Department of Pediatrics and Child Health, Saint Peter Specialized Hospital, Addis Ababa, Ethiopia.
Department of Pediatrics and Adolescent Health, Faculty of Medicine, University of Botswana, Gaborone, Botswana.
Ethiop J Health Sci. 2023 Nov;33(6):955-962. doi: 10.4314/ejhs.v33i6.5.
Surgical treatment has transformed the course and outcome of congenital heart defects in high-income countries, but children with congenital heart diseases in sub-Saharan Africa, where access to cardiac surgery is limited, often experience the natural course of untreated lesions and their complications. The objective of this study was to determine the prevalence of hematologic derangements among Ethiopian children with unoperated cyanoticcongenital heart diseases, to identify factors associated with coagulopathy in this population, and to describe how these complications are managed in this setting.
In this single-center cross-sectional study, we prospectively collected clinical and demographic data from children (<18 years) with cyanotic congenital heart diseases. Blood samples were collected to measure hematologic parameters. Polycythemia was defined as hematocrit >50% and thrombocytopenia as <150,000 per microliter.
Among 70 children recruited, the overall prevalence of polycythemia and thrombocytopenia was 63% (n=44) and 26% (n=18), respectively. On multivariate logistic regression analysis, hematocrit ≥65% (p-value=.024), and oxygen saturation <85% (p-value=.018) were independently associated with moderate or severe thrombocytopenia. Thirty-one (44%) patients had undergone therapeutic phlebotomy, and 84% (26/31) of these patients received iron supplementation.
We report a high prevalence of polycythemia and thrombocytopenia in Ethiopian children with untreated cyanotic congenital heart diseases. There was variable implementation of iron supplementation and therapeutic phlebotomy, highlighting the need to optimize supportive management strategies in this population to mitigate the risk of life-threatening complications.
在高收入国家，外科治疗改变了先天性心脏病的病程和结局，但在获得心脏手术机会有限的撒哈拉以南非洲国家，患有先天性心脏病的儿童往往经历未经治疗的病变及其并发症的自然病程。本研究的目的是确定未经手术治疗的紫绀型先天性心脏病患儿血液学异常的发生率，确定该人群凝血异常的相关因素，并描述在这种情况下如何处理这些并发症。
在这项单中心横断面研究中，我们前瞻性地收集了患有紫绀型先天性心脏病的儿童(<18 岁)的临床和人口统计学数据。采集血样测量血液学参数。红细胞增多症定义为血细胞比容>50%，血小板减少症定义为每微升<150,000。
在 70 名入组的儿童中，总体上红细胞增多症和血小板减少症的发生率分别为 63%(44 例)和 26%(18 例)。多元逻辑回归分析显示，血细胞比容≥65%(p 值=.024)和氧饱和度<85%(p 值=.018)与中重度血小板减少症独立相关。31 名(44%)患者接受了治疗性放血，其中 84%(26/31)接受了铁补充治疗。
我们报告了未经治疗的紫绀型先天性心脏病患儿中红细胞增多症和血小板减少症的高发生率。铁补充和治疗性放血的实施情况存在差异，这突出表明需要优化该人群的支持性管理策略，以降低危及生命的并发症的风险。
