Eur J Cardiothorac Surg 2004;25:465-467
© 2004 Elsevier Science NL
True and pseudo aneurysms of coronary arteries in a patient with Behçet's disease
Murat Ozeren*,
Orhan Veli Dogan,
Süheyla Dogan,
Ertan Yucel
Cardiovascular Surgery Department, Ankara Teaching and Research Hospital, Social Security Organization (SSK), Ankara, Turkey
Received 26 August 2003;
received in revised form 16 November 2003;
accepted 28 November 2003.
* Corresponding author. Address: Kizilirmak cad 61/5, Kizilay, Ankara 06640, Turkey. Tel.: +90-312-4318775; fax: +90-312-4358970
e-mail: mozeren{at}yahoo.com
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Abstract
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A 33-year-old man with a 9-year history of Behçet's disease was hospitalized with a giant pseudo aneurysm of left anterior descending and true aneurysm of right coronary artery. This unusual vascular complication of Behçet's disease treated successfully is presented.
Key Words: Behçet's disease • Coronary artery aneurysm • Vasculitis
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1. Introduction
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Behçet's disease is characterized by the coexistence of oral and genital ulcers and iridocyclitis [1]. The spectrum of the disease is ever expanding. Vascular involvement, a rare but serious form of the disease, is a predictor of poor prognosis [2]. Involvement of coronary arteries is extremely rare in the literature. Surgical approach to these patients is still in dispute. We report on the successful repair of true and pseudo aneurysms of coronary arteries in a 33-year-old patient with Behçet's disease.
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2. Case
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A 33-year-old man with Behçet's disease, diagnosed 9 years earlier was hospitalized for angina pectoris. He had a 2-week history of anterior chest pain and numbness of the left arm. He had class II (Canadian Cardiovascular Society) angina. Diagnosis of Behçet's disease was based on oral and genital ulcers accompanied by relapsing iridocyclitis. In his past history, he had right nephrectomy in 1993, operation for retinal detachment in 1997, right femoral artery true aneurysm repair in 1999, and left femoral artery true aneurysm repair in 2000. He was receiving cyclosporine (100 mg/day), fluocortolon (20 mg/day), colchicine (1.2 mg/day) and dypyridamole (225 mg/day). On physical examination, the patient was afebrile, pulse rate was 70 per min and blood pressure was 110/70 mmHg. Cardiovascular system examination was normal except for bilateral chronic deep venous thrombosis. The chest roentgenogram showed mild cardiomegaly. Electrocardiogram demonstrated ST-T changes in the inferior and the lateral derivations. Transthoracic echocardiogram showed global decrease in left ventricular function, an estimated left ventricular ejection fraction of 48% and akinesis of interventricular septal wall. Electron beam coronary angiography (EBCA) was selected as a first line coronary investigation and revealed 50x43 mm2 giant aneurysm of proximal left anterior descending artery covered with mural thrombus and 22x20 mm2 aneurysm of proximal right coronary artery (Fig. 1a)
. Technetium 99 scintigraphy showed posterobasal fixed perfusion defect (non-viable myocardium) and anteroapical ischemia. Because of increased tendency of enlargement and rupture, the patient was scheduled for surgery after coronary angiography. Coronary angiography revealed pseudoaneurysm originating from proximal left anterior descending artery (Fig. 1b) and true aneurysm of the right coronary artery with fistulization to the right atrium. There was no obstruction along the course of LAD, although compression of aneurysm sac over LAD could not be excluded. Left ventriculogram showed severe hypokinesis and dyskinesis of the basal segment. A whole body computed tomographic scan did not reveal any additional findings. Routine laboratory tests yielded normal findings except for a mildly elevated erythrocyte sedimentation rate of 26 mm/h.
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Fig. 1. (a) Electron beam coronary angiography shows aneurysms located in the left anterior descending and right coronary artery. (b) Coronary angiography shows giant pseudo aneurysm of left anterior descending.
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The patient underwent elective operation and the heart was exposed through midline sternotomy. The pericardium was adherent to the heart. All purse sutures were supported with the pledgets. After institution of cardiopulmonary bypass in the standard fashion, antegrade warm blood cardioplegia was infused. True aneurysm of the right coronary artery was opened and the fistulization point and aneurysm was sewn completely with a 5/0 polypropylene suture and Teflon strips in both sides. As the posterobasal segment infarction ended up in a fixed perfusion defect (non-viable myocardium), bypass to right coronary artery was not performed to prevent the formation of a new aneurysm at the anastomosis. Pseudo aneurysm of left anterior descending artery was occluded proximally and distally to prevent distal embolization of mural thrombus and then the aneurysm was opened and the thrombus was evacuated. A 1x0.5 cm2 hole causing pseudo aneurysm formation over left anterior descending artery was found (Fig. 2)
. The hole was closed with polytetrafluoroethylene (PTFE) patch using 6/0 polypropylene suture. Postoperative course was uneventful. The patient was restarted on fluocortolon (20 mg/day), cyclosporine (100 mg/day), colchicine (1.2 mg day) and dypyridamole (225 mg/day). The patient was discharged at postoperative day 7. Pathological examination of the aneurysm wall revealed non-specific inflammatory process related with Behçet's disease. The patient was asymptomatic at postoperative sixth month and his control EBCA showed patent LAD and occluded RCA without new aneurysm formation.
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3. Discussion
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Behçet's disease is a multisystemic vasculitis affecting all sizes of arteries and veins [3]. The prevalence of coronary involvement in Behçet's disease is 0.5% [2].
The pathology and pathogenesis of the arterial lesions in Behçet's disease have been well documented. An inflammatory obliterative endarteritis of the vasa vasorum, most likely brought about by immune deposition, causes destruction of the media and fibrosis and thus weakens and predisposes the arterial wall to aneurysm formation that eventually ruptures [4].
Arterial involvement of patients with Behçet's disease should be investigated with non-invasive methods. Arterial punctures for angiography or blood gas analysis may provoke development of further aneurysms at the site of vascular interference. EBCA may document location and diameter of coronary aneurysms precisely. But it failed in differentiation of aneurysm as false or true and detection of the coronary artery to atrium fistulization as confirmed with conventional coronary angiography in this case.
Behçet's disease progresses with remissions and attacks. There is still no definite medical treatment. The most common approach is the use of immunosuppressive agents [5]. These include corticosteroids, cytotoxic agents, and cyclosporine. Patients with Behçet's disease also have an increased tendency for graft thrombosis. Some authors recommend anticoagulation, but anticoagulation also increases the risk of pseudoaneurysm formation [6]. There is little experience about the surgical approach for coronary arteries in patients with Behçet's disease. We performed a similar surgical technique for repair of pseudo aneurysm as reported by Rolland and colleagues [7]. We did not perform coronary bypass grafting to the right coronary artery because of the high risk of pseudo aneurysm formation as Ipek et al. [8] met in their case.
In conclusion, young patients presenting with any kind of arterial aneurysm should also be investigated for Behçet's disease among other etiologies. If possible, surgery should be avoided on acute exacerbation of disease with recurrence of major symptom complex and elevated CRP levels. Prompt medical treatment and close follow-up should accompany surgery. Careful manipulation of the aorta and steroid therapy may be useful in decreasing the possibility of aneurysm formation. No-touch techniques on the beating heart can be recommended if possible. Nevertheless, new experimental anastomotic techniques (laser, tissue glues) and endovascular techniques may be more appropriate for these patients.
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References
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Abstract
1. Introduction
