Outcome after prenatal diagnosis of the hypoplastic left heart syndrome
Outcome after prenatal diagnosis of the hypoplastic left heart syndrome
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DOI：
10.1136/hrt.79.4.371
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发表时间：
1998-04-01
期刊：
影响因子：
5.7
通讯作者：
Printz, BF
中科院分区：
文献类型：
--
作者：
Allan, LD;Apfel, HD;Printz, BF
1993年中期至1996年年底，对所有在胎儿生命中出现的左心发育不良综合征病例进行回顾性研究。在妊娠24周前确定的12例中，有4例母亲选择终止妊娠。有一个意向，在24个剩余的fetus.Main结果measure-Survival到6个月的产后life.Results-Of 24个婴儿，5个没有提供诺伍德阶段1，因为三体18（n = 2），不利的心脏解剖（n = 2），或神经功能障碍（n = 1）。另有一名婴儿在胃肠道手术后未能存活至心脏手术。在其余18名患者中，8名患者的特征被认为会增加手术修复的风险。在18例接受诺伍德一期手术的患者中，有9例存活。有70%的婴儿接受手术，没有并发症的特点，50%的所有手术候选人的生存率，和37.5%的生存率从一个意向treatmentposition. Conclusions胎儿发育不良左心综合征的初步诊断，总生存率似乎是小于40%。评估必须包括详细的心外和心内评估，以预测手术治疗的风险。随着怀孕的进展，产前咨询可以根据发现或排除并发因素的情况进行修改。
Objective-To derive figures in the current surgical era for counselling in early pregnancy after the diagnosis of fetal hypoplastic left heart syndrome.Setting-A tertiary referral centre for paediatric cardiology and cardiac surgery.Design-A retrospective study of the outcome in all cases of hypoplastic left heart syndrome presenting in fetal life between mid-1993 and the end of 1996.Patients-The diagnosis was made in 30 fetuses. In four of 12 identified before 24 weeks' gestation the mothers chose to terminate the pregnancy. There was an intention to treat in 24 of the remaining fetuses.Main outcome measure-Survival to six months of postnatal life.Results-Of the 24 infants, five were not offered Norwood stage 1 because of trisomy 18 (n = 2), unfavourable cardiac anatomy (n = 2), or neurological impairment (n = 1). One further infant did not survive to cardiac surgery after gastrointestinal surgery. Of the remaining 18 patients, eight had features that were considered to increase the risk of surgical repair. Of the 18 patients who underwent Norwood stage 1, there were nine survivors. There was a survival rate of 70% in infants undergoing surgery with no complicating features, a 50% survival of the all surgical candidates, and 37.5% survival from an intention to treat position.Conclusions-At the initial diagnosis of fetal hypoplastic left heart syndrome, the overall survival appears to be less than 40%. Evaluation must include detailed extracardiac and intracardiac assessment to predict the risks of surgical treatment. Prenatal counselling can be modified as pregnancy advances, depending on the detection or exclusion of complicating factors.