title: Cardiac magnetic resonance imaging of systemic amyloidosis patients with normal left ventricular ejection fraction: An initial study.
abstract: OBJECTIVE
  The purpose of this study was to find whether Cardiac Magnetic Resonance (CMR) could assess the myocardial interstitium in patients suffering from systemic amyloidosis with normal left ventricular ejection fraction.
  
  
  METHODS
  Twenty Six patients in whom systemic amyloidosis was confirmed by kidney biopsy were investigated. Five patients with normal left ventricular ejection fraction were selected. The heart function of the patients was diagnosed by two-dimensional transthoracic echocardiography. The main MR sequences include an inversion recovery prepared echo planar imaging perfusion sequence, inversion recovery TrueFISP sequence (delayed enhancement) and TrueFISP cine sequence for heart function measurement (including ejection fraction (EF), end diastolic volume (EDV), end systolic volume (ESV), stroke volume (SV) and cardiac output (CO)). Results : Perfusion defects were seen in three patients. In these patients, myocardial enhancement was visible on late gadolinium enhancement images. The enhancement pattern was diffuse in three patients and focal in two patients. Heart dysfunction was mild, as follows: EF normal (range, 56-75%; mean, 69.4%), ESV normal (range, 15.7-30.0; mean, 23.0), EDV decreased (range, 42.1-96.6; mean, 72.7), SV decreased (range, 23.7-68.6; mean, 49.6) and CO normal (range, 2.6-5.9; mean, 3.9). Hematoxylin and eosin stain and Congo red stain demonstrated typical amyloid deposits. Amyloidosis was classified as amyloid light chain by kappa and lambda stain.
  
  
  CONCLUSIONS
  Cardiac Magnetic Resonance could detect abnormal myocardial interstitium in systemic amyloidosis patients with normal left ventricular ejection fraction.
authors.affiliation: Rui Xia, PhD, Resident Doctor, Department of Radiology, West China Hospital, Sichuan University, No. 37 Guoxuexiang, 610041 Chengdu, China.
authors.forename: Rui
authors.initials: R
authors.lastname: Xia
authors.affiliation: Fabao Gao, MD PhD, Professor, Department of Radiology, West China Hospital, Sichuan University, No. 37 Guoxuexiang, 610041 Chengdu, China.
authors.forename: Fabao
authors.initials: F
authors.lastname: Gao
authors.affiliation: Jiayu Sun, PhD, Supervising Technician, Department of Radiology, West China Hospital, Sichuan University, No. 37 Guoxuexiang, 610041 Chengdu, China.
authors.forename: Jiayu
authors.initials: J
authors.lastname: Sun
authors.affiliation: Chunchao Xia, BS, Technician, Department of Radiology, West China Hospital, Sichuan University, No. 37 Guoxuexiang, 610041 Chengdu, China.
authors.forename: Chunchao
authors.initials: C
authors.lastname: Xia
authors.affiliation: Zhangxue Hu, MD PhD, Professor, Department of Nephrology, West China Hospital, Sichuan University, No. 37 Guoxuexiang, 610041 Chengdu, China.
authors.forename: Zhangxue
authors.initials: Z
authors.lastname: Hu
authors.affiliation: Yingkun Guo, MD PhD, Associate Professor, Department of Radiology, West China Second University Hospital, Sichuan University, No. 20 Sec 3 Renmin Road South, 610041 Chengdu, China.
authors.forename: Yingkun
authors.initials: Y
authors.lastname: Guo
keywords: Late gadolinium enhancement; Magnetic resonance imaging; Myocardial amyloidosis; Systemic amyloidosis; echocardiography