• 作者：Maria Teresa Dioní ; sio^a ; ^{teresa.dns@gmail.com} ; Anabela Couceiro^b ; Eulá ; lia Galhano^b ; Luí ; s Matos^b ; José ; Mesquita^b ; Graç ; a Sousa^a ; ^b ; Eduardo Castela^a ; ^b
• 关键词：Sí ; ndrome do Coraç ; ã ; o Esquerdo Hipopl&aacute ; sico ; Cardiopatia congé ; nita ; Gravidez ; Diagnó ; stico pré ; -natal
• 刊名：Diagn贸stico Prenatal
• 出版年：2011
• 期刊代码：pca115_21734127
• 类别：med
• 出版时间：January-March 2011
• 卷：22
• 期：1
• 页码：2-6
• 文件大小：297 K

Introduction

the Hypoplastic Left Heart Syndrome is due to the underdevelopment of left-sided cardiac structures. This syndrome can be diagnosed from 18 weeks of gestation through transabdominal fetal echocardiography.

Aims

description of a prenatal diagnosis team approach in the context of Hypoplastic Left Heart Syndrome, from January 1990 to December 2008, and case follow-up.

Material and methods

retrospective analysis of the Hypoplastic Left Heart Syndrome cases diagnosed prenatally during the course of 19 years. The following parameters were analysed: year of diagnosis, reason for referral, gestational age at diagnosis, gestational age of medical termination of the pregnancy, necropsy findings, fetal deaths, family past history of congenital heart disease, birth weight, gestational age of the newborns and follow-up.

Results

during the studied period 311 congenital heart disease were diagnosed in our department, 67 (21.5%) of which with Hypoplastic Left Heart Syndrome. Twenty-nine (43.3%) parents opted for medical termination of the pregnancy. The median gestational age for medical abortion was 24 weeks, varying from 20 to 35 weeks. In these cases the necropsy confirmed the prenatal diagnosis of Hypoplastic Left Heart Syndrome. There were four fetal deaths. Of the 34 newborns, eight died in the early neonatal period. Regarding the 12 newborns that underwent surgical correction, six are alive; three of them two years post the completion of the Norwood's third stage.

Conclusion

despite advances in neonatal intensive care, surgical techniques and percutaneous therapy, the morbidity and mortality associated with this pathology raises several ethical issues.