Mahle W T, Clancy R R, McGaurn S P, Goin J E, Clark B J
Division of Cardiology, Philadelphia, Pennsylvania, USA.
Pediatrics. 2001 Jun;107(6):1277-82. doi: 10.1542/peds.107.6.1277.
Prenatal echocardiography can identify the fetus that has complex congenital heart disease and may improve early management and surgical outcome. Prenatal diagnosis may be particularly beneficial to patients who have hypoplastic left heart syndrome (HLHS) and who are at risk for hypoxic-ischemic insult at presentation.
We sought to determine whether prenatal diagnosis reduces neurologic morbidity and operative mortality in patients who undergo palliative surgery for the HLHS.
Data from all patients who had HLHS, except for those with lethal genetic anomalies, and who were admitted to our institution between July 1992 and September 1997 were analyzed to assess the impact of prenatal diagnosis on preoperative management, neurologic morbidity, and surgical mortality. The primary outcome measures were hospital mortality and the incidence of adverse neurologic events (seizure or coma).
There were 216 patients who had HLHS and were referred for surgical palliation, 79 (36.6%) of whom had been diagnosed prenatally. All patients who had been diagnosed prenatally were delivered in an advanced nursery and were started on prostaglandin E(1) on the first day of life. Patients whose HLHS was diagnosed postnatally were begun on prostaglandin E(1) later in life (median = day 2 [range = 1-28 days]). There were 4 preoperative deaths and 53 operative or postoperative deaths. Overall hospital mortality was 26.4% and did not differ between patients whose HLHS had been diagnosed prenatally and those whose HLHS had been diagnosed postnatally. With the use of multivariable analysis, prenatal diagnosis was associated with fewer adverse perioperative neurologic events in the patients whose HLHS had been diagnosed prenatally than in those whose HLHS had been diagnosed postnatally (odds ratio = 0.46).
These data suggest that prenatal diagnosis has a favorable impact on treatment of patients who have HLHS and are undergoing staged palliation and reduces early neurologic morbidity. Prenatal diagnosis was not associated with reduced hospital mortality. It is possible that prenatal diagnosis may improve long-term neurologic outcome.
产前超声心动图能够识别患有复杂先天性心脏病的胎儿，并可能改善早期治疗及手术效果。产前诊断对于患有左心发育不全综合征（HLHS）且出生时存在缺氧缺血性损伤风险的患者可能特别有益。
我们试图确定产前诊断是否能降低接受HLHS姑息性手术患者的神经病变发生率及手术死亡率。
分析1992年7月至1997年9月间收治于我院的所有HLHS患者（除患有致死性遗传异常者外）的数据，以评估产前诊断对术前治疗、神经病变发生率及手术死亡率的影响。主要观察指标为住院死亡率及不良神经事件（癫痫或昏迷）的发生率。
共有216例HLHS患者被转诊接受手术姑息治疗，其中79例（36.6%）为产前诊断。所有产前诊断的患者均在高级育婴室分娩，并在出生首日开始使用前列腺素E1。产后诊断为HLHS的患者在出生后较晚开始使用前列腺素E1（中位时间 = 第2天[范围 = 1 - 28天]）。术前死亡4例，手术或术后死亡53例。总体住院死亡率为26.4%，产前诊断为HLHS的患者与产后诊断为HLHS的患者之间无差异。通过多变量分析，产前诊断的HLHS患者围手术期不良神经事件少于产后诊断的患者（优势比 = 0.46）。
这些数据表明，产前诊断对接受分期姑息治疗的HLHS患者的治疗有积极影响，并可降低早期神经病变发生率。产前诊断与降低住院死亡率无关。产前诊断有可能改善长期神经预后。
