Neonatology, Rotunda Hospital, Dublin, Ireland
Paediatrics, Boston Childrens Hospital, Boston, Massachusetts, USA.
Arch Dis Child. 2020 Nov;105(11):1031-1034. doi: 10.1136/archdischild-2019-318178. Epub 2020 Mar 11.
Down Syndrome (DS) is the most common chromosomal abnormality of live born babies. Individuals with DS are at increased risk of cardiopulmonary morbidities in the early neonatal period, infancy and childhood that manifest with elevated pulmonary arterial pressures and altered myocardial performance. Pulmonary hypertension (PH) during the early neonatal period remains under-recognised in this population. PH may occur with or without a congenital heart defect in children with DS and is more common than in the general population. Early detection and continued screening of PH throughout infancy and childhood for these at-risk children is crucial for prompt intervention and potential prevention of long-term sequelae on cardiac function. This review summarises the main physiological concepts behind the mechanisms of PH in children with DS and provides a summary of the current available literature on PH and its impact on myocardial performance.
唐氏综合征（DS）是活产婴儿中最常见的染色体异常。DS 患者在新生儿期、婴儿期和儿童期患心肺疾病的风险增加，表现为肺动脉压升高和心肌功能改变。在这一人群中，新生儿期的肺动脉高压（PH）仍然认识不足。DS 患儿的 PH 可伴有或不伴有先天性心脏病，其发病率高于普通人群。早期发现和持续筛查这些高危儿童在婴儿期和儿童期的 PH 对于及时干预和潜在预防心脏功能的长期后遗症至关重要。本综述总结了 DS 患儿 PH 机制背后的主要生理概念，并概述了目前关于 PH 及其对心肌功能影响的文献。
