The antiphospholipid syndrome and heart valve surgery

Abstract

The antiphospholipid syndrome (APLS) is a complex autoimmune disease often connected to systemic lupus erythematodes. Main features are thromboses, fetal loss and specific antibodies. The involved autoantibodies are directed against plasma proteins such as beta2glycoprotein1 (beta2GPI) or prothrombin which depend on negatively charged phospholipids. Direct antibodies against phospholipids are of no importance for APLS. Clotting tests such as activated partial thromboplastin time or diluted Russell's viper venom test (dRVVT) can show a prolonged time for coagulation despite a prothrombotic state in vivo but the investigator needs awareness about disturbing phospholipid sources and other influential factors. Enzyme linked immuno sorbent assay tests for antibodies against cardiolipin, beta2GPI and prothrombin are valuable solid phase tests with different specificity. Antiphospholipid, anticardiolipin or lupus anticoagulant are misnomers in connection with APLS. They are preserved as a reminiscence of the pioneering work on the way to the still not exactly revealed basics of APLS. Valve operations in APLS patients seem to be rare; a meta-analysis of 57 cases proves that the perioperative management is, at the moment, an empirical approach with high morbidity and mortality in these young patients.

Antiphospholipid syndrome and the heart: A case series and literature review

“…Left sided valves are more frequently affected, probably due to higher shear from jet effect and turbulence, with a predilection for mitral with respect to the aortic valve [24][25][26] especially in secondary APS [27]. Tricuspid valve seems to be seldom affected [12].…”

“…The choice of the surgical procedure (repair vs. substitution), and the type of the valve (mechanical valve vs. a bioprosthesis) is extremely important. Despite the theoretical tendency towards a better outcome with mechanical valves, the use of tissue valves should not be side-lined [27,42]. Due to the hypercoagulable state in APS, mechanical valves might develop thrombosis despite anticoagulation, while tissue valves might have a minimized risk of such complications.…”

“…Heart valve surgery in patients with APS is associated with considerable morbidity and mortality [27,42]. The choice of the surgical procedure (repair vs. substitution), and the type of the valve (mechanical valve vs. a bioprosthesis) is extremely important.…”

Update on Antiphospholipid Antibody Syndrome Management

“…In the case of bleeding due to excessive anticoagulation, protamine sulfate should be continuously administered in small intravenous doses, e.g. 50 mg/h (Gorki et al, 2008), until the bleeding is reduced. Antifibrinolytic drugs, aprotinin or plasmin inhibitors, which are commonly used to stop the bleeding, are not usually given because of the potential risk of postoperative thrombosis.…”

Clinical Update. Clinical Presentations of Pulmonary Embolism in the Emergency Department

“…Literature data shows that the acquired hypercoagulability status emphasized by a thrombotic event can be caused by APLS with an incidence of up to 2% in the general population and can consist in a hypercoagulability state with high implications in the cardiovascular system, with a 5-year mortality rate of 5.3%. [35][36][37][38][39] These patients are at high risk of developing arterial or venous thrombosis, intracardiac thrombus, pulmonary hypertension, coronary artery diseases, valvular disease, and stroke, and also can determine fetal loss. 40,41 The association between atrial fibrillation and PE is still …”