title: [Myocardial changes in heart transplantation recipients with primary restrictive cardiomyopathy].
abstract: OBJECTIVE
  To investigate the histopathological features of primary restrictive cardiomyopathy (PRCM).
  
  
  METHODS
  Nine extransplanted hearts from heart transplantation recipients were examined. Gross and histopathological findings were observed, photographed and final pathological diagnosis was compared to clinical diagnosis. The myocardial ultrastructure changes were determined using transmission electron microscopy.
  
  
  RESULTS
  The hallmark pathologic feature of PRCM was distinguished by myocardial cell degeneration and hyperplastic collagen fibrils around the myocardial cells.Fibrosis was severer in left ventricle free wall than in ventricular septum and right ventricle. The degree of myocardial cell degeneration and poloidal disorder were severer in patients with reduced ejection fraction (EF) than in patients with preserved EF. Transmission electron microscope evidenced severe interstitial fibrosis, myofibrillar changes of sarcomere structure, abnormalities both on intercalated disc number and distribution.
  
  
  CONCLUSIONS
  PRCM is characterized by hyperplastic collagen fibrils around the cardiomyocytes. Fibrosis is severer in left ventricle than in right ventricle. Sarcomere dysplasia is the main cause of PRCM, and ultrastructural examination is helpful for PRCM diagnosis.
authors.affiliation: 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: zhaohongfw@aliyun.com.
authors.forename: Hong
authors.initials: H
authors.lastname: Zhao
authors.forename: Yang
authors.initials: Y
authors.lastname: Sun
authors.forename: Laifeng
authors.initials: L
authors.lastname: Song
authors.forename: Li
authors.initials: L
authors.lastname: Li
authors.forename: Ying
authors.initials: Y
authors.lastname: Tang
authors.forename: Xuejing
authors.initials: X
authors.lastname: Duan
authors.forename: Hongyue
authors.initials: H
authors.lastname: Wang
authors.forename: Qingzhi
authors.initials: Q
authors.lastname: Wang
authors.forename: Yan
authors.initials: Y
authors.lastname: Chu
authors.forename: Jie
authors.initials: J
authors.lastname: Huang
authors.forename: Shengshou
authors.initials: S
authors.lastname: Hu
mesh_terms: D002313:Cardiomyopathy, Restrictive / Q000601:surgery*; D005355:Fibrosis; D016027:Heart Transplantation; D006352:Heart Ventricles; D006801:Humans; D009206:Myocardium / Q000473:pathology*; D032383:Myocytes, Cardiac; D012518:Sarcomeres