bicuspid aortic valve ( bav ) is the most frequent congenital cardiac lesion and is found in 0.9 to 2.0% of the general population ( 1 ) . the congenitally bicuspid aortic valve may have normal function throughout life , or may develop stenosis or regurgitation with or without infection ( 2 ) . case reports of spontaneous aortic thrombosis in the neonate or adult in association with a bicuspid aortic valve are rare , but reported , most of uncertain origin ( 3 ) . the commonest clinical manifestation of antiphospholipid syndrome is with thrombosis . thrombosis in the venous circulation is the most common , but may also occur in the arterial and microvascular compartments . a 51 year old gentleman was referred from coronary care to our unit as an emergency with thrombosis of the aortic valve and ascending aorta . he had been unwell for a two week period prior to admission complaining of generalised malaise and an episode of temporary visual loss in his right eye . his past medical history revealed a mixed picture of arterial and venous thrombosis including a right femoropopliteal bypass for thrombus at the age of 15 , a deep vein thrombosis ( dvt ) in his left leg at the age of 49 and a further embolic event in his left hand . he had been commenced on warfarin at the time of his dvt however this had been stopped 8 weeks before his admission so that he could be investigated at a local haematology clinic . on transfer he was haemodynamically stable in sinus rhythm and clinical examination was essentially normal except for the presence of a soft systolic ejection murmur . transthoracic echocardiography undertaken in coronary care revealed a mobile thrombus , 4.4 x 1.8 cm , attached to the right coronary cusp of the aortic valve , which appeared thickened and calcified . he was taken to theatre as an emergency . a pre - operative transoesophageal echocardiogram ( toe ) confirmed the presence of thrombus on the aortic valve extending into the ascending aorta ( fig 1 ) . after median sternotomy and initiation of cardiopulmonary bypass the ascending aorta was clamped at the level of the innominate artery . a calcified bicuspid aortic valve was excised and replaced with a 27-mm ats open pivot heart valve . no organisms were seen on gram staining of the thrombus however following advice from the microbiological team empirical therapy with vancomycin and gentamicin was initiated . he made an uneventful postoperative recovery and was commenced on intravenous heparin after surgery until he was adequately anticoagulated with warfarin and aspirin . transoesophageal echocardiogram depicting thrombus in the ascending aorta he was subsequently readmitted with pyrexia , general malaise , lethargy , anorexia , dizziness and flushing . repeated culture results were negative and echocardiography of the prosthetic valve showed good function and no evidence of vegetations . despite this it was decided to treat mr x as a culture negative endocarditis , with a six week course of intravenous vancomycin and gentamicin . since discharge he has remained well . haematological investigations taken prior to admission , whilst he was off warfarin , revealed a normal activated partial thromboplastin time and antithrombin concentration . the cardiolipin antibody ( igg and igm ) were within normal range , however lupus anticoagulant antibodies were positive and considered significant particularly in view of the correlation with his clinical picture . spontaneous thrombosis of the ascending aorta is a rare and usually fatal event . in most cases our patient was found to have both a congenital bicuspid aortic valve and elevated lupus anticoagulant antibodies . antiphospholipid syndrome is diagnosed by the presence of antiphospholipid antibody , typically identification of lupus anticoagulant singly or in combination with anticardiolipin antibody ( 4 ) . in this case thrombosis of the bicuspid aortic valve occurred when his warfarin was discontinued for haematological investigations despite 3 previous thrombo - embolic events . lupus anticoagulant comprises a subgroup of antiphospholipid antibodies that disrupt the actions of the prothrombinase complex ( factors xa , va and prothrombin in combination on phospholipid membranes ) . antiphospholipid antibodies behave as acquired inhibitors of anticoagulation , prolonging phospholipid - dependant coagulation tests ( 5 ) . the activated partial thromboplastin time ( aptt ) this report illustrates the significance of testing for prothrombotic disorders in at risk individuals , to prevent similar scenarios perhaps with the early institution of anticoagulant therapy .
we present the case of a 51-year - old man who was admitted as an emergency with spontaneous thrombosis of the aortic valve and ascending aorta . at operation 
 he was found to have a congenitally bicuspid aortic valve and subsequent investigation revealed primary antiphospholipid syndrome . 
 he underwent successful removal of the thrombus combined with mechanical replacement of the aortic valve .