Lee M L, Wu M H, Wang J K, Chang C I, Lue H C
Department of Pediatrics, Changhua Christian Hospital, Taipei, Taiwan, R.O.C.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi. 1995 Jan-Feb;36(1):14-9.
From July 1992 to January 1993, a total of 5 cases of hypoplastic left heart syndrome were diagnosed by echocardiograms and confirmed by available angiographic, surgical or autopsy results. The flow dynamics disclosed by spectrum display in the aortic root and patent ductus arteriosus (PDA), in systole, were: (1) antegrade in the ascending aorta and descending aorta (below PDA), and retrograde in the transverse aorta, and (2) right-to-left shunt in the PDA. While in diastole, there were (1) antegrade in the transverse aorta, and retrograde in the ascending aorta and descending aorta (below PDA), and (2) left-to-right shunt in the PDA. No ventriculocoronary fistulas were identified. With both the characteristic flow pattern and the extremely narrow diameters of the ascending aorta in hypoplastic left heart syndrome, we can differentiate it from those heart diseases associated with secondary hypoplastic left heart due to total anomalous pulmonary venous connection or congenital pulmonary venous stenosis. Both help us understand better the hemodynamics and pathophysiology of hypoplastic left heart syndrome in these patients.
1992年7月至1993年1月，共有5例左心发育不全综合征患者经超声心动图诊断，并经血管造影、手术或尸检结果证实。主动脉根部和动脉导管未闭（PDA）频谱显示的收缩期血流动力学表现为：（1）升主动脉和降主动脉（PDA下方）为正向血流，横主动脉为逆向血流；（2）PDA存在右向左分流。而在舒张期，表现为：（1）横主动脉为正向血流，升主动脉和降主动脉（PDA下方）为逆向血流；（2）PDA存在左向右分流。未发现心室冠状动脉瘘。鉴于左心发育不全综合征具有特征性血流模式以及升主动脉直径极窄，我们能够将其与因完全性肺静脉异位连接或先天性肺静脉狭窄导致继发性左心发育不全的心脏病相鉴别。这两者都有助于我们更好地理解这些患者左心发育不全综合征的血流动力学和病理生理学。
