Bakeet Mohamed Abd Elaal, Mohamed Montaser Mohamed, Allam Ahmed Ahmed, Gamal Rania
M.D., Professor of Pediatrics, Faculty of Medicine, Sohag University, Egypt.
M.D., Lecturer of Pediatrics, Faculty of Medicine, Sohag University, Egypt.
Electron Physician. 2016 Nov 25;8(11):3164-3169. doi: 10.19082/3164. eCollection 2016 Nov.
Cardiomyopathy (CMP) is defined by the World Health Organization (WHO) as a disease of the myocardium associated with cardiac dysfunction. An understanding of CMP is very important, as it is a common cause of heart failure in children, and the most common indication for heart transplantation in children older than one year, but data on CMP in Egypt are scarce. The aim of this study was to determine the number, risk factors, clinical presentation, complications and outcome of different types of childhood cardiomyopathies in Sohag University Hospital.
This cross-sectional hospital based study enrolled fifty children with Cardiomyopathy in Pediatric Departments, Neonatal Care Units, and Cardiac Outpatient Clinics in Sohag University Hospital from March 01, 2014 to February 28, 2015.
Cases with Dilated Cardiomyopathy (DCMP) were 38 (76%), and those who had Hypertrophic Cardiomyopathy (HCMP) were 12 (24%). Dyspnea was the most common presenting complaint in 71% of cases. In cases with DCMP, the mean EF was 33.8, and FS was 17.11, while in cases with HCMP, the mean EF was 70.75, FS was 37. Fifty percent of cases were found to have moderate to severe PHT. Serum CK-MB was elevated in 3 (6%) cases, while serum Troponin I was elevated in 2 (4.2%) cases who diagnosed as having myocarditis. Viral myocarditis was the most common identified etiological agent responsible for 14 (37%) cases with DCMP.
CMP represents a considerable percentage of children with cardiac disorders. DCMP is the most common type, usually presented with congestive heart failure, and the most common cause is myocarditis. L-Carnitine profile was normal in all cases, despite its routine use. Pediatricians need to raise their clinical suspicion to CMPs, as atypical presentations are not uncommon. To do screening for other family members, cardiac enzymes (CK-MB, Troponin I) have to be done in all newly diagnosed CMP cases, along with a revision of the routine prescription of L-Carnitine.
世界卫生组织(WHO)将心肌病(CMP)定义为与心脏功能障碍相关的心肌疾病。了解CMP非常重要,因为它是儿童心力衰竭的常见原因,也是一岁以上儿童心脏移植最常见的指征,但埃及关于CMP的数据很少。本研究的目的是确定索哈杰大学医院不同类型儿童心肌病的数量、危险因素、临床表现、并发症及预后。
这项基于医院的横断面研究纳入了2014年3月1日至2015年2月28日期间在索哈杰大学医院儿科、新生儿护理单元和心脏门诊诊所诊断为心肌病的50名儿童。
扩张型心肌病(DCMP)患者38例(76%),肥厚型心肌病(HCMP)患者12例(24%)。71%的病例中,呼吸困难是最常见的主诉。DCMP患者的平均射血分数(EF)为33.8,缩短分数(FS)为17.11;而HCMP患者的平均EF为70.75,FS为37。50%的病例发现有中度至重度肺动脉高压(PHT)。3例(6%)患者血清肌酸激酶同工酶(CK-MB)升高,2例(4.2%)诊断为心肌炎的患者血清肌钙蛋白I升高。病毒性心肌炎是14例(37%)DCMP患者中最常见的病因。
CMP在患有心脏疾病的儿童中占相当比例。DCMP是最常见的类型,通常表现为充血性心力衰竭,最常见的病因是心肌炎。尽管常规使用左卡尼汀,但所有病例的左卡尼汀水平均正常。儿科医生需要提高对CMP的临床怀疑,因为非典型表现并不少见。对于所有新诊断的CMP病例,除了修订左卡尼汀的常规处方外,还必须检测心脏酶(CK-MB、肌钙蛋白I),以便对其他家庭成员进行筛查。