HỘI CHỨNG SHONE - MỘT HỘI CHỨNG TIM BẨM SINH HIẾM GẶP

Abstract

Background. Shone syndrome (multilevel left heart obstructions with mitral valve involvement) is rare. The outcome of children with this disease is generally poor. This study reviews the results in 3 patients operated in our center.

 Material and method.A total of 3 patients with Shone syndrome underwent operated between 2/2010-7/2011. Male: 2, female: 1. Mean age: 6,5±1,2 ( range 5-7 years) Mean weight: 14,5±0,5 kg (range 14-15kg).All patients had mitral valve lesion: 2 with supravalvular mitral ring, 1 with parachute mitral valve. Surgical procedure: Resection of supramitral ring, mitral valve repair, resection of subaortic stenosis, aortic valve repair.

 Results: There were no perioperative death. 1 patient required implantation of a permanent pacemaker for postoperative complete heart block Mean follow up was 10,3 ±1,5 month (range 8- 12months ). One late death in patient with block AV.

Conclusion: The Shone’s complex is a rare entity, which occurs most frequently in its incomplete form, highlights the importance of a carefully examination because of lesions often coexist and can be missed in the absence of a comprehensive examination.. The outcome of children with Shone’s abnomaly is generally poor.