Zhao Hong, Sun Yang, Song Laifeng, Wang Qingzhi, Chu Yan, Huang Jie, Hu Shengshou
State Key Laboratory of Cardiovascular Disease, Pathology Department, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037, China; Email:
State Key Laboratory of Cardiovascular Disease, Pathology Department, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037, China.
Zhonghua Xin Xue Guan Bing Za Zhi. 2015 May;43(5):418-22.
To investigate the association between clinical and histopathological features in patients with left ventricular non-compaction cardiomyopathy (LVNC).
Histopathological examinations were made on 11 LVNC recipient hearts from June 2004 to June 2014 in Fuwai Hospital, myocardial ultrastructure changes were detected using transmission electron microscopy. Association between clinical and pathological features were analyzed.
Patients were (24 ± 11) years old. There were 6 patients with mucus matrix LVNC, 3 patients with fibrous fatty infiltration, and 2 patients with cardiomyocytes proliferation. The gross morphological changes of LVNC hearts were characterized by numerous and prominent trabeculations with deep intratrabecular recesses in left ventricular myocardium. Ratios of the thicker noncompacted endocardial layer (N) and thin epicardial compacted layer (C) (N/C ratio) were ≥ 2.0, and the most serious lesions were located in the left ventricular apex, and followed by the left ventricular free wall. Histological microscopic examinations evidenced numerous matrix-like material and immature cardiomyocytes on endocardial tissue. Transmission electron microscopy revealed mitochondrial abnormalities on morphology, number, and distribution, underdeveloped cardiomyocytes and anomalies of intercalated disc structure, increased deposition of extracellular matrix-like substance and perinuclear glycogen. Pathological changes on cytoplasmic matrix and intercalated disc were present in all three tissue types of LVNC in this cohort and mitochondria hyperplasia was detected in patients with fibrous fatty infiltration. Heart weight ≥ 350 g is often associated with increased number of mitochondria. Increased cytoplasmic matrix was often detected in patients with LVEF ≥ 30% while intercalated disc anomalies were often detected in patients with LVEF < 30%.
Histological changes were closely related clinical features in patients with LVNC.
探讨左心室致密化不全心肌病（LVNC）患者临床特征与组织病理学特征之间的关联。
对2004年6月至2014年6月期间阜外医院11例LVNC受者心脏进行组织病理学检查，采用透射电子显微镜检测心肌超微结构变化。分析临床特征与病理特征之间的关联。
患者年龄为（24±11）岁。黏液基质型LVNC患者6例，纤维脂肪浸润型患者3例，心肌细胞增殖型患者2例。LVNC心脏的大体形态学改变表现为左心室心肌内有大量突出的肌小梁，肌小梁间有深陷的隐窝。增厚的非致密心内膜层（N）与薄的致密心外膜层（C）的比值（N/C比值）≥2.0，最严重的病变位于左心室心尖，其次是左心室游离壁。组织学显微镜检查显示心内膜组织中有大量基质样物质和未成熟心肌细胞。透射电子显微镜显示线粒体在形态、数量和分布上异常，心肌细胞发育不全，闰盘结构异常，细胞外基质样物质沉积增加，核周糖原增多。该队列中LVNC的所有三种组织类型均存在细胞质基质和闰盘的病理改变，纤维脂肪浸润患者检测到线粒体增生。心脏重量≥350 g常与线粒体数量增加有关。左心室射血分数（LVEF）≥30%的患者常检测到细胞质基质增加，而LVEF<30%的患者常检测到闰盘异常。
LVNC患者的组织学改变与临床特征密切相关。
