Fruitman D S
Faculty of Medicine, Dalhousie University, Halifax, Nova Scotia.
Paediatr Child Health. 2000 May;5(4):219-25. doi: 10.1093/pch/5.4.219.
Few congenital heart malformations have raised as many surgical, ethical, social and economic issues as the therapy for infants diagnosed with hypoplastic left heart syndrome. Before the 1980s, this complex malformation was associated with 95% mortality within the first month of life. In the past two decades, palliative surgery and cardiac transplantation have become management options, in addition to comfort care for the infant. These innovations have forced parents and physicians to make difficult decisions because the long term results of the additional treatment options are not known. This article describes the current risk factors, diagnosis, treatment and outcome of infants with hypoplastic left heart syndrome. Prenatal diagnosis provides families with time for counselling and for becoming more informed about management options. Surgical therapy provides hope for the survival of these infants, but their long term outcomes are not well defined. Comfort care in either the home or hospital remains an acceptable management option. More investigations to determine the long term outcome following palliative surgery and transplantation are needed before they become the standards of care.
很少有先天性心脏畸形像患有左心发育不全综合征的婴儿治疗那样引发如此多的外科、伦理、社会和经济问题。在20世纪80年代之前，这种复杂的畸形与出生后第一个月内95%的死亡率相关。在过去的二十年里，除了对婴儿进行舒适护理外，姑息性手术和心脏移植也已成为治疗选择。这些创新迫使父母和医生做出艰难的决定，因为额外治疗选择的长期结果尚不清楚。本文描述了患有左心发育不全综合征婴儿的当前风险因素、诊断、治疗和结果。产前诊断为家庭提供了咨询时间，并使他们能更多地了解治疗选择。手术治疗为这些婴儿的存活带来了希望，但他们的长期结果尚不明确。在家中或医院进行舒适护理仍然是一种可接受的治疗选择。在姑息性手术和移植成为护理标准之前，需要进行更多研究以确定其长期结果。
