左心发育不良综合征15例超声心动图研究

Echocardiographic analysis of 15 hypoplastic left heart syndrome cases

目的 探讨左心发育不良综合征超声心动图表现 ,以提高对超声心动图诊断该疾病的认识。方法 收集 15例超声心动图资料完整并经证实的左心发育不良综合征患者 ,对其超声心动图顺序节段性研究的结果进行分析。结果 本组左心发育不良综合征患者最常见的心血管表现为左心发育不全、二尖瓣极重度狭窄或无孔二尖瓣、主动脉瓣极重度狭窄或无孔主动脉瓣、主动脉发育不良及依赖于右心室及动脉导管的系统循环。多数患者肺静脉回流经房间隔分流至右房室 ,少数患者左房血流经替代途径回流入右房室 ,分别为心上型肺静脉异位引流、左旋心房主静脉及部分冠状静脉窦无顶 ,其中 1例同时合并三房心及左旋心房主静脉的病例为罕见病例。结论 超声心动图可揭示左心发育不良综合征所有显著的病理解剖及病理生理特征 ,可指导外科手术 ,特别是 Norwood手术的治疗。

ObjectiveTo study the echocardiographic manifestations of hypoplastic left heart syndrome in ordr to improve the knowledge of its echocardiographic diagnosis.MethodsThe confirmed echocardiographic sequential segmental approach results of 15 patients were reviewed.ResultsThe most common cardiovascular anomalies in this group included hypoplastic left heart,extremely severe stenotic or imperforate mitral valve,extremely severe stenotic or imperforate aortic valve,hypoplastic aorta,right ventricle and arterial duct dependent systemic circulation.Pulmonary venous return reach the right ventricle via an atrial septal defect or patent oval foramen in most patients,while in some patients pulmonary venous flow reach the right ventricle via one of the alternative routes including total anomalous pulmonary venous return(supracardiac type),levoatrial cardinal vein or partially unroofed coronary sinus.One patient is a rare case who suffer from cor triatriatum accompanied by levoatrial cardinal vein.ConclusionsEchocardiography is the main diagnostic modality permitting delineation of all the significant pathological and pathophysiological characteristics of hypoplastic left heart syndrome,which benefits the surgical tretment,especial Norwood operation on these patient.