Varnava A M, Elliott P M, Sharma S, McKenna W J, Davies M J
Department of Cardiovascular Pathology, St George's Hospital Medical School, Cranmer Terrace, London SW17 0RE, UK.
Heart. 2000 Nov;84(5):476-82. doi: 10.1136/heart.84.5.476.
To make a quantitative assessment of the relation between disarray, fibrosis, and small vessel disease in hypertrophic cardiomyopathy.
Detailed macroscopic and histological examination at 19 segments of the left and right ventricle and the left atrial free wall.
72 patients with hypertrophic cardiomyopathy who had suffered sudden death or progression to end stage cardiac failure (resulting in death or heart transplantation).
The presence of scarring, atrial dilatation, and a mitral valve impact lesion were noted, and heart weight, wall thickness, per cent disarray, per cent fibrosis, and per cent small vessel disease quantitated for each heart.
Within an individual heart the magnitude of hypertrophy correlated with the severity of fibrosis (p = 0.006) and disarray (p = 0.0002). Overall, however, total heart weight related weakly but significantly to fibrosis (r = 0.4, p = 0.0001) and small vessel disease (r = 0.3, p = 0.03), but not to disarray. Disarray was greater in hearts with mild left ventricular hypertrophy (maximum wall thickness < 20 mm) and preserved systolic function (60.9 (26)% v 43 (20.4)% respectively, p = 0.02) and hearts without a mitral valve impact lesion (26.3% v 18.9%, p = 0.04), but was uninfluenced by sex. Fibrosis was influenced by sex (7% in male patients and 4% in female, p = 0.04), but not by the presence of an impact lesion. No relation was found between disarray, fibrosis, and small vessel disease.
Myocyte disarray is probably a direct response to functional or structural abnormalities of the mutated sarcomeric protein, while fibrosis and small vessel disease are secondary phenomena unrelated to disarray, but modified by factors such as left ventricular mass, sex, and perhaps local autocrine factors.
对肥厚型心肌病中肌束紊乱、纤维化和小血管疾病之间的关系进行定量评估。
对左、右心室及左心房游离壁的19个节段进行详细的大体和组织学检查。
72例肥厚型心肌病患者，这些患者经历了猝死或进展为终末期心力衰竭（导致死亡或心脏移植）。
记录瘢痕形成、心房扩张和二尖瓣撞击病变的存在情况，并对每颗心脏的心脏重量、壁厚、肌束紊乱百分比、纤维化百分比和小血管疾病百分比进行定量。
在个体心脏中，肥厚程度与纤维化严重程度（p = 0.006）和肌束紊乱严重程度（p = 0.0002）相关。然而，总体而言，心脏总重量与纤维化（r = 0.4，p = 0.0001）和小血管疾病（r = 0.3，p = 0.03）呈弱但显著的相关性，但与肌束紊乱无关。在轻度左心室肥厚（最大壁厚<20 mm）且收缩功能保留的心脏中（分别为60.9(26)%和43(20.4)%，p = 0.02）以及没有二尖瓣撞击病变的心脏中（26.3%对18.9%，p = 0.04），肌束紊乱更严重，但不受性别影响。纤维化受性别影响（男性患者为7%，女性为4%，p = 0.04），但不受撞击病变存在的影响。未发现肌束紊乱、纤维化和小血管疾病之间存在关联。
肌细胞紊乱可能是对突变肌节蛋白功能或结构异常的直接反应，而纤维化和小血管疾病是与肌束紊乱无关的继发现象，但受左心室质量、性别以及可能的局部自分泌因子等因素影响。
