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[唐氏综合征中的先天性心脏病]

[Congenital heart disease in Down's syndrome].

作者信息

Arias-Lobo Rebeca, Lupinta-Paredes Edgar, Calderón-Colmenero Juan, Cervantes-Salazar Jorge L, García-Montes José A, Patiño-Bahena Emilia J, Benita-Bordes Antonio

机构信息

Departamento de Cardiología Pediátrica, Instituto Nacional de Cardiología Ignacio Chávez, Ciudad de México, México.

Departamento de Cardiología Intervencionista en Cardiopatías Congénitas, Instituto Nacional de Cardiología Ignacio Chávez, Ciudad de México, México.

出版信息

Arch Cardiol Mex. 2023 Jul 27;93(3):294-299. doi: 10.24875/ACM.22000053.

Abstract

BACKGROUND

Down syndrome is the most common chromosomal abnormality, it is associated with a wide variety of congenital heart defects, being considered as clinical elements of high infant morbidity and mortality. Objective.

OBJECTIVE

To describe the clinical outcomes of patients with Down syndrome undergoing surgery and interventionism as treatment for congenital heart disease at this Institution.

MATERIAL AND METHODS

368 patients with Down syndrome and associated congenital heart disease were diagnosed. The variables studied were weight, stature, sex, age, type of heart disease, corrective procedure, length of stay in the hospital and intensive care unit, morbidity and mortality.

RESULTS

368 pediatric patients underwent surgical or interventional correction. Of which 197 (54%) were female, the median age was 24 months (interquartile range [IQR]: 14-48) in the surgical group and 36 months (IQR: 17-85) in the interventional group. The most frequent congenital heart diseases were: PCA (31%), IVC (28%), CAV (20%), ASD (16%) and tetralogy of Fallot with 4% respectively. Hospital stay was 9 days (IQR: 7-15) in the surgical group and 3 days (IQR: 2-5) in the hemodynamic group. Morbidities were postoperative infection in 30 patients (14%) and complete atrioventricular block in 19 patients (9%). Overall mortality including both surgical and interventional was 2%.

CONCLUSIONS

The therapeutic, surgical and interventional results in children with Down syndrome and congenital heart disease have improved very satisfactorily. The lower prevalence of the atrioventricular canal in the Mexican population is noteworthy. It is essential to carry out a cardiological evaluation of children with Down syndrome and those with congenital heart disease to correct them in a timely manner to promote survival and quality of life.

摘要

背景

唐氏综合征是最常见的染色体异常疾病,与多种先天性心脏缺陷相关,被视为婴儿高发病率和死亡率的临床因素。目的。

目的

描述在本机构接受手术和介入治疗先天性心脏病的唐氏综合征患者的临床结局。

材料与方法

诊断出368例患有唐氏综合征及相关先天性心脏病的患者。研究的变量包括体重、身高、性别、年龄、心脏病类型、矫正手术、住院时间和重症监护病房停留时间、发病率和死亡率。

结果

368例儿科患者接受了手术或介入矫正。其中197例(54%)为女性,手术组的中位年龄为24个月(四分位间距[IQR]:14 - 48),介入组为36个月(IQR:17 - 85)。最常见的先天性心脏病分别为:完全性肺静脉异位引流(31%)、室间隔缺损(28%)、房室通道(20%)、房间隔缺损(16%)和法洛四联症(4%)。手术组的住院时间为9天(IQR:7 - 15),血流动力学组为3天(IQR:2 - 5)。并发症包括30例患者(14%)术后感染和19例患者(9%)完全性房室传导阻滞。手术和介入治疗的总体死亡率为2%。

结论

唐氏综合征合并先天性心脏病患儿的治疗、手术和介入治疗效果有了非常令人满意的改善。墨西哥人群中房室通道患病率较低值得注意。对唐氏综合征患儿和先天性心脏病患儿进行心脏评估并及时矫正至关重要,以提高生存率和生活质量。

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