Abstract Antiphospholipid syndrome is an autoimmune disorder with frequent clinical manifestation of arterial, venous or small vessel thromboembolic events and/or foetal morbidity. Hereby, we present a case report of a patient with a large arterial thrombus originating from the ostium of the right coronary artery accompanied by multiple systemic embolization and right ventricular failure. As a bridge-to-recovery strategy, an extracorporeal right ventricular assist device was successfully applied, representing an effective first choice option in selected patients with isolated acute right heart failure. Antiphospholipid syndrome , Right ventricular failure , Right ventricular assist device INTRODUCTION Antiphospholipid syndrome is a relatively rare autoimmune disorder, which is characterized by the presence of persistent antiphospholipid antibodies (anticardiolipin antibody, anti-beta-2 glycoprotein-I antibody and lupus anticoagulant) and frequent clinical manifestation of arterial, venous or small vessel thromboembolic events and/or foetal morbidity . CASE PRESENTATION Initial presentation Herein, we report a case of a 49-year-old woman without relevant medical history who was admitted to a countryside hospital with severe hypotension and presyncope. Numbness of the left feet started 7 days prior to admission. Blood pressure could not be measured on the left arm. ECG confirmed pathological Q waves, ST-segment elevations and negative T waves in inferior leads (Supplementary Material, Fig. S1). Troponin I level was highly elevated (27 299 ng/l). The chest computed tomography was not explicit to exclude acute aortic syndromes (Fig. 1A, arrows on hypodense structure), and therefore, the patient was referred to our centre with a potential Type A aortic dissection complicated by cardiogenic shock. After the transfer, urgent transoesophageal echocardiography revealed a large (29 × 12 mm), mobile thrombus located in the ascending aorta, which involved the ostium of the right coronary artery (Fig. 1B, arrow on the thrombus and arrowhead on the ostium, Video 1). Transoesophageal echocardiography suggested severely decreased right ventricular (RV) function along with preserved left ventricular function. Video 1 Transoesophageal echocardiography showing a large, mobile thrombus involving the ostium (arrowhead) of the right coronary artery. Video 1 Transoesophageal echocardiography showing a large, mobile thrombus involving the ostium (arrowhead) of the right coronary artery. Close Figure 1: View largeDownload slide Thrombosis of the ascending aorta. (A) Computed tomography, (B) transoesophageal echocardiography, (C) surgical view and (D) subsequent embolization. Figure 1: View largeDownload slide Thrombosis of the ascending aorta. (A) Computed tomography, (B) transoesophageal echocardiography, (C) surgical view and (D) subsequent embolization. Surgery and perioperative anticoagulation management Taking into consideration the patient’s critical condition and the cardiogenic shock, emergent cardiac surgery was performed, and the thrombus was extracted in toto leaving the aortic wall and the coronary ostia intact (Fig. 1C, arrow on the thrombus). Weaning from cardiopulmonary bypass (CPB) was unsuccessful due to persisting RV failure. As a bridge-to-recovery strategy, an extracorporeal RV assist device (RVAD; CentriMag, Thoratec, Pleasanton, CA, USA) was implanted. Postoperative angiography revealed thrombotic occlusion of the left axillary artery (Fig. 1D, arrowhead on occlusion, Video 2), the left and also the right superficial femoral arteries (Fig. 1D, arrows on occlusions); which were treated with surgical embolectomies. Video 2 Postoperative angiography showing the thrombotic occlusion of the left axillary artery (arrowhead). Video 2 Postoperative angiography showing the thrombotic occlusion of the left axillary artery (arrowhead). Close Perioperative anticoagulation was established with unfractionated heparin facilitated with optimized level of antithrombin-III (AT-III). The AT-III level was corrected with 1500 IU AT-III concentrate prior to cardiac surgery to achieve a level of 80% or greater. The required intraoperative dose of unfractionated heparin was 3.9 mg/kg, which resulted in the median activated clotting time of 508 s [interquartile range (IQR) 486–512 s] during the CPB phase. Postoperative anticoagulation was omitted for the first 12 h to minimize the bleeding risk. Afterwards, a continuous unfractionated heparin infusion was started targeting the activated partial thromboplastin time ratio between 2.0 and 2.5. Additionally, the AT-III level was measured every 48 hours until the RVAD explantation. The median activated partial thromboplastin time ratio was 2.44 (IQR 1.93–3.00), and the median AT-III was 106% (IQR 92–111%) in the period of the RVAD support. There was no newly developed thromboembolic complication during the treatment period. Outcome The early postoperative period was complicated with reperfusion-induced rhabdomyolysis and subsequent temporary renal failure. The initially adjusted RVAD support was maintained with a median flow of 3.9 l/min (IQR 3.6–4.0 l/min) for 4.5 days. In the next 10 days, the RVAD assistance was weaned from 3.9 l/min to 2.0 l/min. Regular echocardiographic follow-up confirmed the recovery of RV function. The RVAD was explanted on the 14th postoperative day after a successful weaning facilitated with full dose of levosimendan loading. The patient was discharged home in a good condition on the 34th day. Lupus anticoagulant test has been positive in the early postoperative period and 12 weeks later too, which confirmed the diagnosis of antiphospholipid syndrome. DISCUSSION Previous case reports in this topic demonstrated primarily the occurrence of intracardiac, native aortic valve, aortic and coronary artery thromboses [2, 3]. To the best of our knowledge, this is the first case report regarding ostial right coronary artery thrombosis and consecutive isolated RV failure. Despite the surgical extraction of the thrombus, RV dysfunction persisted that drove to introduce a temporary mechanical circulatory support. As the initial complaints started 7 days prior to hospital admission, the long-standing ischaemia of the RV myocardium was predictive for a longer period of RV recovery. Simultaneously, the left ventricular function was intact. Taking into account this clinical situation, to indicate a temporary RVAD instead of initial venoarterial extracorporeal membrane oxygenation (VA-ECMO) support was the optimal choice providing the necessary assistance for the RV and to prevent the patient from the possible risks of VA-ECMO support and a subsequent operation (i.e. VA-ECMO – RVAD upgrade). In this case, the patient’s critical condition determined the need for an aggressive treatment strategy. However, less invasive thrombus extraction performed using the AngioVac system (AngioDynamics, New York, USA) might also be an option in other circumstances when the thrombosis of the ascending aorta is not complicated with cardiogenic shock . In summary, we reported a rare complication of antiphospholipid syndrome presenting with acute thrombosis of the ascending aorta. The subsequent RV failure was successfully treated by RVAD, representing an effective option in selected patients with isolated acute right heart failure. SUPPLEMENTARY MATERIAL Supplementary material is available at EJCTS online. ACKNOWLEDGEMENTS The authors are grateful to Dr Bálint Károly Lakatos for his substantial contribution to this case report. Conflict of interest: none declared. REFERENCES 1 Rodriguez-Pinto I , Moitinho M , Santacreu I , Shoenfeld Y , Erkan D , Espinosa G et al. Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of 500 patients from the International CAPS Registry . Autoimmun Rev 2016 ; 15 : 1120 – 4 . Google Scholar CrossRef Search ADS PubMed 2 Biceroglu S , Ildizli Demirbas M , Karaca M , Yalcin M , Yilmaz H. Acute thrombotic occlusion of right coronary and left circumflex coronary arteries in a patient with antiphospholipid syndrome: successful stent implantation . Case Rep Med 2010 ; 2010 : 198594. Google Scholar CrossRef Search ADS PubMed 3 Salzberg SP , Nemirovsky D , Goldman ME , Adams DH. Aortic valve vegetation without endocarditis . Ann Thorac Surg 2009 ; 88 : 267 – 9 . Google Scholar CrossRef Search ADS PubMed 4 Brickwedel J , Kölbel T , Rohlffs F , Detter C , Reichenspurner H , Tsilimparis N. Thrombus aspiration from the ascending aorta using the AngioVac device: a case report . Thorac Cardiovasc Surg 2017 ; 65 : S1 – 110 . © The Author(s) 2018. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/about_us/legal/notices)
European Journal of Cardio-Thoracic Surgery – Oxford University Press
Published: Jun 3, 2018
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