Pulmonary hypertension and right ventricular function in Nigerian children with sickle cell anaemia.
Pulmonary hypertension and right ventricular function in Nigerian children with sickle cell anaemia.
复制标题
尼日利亚镰状细胞性贫血儿童的肺动脉高压和右心室功能。
DOI：
10.1093/trstmh/trz038
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发表时间：
2019
影响因子：
2.2
通讯作者：
Bode-Thomas,Fidelia
中科院分区：
文献类型：
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作者：
Peter,IgocheD;Asani,MustafaO;Abdullahi,ShehuU;Aliyu,Ibrahim;Obaro,StephenK;Bode-Thomas,Fidelia
背景肺动脉高压（PH）是镰状细胞性贫血（SCA）的一种并发症，导致相当高的发病率。本研究旨在确定 SCA 儿童中超声心动图提示的 PH 的患病率和相关性。方法我们进行了一项横断面比较研究，涉及 100 名 3-14 岁、处于稳定状态的系统抽样 SCA 受试者与匹配的血红蛋白 AA 表型对照。所有患者均获得临床、实验室和超声心动图数据（包括三尖瓣反流速度 [TRV]、平均肺动脉压 [mPAP] 和三尖瓣环平面收缩期偏移 [TAPSE]）。使用 SPSS 22 版（IBM，Armonk，NY，USA）进行统计分析。 p 值 <0.05 被认为具有统计显着性。 结果 在研究的 100 名 SCA 受试者中，22 名 (22%) 的超声心动图结果提示有 PH，而对照组则没有。与无 PH 组 (2.77 cm [IQR 2.4–3.2]) 相比，PH 组的中位 TAPSE 显着较低 (2.55 cm [四分位数间距 {IQR} 2.2–2.8]) (p=0.03)。 mPAP 与年龄之间不存在显着相关性，研究的任何实验室参数也不存在显着相关性。比值比 (OR) 表明，随着 12 个月内因血管闭塞危象住院频率的增加（OR 15.15 [95% CI 1.57 至 146.35]，p=0.02）和终生输血史（OR 5.44 [95% CI 1.09 至 27.24]， p=0.04）。结论超声心动图提示的 PH 在 SCA 儿童中很常见，并且与较差的右心室功能、频繁的血管闭塞危象和输血相关。
BackgroundPulmonary hypertension (PH), a complication of sickle cell anaemia (SCA), results in considerable morbidity. This study aims to determine the prevalence and associations of echocardiography-suggested PH in children with SCA.MethodsWe performed a cross-sectional comparative study involving 100 systematically sampled SCA subjects 3–14 y of age in their steady state with matched haemoglobin AA phenotype controls. Clinical, laboratory and echocardiography data (including tricuspid regurgitation velocity [TRV], mean pulmonary arterial pressure [mPAP] and tricuspid annular plane systolic excursion [TAPSE]) were obtained from all patients. Statistical analyses were performed using SPSS version 22 (IBM, Armonk, NY, USA). A p-value <0.05 was considered statistically significant.ResultsOf the 100 SCA subjects studied, 22 (22%) had echocardiographic findings suggestive of PH compared with none in the controls. The median TAPSE was significantly lower in the PH group (2.55 cm [interquartile range {IQR} 2.2–2.8]) compared with the no PH group (2.77 cm [IQR 2.4–3.2]) (p=0.03). No significant correlation existed between mPAP and age, nor any laboratory parameters studied. The odds ratio (OR) suggested PH significantly increased with an increase in the frequency of hospitalizations for vaso-occlusive crises within a 12-month period (OR 15.15 [95% CI 1.57 to 146.35], p=0.02) and a lifetime history of blood transfusion (OR 5.44 [95% CI 1.09 to 27.24], p=0.04).ConclusionsEchocardiography-suggested PH is common in children with SCA and is associated with poorer right ventricular function, frequent vaso-occlusive crises and blood transfusions.