Biatrial performance in children with hypertrophic cardiomyopathy: CMR study.

OBJECTIVES: To investigate biatrial mechanics and their relation with left ventricular outflow tract (LVOT) obstruction (LVOTO), the degree of hypertrophy, indices of ventricular diastolic function and fibrosis in children with hypertrophic cardiomyopathy (HCM). METHODS: Fifty-five consecutive, prospectively recruited children with HCM (mean age 12.5 ± 4.6 years, 69.1% male), 19 (34.5%) of whom had LVOTO, underwent cardiac magnetic resonance and echocardiography with quantification of phasic components of biatrial function, biventricular diastolic function and fibrosis. Twenty healthy, sex-matched subjects served as controls. RESULTS: We found a significant increase of left atrial (LA) and right atrial (RA) volumes and reduction in the majority of indices of contractile function, strains and strain rates (p < 0.05) in children with HCM compared with controls. Nearly all of the LA dynamics markers attained a significant association with the LVOT gradient (p < 0.05), the RA volumes and contractile functions were affected by LV fibrosis and mass (p < 0.05), and the RA mechanical components were related to the degree of LVOTO (p < 0.05). The minority of biatrial dynamics markers were associated with indices of ventricular diastolic function. CONCLUSIONS: The majority of biatrial volumetric and functional indices were severely compromised in children with HCM compared with controls. The degree of LVOTO appears to trigger LA volumetric and LA and RA mechanical malfunction. On the other hand, the deterioration of RA volumetric components was linked to LV fibrosis and mass. KEY POINTS: • Biatrial function was severely compromised in children with HCM. • Left atrial malfunction was associated with the degree of LVOTO. • Fibrosis and LV mass were related to RA volumetric and contractile dysfunction. • The degree of LVOTO was linked to right atrial mechanical abnormalities.

Circulating biomarkers of hypertrophy and fibrosis in patients with hypertrophic cardiomyopathy assessed by cardiac magnetic resonance.

BACKGROUND: Myocardial fibrosis in hypertrophic cardiomyopathy (HCM) is associated with worse clinical outcomes. The availability of circulating biomarkers of myocardial fibrosis and hypertrophy would be helpful in clinical practice. OBJECTIVE: The aim of this study was to evaluate usefulness of various biomarkers of myocardial fibrosis and hypertrophy in HCM. METHODS: Levels of biomarkers: soluble ST2 (sST2), galectin-3 (Gal-3), growth differentiation factor-15 (GDF-15), NT-proBNP and high-sensitivity cardiac troponin T (hs-cTnT) were measured in 60 patients with HCM. All patients underwent cardiac magnetic resonance imaging to calculate parameters of hypertrophy and fibrosis. RESULTS: We observed positive correlations among sST2 levels and left ventricular mass (LVM) (r = 0.32, p = 0.012), LV mass indexed for the body surface area (LVMI) (r = 0.27, p = 0.036) and maximal wall thickness (MWT) (r = 0.31, p = 0.015). No correlation was found between Gal-3 and GDF-15 levels and hypertrophy and fibrosis parameters. We observed positive correlations among hs-cTnT levels and LVM (r = 0.58, p < 0.0001), LVMI (r = 0.48, p = 0.0001), MWT (r = 0.31, p = 0.015) and late gadolinium enhancement (LGE) mass (r = 0.37, p = 0.003). There were positive correlations between NT-proBNP levels and LVM (r = 0.33, p = 0.01), LVMI (r = 0.41, p = 0.001), MWT (r = 0.42, p < 0.001) and LGE mass (r = 0.44, p < 0.001). CONCLUSIONS: Although no correlation between sST2 levels and myocardial fibrosis was found, sST2 may provide some additional information about hypertrophy extension. NT-proBNP and hs-cTnT are useful biomarkers in assessment of hypertrophy and fibrosis in HCM.

Quantification of mitral regurgitation in patients with hypertrophic cardiomyopathy using aortic and pulmonary flow data: impacts of left ventricular outflow tract obstruction and different left ventricular segmentation methods.

BACKGROUND: Cardiovascular magnetic resonance (CMR) imaging in patients with hypertrophic cardiomyopathy (HCM) enables the assessment of not only left ventricular (LV) hypertrophy and scarring but also the severity of mitral regurgitation. CMR assessment of mitral regurgitation is primarily based on the difference between LV stroke volume (LVSV) and aortic forward flow (Ao) measured using the phase-contrast (PC) technique. However, LV outflow tract (LVOT) obstruction causing turbulent, non-laminar flow in the ascending aorta may impact the accuracy of aortic flow quantification, leading to false conclusions regarding mitral regurgitation severity. Thus, we decided to quantify mitral regurgitation in patients with HCM using Ao or, alternatively, main pulmonary artery forward flow (MPA) for mitral regurgitation volume (MRvol) calculations. METHODS: The analysis included 143 prospectively recruited subjects with HCM and 15 controls. MRvol was calculated as the difference between LVSV computed with either the inclusion (LVSVincl) or exclusion (LVSVexcl) of papillary muscles and trabeculations from the blood pool and either Ao (MRvolAoi or MRvolAoe) or MPA (MRvolMPAi or MRvolMPAe). The presence or absence of LVOT obstruction was determined based on Doppler echocardiography findings. RESULTS: MRvolAoi was higher than MRvolMPAi in HCM patients with LVOT obstruction [47.0 ml, interquartile range (IQR) = 31.5-60.0 vs. 35.5 ml, IQR = 26.0-51.0; p < 0.0001] but not in non-obstructive HCM patients (23.0 ml, IQR = 16.0-32.0 vs. 24.0 ml, IQR = 15.3-32.0; p = 0.26) or controls (18.0 ml, IQR = 14.3-21.8 vs. 20.0 ml, IQR = 14.3-22.0; p = 0.89). In contrast to controls and HCM patients without LVOT obstruction, in HCM patients with LVOT obstruction, aortic flow-based MRvol (MRvolAoi) was higher than pulmonary-based findings (MRvolMPAi) (bias = 9.5 ml; limits of agreement: -11.7-30.7 with a difference of 47 ml in the extreme case). The differences between aortic-based and pulmonary-based MRvol values calculated using LVSVexcl mirrored those derived using LVSVincl. However, MRvol values calculated using LVSVexcl were lower in all the groups analyzed (HCM with LVOT obstruction, HCM without LVOT obstruction, and controls) and with all methods of MRvol quantification used (p ≤ 0.0001 for all comparisons). CONCLUSIONS: In HCM patients, LVOT obstruction significantly affects the estimation of aortic flow, leading to its underestimation and, consequently, to higher MRvol values than those obtained with MPA-based MRvol calculations.

Cine dyscontractility index: A novel marker of mechanical dyssynchrony that predicts response to cardiac resynchronization therapy.

PURPOSE: To investigate whether magnetic resonance imaging (MRI) cine-derived dyssynchrony indices provide additional information compared to conventional tagged MRI (tMRI) acquisitions in heart failure patients undergoing cardiac resynchronization therapy (CRT). MATERIALS AND METHODS: Patients scheduled for CRT (n = 52) underwent preprocedure MRI including cine and tMRI acquisitions. Segmental strain curves were calculated for both cine and tMRI to produce a range of standard indices for direct comparison between modalities. We also proposed and evaluated a novel index of "dyscontractility," which detects the presence of focal areas with paradoxically positive circumferential strain. RESULTS: Across conventional strain indices, there was only moderate-to-poor (R = 0.3-0.6) correlation between modalities; eight cine-derived indices showed statistically significant (P < 0.05) relations to CRT outcome compared to just two tMRI-based counterparts. The novel dyscontractility index calculated on basal slice cine images (cine dyscontractility index, "CDI") was the single best predictor of clinical response to CRT (area under the curve AUC = 0.81, P < 0.001). While poorly correlated to its tMRI counterpart (R = 0.33), CDI performed significantly better in predicting response to CRT (P < 0.005), and was also numerically better than all other tMRI indices (AUC 0.53-0.76, all P for AUC comparisons <0.17). CONCLUSION: Cine-derived strain indices offer potentially new information compared to tMRI. Specifically, the novel CDI is most strongly linked to response to cardiac resynchronization therapy in a contemporary patient cohort. It utilizes readily available MRI data, is relatively straightforward to process, and compares favorably with any conventional tagging index. J. Magn. Reson. Imaging 2016;44:1483-1492.

PET/CT and SPECT/CT imaging of 90Y hepatic radioembolization at therapeutic and diagnostic activity levels: Anthropomorphic phantom study.

PURPOSE: Prior to 90Y radioembolization procedure, a pretherapy simulation using 99mTc-MAA is performed. Alternatively, a small dosage of 90Y microspheres could be used. We aimed to assess the accuracy of lung shunt fraction (LSF) estimation in both high activity 90Y posttreatment and pretreatment scans with isotope activity of ~100 MBq, using different imaging techniques. Additionally, we assessed the feasibility of visualising hot and cold hepatic tumours in PET/CT and Bremsstrahlung SPECT/CT images. MATERIALS AND METHODS: Anthropomorphic phantom including liver (with two spherical tumours) and lung inserts was filled with 90Y chloride to simulate an LSF of 9.8%. The total initial activity in the liver was 1451 MBq, including 19.4 MBq in the hot sphere. Nine measurement sessions including PET/CT, SPECT/CT, and planar images were acquired at activities in the whole phantom ranging from 1618 MBq down to 43 MBq. The visibility of the tumours was appraised based on independent observers' scores. Quantitatively, contrast-to-noise ratio (CNR) was calculated for both spheres in all images. RESULTS: LSF estimation. For high activity in the phantom, PET reconstructions slightly underestimated the LSF; absolute difference was <1.5pp (percent point). For activity <100 MBq, the LSF was overestimated. Both SPECT and planar scintigraphy overestimated the LSF for all activities. Lesion visibility. For SPECT/CT, the cold tumour proved too small to be discernible (CNR <0.5) regardless of the 90Y activity in the liver, while hot sphere was visible for activity >200 MBq (CNR>4). For PET/CT, the cold tumour was only visible with the highest 90Y activity (CNR>4), whereas the hot one was seen for activity >100 MBq (CNR>5). CONCLUSIONS: PET/CT may accurately estimate the LSF in a 90Y posttreatment procedure. However, at low activities of about 100 MBq it seems to provide unreliable estimations. PET imaging provided better visualisation of both hot and cold tumours.

Left and Right Ventricular Morphology, Function and Myocardial Deformation in Children with Left Ventricular Non-Compaction Cardiomyopathy: A Case-Control Cardiovascular Magnetic Resonance Study.

Background: Left ventricular non-compaction (LVNC) is a rare cardiomyopathy typically involving the left ventricle (LV); however, the right ventricle (RV) can also be affected. This case-control study aimed to assess the morphology and function of LV and RV in children with LVNC. Methods: Sixteen children (13 ± 3 years, six girls) with LVNC were compared with 16 sex- and age-matched controls. LV and RV morphology and function were evaluated in cardiovascular magnetic resonance (CMR) studies. Additionally, LV and RV global radial (GRS), circumferential (GCS), and longitudinal strain (GLS) were assessed using tissue-tracking analysis. Results: Patients with LVNC did not differ from the healthy controls in terms of age, height, weight, and body surface area (BSA). In total, 4/16 subjects with LVNC had mid-wall late gadolinium enhancement (LGE). Compared to the control group, patients with LVNC had higher end-diastolic volume (EDV) indexed for body surface area (BSA), lower ejection fraction (EF), and lower LV strain parameters (all p < 0.05). Children with LVNC also presented with thicker RV apical trabeculation, whereas there were no differences in RV EF and EDV/BSA between the groups. Nevertheless, children with LVNC had impaired RV GRS and GCS (both p < 0.05). Conclusions: LVNC in pediatric patients is associated with LV enlargement and impaired LV systolic function. Additionally, children with LVNC have increased RV trabeculations and subclinical impairment of RV myocardial deformation.

Evaluation of qualitative and quantitative data of Y-90 imaging in SPECT/CT and PET/CT phantom studies.

INTRODUCTION: We aimed to assess the feasibility of SPECT and PET Y-90 imaging, and to compare these modalities by visualizing hot and cold foci in phantoms for varying isotope concentrations. MATERIALS AND METHODS: The data was acquired from the Jaszczak and NEMA phantoms. In the Jaszczak phantom Y-90 concentrations of 0.1 MBq/ml and 0.2 MBq/ml were used, while higher concentrations, up to 1.0 MBq/ml, were simulated by acquisition time extension with respect to the standard clinical protocol of 30 sec/projection for SPECT and 30 min/bed position for PET imaging. For NEMA phantom, the hot foci had concentrations of about 4 MB/ml and the background 0.1 or 0.0 MBq/ml. All of the acquired data was analysed both qualitatively and quantitatively. Qualitative assessment was conducted by six observers asked to identify the number of visible cold or hot foci. Inter-observer agreement was assessed. Quantitative analysis included calculations of contrast and contrast-to-noise ratio (CNR), and comparisons with the qualitative results. RESULTS: For SPECT data up to two cold foci were discernible, while for PET four foci were visible. We have shown that CNR (with Rose criterion) is a good measure of foci visibility for both modalities. We also found good concordance of qualitative results for the Jaszczak phantom studies between the observers (corresponding Krippendorf's alpha coefficients of 0.76 to 0.84). In the NEMA phantom without background activity all foci were visible in SPECT/CT images. With isotope in the background, 5 of 6 spheres were discernible (CNR of 3.0 for the smallest foci). For PET studies all hot spheres were visible, regardless of the background activity. CONCLUSIONS: PET Y-90 imaging provided better results than Bremsstrahlung based SPECT imaging. This indicates that PET/CT might become the method of choice in Y-90 post radioembolization imaging for visualisation of both necrotic and hot lesions in the liver.

Sudden cardiac death risk in hypertrophic cardiomyopathy: comparison between echocardiography and magnetic resonance imaging.

In hypertrophic cardiomyopathy (HCM) patients, left ventricular (LV) maximal wall thickness (MWT) is one of the most important factors determining sudden cardiac death (SCD) risk. In a large unselected sample of HCM patients, we aimed to simulate what changes would occur in the calculated SCD risk according to the European HCM Risk-SCD calculator when MWT measured using echocardiography was changed to MWT measured using MRI. All consecutive patients with HCM who underwent cardiac MRI were included. MWT measured with echocardiography and MRI were compared, and 5-year SCD risk according to the HCM Risk-SCD calculator was computed using four different models. The final population included 673 patients [389 (57.8%) males, median age 50 years, interquartile range (36-60)]. The median MWT was lower measured by echocardiography than by MRI [20 (17-24) mm vs 21 (18-24) mm; p < 0.0001]. There was agreement between echocardiography and MRI in the measurement of maximal LV wall thickness in 96 patients (14.3%). The largest differences between echo and MRI were - 13 mm and + 9 mm. The differences in MWT by echocardiography and MRI translated to a maximal difference of 8.33% in the absolute 5-year risk of SCD, i.e., the echocardiography-based risk was 8.33% lower than the MRI-based estimates. Interestingly, 13.7% of patients would have been reclassified into different SCD risk categories if MRI had been used to measure MWT instead of echocardiography. In conclusion, although there was high general intermodality agreement between echocardiography and MRI in the MWT measurements, the differences in MWT translated to significant differences in the 5-year risk of SCD.

Biventricular mechanics in prediction of severe myocardial fibrosis in patients with dilated cardiomyopathy: CMR study.

PURPOSE: The purpose of this study was to compare the ability of various parameters of myocardial mechanics to predict large amounts of biventricular fibrosis assessed via T1 mapping in patients with dilated cardiomyopathy (DCM). MATERIAL: Cardiovascular magnetic resonance feature tracking analysis and T1 mapping were performed in 26 patients with DCM [mean age: 34.4±9.1years, 15 (57.6%) males]. The values of various parameters of myocardial mechanics at predicting advanced left-ventricle (LV) and right-ventricle (RV) fibrosis were compared using logistic regression analysis and receiver operating characteristic curve (ROC) analysis. RESULTS: There were 7 (26.9%) patients with a large amount of LV fibrosis and 9 (34.6%) patients with severe RV fibrosis. ROC curve analysis revealed that the model of combined LV strain rates (AUC=0.902) offered superb ability at predicting large amounts of LV fibrosis. The models including RV strain rates (AUC=0.974), a combination of RV strains, strain rates and clinical parameters (AUC=0.993) as well as the RV radial strain rate alone model (AUC=0.961) yielded outstanding performance in discriminating large and small amounts of RV fibrosis. In multivariate analysis, the LV circumferential strain (LVCR) and RV radial (RVR) strain rate were the only independent predictors of large amounts of LV and RV fibrosis, respectively. CONCLUSIONS: Indices of myocardial deformation, especially combined with clinical features, offered a superlative ability to differentiate high from low degrees of fibrosis in DCM patients. Among all analyzed parameters of myocardial mechanics, LVCR and RVR rate alone were the independent predictors of high degrees of LV and RV fibrosis, respectively.

Left-ventricular mechanics in children with hypertrophic cardiomyopathy. CMR study.

OBJECTIVES: To assess the magnitude of myocardial displacement abnormalities and their alterations with the fibrosis, left-ventricular (LV) outflow tract obstruction (LVOTO) and hypertrophy in juveniles with hypertrophic cardiomyopathy (HCM). STUDY DESIGN: Fifty-five children [age 12,5±4.6years, 38 (69,1%) males, 19 (34,5%) with LVOTO] with HCM and 20 controls underwent cardiovascular magnetic resonance. The LV feature tracking (FT) derived strain and strain rates were quantified. Results of FT analysis were compared between HCM subjects and controls and between children with and without LVOTO. RESULTS: Children with HCM exhibited decreased strain in both hypertrophied and nonhypertrophied segments versus controls. LV global longitudinal strain (LVGLS) rate (-0.69±0.04 vs -0.91±0.05, p=0,04), LV circumferential strain (LVCR) rate (-0.98±0.09 vs -1.27±0.06, p=0,02), LV radial strain (LVR) (18,5±1.9 vs 27,4±1.4, p<0,01) and LVR rate (0,98±0.1 vs 1,53±0.08, p<0,01) were substantially compromised in subjects with LVOTO vs without. In multivariable regression all LV myocardial dynamics markers, except for LVCR, exhibited a significant association with the degree of LVOTO. LVCR rate (ß=0,31, p=0,02) and LVR (ß=-0.24, p=0,04) were related to LV mass and only LVCR rate (ß=0,15, p=0,03) was associated with the amount of LV fibrosis. CONCLUSIONS: The reduction of all indices of LV myocardial mechanics in juvenile HCM patients was global but particularly pronounced in hypertrophied segments of the LV. The majority of the LV strains and strain rates were substantially compromised in subjects with LVOTO compared to patients without the obstruction. Myocardial mechanics indices seemed to be related to the degree of LVOTO rather than either to mass or the amount of fibrosis.