Echocardiographic characteristics and clinical features of cardiac involvement in Behçet's disease: a retrospective single-center case series.

Background: Cardiac involvement in Behçet's disease (BD) is uncommon but potentially life-threatening and may mimic other cardiovascular disorders, complicating early diagnosis. This study aimed to characterize the clinical and echocardiographic features, treatment, pathological findings, and clinical outcomes of patients with cardiac BD, with particular emphasis on the utility of echocardiography in raising diagnostic suspicion. Methods: We retrospectively screened 186 patients with diagnosed or suspected BD at The Second Affiliated Hospital of Jiangxi Medical College, Nanchang University between October 2020 and January 2026. Of the 112 patients who underwent transthoracic echocardiography, 23 met the International Criteria for BD and were determined by multidisciplinary evaluation to have cardiac lesions attributable to BD after exclusion of alternative etiologies. Clinical, laboratory, electrocardiographic, echocardiographic, pathological, treatment, and follow-up data were descriptively analyzed. Results: The cohort was predominantly male (82.6%), with a mean age of 43.5±13.1 years. The most frequent systemic manifestations were oral ulcers (95.7%), cutaneous lesions (73.9%), and genital ulcers (60.9%). Elevated inflammatory markers, including erythrocyte sedimentation rate (87.0%) and C-reactive protein (82.6%), were frequently observed. B-type natriuretic peptide levels were elevated in 91.3% of patients. Among the 19 patients without a history of cardiac surgery, severe aortic regurgitation was the predominant echocardiographic finding (94.7%) and was most often associated with aortic valve prolapse (78.9%). Other echocardiographic findings included aortic cusp aneurysm with perforation (15.8%), echo-free spaces around the aortic root (21.1%), aortic root and/or ascending aortic dilatation (63.2%), pulmonary artery dilatation (52.6%), left-sided chamber enlargement (84.2%), four-chamber enlargement (10.5%), and reduced left ventricular ejection fraction (21.1%). Among the four patients with a history of cardiac or great-vessel surgery, all three who had undergone aortic valve replacement developed prosthetic valve dehiscence with severe paravalvular leakage within 2-6 months of surgery. Pathological examination of 14 surgical specimens revealed inflammatory-destructive changes involving the aortic wall and valve, characterized by fibrosis, hyalinization, myxoid degeneration, and chronic inflammatory cell infiltration. All patients received glucocorticoid-based immunosuppressive therapy, and 17 also underwent surgical treatment. At the last follow-up, 16 of 23 patients were alive and clinically stable, four were lost to follow-up, and three had died. Conclusions: In this case series, cardiac BD was mainly characterized by destructive valvulo-aortic lesions, particularly severe aortic regurgitation associated with cusp prolapse and periannular or aortic root abnormalities. Echocardiography plays an important role in defining lesion morphology and severity, detecting postoperative prosthetic valve complications, and identifying structural abnormalities that may raise suspicion for cardiac BD in the appropriate clinical context.