Department of Pediatrics, Washington University School of Medicine, 660 S. Euclid Avenue, St Louis, MO 63110, USA.
Blood. 2010 Jul 8;116(1):16-21. doi: 10.1182/blood-2009-06-227447. Epub 2010 Apr 8.
Premature death and cardiac abnormalities are described in individuals with sickle cell disease (SCD), but the mechanisms are not well characterized. We tested the hypothesis that cardiac abnormalities in children with SCD are related to sleep-disordered breathing. We enrolled 44 children with SCD (mean age, 10.1 years; range, 4-18 years) in an observational study. Standard and tissue Doppler echocardiography, waking oxygen saturation averaged over 5 minutes, and overnight polysomnography were obtained in participants, each within 7 days. Eccentric left ventricular (LV) hypertrophy was present in 46% of our cohort. After multivariable adjustment, LV mass index was inversely related to average asleep and waking oxygen saturation. For every 1% drop in the average asleep oxygen saturation, there was a 2.1 g/m(2.7) increase in LV mass index. LV diastolic dysfunction, as measured by the E/E' ratio, was present in our subjects and was also associated with low oxygen saturation (sleep or waking). Elevated tricuspid regurgitant velocity (> or = 2.5 m/sec), a measure of pulmonary hypertension, was not predicted by either oxygen saturation or sleep variables with multivariable logistic regression analysis. These data provide evidence that low asleep and waking oxygen saturations are associated with LV abnormalities in children with SCD.
患有镰状细胞病（SCD）的个体存在过早死亡和心脏异常的情况，但这些机制尚未得到很好的描述。我们检验了这样一个假设，即 SCD 儿童的心脏异常与睡眠呼吸紊乱有关。我们对 44 名患有 SCD 的儿童（平均年龄 10.1 岁；范围，4-18 岁）进行了一项观察性研究。参与者在 7 天内完成了标准和组织多普勒超声心动图、5 分钟平均清醒氧饱和度以及整夜多导睡眠图检查。我们的队列中有 46%的人存在偏心性左心室（LV）肥厚。在多变量调整后，LV 质量指数与平均睡眠和清醒时的氧饱和度呈负相关。平均睡眠时氧饱和度每下降 1%，LV 质量指数就会增加 2.1 g/m2.7。我们的研究对象存在 LV 舒张功能障碍，通过 E/E'比值来衡量，并且与低氧饱和度（睡眠或清醒时）相关。多变量逻辑回归分析显示，三尖瓣反流速度升高（>或= 2.5 m/sec），即肺动脉高压的一种衡量指标，不能用氧饱和度或睡眠变量来预测。这些数据提供了证据表明，低睡眠和清醒时的氧饱和度与 SCD 儿童的 LV 异常有关。
