Reis P M, Punch M R, Bove E L, van de Ven C J
Department of Obstetrics and Gynecology, University of Michigan School of Medicine, Ann Arbor 48109-0264, USA.
Obstet Gynecol. 1999 Apr;93(4):532-5. doi: 10.1016/s0029-7844(98)00462-1.
To report the obstetric and neonatal outcomes of ten infants with hypoplastic left heart syndrome in association with Turner syndrome.
The Pediatric Cardiovascular Surgery database at the University of Michigan was searched from 1990 to 1997, and obstetric and neonatal records of neonates with hypoplastic left heart syndrome and Turner syndrome were reviewed.
There were 406 cases of hypoplastic left heart syndrome admitted during 8 years, of which ten (2.5%) also had Turner syndrome. Nine infants were delivered at term and one at 36 weeks. The mean (+/- standard deviation [SD]) gestational age at delivery was 38 +/- 1.2 weeks, and mean (+/-SD) birth weight was 2991 +/- 438 g. Delivery was vaginal in all cases, and no infant had an Apgar score at 5 minutes less than 7. Karyotype was 45, X in seven cases, and 45, X mosaic in three. Most infants had dysmorphic features at birth. All ten infants had first-stage reconstruction surgery for hypoplastic left heart syndrome. Only two survived and underwent second-stage palliation; both are alive currently, although with significant medical problems.
For infants with hypoplastic left heart and Turner syndromes, regular obstetric management appears appropriate. Although staged reconstruction surgery has improved survival for neonates with isolated hypoplastic left heart syndrome, for those with Turner syndrome, survival appears markedly reduced.
报告10例患有左心发育不全综合征并伴有特纳综合征的婴儿的产科及新生儿结局。
检索了密歇根大学1990年至1997年的小儿心血管外科数据库，并回顾了患有左心发育不全综合征和特纳综合征的新生儿的产科及新生儿记录。
8年间共收治406例左心发育不全综合征患儿，其中10例（2.5%）同时患有特纳综合征。9例婴儿足月分娩，1例36周分娩。分娩时的平均（±标准差[SD]）孕周为38±1.2周，平均（±SD）出生体重为2991±438 g。所有病例均经阴道分娩，5分钟时阿氏评分均不低于7分。核型分析显示，7例为45,X，3例为45,X/嵌合体。大多数婴儿出生时具有畸形特征。所有10例婴儿均接受了左心发育不全综合征的一期重建手术。仅2例存活并接受了二期姑息治疗；目前二者均存活，但存在严重的健康问题。
对于患有左心发育不全综合征和特纳综合征的婴儿，常规产科管理似乎是合适的。尽管分期重建手术提高了单纯左心发育不全综合征新生儿的存活率，但对于患有特纳综合征的新生儿，存活率明显降低。
