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Behcet's disease (BD) is an immune system disease characterized by multi-system vascular inflammation. Its occurrence in patients who experience a Stanford type A aortic dissection (AD) is very rare, but extremely dangerous. A 44-year-old male patient was diagnosed with an acute Stanford type A AD and underwent a standard Bentall procedure and total aortic arch replacement plus descending aortic stented elephant trunk implantation. Aortic valve leakage and an aortic root pseudoaneurysm developed 3 months after surgery. At this time, we suspected that this patient had BD. After immunosuppressive treatment, we performed modified Bentall again; however, the heart failure occurred shortly after the second operation. Finally, we successfully treated this patient with a heart transplant. This is the first report of a heart transplant to treat BD with acute Stanford type A AD. In the diagnosis and treatment of acute Stanford type A AD, in addition to the traditional pathogenic factors, we need to be alert to BD, and heart transplantation may be a good way to treat such patients.

When Behcet’s disease meets Stanford type A aortic dissection, heart transplantation is a reliable treatment