左心发育不良综合征的多普勒超声心动图评判

The evaluation of Hypoplastic left heart syndrome using Echocardiography

目的:评价多普勒超声心动图对左心发育不良综合征(HLHS)解剖结构及心功能状况的诊断价值。方法:本文回顾性分析25例左心发育不良综合征患儿的超声检测结果,并与手术或磁共振结果进行对照。结果:经手术或磁共振检查证实为HLHS者共7例,均与超声诊断结果一致,超声诊断正确率100%。25例HLHS患儿中,Ⅰ型7例(28%),Ⅱ型11例(44%),Ⅲ型5例(20%),Ⅳ型2例(8%)。25例患儿均合并动脉导管未闭和不同程度的肺动脉高压。22例合并房间隔缺损,其中19例存在左向右限制性分流。9例合并中度或重度三尖瓣反流。二尖瓣瓣环直径平均0.56±0.22cm、左室舒张末期内径平均0.84±0.51cm、升主动脉内径平均0.36±0.12cm、右心室心肌工作指数平均0.18±0.11。结论:多普勒超声心动图不仅可以较准确地诊断左心发育不良综合征及其合并畸形,而且还为手术提供有价值的信息。

Objective：To evaluate Echocardiography in diagnosing cardiac anatomy and function in patients with Hypoplastic left heart syndrome （HLHS）. Methods： Totally, 25 HLHS patients were retrospectively investigated. The Echocardiographic diagnosis were compared with either operative findings or MRI findings. Results： In 7 patients, the Echocardiography diagnosis were confirmed either by operative findings or MRI findings. The accuracy of Echocardiography was 100%. In 25 patients, type Ⅰ inclnded 7 patients （28%）, type Ⅱ 11 patients （44%）, type Ⅲ 5 patients （20%） and type Ⅳ 2 patients （8%）. All patients had patent duetus arteriosus （PDA） and different level pulmonary hypertension. Twenty-two patients had atrial septal defects（ASD）, and 19 of them had restrictive left to right shunts, 9 had moderate or severe tricuspid insufficiency. The mean mitral valve annulus was 0.56 ＋ 0.22 cm, the mean left ventrieular diastolic dimcnsion was 0.84 ± 0.51 cm, the mean ascending aorta was 0.36 ± 0.12 cm, and the mean right ventrieular myocardial performance index was 0.18 ± 0.11. Conclusion： Echocardiography can not only accurately diagnose the Hypoplastie left heart syndrome and its associated deformations, but also provide valuable information for surgical decision.