Association between clinical features and histopathological findings in patients with left ventricular non-compaction cardiomyopathy
10.3760/cma.j.issn.0253-3758.2015.05.011
- VernacularTitle:左心室致密化不全的超微结构与临床和病理表现的关系
- Author:
Hong ZHAO
1
;
Yang SUN
;
Laifeng SONG
;
Qingzhi WANG
;
Yan CHU
;
Jie HUANG
;
Shengshou HU
Author Information
1. 100037,中国医学科学院北京协和医学院国家心血管病中心阜外心血管病医院心血管疾病国家重点实验室
- Keywords:
Cardiomyopathies;
Histology;
Microscope,electron,transmission
- From:
Chinese Journal of Cardiology
2015;43(5):418-422
- CountryChina
- Language:Chinese
-
Abstract:
Objective To investigate the association between clinical and histopathological features in patients with left ventricular non-compaction cardiomyopathy (LVNC).Methods 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.Results 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％.Conclusion Histological changes were closely related clinical features in patients with LVNC.
