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Non-invasive imaging markers in patients with repaired tetralogy of Fallot (rTOF) are still being investigated to inform clinical decision making. Atrial function is a prognostic indicator in many acquired and congenital heart diseases. We sought to examine the relationship between cardiac MRI (CMR)-derived indices of left atrial (LA) function, native left ventricular (LV) T1 values, biventricular systolic function, and exercise capacity in rTOF. Sixty-six patients with rTOF without prior pulmonary valve replacement who underwent CMR (median age 18.5 years) were identified. Twenty-one adult rTOF patients (age range 19–32 years) were compared with 20 age-matched healthy volunteers (age range 19–34 years). LA reservoir, conduit, and pump global longitudinal strain (GLS) and strain rate (SR) were determined by tissue tracking. Native LV T1 values were measured on rTOF patients. Pearson correlations were performed to determine bivariate associations. Adult rTOF patients had higher pump GLS, pump:conduit, and pump:reservoir GLS ratios, and lower conduit:reservoir GLS ratio, LV ejection fraction (EF), and right ventricular EF compared to controls (p

Background Cardiac MRI (CMR) derived ventricular global function index (GFI), a ratio of stroke volume to the sum of mean ventricular cavity and myocardial volumes, has demonstrated improved prediction of clinical outcomes in adults with atherosclerotic disease over ejection fraction. We sought to assess CMR derived GFI and a novel modification that accounts for unique loading conditions in patients with repaired tetralogy of Fallot (rTOF) and determine its correlation with exercise performance. Methods and Results Seventy‐five patients with rTOF who underwent CMR were identified. Clinical variables were recorded and biventricular GFI calculated. A right ventricular (RV) effective GFI (eGFI) was derived by incorporating effective stroke volume. Thirty‐five pediatric patients were matched with 29 age‐matched healthy controls. Twenty‐five patients completed cardiopulmonary exercise tests within 6 months of CMR. Stepwise regression models were used to determine univariate and multivariable predictors of indexed and percent predicted peak VO 2 . Median age at CMR was 20 years (interquartile range, 13–28). Pediatric rTOF patients had lower RV eGFI ( P < 0.001), RV ejection fraction ( P =0.002), but higher indexed RV end‐diastolic and end‐systolic volumes ( P < 0.001, P < 0.001) compared with controls. Univariate analysis demonstrated a correlation between indexed peak VO 2 with RV eGFI ( R 2 =0.32, P =0.004), but with neither RVGFI, RV ejection fraction, indexed RV volumes nor RV mass. RV eGFI remained significantly associated with indexed peak VO 2 during multivariable modeling. Conclusions Reduced RV eGFI was associated with reduced exercise capacity in rTOF patients, while RV GFI, RV ejection fraction, indexed RV volumes and mass were not. Our modification of the GFI, RV eGFI, may be a valuable non‐invasive marker of cardiac function in rTOF.

Purpose To investigate right ventricular (RV) strain in patients without identified cardiac pathology using cardiac magnetic resonance tissue tracking (CMR TT). Methods A total of 50 consecutive patients with no identified cardiac pathology were analyzed. RV longitudinal and circumferential strain was assessed by CMR TT. The age range was 4–81 years with a median of 32 years (interquartile range, 15 to 56 years). Results Analysis time per patient was ll ) was − 22.11 ± 3.51%. The peak circumferential strains (E cc ) for global, basal, mid-cavity and apical segments were as follows: − 11.69 ± 2.25%, − 11.00 ± 2.45%, − 11.17 ± 3.36%, − 12.90 ± 3.34%. There were significant gender differences in peak E cc at the base ( P = 0.04) and the mid-cavity ( P = 0.03) with greater deformation in females than in males. On Bland-Altman analysis, peak E ll (mean bias, 0.22 ± 1.67; 95% CI − 3.05 to 3.49) and mid-cavity E cc (mean bias, 0.036 ± 1.75; 95% CI, − 3.39 to 3.47) had the best intra-observer agreement and inter-observer agreement, respectively. Conclusions RV longitudinal and circumferential strains can be quickly assessed with good intra-observer and inter-observer variability using TT.

Right ventricular (RV) size and function in Duchenne muscular dystrophy (DMD) have not been well described. Using cardiac magnetic resonance (CMR) imaging we describe the relationship of RV and left ventricular (LV) size and function in a large DMD cohort.Latest CMR scans of 272 patients consecutively seen at a single tertiary referral center (2011-2014) with skeletal muscle biopsy confirmed DMD were included. 1.5 and 3 Tesla CMR scanners were used. Biventricular ejection fraction (EF), end-diastolic volume index (EDVI), mass and mass index were compared across categories of LVEF.Mean age was 13.5 ± 4.9 years. 71% had normal (≥ 55%) LVEF while mild (EF 45-54%), moderate (EF 30-44%), and severe LV dysfunction (EF30%) was present in 20%, 6% and 3% respectively. The correlation between RVEF and LVEF was weak. Even in patients with severe LV dysfunction, RVEF (49.7% ± 12.9%) was relatively preserved. There were no significant differences in RVEDVI and RV mass index across categories of LV function.In a large DMD cohort, RVEF was relatively preserved and RV size was preserved across categories of LV dysfunction.

The cardiac disease ubiquitously associated in Duchenne Muscular Dystrophy (DMD) has traditionally been considered a progressive dilated cardiomyopathy (DCM). However, left ventricular (LV) dilatation as measured with cardiac MRI has not been a consistent finding in this population, even as circumferential strain (e(cc)) declines with advancing disease. We hypothesized that a distinct pattern of changes in LV geometry, during the course of e(cc) decline, distinguishes DMD associated heart disease from DCM. Using CMR, LV end-diastolic volume (EDV), mass (LVM), ejection fraction, e(cc) and myocardial delayed enhancement (MDE) were determined in DMD patients and normal control subjects. The LV Remodeling Index (LVRI) was calculated as the ratio of LV Mass to Volume (LVM/EDV). Statistical comparisons between all LV parameters and genotype were also performed. Median LVRI in DMD (n = 127) and control subjects (n = 41) were different (0.75 vs. 0.65, P = 0.0150) but within normal range. Furthermore, the median LVRI in DMD boys with reduced LV systolic function was significantly reduced compared to those with normal LV systolic function (0.64 vs. 0.75, P = 0.0974). However, the presence of MDE was associated with a lower median LVRI (0.57 vs. 0.76, P = 0.0471). Regression analysis showed no significant correlation between e(cc) and LVRI (r = -0.03). The LVRI of DMD patients is unexpectedly normal and not correlated with e(cc.) Based on these findings, DMD-associated heart disease exhibits a unique remodeling pattern distinct from DCM.

Background Left atrial (LA) size is a known predictor of adverse cardiovascular events. Echocardiography is the modality of choice for the evaluation of atrial size however cardiac magnetic resonance imaging (CMR) remains the gold standard for cardiac chamber volume quantification. We sought to compare atrial volume assessment using the biplane area-length method by echocardiography with full volume quantification by CMR. Methods