[儿童孤立性心肌致密化不全——病例报告及文献复习]

[Isolated myocardial noncompaction in childhood-case report and review of the literature].

作者信息

Binz G, Knirsch W, Lewin M A G, Haas N A, Koch W, Kandolf R, Uhlemann F

机构信息

Olgahospital Akademisches Lehrkrankenhaus der Universität Tübingen, Bismarckstr. 8, 70176 Stuttgart, Germany.

出版信息

Z Kardiol. 2003 Dec;92(12):1039-44. doi: 10.1007/s00392-003-1017-1.

DOI:10.1007/s00392-003-1017-1

PMID:14663616

Abstract

The diagnostic work-up for the first syncopal episode of a 14-year-old female adolescent revealed the morphology of an isolated myocardial non-compaction on echography. Angiography and biopsy of the left ventricle confirmed the diagnosis. Despite a reduced shortening fraction (FS 21%) and frequent premature atrial beats, there were no further cardiac or extracardiac symptoms. After establishment of therapy with beta-blockade, digitalis, angiotensin converting enzyme inhibition and acetylsalic acid, the follow-up over 24 months was good; the implantation of an automatic implantable cardioverter-defibrillator is planned.

摘要

对一名14岁女性青少年首次晕厥发作的诊断检查显示，超声心动图呈现孤立性心肌致密化不全的形态。左心室造影和活检证实了诊断。尽管缩短分数降低（FS 21%）且频发房性早搏，但未出现进一步的心脏或心外症状。在用β受体阻滞剂、洋地黄、血管紧张素转换酶抑制剂和阿司匹林进行治疗后，24个月的随访情况良好；计划植入自动植入式心脏复律除颤器。