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苏丹患心肌病儿童的临床、超声心动图特征及预后

Clinical and echocardiographic patterns and outcomes of Sudanese children with cardiomyopathy.

作者信息

Mamoun Amna, Ali Sulafa

机构信息

Department of Pediatric Cardiology, Pediatric Cardiology Fellow, Sudan Medical Specialization Board, Khartoum, Sudan.

Department of Pediatric Cardiology, Consultant Pediatric Cardiologist, Gaafar Ibnauf Children's Hospital and Sudan Heart Center, Khartoum, Sudan.

出版信息

Sudan J Paediatr. 2021;21(2):131-136. doi: 10.24911/SJP.106-1600099020.

Abstract

Cardiomyopathy (CMP) constitutes a group of diseases of heart muscle that lead to significant mortality and morbidity, with limited data in Africa. This study aims to describe clinical and echocardiographic (echo) patterns and short-term outcomes of Pediatric CMP. The study was carried at Gaafar Ibnauf Children's Hospital and included all children 0-18 years with an echo diagnosis of CMP. A retrospective part of the study was carried from January 2013 to 2019 and a prospective part from January to June 2019. Clinical and echo data were collected on presentation and on at least one follow up. A total of 146 patients were diagnosed with CMP during the study period. Most patients (48%) presented at 1-6 years of age, neonatal CMP was present in 4%. Familial incidence was detected in 11% of cases. Heart failure was the most common mode of presentation in 96% of patients; 65% needed hospitalisation; a third of them needed intensive care unit admission. The most common type of CMP was the dilated type (67%). Others included noncompaction CMP (19%), restrictive CMP and hypertrophic CMP (each of them in 6% of patients). Hospital mortality was 20% for the whole cohort and 100% for neonates. Other complications occurred in 21% of patients including arrhythmias, cerebrovascular accidents and end stage renal disease. Echo follow up showed that most patients (52%) remained the same, 26% worsened and 21% improved. Genetic and metabolic studies that could help to improve management and outcomes of CMP are needed.

摘要

心肌病(CMP)是一组心肌疾病,可导致严重的死亡率和发病率,而非洲地区关于这方面的数据有限。本研究旨在描述儿童心肌病的临床和超声心动图(超声)特征以及短期预后。该研究在加法尔·伊本纳夫儿童医院开展,纳入了所有0至18岁经超声诊断为心肌病的儿童。研究的回顾性部分涵盖2013年1月至2019年,前瞻性部分为2019年1月至6月。收集了患者就诊时及至少一次随访时的临床和超声数据。在研究期间,共有146例患者被诊断为心肌病。大多数患者(48%)在1至6岁时发病,4%为新生儿心肌病。11%的病例有家族发病情况。心力衰竭是96%患者最常见的就诊原因;65%的患者需要住院治疗;其中三分之一需要入住重症监护病房。最常见的心肌病类型是扩张型(67%)。其他类型包括心肌致密化不全型心肌病(19%)、限制型心肌病和肥厚型心肌病(各占患者的6%)。整个队列的医院死亡率为20%,新生儿为100%。21%的患者出现了其他并发症,包括心律失常、脑血管意外和终末期肾病。超声随访显示,大多数患者(52%)病情稳定,26%恶化,21%改善。需要开展有助于改善心肌病管理和预后的基因和代谢研究。

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