Ann Thorac Surg 1997;64:116-119
© 1997 The Society of Thoracic Surgeons
Original Articles: Cardiovascular
Surgical Management of Behçet's Aortitis: A Report of Eight Patients
Kenji Okada, MD,
Kiyoyuki Eishi, MD,
Shinichi Takamoto, MD,
Motomi Ando, MD,
Yoshio Kosakai, MD,
Kiyoharu Nakano, MD,
Yoshikado Sasako, MD,
Junjiro Kobayashi, MD
Department of Cardiovascular Surgery, National Cardiovascular Center, Osaka, Japan
Accepted for publication January 16, 1997.
 |
Abstract
|
|---|
Background. Behçet's aortitis is one of the most severe manifestations of Behçet's disease. However, there have been few reports of this rare entity. This article we describe our experience with surgical treatment of Behçet's aortitis in 8 patients.
Methods. From May 1978 to February 1995, we operated on 8 patients with Behçet's aortitis. All patients were followed up for a total of 54.4 patient-years (mean, 6.8 years).
Results. There were no operative deaths, but 1 patient died in the hospital of ventricular fibrillation. Another death occurred as a result of pseudoaneurysm rupture 10 months after operation. Fourteen operations were performed on eight patients: a second operation was performed on four patients, and a third operation was performed on two patients. The translocated Bentall procedure, a modified Bentall procedure, was performed six times. One patient required a second operation for graft detachment, but the others have done well without further surgical intervention. Aortic valve replacement was performed six times, and three reoperations were needed after aortic valve replacement.
Conclusions. The translocated Bentall procedure may be effective for Behçet's aortitis, but further investigation is necessary.
|
Introduction
|
|---|
Behçet's disease affects multiple systems. Originally described in 1937, it is characterized by urogenital ulcerations, chronic eye inflammation, and skin lesions. Although this disease has a worldwide distribution, individuals of Japanese and Eastern Mediterranean descent have the highest incidence. Many complications have been recognized for Behçet's disease, but aortitis is one of the most severe. It usually involves aortic regurgitation (AR), aneurysms, or both. After aortic valve replacement (AVR), valve detachment and pseudoaneurysms at the anastomosis occur frequently. Many case reports describe this rare involvement, but few report its long-term outcome. In this article, we describe the surgical treatment of 8 patients with Behçet's aortitis and their long-term outcomes.
|
Material and Methods
|
|---|
From May 1978 through February 1995, we operated on 8 patients with Behçet's aortitis (6 men and 2 women). All patients were followed up for 0.13 to 12.4 years (mean, 6.8 years). Disease classification was the complete type in 1 patient and the incomplete type in 7. The mean patient age was 47.1 ± 8.1 years, and it ranged from 37.4 to 60.0 years at the time of the first operation. The patient's diseases and their first operations are listed in Table 1
.
Since 1987, we have used the translocated Bentall procedure as the treatment of choice for AR, Valsalva aneurysm, valve detachment, perivalvular leakage, and pseudoaneurysm resulting from Behçet's aortitis. The translocated Bentall procedure is a modified Bentall's procedure in which the aortic valve prosthesis is sutured into the graft 1 cm from the end of the graft with a continuous 2-0 polyester suture, forming a composite graft [1]. The composite graft then is implanted into the annulus using everting 3-0 polyester mattress sutures. We investigated the long-term results, pathologic findings of the aortic valve, C-reactive protein (CRP) changes and corticosteroid therapy, and surgical procedures performed.
|
Results
|
|---|
Long-Term Results
In the 4 patients who did not require a second operation, echocardiography detected a grade 
perivalvular leak in only 1 case. Reoperation was needed in the other 4 patients (7.4% per patient-year). The reasons for reoperation were two valve detachments after AVR and two pseudoaneurysms, one after resection of the ascending aorta and one after a Bentall procedure. Two patients died after the second operation. One had an uncontrolled ventricular arrhythmia refractory to drugs and countershock therapy 10 days after the second operation. The other died 10 months after patch closure of a ruptured pseudoaneurysm. A third operation was performed in 2 patients (3.7% per patient-year). A translocated Bentall procedure was performed in both patients, and they were discharged in good condition. Figure 1 shows the survival curve and the reoperation-free curve. Case reports are presented for patients who required reoperation.
|
PATIENT 5.
|
|---|
A 46-year-old woman with Behçet's syndrome was admitted for cardiac failure resulting from AR. An Ionescu-Shiley 21-mm AVR was sutured into a supraannular position. The postoperative course was uneventful. However, 5 years and 7 months later, an AR murmur was audible. Doppler echocardiography demonstrated perivalvular leakage caused by valve detachment and transvalvular AR caused by structural valvular deterioration. At operation 3 years later, the valve was found to be detached through 180 degrees, and a pseudoaneurysm was observed. A translocated Bentall procedure and reimplantation of the coronary arteries was performed. The patient was transferred to the ward and recovered sufficiently to begin eating solid food. However, 10 days after the operation, she had an uncontrollable ventricular arrhythmia that was refractory to drugs and cardioversion and subsequently died.
|
PATIENT 6.
|
|---|
A 41-year-old man with Behçet's disease received a Björk-Shiley 25-mm AVR. One year later, his CRP levels rose to 3+, and a heart murmur was detected. An echocardiogram revealed AR caused by valve detachment, and cardiac failure developed. At reoperation, detachment was found through 270 degrees. An AVR was accomplished by bolstering the mattress sutures with felt strips and inserting sutures from the outside of the aorta. The prosthetic valve was transferred to the supraannular region of the aorta. Eight months later, AR (grade ) resulting from valve detachment was recognized. Ten years later, a translocated Bentall procedure was performed. Six months after the third operation, perivalvular leakage (grade ) was recognized again.
|
PATIENT 7.
|
|---|
A 37-year-old man treated for Behçet's disease had AR and a Valsalva sinus aneurysm diagnosed, and a resection was performed at another hospital. At 45 years of age, a chest roentgenogram demonstrated an abnormal shadow, and an aortogram revealed a pseudoaneurysm. A patch closure was performed with interrupted sutures and an AVR. The postoperative course was uneventful. Ten months later, he died suddenly, and an autopsy revealed a ruptured pseudoaneurysm.
|
PATIENT 8.
|
|---|
A 39-year-old man with Behçet's disease associated with AR and a Valsalva sinus aneurysm underwent a Bentall procedure at another hospital. At 41 years of age, he experienced an episode of chest pain, and a cardiac catheterization revealed a pseudoaneurysm resulting from graft detachment and stenosis of the right coronary artery. A translocated Bentall procedure and coronary artery bypass graft were performed. However, at 42 years of age, graft detachment was found again. The patient had not been taking corticosteroids because of an eye lesion, but because the two operations resulted in poor outcomes, he began corticosteroid therapy. Another translocated Bentall procedure with felt strips was performed. Satisfactory results were obtained, without findings of perivalvular leakage 3 years after the operation.
|
Pathologic Findings of the Aortic Valve
|
|---|
Aortic valve findings are summarized in Table 2. Various types of AR, such as shrinking, thickening, and prolapse, were found, and the site of the lesion also varied. Microscopically, thickening of the intima, tears of the elastica, and inflammatory cell infiltration were common pathologic findings in this type of aortitis. The microscopic findings for patients 1 through 6, who had their first operation at our institution, are summarized in Table 3.
|
C-Reactive Protein Changes and Corticosteroid Therapy
|
|---|
Table 4 summarizes preoperative CRP levels and corticosteroid therapy. Patients 1, 4, 5, and 6 had received corticosteroid therapy before their first operation. Patients 2, 3, and 7 had received corticosteroid therapy after their first operation. Patient 8 had received corticosteroids only after the second operation because he had the complete type of Behçet's disease. At the first operation, 5 patients had low CRP levels, but patient 4 had active infective endocarditis, and the CRP level was slightly elevated (1.6 mg/dL). All of the second or third operations were performed in patients with a low CRP level. The maintenance dose of predonine used was 3 to 30 mg/day.
|
Surgical Procedures
|
|---|
We performed a total of 12 operations on 6 patients (Table 5). The translocated Bentall and AVR procedures each were performed 6 times. After the translocated Bentall procedure, one reoperation was necessary, and one patient died in the hospital of ventricular fibrillation. After AVR, three reoperations were necessary and one death occurred as a result of the rupture of a pseudoaneurysm. Six patients were followed up. One of the four patients in the translocated Bentall group had perivalvular leakage, and one of the two patients in the AVR group had perivalvular leakage.
|
Comment
|
|---|
The cardiovascular manifestations of Behçet's disease have attracted much attention in recent years. Their incidence was reported by Geraint and Thomson [2] to be approximately 30%, and vascular lesions appeared to be the major cause of death in patients with such complications. The underlying pathologic process was thought to be a vasculitis, which affected the vasa vasorum; resected vascular specimens have been shown to have fragmentation and splitting of the elastic fibers in the media, with perivascular mononuclear cell infiltration [2].
Many complications have been reported, such as left ventricular aneurysms [3], aneurysms of a segmental artery of the left lung [4], conduction disturbances [5], myocardial infarctions [6], ventricular arrhythmias [6], aortic and mitral regurgitations [7, 8], aortic arch aneurysms [9], AR [10–12], and false aortic aneurysm formation [13]. However, those studies did not report long-term results. In our study, the reoperation rate was 7.4% per patient-year, and the mortality rate was 3.7% per patient-year. Figure 1
demonstrates that no patient died until 8 years after the operation, and after 10 years, the actuarial survival rate was slightly more than 50%. A second operation was needed in more than 30% of the patients after 1 year.
We evaluated the aortic valve deformities; however, there was no difference between those patients who did and did not require reoperation. This suggests that we cannot predict valve detachment after the first operation. Therefore, we cannot choose the most appropriate procedure based on the macroscopic findings.
Yamana and colleagues [14] described 2 patients with vascular-Behçet's disease who had femoral and popliteal aneurysms, and they studied the resected aneurysms and occluded distal arteries clinicopathologically. They found prominent fibrosis of the adventitia, including the surrounding tissues, as well as venous occlusion, perivasculitis, and the deposition of complement factors C3 and C4 and immunoglobulins A, G, and M in the arterial wall and surrounding tissues. This suggests that the formation of aneurysms in Behçet's disease is caused by the destruction of the intimal and external layers of the arterial wall. However, no common pathogenic mechanisms have been identified. Further study of Behçet's disease using immunohistochemistry is required.
In our study, the pathologic findings were similar among the 8 patients. Valve detachment usually occurs in patients with severe aortitis. However, in patient 6, valve detachment occurred despite the absence of inflammatory findings. It also has been suggested that primary acute arteritis may be a feature of Behçet's disease, but the histologic findings do not suggest a clear course of postoperative therapy.
The preoperative CRP levels in our study were low, except for 1 patient with associated acute infectious endocarditis. This disease is progressive and chronic, and requires strict observation and systemic control of any inflammation. 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 corticosteroid therapy. Any surgical intervention preferably should be undertaken during a relatively quiescent phase of inflammation.
The natural history of Behçet's disease has been described by Geraint and Thomson [2, 15]. They found that cardiovascular complications related to AR and Valsalva sinus aneurysms were the leading cause of death. Composite replacement of the ascending aorta or AVR has become the standard therapy for these patients, but the results have not been satisfactory [10, 16]. Isomura and colleagues [17] described the operative and perioperative treatment of 11 patients with aortic regurgitation caused by aortitis. They concluded that it was important to arrest the inflammatory reaction preoperatively and to reinforce the implanted prosthesis with pledgeted sutures. However, no patient with Behçet's aortitis was included in their study. In our patient 6, we had to reinforce the implanted prosthesis with pledgeted sutures. However, 8 months later, AR recurred and a second operation was needed. The operative findings revealed that the pledgeted sutures had burrowed into the aortic wall, and were found inside the aorta. Therefore, we believe that using pledgeted sutures alone is not sufficient.
Danielson and associates [18] reported the "translocation of the aortic valve" procedure for prosthetic valve endocarditis. The translocated Bentall procedure first was described by Cabrol and coworkers [19] for the treatment of AR associated with an aneurysm of the ascending aorta. Suzuki and colleagues [20] used reimplantation of the valves with Teflon pledgets, which is one of the strategies used in the translocated Bentall procedure to prevent uncontrollable hemorrhage and tension on the anastomosis. We believe that in AVR, the stress of the valve function directly affects the rigid sewing ring, and causes detachment more frequently. In the translocated Bentall procedure, detachment occurs less frequently because the flexible tubular prosthesis cushions this stress.
In our study, 14 operations were performed on 8 patients, and 2 patients died. Valve and graft detachments were recognized after these procedures. In comparing the translocated Bentall and AVR procedures, the incidence of valve detachment was lower in patients who underwent the former. The translocated Bentall procedure was performed a total of six times on five patients, and graft detachment was found in only one patient. That patient subsequently died of ventricular fibrillation, which was thought to be unrelated to the surgical technique.
We conclude that the translocated Bentall procedure may be useful in reducing valve detachment.
|
Footnotes
|
|---|
Address reprint requests to Dr Okada, First Department of Surgery, Hiroshima University School of Medicine, 1-2-3 Kasumi, Minami-ku Hiroshima, Japan, 734.
|
References
|
|---|
- Kawazoe K, Eishi K, Kawashima Y. New modified Bentall procedure: Carrel patch and inclusion technique. Ann Thorac Surg 1993;55:1578–79.[Abstract]
- Geraint JD, Thomson A. Recognition of the diverse cardiovascular manifestations in Behçet's disease. Am Heart J 1982;45:457–58.
- Eusanio GD, Mazzola A, Gregorini R, et al. Left ventricular aneurysm secondary to Behçet's disease. Ann Thorac Surg 1991;51:131–32.[Abstract]
- Salamon F, Weinberger A, Nili M, et al. Massive hemoptysis complicating Behçet's syndrome. The importance of early pulmonary angiography and operation. Ann Thorac Surg 1988;45:566–67.[Abstract]
- Nojiri C, Endo K, Koyanagi H. Conduction disturbance in Behçet's disease. Associated with ruptured aneurysm of the sinus of Valsalva into the left ventricular cavity. Chest 1984;86:636–38.[Abstract/Free Full Text]
- Schiff S, Moffatt R, Mandel WJ, et al. Acute myocardial infarction and recurrent ventricular arrhythmias in Behçet's syndrome. Am Heart J 1982;103:438–40.[Medline]
- Pena JM, Garcia-Alegria J, Garcia-Fernandez F, et al. Mitral and aortic regurgitation in Behçet's syndrome. Ann Rheum Dis 1985;44:637–39.[Abstract/Free Full Text]
- Terada Y, Takamoto T, Nakamura T, et al. Behçet's disease associated with aortic and mitral regurgitation: report of two cases. J Cardiol 1988;18:857–65.[Medline]
- Napaleone CP, Bartolomeo RD, Pierangeli G, et al. Aortic arch aneurysm in Behçet's disease: an unusual association. Cardiovasc Surg 1994;2:379–80.[Medline]
- Comess KA, Zibelli LR, Gordon D, et al. Acute, severe, aortic regurgitation in Behçet's syndrome. Ann Intern Med 1983;99:639–40.
- Gozalez T, Hernandez-Beriain JA, Rodriguez-Lozano B, et al. Severe aortic regurgitation in Behçet's disease. J Rheumatol 1993;20:1807–08.[Medline]
- Tai YT, Fong PC, Ng WF, et al. Diffuse aortitis complicating Behçet's disease leading to severe aortic regurgitation. Cardiology 1991;79:156–60.[Medline]
- Rae SA, Vandenburg M, Scholtz CL. Aortic regurgitation and false aortic aneurysm formation in Behçet's disease. Postgrad Med J 1980;56:438–39.[Abstract]
- Yamana K, Kosuga K, Kinoshita H, et al. Vasculo-Behçet's disease. Immunological study of the formation of aneurysm. J Cardiovasc Surg (Torino) 1988;29:751–55.[Medline]
- Geraint JD. Behçet's syndrome. N Engl J Med 1979;301:431–32.[Medline]
- Chikamori T, Doi YL, Yonezawa Y, et al. Aortic regurgitation secondary to Behçet's disease: a case report and review of the literature. Eur Heart J 1990;11:572–76.[Abstract/Free Full Text]
- Isomura T, Hisatomi K, Yanagi I, et al. The surgical treatment of aortic regurgitation secondary to aortitis. Ann Thorac Surg 1988;45:181–85.[Abstract]
- Danielson GK, Titus JL, DuShane JW. Successful treatment of aortic valve endocarditis and aortic root abscess by insertion of prosthetic valve in ascending aorta and placement of bypass graft to coronary arteries. J Thorac Cardiovasc Surg 1974;67:443–49.[Medline]
- Cabrol C, Pavie A, Grandjbackhch I, et al. Complete replacement of the ascending aorta with reimplantation of the coronary arteries: new surgical approach. J Thorac Cardiovasc Surg 1981;81:309–15.[Abstract]
- Suzuki A, Amano J, Tanaka H, et al. Surgical consideration of aortitis involving the aortic root. Circulation 1989;80(Suppl 1):222–32.
This article has been cited by other articles:
|
|
|
|
 
K. Yoshikawa, H. Hori, S. Fukunaga, E. Tayama, and S. Aoyagi
Aortic Root Replacement in Behcet Disease
Asian Cardiovasc Thorac Ann,
December 1, 2007;
15(6):
521 - 523.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
E. M. Chau, E. Wang, C. S. Chiu, and W.-H. Chow
Non-Infectious Aortitis: an Important Cause of Severe Aortic Regurgitation
Asian Cardiovasc Thorac Ann,
June 1, 2006;
14(3):
177 - 182.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
Y Hama, T Kaji, Y Iwasaki, T Kawauchi, M Yamamoto, and S Kusano
Endovascular management of multiple arterial aneurysms in Behcet's disease
Br. J. Radiol.,
July 1, 2004;
77(919):
615 - 619.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. H. Park, J. W. Chung, J. H. Joh, S. Y. Song, S. J. Shin, K. S. Chung, D. Y. Lee, J. Y. Won, and S. J. Kim
Aortic and Arterial Aneurysms in Behcet Disease: Management with Stent-Grafts—Initial Experience
Radiology,
September 1, 2001;
220(3):
745 - 750.
[Abstract]
[Full Text]
[PDF]
|
|
|
Top
Abstract
Introduction
