a 67 year old woman was hospitalized in the emergency room due to dysarthria started 4 hours before hospitalization .
the patient was diagnosed with systemic sclerosis in 2003 and was taking methylprednisolone 2 mg per day at our clinic 's rheumatic internal medicine department . according to physical examination findings ,
sensorium was normal where as dysarthria , facial palsy , and right deviant of tongue were observed .
blood pressure was 140/80 mmhg , pulse was 102 beats per minute , respiratory rate was 20 times per minute , body temperature was 36.9 , and there were no abnormalities in chest auscultation . according to blood test result , number of white blood cells was increased and platelet level was normal .
cardiomegaly was observed through simple chest x - ray and acute infarction was observed around the large sulcus and parietal lobe through mri ( fig .
ct scan of carotid artery and chest to find out the cause of cerebral infarction , lesion suspicious of aortic arch thrombus was observed . in enhanced image
was observed , no abnormal observations were found in regional wall motion and heart valves , and floating thrombus was found in ascending aorta ( fig .
2 ) . according to hematological test , anti - cardiolibin ab - igg level was 2.0 gpl u / ml ( normal : less than 9.9 ) , anti - cardiolibin ab - igm level was 1.0 mpl u / ml ( normal : less than 6.9 ) , lupis anticoagulant ( - ) , protein s antigen was 114% ( 60~150 ) , protein s activity was 93% ( 58.7~119.2 ) , antiphospholipid ab igg was 1.0 u / ml ( less than 9.9 ) , and antiphospholipid ab igm was 2.0 u / ml ( less than 9.9 ) showing normal values .
however , protein c activity was 167% ( 70~130 ) and protein c antigen was > 160% ( 72~160 ) showing increased levels . due to the facts that size of the thrombus was large , location was from ascending aorta to aortic arch , thrombus was highly mobile and cerebral embolism occurred ,
median sternotomy was done , arterial cannula was inserted through 8 mm vascular graft anastomosed in right axillary artery , venous cannula was inserted in superior and inferior vena cava , started extracorporeal circulation , and decreased central temperature to 22. cardiac arrest was induced by inserting retrograde cardioplegic solution in coronary sinus , right innominate artery and left common carotid artery were cross clamped , and selective antegrade cerebral perfusion through right axillary artery was done eventually leading to circulatory arrest .
incision was made in ascending aorta and 2.5 cm - long intraaortic thrombus was completely removed ( fig .
aortic incision was sutured , right innominate artery and left common carotid artery were unclamped resuming systemic circulation , and weaning from cardiopulmonary bypass was done without complications .
selective antegrade cerebral perfusion was done for 15 minutes and cardiopulmonary bypass was done for 128 minutes .
based on pathological test of the thrombus , there were no evidence of malignant neoplasm , and the patient recovered without complications and was discharged .
factors related with arterial thrombus are arteriosclerosis , arteriodissection , trauma , malignant tumor , and hemostatic disorder . in this case study ,
the patient had systemic sclerosis from several years ago and was taking methylprednisolone 2 mg per day .
there are no clearly known causes of systemic sclerosis , but natural death of cell caused by anti - endothelial cell antibody is suggested as a hypothesis .
this usually affects microvessel causing proliferation of endothelial cell , medial thinning , and thickening of basilar membrane .
eventual destruction of vessel causes angiodyskinesia , functional disorder of endothelial cell , thickening of vessel wall , activation of coagulation factor , reduction of fiber disassociation , and increase in attachment of platelets and lymphocytes . in cases with systemic sclerosis , thrombus in extremity is relatively common and floating thrombus inside the aorta is known to be rare .
thrombus mostly occurs in elder patients , is usually stationary forming atherosclerotic plaque , and often occurs in extremity .
patient of this case did not have past history that is known to cause thrombus such as artery detachment , trauma , or malignant tumor .
evidences of infection malignant tumor , arteriosclerosis , and inflammatory change were not found after postoperative pathological test of specimen .
the patient did not smoke , had no hypertension or diabetes , did not show high parameters in blood test , but only showed higher levels of protein c activity and protein c antigen than normal .
however , long - term use of steroid and vessel damage due to systemic sclerosis might be related to thrombus formation .
since occurrence in ascending aorta is rare , this case can be considered as meaningful .
transesophageal echocardiography is non - invasive and easy method used widely for diagnosing thrombus in heart or aorta .
there are several different methods in aortic thrombus treatment such as anticoagulant therapy , thrombolytic therapy , aspiration thrombectomy , and thrombectomy , but there are no evidence that which method is better . in medical treatment , thrombolytic agent and anticoagulant are used . after using anticoagulant with heparin for several days , size reduction of thrombus must be observed and heparin treatment must be maintained until the thrombus is completely gone . in general , when the size of thrombus is large and thrombus is mobile , surgical removal is preferred as primary treatment because of the risk factors such as systemic embolism , repeated embolism , and medical treatment failure . in this case , heparin was used for 2 days after cerebral infarction prior to surgery .
thrombectomy was executed since it was highly mobile , large in size , did not change in its size through follow - up ultrasonic test , and repetitive cerebral embolism was possible .
this report is written in order to inform a rare case where thrombus in ascending aorta of a patient with systemic sclerosis was surgically treated .
 aortic thrombi are important because it can cause the central and peripheral embolizations . 
 aortic thrombi can occur anywhere in the aorta but extremely rare in ascending aorta without atherosclerosis , aneurysm , cardiosurgical or traumatic state . 
 systemic sclerosis ( ssc ) is an autoimmune disorder of connective tissue and it can involve multisystem . 
 enhanced coagulation pathways , decreased fibrinolysis , and endothelial dysfunction probably contribute to vascular events in ssc . 
 we report a case of a highly mobile thrombus in the ascending aorta , presented as an acute embolic stroke in the patient with systemic sclerosis . 
 surgical removal was performed to prevent recurrent embolic events . 
CASE REPORT
DISCUSSION