Behcet's disease and heart transplantation: a word of caution.
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.
Hollander
Seth A
SA
Department 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
Yasnovsky
Jessica R
JR
Reinhartz
Olaf
O
Chan
Frandics
F
Sandborg
Christy
C
Hunt
Sharon
S
Bernstein
Daniel
D
Chin
Clifford
C
D000293:Adolescent; D000783:Aneurysm / Q000209:etiology; D001528:Behcet Syndrome / Q000150:complications*; D000075202:Contraindications; D006333:Heart Failure / Q000209:etiology* / Q000601:surgery*; D016027:Heart Transplantation / Q000009:adverse effects; D006801:Humans; D006973:Hypertension / Q000209:etiology; D008297:Male; D051437:Renal Insufficiency / Q000209:etiology; D016896:Treatment Outcome