JACC Case Rep. 2026 May 20:108365. doi: 10.1016/j.jaccas.2026.108365. Online ahead of print.
ABSTRACT
BACKGROUND: Behçet disease (BD) is a multisystem vasculitis with variable cardiovascular involvement. Hughes-Stovin syndrome (HSS), featuring pulmonary artery aneurysms and venous thrombosis, is considered a rare variant within the BD spectrum. Intracardiac mass formation in either vasculitis is exceptionally rare.
CASE SUMMARY: A 32-year-old man with prior mucocutaneous ulcers presented with hemoptysis and chest pain. Multimodality imaging revealed pulmonary arterial thrombosis and lobulated intracardiac mass attached to the native tricuspid valve (TV). Histopathology showed inflammatory organizing hematoma. Despite anticoagulation and valve replacement, the patient developed pulmonary artery pseudoaneurysms and died from fatal hemorrhage.
DISCUSSION: Eight similar cases in the literature revealed a pattern of TV masses, pulmonary artery aneurysms, and hemoptysis in young male patients, suggesting a cardiac-predominant BD-HSS overlap syndrome.
TAKE-HOME MESSAGES: An under-recognized syndrome shares overlapping features of BD and HSS, a common denominator being unusual TV masses. In patients with pulmonary artery involvement, immunosuppressive therapy is critical, and anticoagulation requires caution given aneurysmal rupture risk.
PMID:42165773 | DOI:10.1016/j.jaccas.2026.108365