Aortic regurgitation caused by Behçet's disease: surgical experience during an 11-year period.

Abstract

Aortic regurgitation (AR) caused by Behçet's disease is a rare clinical condition and surgical experience is limited. Many patients undergoing simple aortic valve replacement (AVR) required a second or third operation as a result of prosthetic valve dehiscence. We analyze our experience with surgical management for AR in Behçet's disease during an 11-year period.
From April 1997 through December 2008, 10 male patients (mean age, 38.4 ± 6.9 years) with AR caused by Behçet's disease underwent surgical treatment. The primary procedures were AVR in seven cases and aortic root replacement in three (two Bentall, one Cabrol). The duration of follow-up was 5.3 ± 3.6 years.
In the seven patients with simple AVR, valve dehiscence occurred in six after an interval of 2.9 ± 1.7 months, necessitating 11 reoperations (four redo AVRs, one redo redo-AVR, four aortic root replacements, and two heart transplants) with one early death. In aortic root replacement patients, two Bentall patients survived and required no reintervention, and the Cabrol patient died from electromechanical uncoupling one year postoperatively. Of the seven survivors, the definite procedure was aortic root replacement in four cases, heart transplantation in two, and AVR in one.
The rate of valve dehiscence was high after isolated AVR in patients with AR caused by Behçet's disease. At the first operation, aortic root replacement should be the procedure of choice, preferably with a homograft. Heart transplantation might be a feasible alternative to aortic root replacement in selected patients.