CMR scarring in a patient with hypertrophic cardiomyopathy correlates well with histological findings of fibrosis
1I. Medical Department, University Hospital Mannheim, 68167 Mannheim, Germany
2Department of Pathology, University Heidelberg, Heidelberg, Germany
3Department of Radiology, University Hospital Mannheim, 68167 Mannheim, Germany
* Corresponding author. Tel: +49 621 3832512; fax: +49 621 3832012. E-mail address: theano.papavassilliu{at}med.ma.uni-heidelberg.de
A 38-year-old female patient with hypertrophic cardiomyopathy and congestive heart failure underwent late gadolinium enhancement cardiovascular magnetic resonance (CMR). Cine CMR (Panel A) revealed normal dimensions of the left ventricle (left ventricular end-diastolic diameter 45 mm) with moderately reduced left ventricular systolic function (ejection fraction 40%). The maximum wall thickness was 16 mm and there was severe dilation of both atria. Using a T1-weighted inversion-recovery sequence 15 min after the application of 0.2 mmol/kg body weight gadolinium-DTPA, the anteroseptal, inferoseptal, and the anterior walls (Panel B) of the left ventricle showed extensive regions of myocardial enhancement. During her hospital course, the patient developed malignant ventricular arrhythmias and underwent urgent heart transplantation 30 days after the CMR scan. The explanted heart was examined histologically for the extent of fibrosis and disarray. The macroscopic examination of the explanted heart showed massive scarring. Microscopy showed areas of myofibril disarray and scars. These findings were diagnostic of HCM. Regions of gadolinium enhancement correlate well with regions of macroscopic pale myocardium (Panel C) and regions of fibrosis (Panel D).
Panel A. Pre-contrast diastolic long-axis cine image in vivo obtained with cine CMR.
Panel B. Post-contrast image demonstrating myocardial hyperenhancement in vivo. There is a large area of mid-myocardial late gadolinium enhancement of the anterior wall (arrows).
Panel C. Corresponding gross specimen from the explanted heart with well-defined regions of macroscopic mid-myocardial pale myocardium similar to the distribution of late gadolinium enhancement in Panel B.
Panel D. Histological section (haematoxylin and eosin stain) shows a magnification of the box in Panel C with pronounced fibrosis (sheets of collagen stained pink). There is a concordance between regions of gadolinium enhancement in vivo and regions of fibrotic tissue.
CiteULike 
Connotea 
Del.icio.us What's this?
This article has been cited by other articles:
|
|
 |
|
  M. Michels, O. I.I. Soliman, J. Phefferkorn, Y. M. Hoedemaekers, M. J. Kofflard, D. Dooijes, D. Majoor-Krakauer, and F. J. Ten Cate Disease penetrance and risk stratification for sudden cardiac death in asymptomatic hypertrophic cardiomyopathy mutation carriers Eur. Heart J., November 1, 2009; 30(21): 2593 - 2598. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. S. Maron, I. Olivotto, B. J. Maron, S. K. Prasad, F. Cecchi, J. E. Udelson, and P. G. Camici The case for myocardial ischemia in hypertrophic cardiomyopathy. J. Am. Coll. Cardiol., August 25, 2009; 54(9): 866 - 875. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Rudolph, H. Abdel-Aty, S. Bohl, P. Boye, A. Zagrosek, R. Dietz, and J. Schulz-Menger Noninvasive detection of fibrosis applying contrast-enhanced cardiac magnetic resonance in different forms of left ventricular hypertrophy relation to remodeling. J. Am. Coll. Cardiol., January 20, 2009; 53(3): 284 - 291. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. S. Maron, E. Appelbaum, C. J. Harrigan, J. Buros, C. M. Gibson, C. Hanna, J. R. Lesser, J. E. Udelson, W. J. Manning, and B. J. Maron Clinical Profile and Significance of Delayed Enhancement in Hypertrophic Cardiomyopathy Circ Heart Fail, September 1, 2008; 1(3): 184 - 191. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. W. Hansen and N. Merchant MRI of Hypertrophic Cardiomyopathy: Part I, MRI Appearances Am. J. Roentgenol., December 1, 2007; 189(6): 1335 - 1343. [Abstract] [Full Text] [PDF]
|
|
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||