危地马拉唐氏综合征患儿的先天性心脏病

Congenital cardiac disease in children with Down's syndrome in Guatemala.

作者信息

Vida Vladimiro L, Barnoya Joaquín, Larrazabal Luis A, Gaitan Guillermo, de Maria Garcia Flor, Castañeda Aldo R

机构信息

Unidad de Cirugía Cardiovascular de Guatemala (Pediatria), Gautemala.

出版信息

Cardiol Young. 2005 Jun;15(3):286-90. doi: 10.1017/S1047951105000582.

DOI:10.1017/S1047951105000582

PMID:15865831

Abstract

BACKGROUND

Congenital cardiac disease is the greatest cause of death in patients with Down's syndrome during the first two years of life, with from two-fifths to two-thirds of those with Down's syndrome also having congenital cardiac malformations. The lesions within the heart can be single or multiple. Our objective was to evaluate the frequency and type of such congenital cardiac malformations in patients born with Down's in Guatemala, and to provide baseline information for further research.

METHODS

We reviewed all patients with Down's syndrome who underwent a cardiologic screening examination between January, 1997, and December, 2003, in the only department dealing with Paediatric Cardiology in Guatemala.

RESULTS

Of the 349 patients reviewed, 189 (54.1 per cent) also had an associated congenital cardiac malformation. The median age at diagnosis was 6 months, with a range from 2 to 13 months. In 152 patients (80.4 per cent), the cardiac lesion was isolated, while 37 patients (19.6 per cent) had multiple defects. The most common single defect was patency of the arterial duct, found in 54 of the 189 patients (28.6 per cent), followed by ventricular septal defect in 27.5 per cent, atrial septal defect in 12.7 per cent, and atrioventricular septal defect with common atrioventricular junction in 9.5 per cent. The most frequent concomitant malformation found co-existing with other congenital cardiac lesions was patency of the arterial duct, found in 17.5 per cent.

CONCLUSIONS

As far as we are aware, ours is the first epidemiologic study concerning the frequency and type of congenital cardiac disease found in Guatemalan children with Down's syndrome. The high frequency of patency of the arterial duct, and the differential distribution of the cardiac malformations associated with Down's syndrome among Guatemalan children, differ from what has been reported in the United States of America, Europe, and Asia. This difference warrants further research.

摘要

背景

先天性心脏病是唐氏综合征患者在出生后两年内死亡的最主要原因，五分之二至三分之二的唐氏综合征患者伴有先天性心脏畸形。心脏内的病变可以是单一的，也可以是多个的。我们的目的是评估危地马拉唐氏综合征患儿先天性心脏畸形的发生率和类型，并为进一步研究提供基线信息。

方法

我们回顾了1997年1月至2003年12月期间在危地马拉唯一的儿科心脏病科接受心脏筛查检查的所有唐氏综合征患者。

结果

在回顾的349例患者中，189例（54.1%）还伴有先天性心脏畸形。诊断时的中位年龄为6个月，范围为2至13个月。152例患者（80.4%）的心脏病变为单发，37例患者（19.6%）有多个缺陷。最常见的单一缺陷是动脉导管未闭，在189例患者中有54例（28.6%），其次是室间隔缺损占27.5%，房间隔缺损占12.7%，房室间隔缺损伴共同房室交界占9.5%。与其他先天性心脏病变同时存在的最常见合并畸形是动脉导管未闭，占17.5%。