Perfusion Strategies in Rare Diseases: Behçet Syndrome.

Abstract

Behçet's syndrome is considered a rare disease, it is a chronic inflammatory disorder of unknown origin, it affects veins and arteries of all sizes causing an alteration of endothelial function ' and it is expressed clinically with organic lesions at various levels. Genetic, microbial and immunological factors are involved in pathophysiology. The most common symptoms are oral, ocular, genital ulcers, skin lesions, and arthritis. Case presentation: 52-year-old female patient with a history of Behçet's syndrome, severe mitral valve regurgitation, significant decrease in functional class, rheumatic fever, hypothyroidism, uveitis, and ventricular arrhythmias treated with radioablation. Mitral plasty was performed with 34 mm ring and triangular resection of the P2 segment plus chordal resection. Extracorporeal circulation time: 133 min, Aortic clamping time: 97 min, antinflamatory measures were adopted during the conduct of cardiopulmonary bypass, the patient was successfully discharged 7 days after the procedure. Conclusion: Behçet's syndrome is an entity that affects blood vessels, the aortic valve, coronary arteries and the cardiac chambers. The use of appropriate techniques that involve less time of exposure to extracorporeal circulation, and strategies aimed at reducing the inflammatory response and blood losses will significantly reduce morbidity and mortality in these cases.