scholarly journals Behçet's Disease: Successful Aortic Root Reconstruction in Severely Dilated Aortoventricular Junction After Aortic Valve Replacement With Novel Surgical Method - a Case Report

2020 ◽  
Author(s):  
Miklós Pólos ◽  
Ádám Koppányi ◽  
Kálmán Benke ◽  
László Daróczi ◽  
Attila Oláh ◽  
...  

Abstract Background: Behçet’s disease is an auto-inflammatory disorder categorized as a primer systemic vasculitis of unknown aetiology. Genetic factors, infectious agents and the irregularity of T-cell homeostasis are presumed to be responsible for the emergence of Behçet’s disease. Characteristic symptoms are multisystemic. Although cardiovascular involvement is rare, it should be noted due to the difficulty of surgical treatment options.Case Presentation: Our 44-year-old male patient underwent aortic valve replacement due to aortic regurgitation. At the 15-month follow-up, echocardiography showed detachment of the prosthetic valve and in the aortic root, multiple pseudo-aneurysms were identified. We performed an aortic root reconstruction with a Bentall procedure using a special „skirted” conduit to reduce strain in the suture line between the conduit and the extremely dilated left ventricular outflow tract. Conclusions: The surgical treatment of cardiovascular manifestations of Behçet’s disease remains challenging. This new technique may be beneficial in well-selected cases where the annulus of the aorta is extremely dilated or annular tissue disorder is present.

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Miklós Pólos ◽  
Ádám Koppányi ◽  
Kálmán Benke ◽  
László Daróczi ◽  
Attila Oláh ◽  
...  

Abstract Background Behçet’s disease is an auto-inflammatory disorder categorized as a primer systemic vasculitis of unknown aetiology. Genetic factors, infectious agents and the irregularity of T-cell homeostasis are presumed to be responsible for the emergence of Behçet’s disease. Characteristic symptoms are multisystemic. Although cardiovascular involvement is rare, it should be noted due to the difficulty of surgical treatment options. Case presentation Our 44-year-old male patient underwent aortic valve replacement due to aortic regurgitation. At the 15-month follow-up, echocardiography showed detachment of the prosthetic valve and in the aortic root, multiple pseudo-aneurysms were identified. We performed an aortic root reconstruction with a Bentall procedure using a special „skirted” conduit to reduce strain in the suture line between the conduit and the extremely dilated left ventricular outflow tract. Conclusions The surgical treatment of cardiovascular manifestations of Behçet’s disease remains challenging. This new technique may be beneficial in well-selected cases where the annulus of the aorta is extremely dilated or annular tissue disorder is present.


BMC Surgery ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Xiaoli Qin ◽  
Weitao Liang ◽  
Honghua Yue ◽  
Zhong Wu

Abstract Background Prosthetic valve detachment is not rare after aortic valve replacement in Behcet’s disease. However, destruction of the intervalvular fibrous body (IFB) due to Behcet’s disease was rarely reported. Case presentation We report a case of 30 year-old woman, with valve detachment and IFB separation. The patient received aortic valve replacement three months ago. Her medical history included recurrent oral ulcers and cutaneous lesions. Finally, reoperation was performed and peri-operative steroid therapy was carried out. Conclusions The case presented a rare complication of valve detachment in Behcet’s disease.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1207.1-1207
Author(s):  
L. Sun ◽  
J. Liu ◽  
W. Zheng

Background:Cardiovascular involvement in Behçet’s syndrome (BS) is associated with poor prognosis and accounts for the leading cause of mortality and morbidity in BS1, 2. Perioperative management of severe aortic valve regurgitation (AR) caused by BS is critical while challenging. AR caused by BS is associated with a high incidence of severe postoperative complications, especially paravalvular leakage (PVL) due to the fragilities of aortic lesions and tissue inflammation, leading to a significantly increased risk of re-operation and life-threatening conditions3-5. For the perioperative management of patients with AR caused by BS, many patients respond inadequately to the combination therapies of glucocorticoids (GCs) and immunosuppressants. Meanwhile, rapid and efficient control of inflammation is critical in preserving cardiac function and surgical management. However, the application of biologics in the perioperative treatment of AR attributable to BS has not yet been evaluated.Objectives:To investigate the efficacy and safety of biologics in the perioperative management of severe AR caused by BS.Methods:We retrospectively analyzed twenty patients with severe AR caused by BS and were treated with biologics during the perioperative cardiac surgeries in our center between February 2016 and October 2020.Results:Twenty patients with severe AR were enrolled, including 19 males and 1 female, with a mean age of 39.1±8.8 years and median course 8 (IQR, 5.25-10) years. Before biologics administration, 92.9% of the patients who underwent aortic valve replacement surgeries had failed conventional therapy and developed postoperative paravalvular leakage (PVL) at a median interval of 4 months. Biologics was administered during the perioperative period of 22 aortic valve surgeries, including preoperatively with a median interval of 3.5 (IQR, 2.75-4.25) months in 13 cases, or within three-month postoperatively in 9 cases, with background glucocorticoids (GCs) and immunosuppressants. After a median follow-up of 21 (IQR, 15-32) months, 11 out of 13 cases (84.6%) preoperatively and 8 out of 9 cases (88.9%) postoperatively treated with biologics were event-free. The BDCAF score improved significantly (7 vs. 0, median, p<0.0001). Significant decrease of ESR (25.0 (IQR, 11-36.25) mm/h vs. 6.5 (IQR, 4-8.8) mm/h, p<0.001), and CRP (20.77 (IQR, 7.19-29.58) mg/L vs. 1.53 (IQR, 0.94-2.92) mg/L, p=0.001) were achieved rapidly and effectively. The dosage of GCs tapered from 40 (IQR, 30-60) mg/d to 10 (IQR, 5-11.25) mg/d, p<0.0001. Immunosuppressants were tapered in number and dosage in 6 (30%) and 20 patients (100%), respectively. No serious adverse event was observed.Conclusion:Our study suggests that biologics was effective and well-tolerated for the perioperative management of severe and refractory AR caused by BS, which significantly reduced the occurrence of postoperative PVL and had a favorable GCs- and immunosuppressants- sparing effect.References:[1]Saadoun D, Wechsler B, Desseaux K, et al. Mortality in Behcet’s disease. Arthritis Rheum 2010; 62: 2806-2812.[2]Thomas T, Chandan JS, Subramanian A, et al. Epidemiology, morbidity and mortality in Behcet’s disease: a cohort study using The Health Improvement Network (THIN). Rheumatology (Oxford) 2020; 59: 2785-2795.[3]Ando M, Kosakai Y, Okita Y, et al. Surgical treatment of Behcet’s disease involving aortic regurgitation. Ann Thorac Surg 1999; 68: 2136-2140.[4]Han JK, Kim HK, Kim YJ, et al. Behcet’s disease as a frequently unrecognized cause of aortic regurgitation: suggestive and misleading echocardiography findings. J Am Soc Echocardiogr 2009; 22: 1269-1274.[5]Guo X, Tian Z, Liu Y, et al. Preoperative immunosuppressive therapy reduces paravalvular leakage after aortic valve surgery in patients with aortic regurgitation attributable to Behcet’s disease. Clin Exp Rheumatol 2016; 34: S26-S33.Disclosure of Interests:None declared


2021 ◽  
Vol 12 ◽  
Author(s):  
Lanlan Li ◽  
Yang Liu ◽  
Ping Jin ◽  
Jiayou Tang ◽  
Linhe Lu ◽  
...  

ObjectOur goal was to assess the implant depth of a Venus-A prosthesis during transcatheter aortic valve replacement (TAVR) when the areas of eccentric calcification were distributed in different sections of the aortic valve.MethodsA total of 53 patients with eccentric calcification of the aortic valve who underwent TAVR with a Venus-A prosthesis from January 2018 to November 2019 were retrospectively analyzed. The patients were divided into three groups (A, B, and C) according to the location of the eccentric calcification, which was determined by preprocedural computerized tomography angiography (CTA) images. The prosthesis release process and position were evaluated by contrast aortography during TAVR, and the differences in valve implant depths were compared among the three groups. The effects of different aortic root structures and procedural strategies on prosthesis implant depth were analyzed.ResultsEleven patients had eccentric calcification in region A; 19 patients, in region B; and 23 patients, in region C. The patients with eccentric calcification in region B had a higher risk of prosthesis migration (10.5% upward and 21.1% downward), and the position of the prosthesis after TAVR in group B was the deepest among the three groups. When eccentric calcification was located in region A or C, the prosthesis was released at the standard position with more stability, and the location of the prosthesis was less deep after TAVR (region A: 4.12 ± 3.4 mm; region B: 10.2 ± 5.3 mm; region C: 8.4 ± 4.0 mm; region A vs. region B, P = 0.0004; region C vs. region B; and P = 0.0360). In addition, the left ventricular outflow tract (LVOT) (P = 0.0213) and aortic root angulation (P = 0.0263) also had a significant effect on implant depth in the aortic root structure of the patients. The prosthesis size was 28.3 ± 2.4 in the deep implant group and 26.4 ± 2.0 in the appropriate implant group (P = 0.0068).ConclusionThe implant depth of the Venus-A prosthesis is closely related to the distribution of eccentric calcification in the aortic valve during TAVR. Surgeons should adjust the surgical strategy according to aortic root morphology to prevent prosthesis migration.


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