Search

Background: Cardiomyopathy is the leading cause of death in boys with Duchenne muscular dystrophy (DMD). While cardiac magnetic resonance (CMR) is routinely used to assess fibrosis and left ventricular (LV) ejection fraction, CMR measures of LV filling and ejection in DMD have not been reported., Methods: Patients with DMD (n=179) and healthy controls (n=96) were prospectively enrolled and underwent CMR. The DMD cohort was followed clinically at multiple institutions, and clinical data were recorded. Standard volumes and functions were calculated, and LV filling and ejection curves were measured from baseline CMR. Multivariable linear regressions were used to compare ventricular filling and ejection measures between groups, adjusting for baseline differences. Cox regressions were used to evaluate the relationship between diastolic function measures and mortality in the DMD cohort., Results: Patients with DMD had significantly smaller stature and ventricular volumes than healthy control patients ( P <0.001). They had lower baseline LV ejection fraction ( P <0.001), though most had normal systolic function. When adjusted for age, sex, heart rate, body surface area, and LV end-diastolic volume, patients with DMD had slower peak filling rates ( P <0.001) and peak ejection rates ( P <0.001), as well as slower time to peak ventricular ejection rate ( P =0.011). When adjusted for heart rate, a lower peak ventricular ejection rate ( P =0.007) and peak filling rate ( P =0.033), normalized to LV end-diastolic volume, were associated with mortality in patients with DMD., Conclusions: Patients with DMD have significantly different baseline CMR filling and ejection indices compared with controls. Some filling indices are associated with mortality and may be useful prognostic measures. Further research is needed in larger cohorts to determine the prognostic value of these differences., Competing Interests: None.

Background: quantitative assessment and validation of left ventricular (LV) volumes and mass in neonates and infants with complex congenital heart disease (CHD) is important for clinical management but has not been undertaken. We compared matrix-array 3D echocardiography (3D echo) measurements of volumes, mass, and ejection fraction (EF) with those measured by cardiac MRI in young patients with CHD and small LVs because of either young age or LV hypoplasia., Methods and Results: thirty-five patients aged <4 years (median, 0.8 years) undergoing MRI were prospectively enrolled. Three-dimensional echo was acquired immediately after MRI, and volume, mass, and EF measurements, using summation of discs methodology, were compared with MRI. Three-dimensional echo end-diastolic volume (24.4±15.7 versus 24.8±46.4 mL; P=0.01; intraclass correlation coefficient [ICC], 0.96) and end-systolic volume (12.3±8.6 versus 9.6±6.8 mL; P<0.001; ICC, 0.90) correlated with MRI with small mean differences (-0.49 mL [P=0.6] and 2.7 mL [P=0.001], respectively). Three-dimensional echo EF was smaller than MRI by 9.3% (P<0.001), and 3D echo LV mass measurements were comparable to MRI (17.3±10.3 versus 17.6±12 g; P<0.77; ICC, 0.93), with a small mean difference (1.1 g; P=0.28). There was good intra- and interobserver reliability for all measurements., Conclusions: in neonates and infants with CHD and small LVs (age appropriate or hypoplastic), matrix-array 3D echo measurements of mass and volumes compare well with MRI, providing an important modality for ventricular size and performance analysis in these patients, particularly in those with left-side heart obstructive lesions.