Ann Thorac Surg 1999;68:2136-2140
© 1999 The Society of Thoracic Surgeons
Original Articles: Cardiovascular
Surgical treatment of behçet’s disease involving aortic regurgitation
Motomi Ando, MDa,
Yoshio Kosakai, MDa,
Yutaka Okita, MDa,
Kiyoharu Nakano, MDa,
Soichiro Kitamura, MDa
a Department of Cardiovascular Surgery, National Cardiovascular Center, Osaka, Japan
Address reprint requests to Dr Ando, Department of Cardiovascular Surgery, National Cardiovascular Center, 5-7-1 Fujishirodai, Suita, Osaka 565-8565, Japan
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Abstract
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Background. Behçet’s disease involving aortic regurgitation is rare, and prosthetic valve detachment after aortic valve replacement is one of the most serious complications reported. We investigated the surgical results in 10 patients with aortic regurgitation caused by Behçet’s disease.
Methods. Between 1981 and 1997, 10 patients with aortic regurgitation secondary to Behçet’s disease had surgery. There were 8 men and 2 women, and their ages ranged from 33 to 60 years (mean, 46 ± 8 years). The surgical procedures for aortic regurgitation were aortic valve replacement in 6 patients and Bentall type operation in 4.
Results. No patient died during the hospital stay. The follow-up periods ranged from 11 to 185 months (mean, 87 months). Two patients died during the follow-up period. The acturial survival rate was 89% at 5 years and 67% at 10 years. Prosthetic valve detachment or suture detachment requiring redo operation occurred in 4 patients, 3 of whom had redo operations twice. Four patients had a composite graft replacement, and 1 patient died after the operation. No prosthetic valve detachment was noted in 64% of the patients at 5 years and in 43% at 10 years.
Conclusions. The rate of prosthetic valve detachment was 40% (4 of 10 patients), with a higher incidence in patients with Behçet’s disease than in those treated during the same period at the same hospital for aortitis caused by other diseases. Surgical techniques for treatment of this condition should be modified to improve the surgical outcome in these patients.
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Introduction
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Aortic regurgitation (AR) caused by Behçet’s disease is rare. Surgical treatment for Behçet’s disease is difficult because the surgeon must handle fragile, inflamed tissue. Valve detachment after aortic valve replacement (AVR) and pseudoaneurysmal formation are serious postoperative complications. We evaluated our surgical results in patients with AR caused by Behçet’s disease and their long-term outcomes.
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Patients and methods
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Ten patients with AR caused by Behçet’s disease were surgically treated at our hospital between 1981 and December 1997 (Table 1). There were 8 men and 2 women, and their ages ranged from 33 to 60 years (mean, 46 ± 8 years). The diagnosis of Behçet’s disease was based on clinical examination and intraoperative pathology of the aortic wall. Disease classification was the complete type in 3 patients and the incomplete type in 7. Preoperative corticosteroids were administered to 7 patients who had inflammatory findings (C-reactive protein >1.0 mg/dL, erythrocyte sedimentation rate >20 mm/h). In addition to AR, 2 patients had annuloaortic ectasia, 1 had mitral regurgitation, 1 had pseudoaneurysm of the ascending aorta, and 2 had Valsalva aneurysm.
Operative techniques
As an operation for AR, AVR was performed using everted 2-0 polyester mattress sutures on the para-annular position in 6 patients, and a valved conduit was placed in 4 patients. Figure 1 shows the intraoperative findings and the operative procedure in case 5. In this case, a valved conduit operation was done. Figure 2 shows magnetic resonance imaging and digital subtraction angiography results in cases 8 and 10. These 2 patients had annuloaortic ectasia with aneurysmal dilatation of the ascending aorta and had a valved conduit procedure. The valved conduit procedure was a modified Bentall operation in which the aortic valve prosthesis was sutured into the graft 1 cm from the end of the graft with a continuous 3-0 polyester suture, forming a composite graft. The composite graft was then implanted into the annulus using everting 2-0 polyester mattress sutures by the coronary reimplantation method. Ionescu-Shiley porcine xenograft was used for cases 1 and 2, but for the other 8 patients, a mechanical valve was chosen for AVR or for valved conduit. The concomitant operations that were done included coronary artery bypass graft in 1 patient, mitral annuloplasty in 1, aneurysmectomy of the ascending aorta in 1, and aneurysmectomy of the Valsalva sinus in 2 (Table 1).
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Fig 1. Case 5. (A, B) Operative findings. This patient had pseudoaneurysm of a noncoronary sinus. (C) Valved conduit operation with reimplantation of the coronary ostium was done. (D) Postoperative DSA. (Ao = aorta; LAD = left anterior descending artery; LCA = left coronary artery; LCX = left circumflex artery; RA = right atrium; RCA = right coronary artery.)
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Fig 2. Magnetic resonance imaging and digital subtraction angiographic findings in cases 8 and 10. These 2 patients had aneurysm of the ascending aorta and had valved conduit procedures. (Postop. = postoperative; Preop. = preoperative.)
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Results
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There was no operative death in any of the 10 patients (Table 1). Postoperative steroids were given to all patients to prevent suture detachment. Surgical results in the follow-up period are shown in Table 2. The duration of follow-up ranged from 11 to 185 months (mean, 87 months). In case 4, echocardiography detected grade 
perivalvular leakage, but it did not require a second operation. Valve detachment or suture detachment that required redo operation complicated the clinical course in 4 patients (40%), 3 of whom had redo operations twice. There was higher incidence of valve detachment among patients with Behçet’s disease who had AVR than among patients treated during the same period at the same hospital for aortitis caused by other diseases. Patient 1 had both prosthetic valve failure and aneurysmal formation of the ascending aorta in addition to valve detachment; that patient subsequently had a valved conduit operation 103 months after the first operation. However, she died of arrhythmia 10 days after the redo operation. Patients 3 and 6 had repeat AVR as a second operation, and a valved conduit operation as a third operation. Figure 3 shows first and second operative findings in case 3. Patient 7 had valved conduit operations twice, 14 months and 30 months after the initial operation. Patients 3, 6, and 7 were discharged in good condition. Patient 2 died of ruptured pseudoaneurysm of the ascending aorta 9 months after the operation. A freedom from reoperation curve for valve detachment or pseudoaneurysm is shown in Figure 4. No prosthetic valve detachment was noted in 64% of the patients at 5 years and in 43% at 10 years. The actuarial survival curve is also shown in Figure 4. The acturial survival rate was 89% at 5 years and 67% at 10 years.
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Fig 3. Case 3. (A) First operative finding. (B) Second operation was done 15 months later. Detached valve is shown. (C) Redo aortic valve replacement was done with placement of thick, belt-like Teflon felt on the outer side of the aortic wall for reinforcement using porcine xenograft. However, valve detachment recurred, and a valved conduit operation was necessary 128 months later.
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Fig 4. Freedom from reoperation curve for valve detachment or suture insufficiency at the aortic annulus and actuarial survival curve in surgical patients with aortic regurgitation caused by Behçet’s disease.
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Comment
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Behçet’s disease causes lesions not only in the aorta and arteries branching from the aorta, but also in the aortic valve [1–6]. The natural history of Behçet’s disease has been described by Geraint and Thomson [7]. They found that cardiovascular complications related to AR and Valsalva sinus aneurysms were the leading cause of death in patients with Behçet’s disease. After AVR for AR associated with Behçet’s disease, valve detachment can occur postoperatively, requiring redo operation in some patients. In patients with Behçet’s disease complicated by AR and annuloaortic ectasia who had valved conduit reconstruction, aneurysms at the anastomotic site sometimes occurred [4]. The aneuryms were caused by the fragility of the aortic wall and aortic annular tissue in this disease and by recurrent inflammation. In this study, the occurrence of valve detachment and suture detachment at the aortic annulus among surgically treated patients with AR associated with Behçet’s disease was evaluated. The diagnosis of Behçet’s disease was based not only on pathologic findings in aortic wall specimens obtained during the operation, but also on clinical findings.
Behçet’s disease is clinically manifested with recurrent, painful aphthous stomatitis, genital ulcers, and iridocyclitis [8]. This disease occurs more commonly in men than in women. Venous and peripheral arterial involvement has been well documented in Behçet’s disease. Arterial lesions are characterized by the formation of aneurysms in the abdominal aorta and in the femoral, subclavian, and carotid arteries. This lesion is rare in the ascending aorta [9]. AR is primarily due to valvulitis.
There have been only a few studies on the incidence of valve detachment after AVR for AR caused by Behçet’s disease. We surgically treated 90 patients with AR caused by nonspecific aortitis in the same period as the present study. In our patients, valve detachment was observed in 13.3% (12 of 90 patients), in 4.6% (3 of 65 patients) of the patients who had Takayasu’s arteritis, 40% (4 of 10) of those with Behçet’s disease, 33% (5 of 15) of those with aortitis of unknown origin, and 28.6% (8 of 28) of all men with aortitis. In our series, the incidence of valve detachment was high in the patients with Behçet’s disease, aortitis of unknown origin, and in male patients. The reoperation-free rates after 5 years and 10 years, due to the absence of valve detachment or suture insufficiency, were 96% and 94% in patients with Takayasu’s arteritis, in contrast to 64% and 43% of those with Behçet’s disease. These differences probably result from a more fragile aortic wall in Behçet’s disease caused by recurrent and uncontrollable inflammation.
To prevent valve detachment, technical improvements have been devised, including buttress sutures from the lateral side of the aortic wall for fixation of the prosthetic valve at the aortic annulus, placement of thick, belt-like Teflon felt on the lateral side of the aortic wall for reinforcement, or the use of a valved conduit or homograft even in patients without annuloaortic ectasia. Composite graft reconstruction is helpful because circumferential fixation outside the aortic wall and double fixation at the aortic annulus are possible, and the prosthetic valve does not apply direct pressure on the aortic annulus. We believe that in AVR, the pressure of the valve function directly affects the rigid sewing ring, thereby causing a higher detachment rate. In composite graft reconstruction, the original Bentall operation [10] for this disease is not indicated because of a high risk of suture insufficiency. Both coronary arteries are reconstructed by reimplantation of the coronary ostium [11] or by the interposition method [12] using middle-caliber artificial vessels.
Our evaluation of surgical techniques suggested that Takayasu’s arteritis can be treated by conventional valve replacement because those patients have a low incidence of valve detachment. However, valved conduit reconstruction is indicated in patients with AR associated with Behçet’s disease, in whom prevention of valve detachment is difficult even by current valve fixation methods. The incidence of valve detachment was lower in patients who had valved conduit procedures than in those who had AVR procedures. The valved conduit procedure was done a total of nine times on 7 patients, and graft detachment was found in only 1 patient.
Opinion is divided on the use of steroids to prevent valve detachment [4] by correcting poor postoperative healing of tissue and susceptibility to infection. Preoperative steroid administration to decrease inflammation and its postoperative use to control inflammation could be important. We believe that the most important aspect of therapy for Behçet’s aortitis is not simply to correct the detachment, but to prevent and treat the causative inflammatory reaction with steroid therapy. We administered steroids preoperatively in 7 of the 10 patients, and postoperatively in all patients who had inflammatory signs. They had no complications caused by the use of steroids.
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Accepted for publication May 3, 1999.
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Abstract
Introduction
