Division of Pediatric Pulmonology and Sleep Medicine, Department of Pediatrics, Penn State College of Medicine, Hershey, PA, USA.
Division of Pediatric Cardiology, Department of Pediatrics, Penn State College of Medicine, Hershey, PA, USA.
Pediatr Res. 2019 Mar;85(4):506-510. doi: 10.1038/s41390-018-0125-6. Epub 2018 Jul 24.
Pulmonary hypertension (PH) is multifactorial in origin and may develop early in children with sickle cell disease (C-SCD). Potential etiologies are hemolysis-induced endothelial dysfunction, left ventricular (LV) dysfunction, and chronic hypoxia. Nocturnal hypoxia (NH) in C-SCD is known to be a sequela of obstructive sleep apnea (OSA). The primary objective of this study is to correlate polysomnographic evidence NH with echocardiographic measures of PH in C-SCD.
We performed a retrospective chart review of 20 C-SCD (Hemoglobin SS), who had polysomnography and echocardiogram performed within a narrow time interval, and 31% of them had pre-existing cardiac conditions. Tricuspid regurgitant jet velocity (TRJV) ≥ 2.5 m/s was considered as an indicator of PH.
Twenty-five percent of the subjects had NH. Forty percent of C-SCD, predominantly male, had evidence of PH based on an elevated TRJV. Children with NH compared to non-NH had significantly worse baseline hypoxemia (p < 0.001), higher TRJV (p = 0.005), and higher LV end-diastolic diameters (p = 0.009). The severity of NH was influenced by OSA. However, PH was not associated with OSA or duration of hydroxyurea therapy.
Our study indicates that NH is associated with PH in C-SCD, and that screening for NH may help to identify C-SCD with higher morbidity risk.
肺动脉高压（PH）的病因复杂，可能在镰状细胞病（C-SCD）患儿早期就已出现。潜在病因包括溶血诱导的内皮功能障碍、左心室（LV）功能障碍和慢性缺氧。已知 C-SCD 中的夜间缺氧（NH）是阻塞性睡眠呼吸暂停（OSA）的后遗症。本研究的主要目的是将 NH 的多导睡眠图证据与 C-SCD 中 PH 的超声心动图测量结果相关联。
我们对 20 名 C-SCD（血红蛋白 SS）患者进行了回顾性图表审查，他们在狭窄的时间间隔内进行了多导睡眠图和超声心动图检查，其中 31%的患者存在先前存在的心脏状况。三尖瓣反流速度（TRJV）≥2.5m/s 被认为是 PH 的指标。
25%的患者有 NH。40%的 C-SCD 患者（主要为男性）根据升高的 TRJV 存在 PH 的证据。与非 NH 患者相比，NH 患者的基线低氧血症明显更差（p<0.001），TRJV 更高（p=0.005），LV 舒张末期直径更高（p=0.009）。NH 的严重程度受 OSA 的影响。然而，PH 与 OSA 或羟基脲治疗时间无关。
我们的研究表明，NH 与 C-SCD 中的 PH 相关，筛查 NH 可能有助于识别具有更高发病率风险的 C-SCD。
