Asian Cardiovasc Thorac Ann 2007;15:521-523
© 2007 Asia Publishing EXchange Ltd
Aortic Root Replacement in Behçet Disease
Kazuhiro Yoshikawa, MD,
Hidetsugu Hori, MD,
Shuji Fukunaga, MD,
Eiki Tayama, MD,
Shigeaki Aoyagi, MD
Department of Surgery, Kurume University School of Medicine Kurume, Japan
For reprint information contact: Kazuhiro Yoshikawa, MD Tel: 81 94 235 3311 Fax: 81 94 235 8967 Email: yosshi{at}med.kurume-u.ac.jp, Department of Surgery, Kurume University School of Medicine, 67 Asahi-machi, Kurume 830-0011, Japan.
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ABSTRACT
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The patient presented with a history of recurrent aphthous stomatitis, genital ulceration, and a family history of positive for collagen disease. Echocardiography and retrograde aortography revealed aneurysm formation of the sinus of Valsalva, and dilatation of the aortic valve annulus with severe aortic regurgitation. On diagnosis of an aneurysm of the sinus of Valsalva and aortic regurgitation associated with Behçet’s disease, aortic root replacement with the modified Bentall technique was successfully performed.
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INTRODUCTION
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Behçet’s disease is a characteristic inflammatory disorder of unknown origin that can affect the aorta, its major branches, and the aortic valve.1 The cardiovascular complications of Behçet’s disease occasionally require surgical intervention; however, surgical treatment for cardiovascular lesions presents many difficulties. These include difficulties due to the extensive and severe lesions as well as problems due to the necessity of manipulating fragile and inflamed tissue. For these reasons, postoperative complications such as hemorrhage, pseudoaneurysm formation, valve detachment, or paravalvular leakage are often encountered after the initial or even the second operation.2,3 We report here a patient with an aneurysm of the sinus of Valsalva and aortic regurgitation associated with Behçet’s disease who was successfully treated by replacement of the aortic root using a valved conduit.
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CASE REPORT
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A 31-year-old Japanese man was admitted with a chief complaint of chest pain. He had a history of recurrent aphthous stomatitis and genital ulceration, and his family history included collagen disease in his aunt. On admission, the patient was slightly anemic but afebrile. Aphthous stomatitis was found on the tongue. A grade 4/6 to and fro murmur was heard on the 3rd intercostal space along the left sternal border. Hematologic examination showed high C-reactive protein (CRP) (3.23 mg·dL–1) and a high erythrocyte sedimentation rate (ESR) (74 mm·hr–1), however the white blood cell count (7.8 K·µL–1) was not raised. HLA typing was B51. Chest X-Ray revealed cardiomegaly (with a cardiothoracic ratio of 60%) and a widened silhouette of the ascending aorta. Echocardiography revealed aneurysm formation of the sinus of Valsalva and dilatation of the aortic valve annulus with severe regurgitation. The left ventricular (LV) end-diastolic and end-systolic dimensions were 67 mm and 39 mm, respectively, and the LV ejection fraction was 0.71.
Cardiac catheterization revealed normal (20/11 mm Hg) pulmonary artery pressure and extremely low (152/22 mm Hg) diastolic pressure of the aorta resulting in wide pulse pressure. Retrograde aortography revealed a grade 3/4 aortic regurgitation (AR) and aneurysm formation of the sinuses of Valsalva (Figures 1
and 2). Based on these findings, the diagnosis of aneurysm of the sinus of Valsalva and AR associated with an incomplete form of Behçet’s disease was made.
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Figure 2. Aortogram showing severe Aortic regurgitation and aneurysm formation of the sinus of Valsalva.
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As the patient’s condition was suspected as being in the active phase, steroid therapy with prednisolone (40 mg·day–1) was started. The dose of prednisolone was gradually tapered to 30 mg·day–1 over one month, and the inflammatory signs became negative (CRP: 0.04 mg·dL–1, ESR: 10 mm·hr–1) before the operation.
During surgery, aneurysm formation at the ascending aorta and the sinus of Valsalva was observed. After establishment of cardiopulmonary bypass, the ascending aorta was longitudinally opened under aortic crossclamping and myocardial protection with cold blood cardioplegia. The aortic wall was thickened but no calcification existed. Although thickening and stiffening of the aortic valve were not observed, dilatation of the aortic valve annulus and retraction of the aortic cusps with resultant failure to coapt were found. A high take-off of the coronary artery was also present. In addition to graft replacement of the ascending aorta and the proximal aortic arch, aortic root replacement with the modified Bentall technique was performed using a composite valved graft in which the aortic valve prosthesis was sutured 1 cm above the proximal end of the graft (Figure 3). The composite graft was then implanted into the annulus with pledgeted everting mattress sutures. The coronary arteries were reconstructed by the aortic button technique.
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Figure 3. A photograph of the valve conduit including the prosthetic valve 1 cm away from the proximal end of the graft.
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Postoperative steroid therapy was started immediately after the operation. Prednisolone (10 mg·day–1) was given intravenously on the day of the operation, and oral administration of prednisolone 30 mg was started on the first postoperative day. No significant increase of CRP values was observed throughout the postoperative period.
Histologic examination of the excised aortic wall and valve tissue showed chronic inflammatory reaction with degenerative changes that were compatible with Behçet’s disease at the scar stage. The patient was doing well 42 months after surgery with daily oral administration of prednisolone 10 mg.
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DISCUSSION
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Behçet’s disease is clinically manifested by recurrent, painful aphthous stomatitis, genital ulcers, and iridocyclitis. This disease often affects young adults and is more common in men than women. Arterial involvement has been well documented in Behçet’s disease. Aortic regurgitation is seen in 5% to 19% of patients with Behçet’s disease. James and Thomson1 demonstrated that cardiovascular complications related to AR, and Valsalva sinus aneurysms were the leading causes of death in patients with Behçet’s disease.
Aortic valve replacement (AVR) in Behçet’s disease requires special considerations because postoperative complications such as valve detachment and pseudoaneurysm formation are not rare.4 Although no accurate estimate of the incidence of valve detachment has been made, it has been reported to be as high as 36% (4 of 11 patients).4 To prevent valve detachment, various techniques have been used, including the use of pledgeted interrupted sutures and transmural buttress sutures to fix a prosthetic valve at the aortic annulus, and the use of a valved conduit or homograft.5 Among these techniques, Ando and colleagues4,5 have emphasized the usefulness of a modified Bentall procedure. In their modified procedure, a composite graft consisting of a vascular graft 1.0 to 2.0 cm away from the proximal extremity of the vascular tube graft is developed, and is then implanted into the aortic valve annulus by means of buttressed everting mattress sutures with circumferential belt-like felt outside the aortic wall. Placement of the prosthetic aortic valve into a vascular graft or the use of an aortic homograft results in reduction of the tension on the suture line and the native aortic valve annulus relative to the tension observed in orthotopic valve replacement with a mechanical valve.
According to the experience of Okada et al,6 dehiscence of the suture line at the aortic valve annulus occurred in only one of 8 patients who underwent replacement of a detached prosthetic valve using this modified Bentall technique or an aortic homograft. Thus, they have recommended that aortic root replacement using their modified Bentall technique or an aortic homograft should be performed in patients with a risk of valve detachment such as patients with Behçet’s disease and those with Takayasu’s arteritis.6 In the present patient, we used a technique similar to their modified technique, except that we fixed the valve conduit to the native aortic valve annulus with buttressed everting mattress sutures. However, no detachment of the valve conduit nor pseudoaneurysm formation at the aortic valve annulus were found on echocardiograms obtained in the 40th postoperative month.
Control of the inflammatory process is important to prevent complications occurring after AVR.7,8 It is preferable that surgical procedures be performed during the chronic phase of the inflammation. The effectiveness of steroids and other non-steroid anti-inflammatory drugs has been described in several reports. Although the administration of steroids in the early postoperative period has some potential hazards such as susceptibility to infection and disturbance of the wound-healing process, we believe that steroid therapy should be started in the early postoperative period in patients who are operated on during the active phase of Behçet’s disease, and should be maintained until the CRP and ESR values become normalized.
In conclusion, we report a patient who had an aneurysm of the sinus of Valsalva and AR associated with Behçet’s disease. Aortic root replacement with the modified Bentall technique was successfully performed, and inflammation was controlled with steroid therapy.
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ACKNOWLEDGMENTS
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This work was supported in part by the Grant-in-Aid for Encouragement of Young Scientists, Japan Society for the promotion of Science (7303-8587-15790954).
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REFERENCES
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- James DG, Thomson A. Recognition of the diverse cardiovascular manifestation in Behcet’s disease. Am Heart J 1982;103:457–8.[Medline]
- Aoyagi S, Kosuga K, Fukunaga S, Akashi H, Oryoji A, Oishi K. Valve detachment after aortic valve replacement-a successfully reoperated case with the Cabrol technique. Jpn Circ J 1996;60:377–81.[Medline]
- Tanimoto K, Ishizuka N, Ram SB, Okada M, Nakamura K, Kasanuki H, et al. Treatment of cardiovascular Behcet’s disease: a problem with valve replacement. J Cardiol 1998;31 Suppl 1:75–82[Medline]
- Ando M, Okita Y, Sasako Y, Kobayashi J, Tagusari O, Kitamura S. Surgery for aortic regurgitation caused by Behcet’s disease: a clinical study of 11 patients. J Card Surg 1999;14:116–21.[Medline]
- Ando M, Sasako Y, Okita Y, Tagusari O, Kitamura S. Valved conduit operation for aortic regurgitation associated with Behcet’s disease. Jpn J Thorac Cardiovasc Surg 2000;48:424–7.[Medline]
- Okada K, Eishi K, Takamoto S, Ando M, Kosakai Y, Nakano K, et al. Surgical management of Behcet’s aortitis: a report of eight patients. Ann Thorac Surg 1997;64:116–9.[Abstract/Free Full Text]
- Isomura T, Hisatomi K, Yanagi I, Shimada S, Uraguchi K, Aoyagi S, et al. The surgical treatment of aortic regurgitation secondary to aortitis. Ann Thorac Surg 1988;45:181–5.[Abstract]
- Moro H, Hayashi J, Ohzeki H, Sogawa M, Nakayama T, Namura O. Surgical management of cardiovascular lesions caused by systemic inflammatory diseases. Thorac Cardiovasc Surg 1999;47:106–10.[Medline]
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ABSTRACT
INTRODUCTION
