Kleinert S, Geva T
Lillie Frank Abercrombie Section of Pediatric Cardiology, Texas Children's Hospital, Houston 77030.
J Am Coll Cardiol. 1993 Nov 1;22(5):1501-8. doi: 10.1016/0735-1097(93)90563-g.
This study was designed to identify, by echocardiography, morphometric abnormalities of the left ventricular outflow tract in children with fixed subaortic stenosis and to determine whether these abnormalities precede the development of subaortic obstruction.
Fixed subaortic stenosis typically develops and progresses after the 1st year of life and is therefore often regarded as an acquired lesion. Although it has been speculated that there may be an underlying anatomic substrate, there are no data to support this hypothesis.
The size of the aortic annulus, mitral-aortic valve separation, aorto-left ventricular septal angle and degree of aortic override were determined in two groups of children. Group 1 comprised 35 patients with isolated subaortic stenosis noted on initial echocardiogram who were compared with an age- and weight-matched normal control group (Group 1A). Group 2 comprised 23 patients with ventricular septal defect or coarctation of the aorta, or both, who had no subaortic stenosis on initial echocardiogram but who developed it subsequently. This group was compared with an age-, weight- and lesion-matched control group (Group 2A).
Compared with control subjects, patients with isolated subaortic stenosis had a significantly wider mitral-aortic separation ([mean +/- SD] 5.1 +/- 1.3 vs. 3.4 +/- 0.9 mm, p < 0.001), a steeper aortoseptal angle (131 +/- 6 degrees vs. 144 +/- 5 degrees, p < 0.001) and an exaggerated aortic override (p < 0.05). Similar differences were found on initial echocardiogram in Group 2 patients before development of subaortic stenosis: wider mitral-aortic separation (4.2 +/- 1.2 vs. 2.5 +/- 0.7 mm, p < 0.001), a steeper aortoseptal angle (132 +/- 7 degrees vs. 145 +/- 7 degrees, p < 0.001) and an exaggerated aortic override (p < 0.05).
A left ventricular outflow tract malformation characterized by a wider mitral-aortic separation, an exaggerated aortic override and a steeper aortoseptal angle are present in children with ventricular septal defect or coarctation of the aorta, or both, who subsequently develop subaortic stenosis. These morphometric features can be used to identify by echocardiography patients who are at risk for developing fixed subaortic stenosis.
本研究旨在通过超声心动图识别固定性主动脉瓣下狭窄患儿左心室流出道的形态学异常,并确定这些异常是否先于主动脉瓣下梗阻的发生。
固定性主动脉瓣下狭窄通常在1岁以后发生并进展,因此常被视为后天性病变。尽管有人推测可能存在潜在的解剖学基础,但尚无数据支持这一假设。
测定两组儿童的主动脉瓣环大小、二尖瓣-主动脉瓣间距、主动脉-左心室间隔角及主动脉骑跨程度。第1组包括35例初次超声心动图检查发现孤立性主动脉瓣下狭窄的患者,并与年龄和体重匹配的正常对照组(第1A组)进行比较。第2组包括23例室间隔缺损或主动脉缩窄或两者皆有的患者,他们初次超声心动图检查时无主动脉瓣下狭窄,但随后发生了该病变。该组与年龄、体重和病变匹配的对照组(第2A组)进行比较。
与对照组相比,孤立性主动脉瓣下狭窄患者的二尖瓣-主动脉瓣间距明显更宽([均值±标准差]5.1±1.3对3.4±0.9mm,p<0.001),主动脉-间隔角更陡(131±6°对144±5°,p<0.001),主动脉骑跨更明显(p<0.05)。在第2组患者主动脉瓣下狭窄发生前的初次超声心动图检查中也发现了类似差异:二尖瓣-主动脉瓣间距更宽(4.2±1.2对2.5±0.7mm,p<0.001),主动脉-间隔角更陡(132±7°对145±7°,p<0.001),主动脉骑跨更明显(p<0.05)。
室间隔缺损或主动脉缩窄或两者皆有的患儿,若随后发生主动脉瓣下狭窄,则存在以二尖瓣-主动脉瓣间距增宽、主动脉骑跨明显及主动脉-间隔角更陡为特征的左心室流出道畸形。这些形态学特征可用于通过超声心动图识别有发生固定性主动脉瓣下狭窄风险的患者。