Division of Prenatal Medicine, Department of OB/GYN, Justus-Liebig-University, Giessen.
Pediatric Heart Center, Justus-Liebig-University, Giessen.
Ultraschall Med. 2012 Dec;33(7):E51-E56. doi: 10.1055/s-0032-1312830. Epub 2012 Dec 18.
Hypoplastic left heart (HLH) is one of the most common forms of cardiac abnormality detectable during gestation by fetal echocardiography. Antenatal diagnosis allows for appropriate counseling and time to consider treatment options. We report the actual outcome data after fetal diagnosis of HLH.
Retrospective analysis of the outcome in all cases with HLH from 1994 - 2011 presenting in fetal life at two tertiary referral centers for prenatal diagnosis and pediatric cardiology.
105 cases were included and the overall survival is 40.9 % (43/105) after prenatal diagnosis. There was an 81.1 % survival rate in infants undergoing surgery and a 64.1 % survival rate from an intention-to-treat position. Two neonates died due to tamponade and cardiac arrest following balloon septostomy and one neonate from sepsis before surgery. Extracardiac anomalies occurred in three fetuses, and karyotype anomalies in seven fetuses (18.9 %). In 4 of 5 babies born with additional extracradiac or karyotype anomalies, parents opted for compassionate care. The first had trisomy 13, the second had trisomy 18, the third neonate presented with spina bifida, and the fourth presented with hydronephrosis and pulmonary atresia. Termination of pregnancy took place in 17 cases (16.1 %).
Thorough antenatal evaluation should include karyotyping, detailed extracardiac and intracardiac assessment to accurately predict the risks of surgery. Prenatal counseling might be modified after the exclusion of additional anomalies. These data provide up-to-date information for parental counseling.
左心发育不良（HLH）是胎儿超声心动图在妊娠期间可检测到的最常见的心脏异常之一。产前诊断可提供适当的咨询和时间来考虑治疗方案。我们报告了胎儿诊断为 HLH 后的实际结局数据。
对 1994 年至 2011 年在两家产前诊断和儿科心脏病学三级转诊中心就诊的所有 HLH 胎儿病例进行回顾性分析。
共纳入 105 例病例，产前诊断后总体存活率为 40.9%（43/105）。接受手术的婴儿存活率为 81.1%，意向治疗的存活率为 64.1%。两名新生儿在球囊房间隔造口术后因心脏填塞和心脏骤停死亡，一名新生儿在手术前因败血症死亡。3 例胎儿存在心脏外畸形，7 例胎儿存在染色体异常（18.9%）。在 5 例伴有额外心脏外或染色体异常的婴儿中，有 4 例父母选择了同情护理。第一个婴儿患有三体 13，第二个婴儿患有三体 18，第三个新生儿患有脊柱裂，第四个新生儿患有肾积水和肺动脉闭锁。17 例（16.1%）病例终止妊娠。
全面的产前评估应包括染色体核型分析、详细的心脏内外评估，以准确预测手术风险。在排除其他异常后，产前咨询可能会发生改变。这些数据为家长提供了最新的咨询信息。
