心室致密化不全诊治进展文件材料.pptVIP

心室致密化不全诊疗进展 周更须 概述 心肌致密化不全(noncompaction ventricular myocardium,NVM)又称海绵状心肌(spongy myocardium)，临床上较为少见。近年来国内外对该病逐渐认识，2006年心肌病分类为遗传性心肌病 心肌致密化不全是一种罕见的先天性心肌病，其病因及发病率不明，可能是由于在胚胎早期网状肌小梁致密化过程失败导致小梁化的心肌持续存在所致。多见于左心室，也可见于右心室及双心室，可单发或呈家族聚集性。 病理病理生理 A section has been taken from the interventricular septum B Low-power view of histologic section showing a deep myocardial recess in close proximity to the epicardial adipose tissue (hematoxylin and eosin stain; Original magnification, 40) C Low-power view of histologic section showing multiple trabeculations and myocardial projections, with extensive fibrosis (Klatskin trichrome stain; original magnification, 10) D Low-power view of histologic section showing a thickened endocardium overlying the myocardial recesses, with areas of patchy fibrosis (Klatskin stain; original magnification, 40) 病理病理生理 Microphotograph of a transverse section at the level of both ventricles of a heart that shows extensively developed trabeculae that fill the ventricular lumen. Note the form of the more compacted ventricular septum 临床特点 儿童多见，本病在成人发病率高于预期，有家族倾向 临床表现无特异性，心肌致密化不全患者出现症状的年龄和临床表现程度个体差异很大，从无症状到严重心功能不全，或发生猝死 虽然心肌致密化不全是先天性心肌病，但心脏病症状的出现常在成年 临床特点 Demographic characteristics of 53 patients Male gender 25 (47.2%) Age at Diagnosis, All 43.2 ± 14.76 (range 16–74) Men 40.9 ± 13.65( range 16–60) Women 45.2 ± 15.65(range 21–74) Age, years Number Percent 10–20 2 (3.8%) 21–30 13 (24.5%) 31–40 7 (13.2%) 41–50 11 (20.8%) 50 20 (37.7%) Duration 7 ± 5 months( range 1–24) Prevalence in the INCICh 4/10,000/5 years 临床特点 Clinical and electrocardiographic characteristics IN 53 CASES OF NVM Precordial pain 11 （21%） Syncope 5 （9.4%） NYHA Functional class I/II 40 （75%） NYHA Functional class III/IV 13 （25%） Familiar occurrence 16 （30.2%） Cardiac Rhythm Sinus 48 （90.5%） Atrial fibrillation 3 （5.7%） Pacemaker 2 （3.8%） Bundle branch block 25 （47.2%