Aortic Root Replacement in Behçet Disease

Abstract

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.

Cardiac manifestations in Behcet's disease

“…These aneurysms are isolable, and most are evident together with coronary stenosis and are sometimes seen together with several arterial aneurysms (30,31). Sinus Valsalva aneurysms and aortitis are the most frequently reported cardiac complications, with BD particularly involving the root of the aorta (31)(32)(33). Sinus Valsalva aneurysms may be seen alone or with other sinus aneurysms, and may lead to acute or chronic aortic failure.…”

Aortic Regurgitation Caused by Behçet's Disease: Surgical Experience during an 11-Year Period

“…Surgical treatment for AR in Behçet's disease carries a high risk of postoperative morbidity and mortality as a result of valve dehiscence in patients receiving simple aortic valve replacement (AVR), due to the fragilities of aortic structures and tissue inflammation, 6, 10,11 necessitating a second or third operation. Surgical experience with AR in Behçet's disease is limited 6,7,10 , 12–20 . In this study, we reviewed our institutional experience of surgical treatment for patients with AR caused by Behçet's disease during an 11‐year period.…”

Behçet's Disease, Echocardiographers, and Cardiac Surgeons: Together is Better

“…Sinus of Valsalva aneurysms and aortitis are the most frequently reported cardiac complications with BD. Aortitis involves particularly the root of the aorta . A study detected aneurysmal dilatation of the aorta in up to 48% of the cases with BD examined by echocardiography, while other reported a frequency varying between 5% and 30% .…”

“…The diagnosis is established by echocardiography, aortography, ventriculography, and CT. Echocardiographic findings of marked thinning and redundancy of aortic leaflets, with or without mobile masses and/or echo free space within the annulus, may be pathognomonic of BD . Treatment frequently requires root replacement and not only conventional aortic valve replacement irrespective of aortic size . It is to be noted, however, that because of tissue fragility, surgical treatment for aortic regurgitation in BD can be associated with recurrence due to valvular detachment and prosthetic dehiscence .…”

“…Aortitis involves particularly the root of the aorta. [41][42][43] A study detected aneurysmal dilatation of the aorta in up to 48% of the cases with BD examined by echocardiography, 44 while other reported a frequency varying between 5% and 30%. 45 Aortic root dilatation could be an early stage of aneurysms in this disorder (Fig.…”