Ma Xiao-jing, Huang Guo-ying, Liang Xue-cun, Liu Xiao-qin, Jia Bing
Children's Hospital of Fudan University, Shanghai, China, 399 Wan Yuan Road, Shanghai, 201102, People's Republic of China.
Pediatr Cardiol. 2011 Apr;32(4):442-8. doi: 10.1007/s00246-011-9886-y. Epub 2011 Jan 29.
Shone's complex is a rare and severe type of congenital left-sided obstructive lesion of multiple cardiovascular levels. This report aims to present the authors' experience diagnosing atypical Shone's complex using echocardiography. Atypical Shone's complex was diagnosed for 38 consecutive patients (18 boys and 20 girls, ages 1 day to 15 years) using echocardiography. All the diagnoses were confirmed by cardioangiography, surgery, or both. Among the congenital left-sided obstructive lesions, four levels of obstruction coexisted in 3 cases (7.9%), three levels in 10 cases (26.3%), and two levels in 25 cases (65.8%). Supra-annular mitral stenosis caused by a supravalvular mitral ring was diagnosed in 1 case. Mitral valvular stenosis was diagnosed in 24 cases including a parachute mitral valve (4 cases), a double-orifice mitral valve (1 case), and partly fused thickened mitral valvular leaflets (19 cases). Subaortic stenosis was diagnosed in 10 cases including circumferential membranous subaortic stenosis (1 case), tunnel-type subaortic narrowing (2 cases), and asymmetric obstructive fibrous ridge below the aortic valve (7 cases). Aortic stenosis was diagnosed in 25 cases including the bicuspid aortic valve (12 cases), the hypoplasia aortic annulus (3 cases), and partly fused or thickened tri-leaflet aortic valves (10 cases). Supra-aortic stenosis was diagnosed in 6 cases including the localized type (4 cases) and the diffused type (2 cases). Coarctation of the aorta was diagnosed in 26 cases. Of these, focal coarctation was described in 15 cases, long segment coarctation in 7 cases, and dysplastic isthmus and/or descending aorta in 4 cases. Secondary changes in Shone's complex manifested as left atrial dilation (26 cases), left ventricular hypertrophy (26 cases), and pulmonary hypertension (19 cases). Echocardiography plays an important role in the diagnosis of atypical Shone's complex, in comprehensive evaluation of the number, position, morphology, and severity of left-sided obstructions.
肖恩综合征是一种罕见且严重的先天性左侧多心血管水平梗阻性病变。本报告旨在介绍作者使用超声心动图诊断非典型肖恩综合征的经验。通过超声心动图对38例连续患者（18例男孩和20例女孩，年龄1天至15岁）诊断为非典型肖恩综合征。所有诊断均经心血管造影、手术或两者证实。在先天性左侧梗阻性病变中，4级梗阻共存3例（7.9%），3级梗阻10例（26.3%），2级梗阻25例（65.8%）。1例诊断为瓣上二尖瓣环导致的瓣上二尖瓣狭窄。二尖瓣狭窄诊断24例，包括降落伞样二尖瓣（4例）、双孔二尖瓣（1例）和部分融合增厚的二尖瓣叶（19例）。主动脉瓣下狭窄诊断10例，包括环周膜性主动脉瓣下狭窄（1例）、隧道型主动脉瓣下狭窄（2例）和主动脉瓣下方不对称梗阻性纤维嵴（7例）。主动脉狭窄诊断25例，包括二叶式主动脉瓣（12例）、主动脉瓣环发育不全（3例）和部分融合或增厚的三叶式主动脉瓣（10例）。主动脉上狭窄诊断6例，包括局限性类型（4例）和弥漫性类型（2例）。主动脉缩窄诊断26例。其中，局灶性缩窄15例，长节段缩窄7例，发育不良的峡部和/或降主动脉4例。肖恩综合征的继发性改变表现为左心房扩大（26例）、左心室肥厚（26例）和肺动脉高压（19例）。超声心动图在非典型肖恩综合征的诊断以及对左侧梗阻的数量、位置、形态和严重程度的综合评估中发挥着重要作用。
