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法洛四联症

Tetralogy of Fallot.

作者信息

Apitz Christian, Webb Gary D, Redington Andrew N

机构信息

Division of Cardiology, Labatt Family Heart Centre, Hospital for Sick Children, Toronto, ON, Canada.

出版信息

Lancet. 2009 Oct 24;374(9699):1462-71. doi: 10.1016/S0140-6736(09)60657-7. Epub 2009 Aug 14.

Abstract

Tetralogy of Fallot is the most common form of cyanotic congenital heart disease, and one of the first to be successfully repaired by congenital heart surgeons. Since the first procedures in the 1950s, advances in the diagnosis, perioperative and surgical treatment, and postoperative care have been such that almost all those born with tetralogy of Fallot can now expect to survive to adulthood. The startling improvement in outcomes for babies born with congenital heart disease in general-and for those with tetralogy of Fallot in particular-is one of the success stories of modern medicine. Indeed, in many countries adults with tetralogy of Fallot outnumber children. Consequently, new issues have emerged, ranging from hitherto unpredicted medical complications to issues with training for caregivers and resource allocation for this population of survivors. Therefore, evolution of treatment, recognition of late complications, research on disease mechanisms and therapies-with feedback to changes in care of affected children born nowadays-are templates on which the timely discussion of organisation of care of those affected by congenital heart diseases from the fetus to the elderly can be based. Here, we focus on new developments in the understanding of the causes, diagnosis, early treatment, and late outcomes of tetralogy of Fallot, emphasising the continuum of multidisciplinary care that is necessary for best possible lifelong treatment of the 1% of the population born with congenital heart diseases.

摘要

法洛四联症是最常见的青紫型先天性心脏病,也是最早由先天性心脏病外科医生成功修复的疾病之一。自20世纪50年代首次开展相关手术以来,在诊断、围手术期及手术治疗以及术后护理方面均取得了进展,以至于几乎所有患法洛四联症的患儿现在都有望存活至成年。总体而言,先天性心脏病患儿尤其是法洛四联症患儿治疗效果的惊人改善是现代医学的成功案例之一。事实上,在许多国家,患法洛四联症的成年人数量超过了儿童。因此,出现了新的问题,从以前未曾预料到的医学并发症到护理人员培训问题以及为这群幸存者的资源分配问题。所以,治疗方法的演变、晚期并发症的识别、疾病机制和治疗方法的研究——并反馈到如今出生的患病儿童护理的变化——是可以据此适时讨论从胎儿到老年人先天性心脏病患者护理组织的模板。在此,我们重点关注法洛四联症病因、诊断、早期治疗和晚期结局认识方面的新进展,强调多学科护理的连续性,这对于为1%患有先天性心脏病的人群提供尽可能最佳的终身治疗是必要的。

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