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Behcet's disease and heart transplantation: a word of caution
Seth A Hollander1, Jessica R Yasnovsky, Olaf Reinhartz
1Department of Pediatrics (Cardiology), Lucile Salter Packard Children's Hospital, Stanford University Medical Center, 750 Welch Road, Palo Alto, CA 94304, USA. seth.hollander@stanford.edu
Insights
This study reports the first successful heart transplant in a patient with Behcet's disease, achieving over one year of survival. However, recurrent vascular issues remain a concern for future cardiac transplant candidates with this autoimmune condition.
Area of Science:
- Cardiology
- Immunology
- Vascular Surgery
Background:
- Behcet's disease is a rare autoimmune disorder causing oral/genital ulcers, arthritis, neurological issues, and vasculitis.
- Vascular complications like aneurysms and thrombosis, along with a heightened inflammatory response (pathergy), pose significant surgical risks.
- Cardiac transplantation has been considered contraindicated in Behcet's disease due to the complexity of anastomoses and potential for vascular complications.
Observation:
- This case study details the first orthotopic heart transplant performed in a patient diagnosed with Behcet's disease.
- The procedure involved complex atrial and large-vessel anastomoses, typically challenging in patients with this condition.
- Despite significant complications, the patient achieved survival exceeding one year post-transplant.
Findings:
- The study demonstrates that cardiac transplantation is achievable in select Behcet's disease patients.
- Major complications were encountered, highlighting the inherent risks associated with the procedure in this patient population.
- Long-term success remains uncertain due to the underlying propensity for recurrent vascular pathology in Behcet's disease.
Implications:
- This pioneering case offers a glimmer of hope for Behcet's disease patients requiring advanced cardiac interventions.
- Further research and advancements in managing vascular inflammation are crucial for improving outcomes of cardiac transplantation in these patients.
- The findings underscore the need for innovative strategies to mitigate the risk of aneurysm recurrence and thrombosis after major vascular surgery in Behcet's disease.
Abstract:
Behcet's disease is a rare autoimmune disease characterized by oral and genital ulcers, and by multisystem disease, including arthritis, neurologic complications and vasculitis. Large-vessel and coronary artery aneurysms are often an indication for surgery, but the return of aneurysms, thrombosis, and the tendency to exhibit an exaggerated inflammatory response at puncture sites (pathergy) complicate surgical recovery. As such, cardiac transplantation, which requires atrial and large-vessel anastomoses, has not been reported in patients with Behcet's disease. We report the first orthotopic heart transplant with >1-year survival in a patient with Behcet's disease despite major complications. The investigators remain pessimistic about cardiac transplantation in patients with Behcet's disease until advances in preventing recurrent vascular pathology ensue.
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