a 38-year - old male patient was referred to asan medical center due to severe shock attributable to contained aortic rupture around the aortic root .
the patient had been treated with systemic corticosteroids after being diagnosed with behcet s disease 14 years previously .
the patient had previously undergone aortic valve surgery three times in another hospital for the management of aortitis and consequent aortic regurgitation associated with underlying behcet s disease .
the first two mechanical aortic valve replacement ( avr ) procedures had been performed 14 years previously , followed by a ross procedure two years after the second operation .
when the patient presented to our emergency department , he was in severe shock ( systolic blood pressure , 65 mmhg ) despite maximal intravenous inotropic support ( epinephrine and norepinephrine ) , and exhibited a drowsy mental state .
on enhanced computed tomography , a contained aortic rupture was found around the aortic root and the ascending aorta , abutting the sternum .
1 ) . an emergency operation was planned . a transesophageal echocardiography performed in the operating room showed mild aortic regurgitation ( ar ) and severely depressed cardiac function with a left ventricular ( lv ) ejection fraction of 17% despite full inotropic support .
massive hemorrhage during the reopening of the sternum and consequent catastrophic events were anticipated ; therefore , deep hypothermic circulatory arrest ( dhca ) was implemented before opening the sternum , using femoral cardiopulmonary bypass ( cpb ) with direct trans - apical lv venting through a left - side mini - thoracotomy ( fig .
once the nasopharyngeal temperature cooled to 18c , sternal opening was attempted using an oscillating saw .
as expected , a massive hemorrhage occurred from the ruptured aorta while the hematoma was being removed after sternal opening , at which point the cpb was stopped .
distal ascending aorta clamping was achieved securely within one minute of dhca , and cpb was restarted .
two discrete rupture sites were found in the aorta : one at the distal anastomosis site of the previous ross procedure and the other at the right coronary button site ( fig .
3 ) . after complete adhesiolysis of the mediastinum and the removal of the previously implanted pulmonic autograft ,
several tiny pseudoaneurysms were identified at the level of the aorto - ventricular junction , and further resection of this area was therefore conducted .
dhca was then reimplemented ( 15 minutes ) in order to conduct hemiarch replacement using a 28-mm hemashield vascular graft ( boston scientific , boston , ma , usa ) , which was followed by the reimplementation of cpb and systemic rewarming . during this period ,
complete root replacement was carried out , using a 27-mm composite mechanical valved conduit ( st .
, st paul , mn , usa ) . the circulatory arrest and cardiac ischemic times were 16 minutes and 188 minutes , respectively .
the patient failed to be weaned from cpb due to profound lv dysfunction , therefore , cpb was substituted with venous - arterial extracorporeal membrane oxygenation ( ecmo ) .
massive hemorrhage from the surgical sites continued despite meticulous hemostatic maneuvers and massive blood product transfusions .
the patient was transferred to the intensive care unit ( icu ) with an open sternum .
the transfusion of large quantities of blood products for two days resulted in adequate hemostasis .
follow - up echocardiography showed a significant recovery of lv function at this point , and the patient was successfully weaned off ecmo after four days of ecmo support , after which a delayed sternal closure was performed .
the follow - up computed tomography images and echocardiogram were unremarkable in that neither paravalvular dehiscence nor leakage on the anastomosis site was observed .
lv function was found to be normal , with an ejection fraction of 56% without any inotropic support .
the patient was discharged on postoperative day 31 without any neurologic sequelae or residual complications . on the echocardiographic follow - up
performed six months after the surgery , cardiac function was completely normal , with adequate function of the mechanical valve and no pathologic findings around the aortic root and ascending aorta .
the patient has undergone 14 months of follow - up while prescribed an oral corticosteroid medication , and has exhibited no cardiovascular complications .
cardiovascular involvement in behcet s disease is a rare but life - threatening condition ; in particular , involvement of the aortic root is known to be the leading cause of death in patients with behcet s disease .
surgical treatment of the aortic valve is required in these conditions , however , conventional avr is frequently complicated by prosthetic valve detachment due to the fragility of the aortic tissue and recurrent inflammation .
various surgical methods have been suggested to improve the outcomes of the surgical treatment of aortic root diseases associated with behcet s disease [ 46 ] .
since observations have revealed that the inflammation in behcet s disease primarily involves the aorto - ventricular junction of the aortic root , the complete resection of the inflammatory tissue in this area has been considered important for preventing the recurrence of the disease . with this in mind , aortic root replacement ( arr ) is considered an ideal surgical option for ar attributable to behcet s disease .
the largest series of aortic valve operations in patients with behcet s disease presented 19 patients who underwent 46 aortic valve procedures and demonstrated an 80% reoperation rate after avr alone due to valve dehiscence , whereas excellent results were observed with arr .
the authors suggested that arr should be performed as the initial surgical treatment of ar in patients with behcet s disease .
ando et al .   also reported excellent results of arr procedures in eight patients with behcet s disease ; only one patient died of arrhythmia , while the other seven patients survived through a long follow - up period .
of those seven patients , only one patient required reoperation due to valve detachment over the course of a mean follow - up period of 74 months . in agreement with these previous studies ,
we believe that ar attributable to behcet s disease should be treated with arr as the initial surgical procedure in order to prevent prosthetic valve detachment . in the present case ,
ar attributable to behcet s disease was initially treated with conventional avr that was followed by a repeated avr procedure and a ross procedure due to recurrent prosthetic valve detachment .
the patient ultimately presented with contained aortic rupture with pseudoaneurysm formation around the aortic root and ascending aorta twelve years after the third surgery .
since a massive hemorrhage and consequent cardiac arrest were anticipated during the opening of the sternum , we decided to induce dhca with transapical lv venting before opening the sternum . through a left - side mini - thoracotomy , it was possible to achieve lv venting without encountering severe mediastinum adhesion or fatal hemorrhage from the ruptured aorta .
this strategy enabled the prompt identification of the ruptured location and the complete resection of the diseased aortic tissue .
since this patient had been suffering from cardiac insufficiency attributable to preoperative shock , preventing lv distention injury during dhca was believed to be crucial for minimizing further myocardial damage associated with the surgical procedures .
the intensive transfusion of blood products , including platelet concentrate and fresh frozen plasma , was performed after transferring the patient to the icu .
serious mediastinal bleeding and a coexisting low cardiac output state requiring ecmo support gradually improved during postoperative icu care after the correction of coagulopathy .
we believe that an optimal surgical strategy and dedicated intensive care allowed the patient to recover from a desperate condition . in conclusion , we report the case of a patient with behcet s disease presenting with a contained aortic rupture in the previous surgical sites of the aortic root , who was treated with a sophisticated surgical approach and postoperative care .
the long - term outcomes associated with this procedure need to be addressed in further studies .
 cardiovascular involvement in cases of behcet s disease is a rare but life - threatening condition , and prosthetic valve detachment is a frequent and serious complication attributable to behcet s disease following the surgical repair of aortic regurgitation . 
 we report the case of a patient with behcet s disease presenting with contained aortic rupture around the aortic root . 
 the patient had previously undergone aortic valve surgery three times due to recurrent prosthetic valve detachment . 
 an emergency operation was performed , consisting of aortic root replacement ( arr ) using a composite valved conduit and the replacement of the hemiarch . 
 arr may be an appropriate surgical option for patients with behcet s disease in order to prevent recurrence of the disease . 