Ribera Melissa C V, Ribera Ricardo B, Koifman Rosalina J, Koifman Sérgio
1Master's Program in Public Health,Acre Federal University,Rio Branco,Acre,Brazil.
2Children's Hospital,Pediatric Cardiology Service,Rio Branco,Acre,Brazil.
Cardiol Young. 2015 Jan;25(1):63-9. doi: 10.1017/S104795111300156X. Epub 2013 Oct 24.
Cardiac abnormalities in sickle cell anaemia are frequent and early, despite being more evident in adulthood. The study on cardiac abnormalities is essential in the current context, as, owing to improved health, children are increasingly able to reach adulthood and suffering the consequences of chronic cardiac injury.
The aim of this study was to determine the prevalence of echocardiographic changes in patients under 20, suffering from sickle cell disease in Rio Branco, Brazilian Western Amazon.
The descriptive epidemiological study compare two sets of children and adolescents, one including sickle cell anaemia patients (n=45), and other one (n=109) without sickle cell anaemia or heart disease. The echocardiographic measurements were indexed according to body surface using z-scores, and the prevalence of echocardiographic changes in both groups, with their respective 95% confidence intervals, ascertained and compared.
Compared with the non-sickle cell anaemia series, the sickle cell anaemia group showed z-scores 13.1-fold higher for the diastolic diameter of the left ventricle, 5.2 times higher for the thickness of the posterior wall, 4.9 higher for the left atrium, 2.5 times higher for the right ventricle and 2.0 times higher for the septum thickness. Also the rate of left ventricular mass, systolic pressure of the right ventricle and the relative wall thickness were significantly higher in sickle cell anaemia set.
Cardiac abnormalities were observed in 93.5% of patients. Early detection of cardiac abnormalities and quantifying them using the indexation of echocardiographic measurements according to body surface will allow proper identification and attendance of these children.
镰状细胞贫血患者的心脏异常很常见且出现较早，尽管在成年期更为明显。在当前背景下，对心脏异常的研究至关重要，因为随着健康状况的改善，儿童越来越能够活到成年并承受慢性心脏损伤的后果。
本研究的目的是确定巴西西部亚马逊地区里奥布兰科20岁以下镰状细胞病患者超声心动图改变的患病率。
这项描述性流行病学研究比较了两组儿童和青少年，一组包括镰状细胞贫血患者（n = 45），另一组（n = 109）无镰状细胞贫血或心脏病。超声心动图测量值根据体表面积使用z评分进行索引，并确定和比较两组超声心动图改变的患病率及其各自的95%置信区间。
与非镰状细胞贫血组相比，镰状细胞贫血组左心室舒张直径的z评分高13.1倍，后壁厚度高5.2倍，左心房高4.9倍，右心室高2.5倍，室间隔厚度高2.0倍。镰状细胞贫血组的左心室质量、右心室收缩压和相对壁厚率也显著更高。
93.5%的患者观察到心脏异常。早期发现心脏异常并根据体表面积对超声心动图测量值进行索引量化，将有助于对这些儿童进行正确识别和照料。
