Surgical Experiences of Shone's Syndrome

Won, Tae Hee; Lee, Jeong Ryul; Kim, Yong Jin; Rho, Joon Ryang

Abstract

Shone's syndrome is a congenital cardiac malformation that consists of multiple levels of left heart obstruction including supravalvular mitral ring, congenital mitral stenosis(parachute mitral valve), subaortic stenosis, and coarctation of aorta. This syndrome is a very rare congenital anomaly and its prognosis is poor. We experienced 9 patients with Shone's syndrome between 1985 and 1994. There were 8 male and 1 female patients, and mean age was 33.0+/-31.0 months ranged from 2 months to 11 years. The congenital mitral stenosis and coarctation of aorta existed in all patients and the supravalvular mitral ring and subaortic stenosis in 4 patients. Two patients had all four anatomic lesions. 3 patients underwent one stage total correction and the other 6 patients underwent two staged operation that was initial coarctoplasty with thoracotomy and later correction of intracardiac anomalies with median sternotomy. A third operation was performed in 2 patients. These procedures included reoperation for coarctation and replacement of mitral valve for persistent mitral stenosis. There was no operative death at the first operation but two operative deaths at the second operation. The cause of death in two cases was severe heart failure secondary to left ventricular hypoplasia. There was no operative death at the third operation. The seven survivors have been followed from 11 months to 12 years(mean follow-up : 6.7+/-3.6 years). There was no late death and the New York Heart Association activity level was class I for all patients. We conclude that a good long-term outcome can be expected by proper surgical treatment tailored to each individual's anatomy and pathophysiology although the operative mortality and morbidity of Shone's syndrome are high.