Oppression of Left Main Trunk Due to Pseudoaneurysm With Graft Detachment in Patients With Behcet Disease Previously Treated by Bentall Procedure
A 35-year-old Japanese man was admitted to our hospital because of congestive heart failure in January 2006. Echocardiography showed severe aortic regurgitation and partial ruptured aneurysm of the Valsalva sinus. Because he had already presented oral aphthae, uvetis, skin lesions with bouton, and genital ulceration, this case was diagnosed as a complete form of Behcet disease and the Bentall operation was performed. He was discharged without any complications. However, he experienced chest pain with ST-segment depression 3 months after the Bentall operation although no significant stenosis could be seen by preoperative coronary angiography in January 2006. This patient had III/VI systolic murmur of intensity of Levine on the third left sternal border. Transthoracic and transesophageal echocardiography revealed the echo-free space around the aortic graft and an abnormal jet from the left ventricular outflow to that echo-free space (online-only Data Supplement Movies I and II). Multislice computed tomography showed the development of multiple pseudoaneurysms around the aortic graft, which oppressed the left main trunk and caused severe stenosis of the left main trunk (Figure 1A and 1B). Because he had progressive angina with severe ST-segment changes (Figure 2), the Bentall operation was reperformed on an urgent basis, in addition to coronary artery bypass grafting. The aortic graft was detached at the right coronary cusp and multiple pseudoaneurysms were seen at the right and left coronary cusps. In addition, a pseudoaneurysm at the left coronary cusp oppressed the left main trunk, and severe stenosis in the left main trunk was observed. After the previously implanted composite graft was resected, new composite graft was implanted into the annulus. The left coronary ostia was reimplanted using the button technique, and coronary artery bypass grafting was performed for the right coronary artery. Histological examination of the explanted synthetic graft of the aortic root revealed poor organization with severe inflammatory cell infiltration such as neutrophils and macrophages (Figure 3). Numerous Gram-positive cocci were also recognized in the graft (Figure 3).
Graft detachment or pseudoaneurysm after the Bentall operation in Behcet disease occurs in 20% to 40% of cases.1,2 However, there are no case reports like the present case. In this case, the inflammation level was not satisfactorily controlled and the C-reactive protein level on admission was high (9.6 mg/dL). Therefore, inflammation leading to fragility of the aortic wall seems to cause these rare complications. After reoperation and strict control of inflammation using methylprednisolone, the patient had no recurrence of psuedoaneurysm formation during a 2-year follow-up period.
We would like to thank Mrs Hiromi Maeda for her excellent secretarial assistance, and the nurses and residents in the coronary care unit of the National Cardiovascular Center for their cooperation.
The online-only Data Supplement is available with this article at http://circ.ahajournals.org/cgi/content/full/119/21/2528/DC1.