Soongswang Jarupim, Sangtawesin Chaisit, Sittiwangkul Rekwan, Wanitkun Suthep, Muangmingsuk Sunthorn, Sopontammarak Somkiat, Klungratana Chamaporn, Kangkagate Charuwan
Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
J Med Assoc Thai. 2002 Aug;85 Suppl 2:S648-57.
Myocardial diseases are among the important causes of mortality and morbidity in children. This drew the authors attention to the study of myocardial diseases in children to find out the outcome, factors affecting the outcome, and management strategies. The authors retrospectively studied children who had been diagnosed with primary myocardial diseases at six university hospitals in Thailand from January 1996 to December 2000. The total number of cases was 209 which accounted for 1.2 per cent of cardiovascular diseases in children. The patients' ages ranged from 0.1-15 years. These myocardial diseases included dilated cardiomyopathy (DCM) 45 per cent, acute myocarditis 27.3 per cent, hypertrophic cardiomyopathy (HCM) 18.2 per cent, hypertrophic obstructive cardiomyopathy (HOCM) 8.1 per cent and restrictive cardiomyopathy (RCM) 1.4 per cent. Fifty-six per cent of the patients were female. Congestive heart failure was the most common presenting symptom (75%). Median ejection fraction (EF) of acute myocarditis was 42 per cent (15-79%) which was significantly higher than DCM (33.5%, 10-57%). Serum cardiac troponin T (cTnT) was also significantly higher in acute myocarditis than in DCM (0.08 ng/ml, 0.01-0.16 vs 0.01 ng/ml, 0.01-0.10). Within the follow-up period of 1 year (0.1-5.5 years), the mortality rates were 18.8 per cent, 17.0 per cent, 5.4 per cent and 33.3 per cent in DCM, acute myocarditis, HCM and RCM respectively. Factors associated with the mortality rate in acute myocarditis were admission to ICU and low left ventricular EF at presentation while IVIG administration and cTnT level did not.
Primary myocardial diseases are uncommon. Most of the patients had compromised cardiovascular reserve. Admission to ICU and low EF were factors that affected the mortality in acute myocarditis while intravenous immunoglobulin administration did not. Mortality rate in the subacute follow-up period was high in all groups.
心肌病是儿童死亡和发病的重要原因之一。这引起了作者对儿童心肌病的研究兴趣,以了解其预后、影响预后的因素及治疗策略。作者回顾性研究了1996年1月至2000年12月期间在泰国六所大学医院被诊断为原发性心肌病的儿童。病例总数为209例,占儿童心血管疾病的1.2%。患者年龄在0.1至15岁之间。这些心肌病包括扩张型心肌病(DCM)占45%,急性心肌炎占27.3%,肥厚型心肌病(HCM)占18.2%,肥厚型梗阻性心肌病(HOCM)占8.1%,限制型心肌病(RCM)占1.4%。56%的患者为女性。充血性心力衰竭是最常见的首发症状(75%)。急性心肌炎的中位射血分数(EF)为42%(15 - 79%),显著高于扩张型心肌病(33.5%,10 - 57%)。急性心肌炎患者血清心肌肌钙蛋白T(cTnT)也显著高于扩张型心肌病患者(0.08 ng/ml,0.01 - 0.16对比0.01 ng/ml,0.01 - 0.10)。在1年(0.1 - 5.5年)的随访期内,扩张型心肌病、急性心肌炎、肥厚型心肌病和限制型心肌病的死亡率分别为18.8%、17.0%、5.4%和33.3%。与急性心肌炎死亡率相关的因素是入住重症监护病房(ICU)和就诊时左心室EF值低,而静脉注射免疫球蛋白(IVIG)的使用和cTnT水平则无关。
原发性心肌病并不常见。大多数患者的心血管储备功能受损。入住ICU和低EF值是影响急性心肌炎死亡率的因素,而静脉注射免疫球蛋白的使用则不是。所有组在亚急性随访期的死亡率都很高。