对唐氏综合征婴儿心功能和肺血流动力学的连续评估。
Serial Assessment of Cardiac Function and Pulmonary Hemodynamics in Infants With Down Syndrome.
机构信息
Department of Neonatology, Rotunda Hospital, Dublin, Ireland.
Department of Pediatrics, Boston Children's Hospital, Boston, Massachusetts; Department of Pediatrics, Harvard Medical School, Boston, Massachusetts.
出版信息
J Am Soc Echocardiogr. 2022 Nov;35(11):1176-1183.e5. doi: 10.1016/j.echo.2022.07.012. Epub 2022 Jul 19.
BACKGROUND
There is a dearth of longitudinal data describing the evolution of cardiopulmonary hemodynamics in infants with Down syndrome (DS) beyond infancy. We hypothesized that babies with DS, independent of the presence of congenital heart disease (CHD), demonstrate biventricular systolic and diastolic impairment and sustained elevation of pulmonary pressures compared with controls over the first 2 years of age.
METHODS
This was a prospective observational cohort study of 70 infants with DS (48 with CHD and 22 without CHD) and 60 controls carried out in 3 tertiary neonatal intensive care units in Dublin, Ireland. Infants with DS with and without CHD and non-DS controls underwent serial echocardiograms at birth, 6 months, 1 year, and 2 years of age to assess biventricular systolic and diastolic function using deformation analysis. Pulmonary vascular resistance was assessed using pulmonary artery acceleration time and left ventricular (LV) eccentricity index.
RESULTS
Infants with DS exhibited smaller LV (birth: 27 ± 4 vs 31 ± 2 mm, P < .01; 2 years: 43 ± 5 vs 48 ± 4 mm, P < .01) and right ventricular (birth: 28 ± 3 vs 31 ± 2 mm, P < .01; 2 years: 40 ± 4 vs 44 ± 3 mm, P < .01) lengths and lower LV (birth: -19% ± 3% vs -22% ± 2%, P < .01; 2 years: -24% ± 2% vs -26% ± 2%, P < .01) and right ventricular (birth: -19% ± 4% vs -22% ± 3%, P < .01; 2 years: -29% ± 6% vs -33% ± 4%, P < .01) systolic strain over the 2-year period. Pulmonary artery acceleration time was lower in the DS group throughout the study period (birth: 44 ± 10 vs 62 ± 14 ms, P < .01; 2 years 71 ± 12 vs 83 ± 11 ms, P < .01). No differences were observed between DS infants with and without CHD (all P > .05).
CONCLUSIONS
Infants with DS exhibit impaired maturational changes in myocardial function and pulmonary vascular resistance. Such novel findings provide valuable insights into the pathophysiology affecting cardiorespiratory morbidity in this population.
背景
目前缺乏描述唐氏综合征（Down syndrome，DS）婴儿心肺血液动力学演变的纵向数据，这些婴儿的年龄已超过婴儿期。我们假设，无论是否存在先天性心脏病（congenital heart disease，CHD），DS 婴儿的左、右心室收缩和舒张功能均存在损伤，并且与对照组相比，其肺动脉压力在 2 岁前持续升高。
方法
这是一项在爱尔兰都柏林的 3 家三级新生儿重症监护病房进行的前瞻性观察性队列研究，共纳入 70 名 DS 婴儿（48 名合并 CHD，22 名无 CHD）和 60 名对照婴儿。DS 婴儿合并和不合并 CHD 以及非-DS 对照组婴儿在出生时、6 个月、1 岁和 2 岁时接受了多次超声心动图检查，以使用应变分析评估左、右心室收缩和舒张功能。通过肺动脉加速时间和左心室（left ventricular，LV）偏心指数评估肺血管阻力。
结果
DS 婴儿的 LV（出生时：27 ± 4 比 31 ± 2mm，P < 0.01；2 岁时：43 ± 5 比 48 ± 4mm，P < 0.01）和右心室（出生时：28 ± 3 比 31 ± 2mm，P < 0.01；2 岁时：40 ± 4 比 44 ± 3mm，P < 0.01）长度更小，LV（出生时：-19% ± 3% 比-22% ± 2%，P < 0.01；2 岁时：-24% ± 2% 比-26% ± 2%，P < 0.01）和右心室（出生时：-19% ± 4% 比-22% ± 3%，P < 0.01；2 岁时：-29% ± 6% 比-33% ± 4%，P < 0.01）的收缩应变在 2 年内均逐渐降低。整个研究期间，DS 组的肺动脉加速时间均较低（出生时：44 ± 10 比 62 ± 14ms，P < 0.01；2 岁时：71 ± 12 比 83 ± 11ms，P < 0.01）。DS 婴儿合并和不合并 CHD 组之间无差异（所有 P > 0.05）。
结论
DS 婴儿的心肌功能和肺血管阻力的成熟变化受损。这些新发现为了解影响该人群心肺发病率的病理生理学提供了有价值的见解。
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