MR imaging and MR spectroscopy for characterization of cardiomyopathies in adolescents - preliminary results

Cardiomyopathy is a rare but life-threatening disease in children and adolescents. Recent studies reported morphological, functional or metabolic alterations of the heart. We discuss a combined MR imaging and (31)P MR spectroscopy (MRS) protocol allowing the analysis of interdependencies between these parameters. Since normal values of cardiac MR parameters in this age group are not available, we included studies of age-matched healthy adolescents. 2D-CINE was used to assess left ventricular (LV) parameters. Additional 3D-Chemical Shift Imaging (3D-CSI) and Spectral Localization with Optimal Pointspread Function (SLOOP) reconstruction allowed quantification of the cardiac energy metabolism. Patients (n = 4; all male; age 16.8 +/- 2.9 years) were included on the basis of an echocardiographic diagnosis of possible cardiomyopathy. The same protocol was applied to healthy young volunteers (n = 4; 1 female, 3 male; age 15.5 +/- 0.6 years). The patients had a significantly higher LV mass index compared to the control group (147 +/- 41 g/m (2) versus 97 +/- 16 g/m2; p = 0.04). The other LV parameters (including LV EF with 59 +/- 22 % versus 67 +/- 10 %) showed no significant differences. The phosphocreatine to adenosine triphosphate ratio (PCr/ATP-ratio) of the patients was reduced to 1.71 +/- 0.40 versus 2.44 +/- 0.30 (p = 0.01), combined with a tendency towards decreased PCr concentrations of 9.1 +/- 2.5 versus 7.9 +/- 1.0 mmol/kg. The combination of (31)P MR spectroscopy and MR imaging allows quantitative determination of morphologic, functional and metabolic alterations in adolescents with suspected cardiomyopathy in one examination procedure. The reduction of energy metabolism combined with unaltered global function may indicate a primary role of metabolism in the pathogenesis of cardiomyopathies in adolescents.