The feasibility of using hyperpolarized 13C to interrogate in vivo human metabolism in the healthy heart has recently been demonstrated. In this abstract we demonstrate the feasibility of using hyperpolarized 13C imaging to detect metabolic alterations in human hypertrophic cardiomyopathy. Results show significantly elevated 13C-bicarbonate-to-pyruvate ratio near the apex of the heart, corresponding to the known location of disease. The 13C-bicarbonate images also show a different spatial distribution from those observed in healthy volunteers. These results show good prospects for imaging the altered cardiac energetics in the diseased heart using this technology.
One patient with non-obstructive apical hypertrophic cardiomyopathy (HCM) and six healthy male subjects (N=6) were recruited and gave written informed consent under a protocol approved by the institutional Research Ethics Board and approved by Health Canada as a Clinical Trial Application. An oral carbohydrate load (35g Gatorade powder in water) was administered approximately 1 hour before the pyruvate injection.
Imaging was performed with a GE MR750 3T. 1H-SSFP-CINE images were acquired using the body coil for anatomical reference and cardiac functional assessment.
The substrate [1-13C]pyruvic acid was polarized in a SpinLab DNP polarizer (GE Healthcare) as previously described[3]. Following dissolution, neutralization, and quality control checks, the pyruvate dose (0.1 mmol/kg) was injected at a rate of 5 mL/s, followed by 25 mL saline flush. 13C imaging was started at the end of the saline flush. Short-axis images of 13C-bicarbonate, [1-13C]lactate, and [1-13C]pyruvate were obtained using a multi-slice, spectrally-selective sequence covering the left ventricle (1x1x1 cm3 resolution, 6 slices, scan time 18 cardiac cycles, volume transmit coil, 8 channel 13C receive coil)[4]. Following 13C imaging, dynamic 13C MRS was used to monitor residual metabolic signals from the entire heart.
Images were manually segmented according to left ventricular (LV) and myocardial components. Mean myocardial 13C-bicarbonate and 13C-lactate signals were used to index PDH and LDH activity. Metabolite ratios (Bic/Pyr and Lac/Pyr) were calculated by normalizing to the mean LV pyruvate signal within each slice to remove inter-subject variations in coil positioning, polarization, excitation flip angle, and perfusion.
Hypertrophic cardiomyopathy is characterized by increased glucose metabolism. We hypothesized that metabolic ratios (Bic/Pyr and Lac/Pyr) within the region of disease would reflect altered metabolism, relative to the hearts of healthy subjects. For each slice, disease Bic/Pyr and Lac/Pyr signals were compared to pooled control values from the same slice.
In general, we hypothesized that the presence of disease would be linked with greater dispersion of metabolic ratio across the different slices of the heart. To test this, SD(Bic/Pyr) and SD(Lac/Pyr) were computed across the different image slices, for each subject. SD is known to be sensitive to the presence of outliers.
Signals (Bic/Pyr, Lac/Pyr, and SDs) were modeled as normally distributed across subjects. P-values corresponding to null hypothesis rejection were obtained using the difference between disease and control values, expressed in SDs from the mean.
Figure 1 shows summed 13C images from both healthy volunteers and a HCM patient. The images show pyruvate distributed within the blood pool, and 13C-bicarbonate spatially localized to the myocardium. In the HCM patient, bicarbonate signal was highest towards the apex of the heart, which corresponded to the apical hypertrophy (Figure 2). Figure 3 demonstrates that the apical Bic/Pyr is 2.4 SDs above the mean (p=0.017). All other ratios lie at most 1 SD above the mean value in their respective slices. Figure 4 shows that the standard deviation of Bic/Pyr across slices in the HCM patient was 3.1 SDs above the mean (p=0.0019). A significant difference in Lac/Pyr was not detected between control and disease groups.
The 13C MRS data (Figure 5) agree with elevated Bic/Pyr in the HCM patient relative to control. A diffuse pattern was also observed in the bicarbonate images; this was due to an off-resonance mis-setting of the transmitter frequency by 90 Hz, which resulted in contamination by pyruvate hydrate signal within the bicarbonate image.