Disease characteristics and surgical effect of 22 children with Shone′s syndrome / 中华实用儿科临床杂志

Objective:To analyze the characteristics and surgical outcomes of Shone′s syndrome in children, and to explore the surgical treatment strategy and technical key.Methods:Retrospective study.Children with Shone′s syndrome treated in the Pediatric Heart Center, Beijing Anzhen Hospital, Capital Medical University from May 2013 to June 2019 were retrospectively analyzed for their baseline characteristics and surgical data.The data were compared by Student t-test. Results:A total of 22 children with Shone′s syndrome were recruited, involving 15 males (68.2%) and 7 females (31.8%). There were 6 cases (27.3%) of complete form of Shone′s syndrome and 16 cases (72.7%) of incomplete form.No deaths were reported.The postoperative mitral valve velocity [(149.7±38.2) cm/s vs.(234.9±34.0) cm/s, t=7.341, P<0.05], left ventricular outflow tract velocity [(202.0±105.0) cm/s vs.(328.6±120.3) cm/s, t=6.575, P<0.05] and aortic arch coarctation velocity [(186.1±60.9) cm/s vs.(347.9±100.8) cm/s, t=7.630, P<0.05]were significantly lower than those of preoperative levels.There were no complications occurred at 1-year follow-up, and 91.7% of the patients were followed up for 3 years, and 80.2% were followed up for 5 years without complications, 2 cases needed reoperation. Conclusions:Surgical treatment of Shone′s syndrome achieved satisfactory outcomes.Early diagnosis and early intervention are beneficial to children with Shone′s syndrome, although they need to be followed up and have the risk of reoperation in the long term.

Síndrome de Shone incompleto: a propósito de un caso diagnosticado en la adultez / Incomplete Shone's syndrome. Apropos of a case diagnosed in adulthood

El síndrome de Shone constituye una rara entidad cardíaca congénita que consiste en la presencia de lesiones estructurales obliterantes de hemicardio izquierdo que incluyen: anillo supravalvular, válvula mitral en paracaídas, estenosis sub-aórtica y coartación aórtica. Es poco frecuente que curse en su forma incompleta. El caso que a continuación se presenta, describe el síndrome de Shone incompleto detectado en un adulto

Shone's syndrome is a rare congenital heart disease consisting in the presence of obliterating structural lesions on the left side of the heart, including supravalvular ring, parachute mitral valve, subaortic stenosis and aortic coarctation. The incomplete form of the disease is not common. The presentation describes a case of incomplete Shone's syndrome diagnosed in adulthood

Surgical Experiences of Shone's Syndrome / 대한흉부외과학회지

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.