Background/Purpose:  Aortic regurgitation (AR) in Behcet disease is a rare but very fatal condition. Many patients require a second or third operation after simple aortic valve replacement (AVR) as a result of prosthetic valve dehiscence or destruction due to flare. Recently, several case series have reported favorable outcomes after aortic root replacement (ARR). We evaluated if the surgical outcomes of AR in Behcet disease was dependent on surgical methods or materials. Here, we compared the long-term outcome of AR in Behcet disease who had undergone surgical treatment.

Methods:  We have identified 33 patients who had been surgically treated for AR associated with Behcet disease from January 1996 through December 2013. A total of 23 patients fulfilled the international criteria for Behcet disease. AVR was performed in 9 patients and ARR in 14 patients. Bioprosthesis ARR was performed in 8 patients and composite graft ARR in 6 patients. The duration of follow-up was 10.7 years (median; IQR = 8.9-13.5) for the bioprosthesis ARR group and 6.4 years (median; IQR = 4.8-7.7) for the composite graft ARR group. The definition of the event was as follows; aortic valve/graft problem, infective endocarditis, cerebral infarction caused by thromboembolism or re-operation of aortic valve. We compared the events after first operation between the bioprosthesis ARR and composite graft ARR groups.

Results:  In the 9 patients with AVR, events occurred in 6 patients (2.3 years after operation [median; IQR = 0.3-10.3]) and 11 cases required re-operation. In the 14 patients with ARR, events occurred in 7 patients (4.7 years after operation [median; IQR = 1.6-6.9] years after operation) and 6 cases required re-operation. However, steroid was prescribed for significantly more patients and with higher dosage in ARR group than those of AVR group.

In the 8 patients with bioprosthesis ARR, events occurred in 6 patients (3.0 years after operatin [median; IQR = 1.5-5.4)) and re-operation was performed in 6 cases. Interestingly, in the 6 patients with composite graft ARR, events occurred in 1 patient (6.2 years after operation [median; IQR = 4.8-7.5]), there was no case requiring re-operation. Kaplan-Meier curves displayed higher event free rate in composite graft ARR group compared to bioprosthesis ARR group (Figure 1). Overall mortality was 14.3% (2 of 8 patients in bioprosthesis ARR group, 0 of 6 patients in composite graft ARR group). The administration of steroid and immunosuppressants after operation were not significantly different between both groups.

Conclusion:  In patients with AR related with Behcet disease, the rate of event was lower in patients with composite graft ARR compared to those with bioprosthesis ARR. Composite graft ARR was shown to be a feasible surgical option in patients requiring ARR for aortic root involvement of Behcet disease.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/surgical-method-for-aortic-root-involvement-of-behcet-disease/