超声心动图对左心发育不良综合征术前诊断及术后评价的意义

Utility of pre/post operative echocardiography in the surgical management of hypoplastic left heart syndrome

目的 总结 1998年在美国贝勒医学院工作期间 ,左心发育不良综合征 (HLHS)患者改良NorwoodⅢ期心脏重建手术前后超声心动图诊断的资料和经验 ,评价其价值并为国内同行借鉴。方法 HLHS患者 18例 ,NorwoodⅠ期手术 10例 ,Ⅱ期手术 6例 ,Ⅲ期手术 2例。术前超声心动图结果与术中外科诊断比较 ,分诊断符合、诊断不符和轻微差别。结果 NorwoodⅠ期手术 10例患者均无术前心导管检查 ,超声心动图术前诊断与术中外科诊断达到 10 0 %的诊断符合。Ⅱ期手术患者 6例 ,超声心动图对Ⅰ期手术术后评价的诊断符合率为10 0 % ,分支肺动脉的评价 33 .3 %诊断符合 ,6 6 .7%有轻微差别。Ⅲ期手术的 2例病患者超声心动图与术中诊断均相符。结论 单靠超声心动图能够确立HLHS的诊断并为NorwooⅠ期手术提供足够的信息 ,对NorwoodⅡ期和Ⅲ期术前及术后诊断超声心动图能够提供大量正确的信息 。

Objective To evaluate the value of pre/post operative echocardiography in hypoplastic left heart syndrome(HLHS) patients who underwent modified Norwood staged procedure,which was reviewed in all HLHS patients in 1998 at Baylor Medical College and to provide the information for the use of the colleagues in China.Methods There were 18 patients with HLPS,10 underwent modified Norwood stage Ⅰ procedure,6 stage Ⅱ and 2 stage Ⅲ. The echocardiographic diagnosis was compared with intraoperative findings,including following results: coincidence,echocardiography had same diagnosis with intraoperative findings; major discrepancy,echocardiographic diagnosis impacted on surgery and necessitated a change in the operative procedure or increased the surgical risk; minor discrepancy,echocardiography diagnosis had minor difference with surgical finding but no impact on surgery and was not necessitated a change in the operative procedure or increased the surgical risk.Results 100% echocardiographic diagnoses coincide with intraoperative findings on all 10 cases undergoing modified Norwood stage Ⅰ procedure without catheterization.Six staged Ⅱ patients had 100% coincidence on postoperative assessment diagnosis but 33.3% coincide with the diagnosis of 66.7% minor discrepancy on branch pulmonary arteries evaluations.Two staged Ⅲ patients had 100% coincidence with echocardiographic diagnosis.Conclusions Echocardiography alone is reliable and accurate for preoperative diagnosis in Norwood stage Ⅰ procedure for HLHS.Echocardiography also can provide a lot of accurate pre/post operative evaluative information for the patients on Norwood stage Ⅱ and stage Ⅲ,but it has limitation on evaluation of detail anatomy and pressure on branch pulmonary arteries and pulmonary veins for some patients.