Tel: 82-2-2072-2465, Fax: 82-2-747-5639, jeonyunseok@ 123456gmail.com
                Article
                DOI: 10.4097/kjae.2014.66.2.164
                PMC ID: 3948446
                SO-VID: 05f6a4ef-83a9-4d86-a3f0-96e1baa45780
                Copyright © Copyright © the Korean Society of Anesthesiologists, 2014
                License:

                This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/3.0/), which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 17 September 2012
                Date revision received : 03 January 2013
                Date accepted : 06 February 2013
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Anesthesiology & Pain management
                Keywords: antiphospholipid syndrome,cardiopulmonary bypass,cardiac surgery
                Data availability:
                ScienceOpen disciplines: Anesthesiology & Pain management
                Keywords: antiphospholipid syndrome, cardiopulmonary bypass, cardiac surgery

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