Cardiac magnetic resonance imaging of myocardial contrast uptake and blood flow in patients affected with idiopathic or familial dilated cardiomyopathy
- 1 September 2008
- journal article
- research article
- Published by American Physiological Society in American Journal of Physiology-Heart and Circulatory Physiology
- Vol. 295 (3), H1234-H1242
- https://doi.org/10.1152/ajpheart.00429.2008
Abstract
Idiopathic dilated cardiomyopathy (IDC) is characterized by left ventricular (LV) enlargement with systolic dysfunction, other causes excluded. When inherited, it represents familial dilated cardiomyopathy (FDC). We hypothesized that IDC or FDC would show with cardiac magnetic resonance (CMR) increased myocardial accumulation of gadolinium contrast at steady state and decreased baseline myocardial blood flow (MBF) due to structural alterations of the extracellular matrix compared with normal myocardium. CMR was performed in nine persons affected with IDC/FDC. Healthy controls came from the general population ( n = 6) or were unaffected family members of FDC patients ( n = 3) without signs or symptoms of IDC/FDC or any structural cardiac abnormalities. The myocardial partition coefficient for gadolinium contrast (λGd) was determined by T1 measurements. LV shape and function and MBF were assessed by standard CMR methods. λGd was elevated in IDC/FDC patients vs. healthy controls (λGd = 0.56 ± 0.15 vs. 0.41 ± 0.06; P = 0.002), and correlated with LV enlargement ( r = 0.61 for λGd vs. end-diastolic volume indexed by height; P < 0.01) and with ejection fraction ( r = −0.80; P < 0.001). The extracellular volume fraction was higher in IDC patients than in healthy controls (0.31 ± 0.05 vs. 0.24 ± 0.03; P = 0.002). Resting MBF was lower in IDC patients (0.64 ± 0.13 vs. 0.91 ± 0.22; P = 0.01) than unaffected controls and correlated with both the partition coefficient ( r = −0.57; P = 0.012) and the extracellular volume fraction ( r = −0.56; P = 0.019). The expansion of the extracellular space correlated with reduced MBF and ventricular dilation. Expansion of the extracellular matrix may be a key contributor to contractile dysfunction in IDC patients.This publication has 45 references indexed in Scilit:
- First‐pass dynamic contrast‐enhanced MRI with extravasating contrast reagent: evidence for human myocardial capillary recruitment in adenosine‐induced hyperemiaNMR in Biomedicine, 2008
- Quantitative myocardial distribution volume from dynamic contrast-enhanced MRIMagnetic Resonance Imaging, 2008
- Coding Sequence Mutations Identified in MYH7, TNNT2, SCN5A, CSRP3, LBD3, and TCAP from 313 Patients with Familial or Idiopathic Dilated CardiomyopathyClinical and Translational Science, 2008
- Clinical Characteristics of 304 Kindreds Evaluated for Familial Dilated CardiomyopathyJournal of Cardiac Failure, 2006
- Feasibility of the single‐bolus strategy for measuring the partition coefficient of Gd‐DTPA in patients with myocardial infarction: Independence of image delay time and maturity of scarMagnetic Resonance in Medicine, 2006
- Determining the extent to which delayed‐enhancement images reflect the partition‐coefficient of Gd‐DTPA in canine studies of reperfused and unreperfused myocardial infarctionMagnetic Resonance in Medicine, 2004
- The influence of myocardial blood flow and volume of distribution on late Gd‐DTPA kinetics in ischemic heart failureJournal of Magnetic Resonance Imaging, 2004
- Treatment of Dilated Cardiomyopathy With Electroporation of Hepatocyte Growth Factor Gene Into Skeletal MuscleHypertension, 2004
- Histopathologic findings in explanted heart tissue from patients with end-stage idiopathic dilated cardiomyopathyTransplant International, 2001
- Guidelines for the study of familial dilated cardiomyopathiesEuropean Heart Journal, 1999