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Fatal hemorrhea due to ascending aorta pseudoaneurysm rupture after Bentall procedure in a patient with Behcet's disease

Zhang, Zhen; Wang, Zhenkang; Wang, Wujun

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doi: 10.3760/cma.j.issn.0366-6999.20131871
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To the editor: A 24-year-old symptomless man was admitted to local hospital for aortic valve regurgitation and ascending aorta aneurysm in August 2008, he received aortic valve and ascending aorta replacement and recovered well. In October 2009, he began to have progressive exertional dyspnoea, echocardiography showing prosthetic valve detached and severe paravalvular leakage. The patient ever had recurrent oval aphthae, genital ulcer and folliculitis, and blood analysis showed higher level of C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR); then he was diagnosed as Behcet's disease. He was transferred to our hospital and received Bentall procedure one week later. As we cannot find the ostia of right coronary atery, we only reimplanted left coronary atery with button technique. This patient survived from severe low cardiac output syndrome and was extubated on post operative day (POD) 5. CT scan showed right coronary ostia atresia and distal branch connected with left coronary artery by collaterals. Another complication was complete atrial-ventricle block. He was discharged after a permanent pace maker implantation.

In August 2012, he noticed a slowly growing pulsive soft mass at the sternal angle, and enhancement CT scan did not show anything remarkable. One month later, the mass ruptured with fatal hemorrhea when this patient was walking. The patient was unconsciousness and was sent to local hospital. After wound pressure dressing and blood transfusing of about 3000 ml, he was transferred to our hospital again. A 64-slice enhancement CT scan revealed small ascending aorta pseudoaneurysm just behind sternal angle protruded into sternum. The patient accepted covered stent implantation for isolation of pseudoaneurysm from aorta lumen and recovered well.

Behcet's disease mainly causes nonspecific vasculitis which may lead to aortic valve prolapse or perforation. Bentall procedure was regarded as the optimal choice for simple valve replacement, but may cause intolerable paravalve leakage.1,2 But graft detachment and pseudoaneurysms may occur in patients who ever accepted Bentall procedure.1-3 It is believed that recurrent local inflammation can cause poor healing between prosthetic valve or graft and aortic annular. If doctors wait long enough, perhaps more and more patients may encounter this complication. Yamamoto et al4 recommend the use of homograft for its better histocompatibility. This patient suffered from fatal hemorrhae caused by pseudoaneurysm on distal anastomosis. The reasons include osteoporosis due to long-term prednisone intake. The use of covered stent for intra-lumen isolation is a proximal choice for less invasiveness and safety.

Figure 1. A:
Figure 1. A::
Enhancment CT revealed pseudoaneurysm protruding into sternum (white arrow). B: Reconstructed picture revealed pseudoaneurysm protrude into stermum just behind sternal angle (white arrow). C: Right coronary ostia atresia, distal branch connected with left coronary artery by collaterals.


1. Ando M, Kosakai Y, Okita Y, Nakano K, Kitamura S. Surgical treatment of Behcet's disease involving aortic regurgitation.Ann Thorac Surg 1999; 68: 2136-2140.
2. Jeong DS, Kim KH, Kim JS, Ahn H. Long-term experience of surgical treatment for aortic reguigitation attributable to Behcet's disease. Ann Thorac Surg 2009; 87: 1775-1782.
3. Kataoka Y, Tsutsumi T, Ishibashi K, Higashi M, Morii I, Kawamura A, et al. Oppression of left main trunk due to pseudoaneurysm with graft detachment in patients with Behcet disease previously treated by bentall procedure. Circulation 2009; 119: 2858-2859.
4. Yamamoto T, Makuuchi H, Naruse Y, Kobayashi T, Goto M, Nonaka K. Aortic root replacement by crypreserved homograft for prosthetic valve detachment case due to aortitis. Jpn J Thorac Cardiovasc Surg 1998; 46: 565-569.
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