Roche K J, Genieser N B, Ambrosino M M, Henry G L
Department of Radiology, New York University Medical Center, Tisch Hospital, Rm. 236, 560 First Ave., New York, NY 10016, USA.
Pediatr Radiol. 1998 Nov;28(11):841-5. doi: 10.1007/s002470050478.
Shone's complex is a series of four obstructive or potentially obstructive left-sided cardiac lesions (supravalvular mitral ring, parachute deformity of the mitral value, subaortic stenosis, and coarctation of the aorta). Both the complete form (all four lesions) and incomplete forms (less than four lesions) have been described.
To determine which abnormalities of Shone's complex could be characterized by MR.
MR examinations in three patients (one complete, two incomplete) were retrospectively reviewed.
A supravalvular mitral ring, found at surgery in one patient, was not identified. Regurgitant and stenotic flow across the mitral valve, abnormal motion of the valve leaflets and abnormalities of the papillary muscles were identified. Individual chordal attachments were difficult to resolve. Narrowing in the subaortic region and abnormal flow from the subaortic region through the valve plane were demonstrated. A discrete subaortic diaphragm in one patient was not resolved. Both focal and diffuse types of coarctation of the aorta were well characterized.
MR imaging is suited to evaluation of patients with Shone's complex. Individual chordal attachments and thin diaphragms of the mitral and aortic valves were difficult to resolve.
肖恩综合征是一系列四种阻塞性或潜在阻塞性的左侧心脏病变（二尖瓣上环形狭窄、二尖瓣降落伞样畸形、主动脉瓣下狭窄和主动脉缩窄）。完整形式（所有四种病变）和不完整形式（少于四种病变）均有描述。
确定肖恩综合征的哪些异常可通过磁共振成像（MR）来表征。
回顾性分析了3例患者（1例完整型，2例不完整型）的MR检查结果。
1例患者手术中发现的二尖瓣上环形狭窄未被识别。发现二尖瓣反流和狭窄血流、瓣叶异常运动及乳头肌异常。单个腱索附着难以分辨。显示主动脉瓣下区域变窄以及主动脉瓣下区域通过瓣膜平面的异常血流。1例患者的离散型主动脉瓣下隔膜未分辨清楚。主动脉缩窄的局灶型和弥漫型均得到很好的表征。
MR成像适用于肖恩综合征患者的评估。二尖瓣和主动脉瓣的单个腱索附着及薄隔膜难以分辨。
