Treatment outcomes in patients with non-infectious aortic valvulitis undergoing aortic valve replacement: implication for the treatment of aortic valve involvement in Behcet’s disease

Abstract

Behcet’s disease (BD) is characterized by inXammation in the media and adventitia of the large vessel and is well known to be one of the causes of aortic regurgitation (AR) [1, 2]. It can lead to unexpected results such as aortic valve replacement (AVR), aortic root replacement, or extensive debridement because these patients often show simultaneous inXammation of the ascending aorta, aortic branch, and aortic valve, which leads to subsequent AR development in up to 44% of patients [1, 3]. It has been reported that 50% of patients with BD aortitis undergoing AVR require re-operation due to aortic valve detachment or paravalvular leakage [4], while patients undergoing surgical treatment for primary AR have more favorable outcomes [5]. Controversy still exists in the literature regarding the best surgical interventions and medical therapies for the perioperative period in these patients. Thus, we examined ten patients who underwent AVR due to non-infectious aortic valvulitis/aortitis and who seemed likely to have BD. InXammatory aortic valvulitis/aortitis was recognized from the intraoperative gross appearance and/or pathologic Wndings of the aortic valve and aortic wall. The pathologic Wndings of the aortic valve and ascending aorta revealed chronic active inXammation, Wbrinoid necrosis, or Wbrosis without evidence of infection, which is similar to the pathologic Wndings of aortic valvulitis/aortitis in BD patients, as reported by Lee et al. [2]. No microorganisms were identiWed by blood or tissue culture in any patients. The clinical characteristics of the patients are shown in Table 1. The study group comprised nine men and one woman with a mean age of 41.5 (§10.5) years at the time of Wrst operation. The mean follow-up duration was 72.4 (§49.7) months. All cases had a recurrent oral ulcer, and a genital ulcer (50%) and skin lesion such as folliculitis were frequent manifestations. No evidence of ocular involvement was documented on examination. Laboratory data were available in 87.5% of patients at the time of surgery, showing that erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels were elevated to 31.29 (§21.09) mm/h and 1.84 (§1.83) mg/dl, respectively. These clinical features in our subjects were similar to those in BD patients. Alike our cases, many studies have found that BD is not easy to diagnose at the time of AVR because AR is a rare complication of BD and most patients do not manifest clinical symptoms and signs compatible with BD [2, 3, 6, 7]. A total of 26 operations were performed on the ten patients. All but one patient (patient 6) underwent re-operation (90%). Wound dehiscence developed following Wve operations (19.2%). The event-rate of paravalvular leakage and aortic valve detachment in BD patients with AR is known to be high [2, 4, 6, 8], while it has been reported that paravalvular leakage required re-operation in 0.1 events per 100 patient-years in a cohort of 816 consecutive, unselected patients with primary aortic valve disease [5]. Our patients also experienced frequent development of paravalvular leakage and valve dehiscence. J. K. Ahn · H. Kim · J. Lee · E.-M. Koh · H.-S. Cha (&) Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea e-mail: hoonsuk.cha@samsung.com