Department of Pediatrics and Cardiovascular Research Institute, Baylor College of Medicine, Houston, TX (S.A.M., D.J.P.); Texas Department of State Health Services, Austin, TX (M.K.E., M.A.C.); Dan L. Duncan Institute for Clinical and Translational Research, Baylor College of Medicine, Houston, TX (C.G.M.); Department of Pediatrics, UT Southwestern Medical Center, Dallas, TX (D.E.F.); and the Department of Epidemiology & Biostatistics, University of South Florida, Tampa, FL (W.N.N.).
Circulation. 2014 Jan 21;129(3):285-92. doi: 10.1161/CIRCULATIONAHA.113.003711. Epub 2013 Oct 17.
Most studies have not demonstrated improved survival after prenatal diagnosis of critical congenital heart disease, including hypoplastic left heart syndrome (HLHS). However, the effect of delivery near a cardiac surgical center (CSC), the recommended action after prenatal diagnosis, on HLHS mortality has been poorly investigated.
Using Texas Birth Defects Registry data, 1999 through 2007, which monitored >3.4 million births, we investigated the association between distance (calculated driving time) from birth center to CSC and neonatal mortality in 463 infants with HLHS. Infants with extracardiac birth defects or genetic disorders were excluded. The associations between prenatal diagnosis, CSC HLHS volume, and mortality were also examined. Neonatal mortality in infants born <10 minutes from a CSC was 21.0%, 10 to 90 minutes 25.2%, and >90 minutes 39.6% (P for trend <0.001). Prenatal diagnosis alone was not associated with improved survival (P=0.14). In multivariable analysis, birth >90 minutes from a CSC remained associated with increased mortality (odds ratio, 2.03; 95% confidence interval, 1.19-3.45), compared with <10 minutes. In subanalysis, birth >90 minutes from a CSC was associated with higher pretransport mortality (odds ratio, 6.69; 95% confidence interval, 2.52-17.74) and birth 10 to 90 minutes with higher presurgical mortality (odds ratio, 4.45; 95% confidence interval, 1.17-17.00). Higher surgical mortality was associated with lower CSC HLHS volume (odds ratio per 10 patients, 0.88; 95% confidence interval, 0.84-0.91).
Infants with HLHS born far from a CSC have increased neonatal mortality, and most of this mortality is presurgical. Efforts to improve prenatal diagnosis of HLHS and subsequent delivery near a large volume CSC may significantly improve neonatal HLHS survival.
大多数研究并未表明产前诊断出严重先天性心脏病（包括左心发育不全综合征[HLHS]）后生存率有所提高。然而，产前诊断后接近心脏外科中心（CSC）的分娩（推荐的做法）对 HLHS 死亡率的影响尚未得到充分研究。
我们利用德克萨斯州出生缺陷登记处的数据（1999 年至 2007 年），该数据监测了超过 340 万例分娩，调查了距分娩中心到 CSC 的距离（计算驾驶时间）与 463 例 HLHS 新生儿死亡率之间的关系。排除了伴有心脏外出生缺陷或遗传疾病的婴儿。还检查了产前诊断、CSC 中 HLHS 容量和死亡率之间的关联。距离 CSC 不足 10 分钟出生的婴儿的新生儿死亡率为 21.0%，10 至 90 分钟为 25.2%，超过 90 分钟为 39.6%（趋势 P<0.001）。单独进行产前诊断与生存率的提高无关（P=0.14）。在多变量分析中，与距离 CSC 不足 10 分钟相比，出生距离 CSC 超过 90 分钟仍与死亡率增加相关（优势比，2.03；95%置信区间，1.19-3.45）。在亚组分析中，距离 CSC 出生超过 90 分钟与较高的转运前死亡率相关（优势比，6.69；95%置信区间，2.52-17.74），出生 10 至 90 分钟与较高的术前死亡率相关（优势比，4.45；95%置信区间，1.17-17.00）。较高的手术死亡率与 CSC 中 HLHS 容量较低相关（每 10 例患者的优势比，0.88；95%置信区间，0.84-0.91）。
距离 CSC 较远出生的 HLHS 婴儿的新生儿死亡率较高，且大部分死亡率发生在术前。努力改善 HLHS 的产前诊断以及随后在大容量 CSC 附近分娩，可能会显著提高新生儿 HLHS 的生存率。
