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唐氏综合征与先天性心脏病(作者译)

[Down's dyndrome and congenital heart disease (author's transl)].

作者信息

Sáenz de Buruaga J D, Alegría E, Vallés V, Téllez J, Monreal F, Elizalde J, Alonso A

出版信息

An Esp Pediatr. 1980 Jan;13(1):43-50.

PMID:6445172
Abstract

Out of a group of 113 cases of Down's syndrome 50 were associated with congenital heart disease, confirmed by haemodynamic, angiographic and surgical means. The mean age was 36.6 months. Twelve were cyanotic, six of them because of an inverted previous left-toright shunt. The endocardial cushion defect, alone or in association, accounted for 40% of these anomalies, and ventricular septal defect for 26%. If the cases with right ventricle outflow obstruction are excluded, 50% presented pulmonary pressures over 70 mmHg. In addition, 80% of endocardial cushion defects presented pulmonary hypertension, being the three fourths under one year of life. On the basis of the data presented, authors remark the need of an early approach to specialized diagnosis of these children, in order to allow an earlier surgical correction or palliation than in children without Down syndrome.

摘要

在113例唐氏综合征患者中,有50例合并先天性心脏病,通过血流动力学、血管造影和手术方法得以确诊。平均年龄为36.6个月。12例有发绀症状,其中6例是由于先前的左向右分流倒置所致。心内膜垫缺损单独或合并存在占这些异常情况的40%,室间隔缺损占26%。如果排除右心室流出道梗阻的病例,50%的患者肺动脉压超过70mmHg。此外,80%的心内膜垫缺损患者出现肺动脉高压,其中四分之三在1岁以下。基于所呈现的数据,作者指出需要对这些儿童尽早进行专业诊断,以便能比非唐氏综合征儿童更早地进行手术矫正或姑息治疗。

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