Association Between Down Syndrome and In-Hospital Death Among Children Undergoing Surgery for Congenital Heart Disease

Abstract

Background— The prevalence of Down syndrome (DS)–affected births has increased during the past 30 years; moreover, children with DS have a higher incidence of congenital heart disease compared with their peers. Whether children with DS have better or worse outcomes after repair of congenital heart disease is unclear. We sought to identify differences in in-hospital mortality after cardiac surgery in pediatric patients with and without DS using a large national database. Methods and Results— Children aged <18 years who underwent surgical intervention for congenital heart disease were identified using the Kids’ Inpatient Database (2000, 2003, 2006, and 2009). Patients were stratified using the Risk Adjustment for Congenital Heart Surgery algorithm. A total of 4231 (8.2%) of the 51 309 patients studied had a diagnosis of DS. In-hospital death for patients with DS was significantly lower than that for patients without DS overall (1.9% versus 4.3%; P <0.05) as well as within risk categories 2 (1.0% versus 1.8%; P <0.05) and 3 (2.3% versus 5.1%; P <0.05). Multivariable logistic regression showed a lower odds of death among children with DS (odds ratio=0.60; 95% confidence interval, 0.47–0.76; P <0.05) after adjusting for Risk Adjustment for Congenital Heart Surgery risk category, premature birth, major noncardiac structural anomaly, and age. Conclusions— In this large national study, children with DS who underwent repair of congenital heart disease were more likely to survive to discharge than children without DS. Future work is needed to better understand the factors underlying these differences.

Cardiopatia Congénita em Crianças com Síndrome de Down: O que Mudou nas Últimas Três Décadas?

“…5,11 O último óbito registado ocorreu em 1998, pelo que a taxa de mortalidade a 30 dias entre 2000 e 2013 foi de 0%. No entanto, a relação entre a mortalidade e o ano em que foi realizada a cirurgia não foi significativa.…”

“…4 Apesar destes factos, a mortalidade hospitalar Revista Científica da Ordem dos Médicos www.actamedicaportuguesa.com Dias FM, et al Cardiopatia congénita em síndrome de Down nas últimas três décadas, Acta Med Port 2016 Oct;29(10):613-620 associada a cirurgia cardíaca corretiva é significativamente menor para os doentes com SD quando comparados com doentes sem SD. 5 Os doentes com SD apresentam complicações a nível de outros órgãos ou sistemas com prevalência superior à da população geral, das quais se destacam obesidade, hipotiroidismo, maior probabilidade de desenvolvimento de neoplasias hematológicas (com um perfil genético e de resposta à quimioterapia particulares), maior risco de infeção (em parte devido a défices específicos de imunoglobulinas), assim como maior risco de autoimunidade. 4 A população com SD constitui assim cada vez mais um grupo particular dentro de toda a medicina, uma vez que a sua prevalência e sobrevida têm aumentado significativamente ao longo dos anos.…”

El niño con Síndrome de Down en la Unidad de Cuidados Intensivos

“…Finalmente, se ha demostrado en importantes estudios de cohorte que el neonato y niño con SD presenta el mismo 9 o menor riesgo de muerte a corto plazo 41 que el niño sin SD post cardiocirugía. …”

Should We Mend Their Broken Hearts? The History of Cardiac Repairs in Children With Down Syndrome

“…In May 2014, Evans et al 1 reported that children with Down syndrome had lower rates of in-hospital death after cardiac surgery, compared with children without Down syndrome. Forty years ago, these results would have been unthinkable, as heart defects were not repaired in the majority of children with Down syndrome.…”