Abstract
We report a rare case of Behçet's disease (BD) causing aortic regurgitation and complete atrioventricular block (AVB) in conjunction with pacemaker pocket infection. We emphasize the need to consider the possibility of BD in young and middle-aged patients who present with aortic valve prolapse with regurgitation combined with complete AVB with elevated inflammatory markers.
Key Words: aortic regurgitation, Behçet's disease, complete atrioventricular block, heart failure, pacemaker pocket infection, perivalvular leakage
Graphical Abstract
Behçet's disease (BD) is a progressive diffuse inflammatory vasculitis characterized by recurrent oral and genital ulcers and ocular inflammation. Cardiac involvement is a rare complication of BD. Two cases of BD causing aortic regurgitation (AR) as well as complete atrioventricular block (AVB) have been reported previously; however, in this case report, we describe a case in which BD not only caused AR and complete AVB, but also coexisted with pacemaker pocket infection.
Take-Home Messages
-
•
The possibility of systemic immune-inflammatory–mediated diseases must be considered in young and middle-aged individuals presenting with cardiac multitissue structures such as valvular, conduction system, and myocardium involvement.
-
•
BD has an insidious presentation, lacks characteristic laboratory tests, and cardiac involvement is rare.
History of Presentation
A 46-year-old man presented to our hospital with dyspnea persisting for 2 weeks. Echocardiography revealed severe AR (Figure 1).
Figure 1.
Aortic Regurgitation
Past Medical History
The patient had a medical history of hypertension and an old cerebral infarction.
Investigations
Upon admission, comprehensive arterial computed tomography angiography showed no significant abnormalities in the aorta. However, a small aneurysm was detected in the left coronary sinus, and a severe stenosis of the left posterior cerebral artery was observed. Notably, the autoantibodies were within normal ranges. On the third day of hospitalization, he underwent thoracoscopic-assisted aortic valve replacement surgery, which proceeded smoothly. Postoperative pathology indicated mucoid degeneration, hyaline degeneration, and degenerative changes. The patient recovered well and was discharged after 2 weeks.
Forty-five days postsurgery, he was readmitted with palpitations. Holter monitoring detected second-degree type I and type II AVB (2:1), along with intermittent incomplete right bundle branch block. Electrophysiological testing localized the block to the atrioventricular node. During hospitalization, the patient experienced recurrent low-grade fevers, peaking at 38 °C, with normal white blood cell count. Despite multiple negative blood cultures and pathogenic screenings, levels of C-reactive protein and erythrocyte sedimentation rate remained elevated. Repeated inquiries regarding oral and genital aphthosis were made, and the patient described recurrent episodes more than 10 years prior, with no occurrences in the past 5 years. He also had a history of folliculitis. These findings prompted us to consider a diagnosis of BD. However, at that point in time, some results were still pending, and therefore, glucocorticoid and immunosuppressive therapy had not yet been initiated. Two months later, the patient had an acute exacerbation of heart failure and rapidly developed complete AVB within 24 hours of admission (Figure 2). An emergency temporary pacemaker was implanted, and he was transferred to the intensive care unit. Subsequent echocardiography indicated improper fixation of the bioprosthetic aortic valve and perivalvular leakage (Video 1).
Figure 2.
Complete Atrioventricular Block
Differential Diagnosis
When considering the differential diagnosis, it is important to include infective endocarditis and immune-inflammatory response due to BD. In this particular case, the patient did not exhibit any symptoms or signs at the site of infection, nor were there any vascular or immune-related signs. Additionally, repeated blood cultures came back negative. In light of these findings, only 2 secondary criteria were met: the presence of fever and a history of heart valve surgery. Taking into account the patient's history of BD, along with the presence of fever and poor fixation of the bioprosthetic aortic valve, it is reasonable to consider immune inflammation as a potential cause in this case.
Management
After subcutaneous injections with a tumor necrosis factor receptor-fusion protein, an emergency aortic valve and ascending aorta replacement, along with coronary artery bypass grafting (Bentall procedure) was performed, revealing pronounced inflammatory responses intraoperatively. Pathology reported vasculitis with features including disruption of the medial elastic lamina of the aortic wall tissue (Figure 3), microabscesses (Figure 4), and infiltration of inflammatory cells (Figure 5). Following this, a permanent pacemaker was implanted to pace the left bundle branch area, and the patient continued on regular treatment for BD postdischarge. Approximately 6 months after pacemaker implantation, the patient experienced redness, swelling, and ulceration at the pacemaker wound site (Figure 6). Vancomycin was administered for infection, and BD-related treatments were temporarily halted. The pacemaker was removed 2 weeks later during debridement of the pacemaker pocket, and a Micra AV leadless pacemaker (Medtronic) was implanted concurrently.
Figure 3.
Disruption of the Medial Elastic Lamina of the Aortic Wall Tissue
Figure 4.
Microabscesses
Figure 5.
Infiltration of Inflammatory Cells
Figure 6.
Redness, Swelling, and Ulceration at the Pacemaker Wound Site
Outcome and Follow-Up
Currently, the patient has stable pacemaker and aortic valve function, controlled inflammatory indices, and presents no symptoms of heart failure.
Discussion
BD is an autoinflammatory disorder, often presenting insidiously, and lacks specific laboratory markers.1 The incidence and prevalence of BD is higher in areas along the Silk Road, with men affected more than women.2 It can cause multitissue structural damage to the heart.3 To date, only 2 cases of BD causing AR with complete AVB have been reported,4,5 and there have been no reported cases of combined pacemaker pocket infection.
Conclusions
Cardiac involvement is a rare complication of BD. In this case report, we describe a case in which BD caused not only AR and complete AVB, but also coexisted with pacemaker pocket infection.
Funding Support and Author Disclosures
This work was supported by grants from the Sanming Project of Medicine in Shenzhen, China (No. SZSM201911020), and the HKU-SZH Fund for Shenzhen Key Medical Disciplines (No. SZXK2020081). The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
Footnotes
The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the Author Center.
Appendix
For a supplemental video, please see the online version of this paper.
Contributor Information
Mingya Liu, Email: liumy@hku-szh.org.
Kai-Hang Yiu, Email: khkyiu@hku.hk.
Appendix
Poor fixation of the bioprosthetic aortic valve
References
- 1.Salehi-Abari I. 2015 Persian Gulf criteria for early diagnosis of Behcet’s disease. Open Sci J Clin Med. 2015;5(3):188–190. [Google Scholar]
- 2.di Meo N., Bergamo S., Vidimari P., Bonin S., Trevisan G. Analysis of diagnostic criteria in Adamantiades-Behçet disease: a retrospective study. Indian J Dermatol. 2013;58(4):275–277. doi: 10.4103/0019-5154.113936. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 3.Shadmanfar S., Masoumi M., Davatchi F., et al. Cardiac manifestations in Iranian patients with Behcet's disease. J Tehran Heart Cent. 2021;16(3):109–112. doi: 10.18502/jthc.v16i3.8187. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 4.Or I., Roguin N., Yahalom M., Birjiniuk V., Amikam S. Pacemaker implantation in a patient with Behçet’s disease associated with superior vena cava obstruction. Cardiovasc Intervent Radiol. 1986;9(1):13–14. doi: 10.1007/BF02576975. [DOI] [PubMed] [Google Scholar]
- 5.Yu J.S., Cho E.J., Ji E.H., et al. Rapidly progressive cardiac manifestation of Behcet's disease involving conduction system and aortic valve. J Cardiovasc Ultrasound. 2011;19(4):199–202. doi: 10.4250/jcu.2011.19.4.199. [DOI] [PMC free article] [PubMed] [Google Scholar]
Associated Data
This section collects any data citations, data availability statements, or supplementary materials included in this article.
Supplementary Materials
Poor fixation of the bioprosthetic aortic valve