Association between dysplastic development of ventricular wall and primary cardiomyopathy:pathomorphological observations on 92 hearts from transplant recipients
10.3321/j.issn:0253-3758.2008.10.009
- VernacularTitle:心壁错构性发育不良与原发性心肌病
- Author:
Lai-Feng SONG
1
;
Hong-Yue WANG
;
Hong ZHAO
;
Feng-Ying L(U)
;
Sheng-Shou HU
;
Jie HUANG
;
Li LI
;
Lei LIU
;
Lin-Lin WANG
Author Information
1. 中国医学科学院北京协和医学院心血管病研究所阜外心血管病医院
- Keywords:
Cardiomyopathies;
Heart transplantation;
Cardiac wall displasia
- From:
Chinese Journal of Cardiology
2008;36(10):897-902
- CountryChina
- Language:Chinese
-
Abstract:
Objective To investigate the morphological characteristics and types of ventricular wall with dysplastic development and their associations to primary cardiomyopathy.Methods Ninety-two hearts from heart transplant patients were studied soon after explanation from 2004 to 2007.Gross examination/measurement,histopathology and photography were performed.Results Dysplastic development of ventricular wail could be evidenced in patients with various heart diseases but more often in patients with primary cardiomyopathy,though the extension and distribution of dysplastic development of ventricular wall varied between patients with or without primary cardiomyopathy.Severe dysplastic development of ventricular wall is associated with clinical dysplastic cardiomyopathy.The range of extension and degree of dysplasia in the ventricular wall correlated positively to heart dilation/failure and time point of heart failure development.The incidence of severe vantricular wall dysplasia was 27.17% in all transplanted hearts and was 43.1%(25/58) in hearts diagnosed as primary cardiomyopathy (P＜0.05).The main pathological changes of dysplastic hearts were:(1) extensive proliferative hypertrophy of the heart wall,(2) fibrous/fat or fat/fibrous tissue replacement of normal myocardium,(3) disarrangement of myocardial fibers,(4)dysplastic change in the medium-sized intramural arteries.Dysplastic cardiomyopathy was presented mainly as a combination of several forms of dysplasia.The same clinical manifestations of dysplastic cardiomyopathy patients did not always show the same pathologic changes.Fibrous-fat tissue replacement was commonly found in dilated cardiomyopathy and arrhythmogenic right ventricular eardiomyopathy.Disarrangement of myocardium was often accompanied by hypertrophic cardiomyopathy.Dysplasia of intramural arteries could result in heart dilatation due to myocardial ischemia.Conclusion Dysplasia of ventricular wall is a common variation of heart structure.Only severe or diffuse types of dysplasia is associated with cardiomyopathy,especially primary cardiomyopathy.
