BackgroundThe presence and extent of late gadolinium enhancement (LGE) has been associated with adverse events in patients with hypertrophic cardiomyopathy (HCM). Signal intensity (SI) threshold techniques are routinely employed for quantification; Full-Width at Half-Maximum (FWHM) techniques are suggested to provide greater reproducibility than Signal Threshold versus Reference Mean (STRM) techniques, however the accuracy of these approaches versus the manual assignment of optimal SI thresholds has not been studied. In this study, we compared all known semi-automated LGE quantification techniques for accuracy and reproducibility among patients with HCM.MethodsSeventy-six HCM patients (51 male, age 54 ± 13 years) were studied. Total LGE volume was quantified using 7 semi-automated techniques and compared to expert manual adjustment of the SI threshold to achieve optimal segmentation. Techniques tested included STRM based thresholds of >2, 3, 4, 5 and 6 SD above mean SI of reference myocardium, the FWHM technique, and the Otsu-auto-threshold (OAT) technique. The SI threshold chosen by each technique was recorded for all slices. Bland-Altman analysis and intra-class correlation coefficients (ICC) were reported for each semi-automated technique versus expert, manually adjusted LGE segmentation. Intra- and inter-observer reproducibility assessments were also performed.ResultsFifty-two of 76 (68%) patients showed LGE on a total of 202 slices. For accuracy, the STRM >3SD technique showed the greatest agreement with manual segmentation (ICC = 0.97, mean difference and 95% limits of agreement = 1.6 ± 10.7 g) while STRM >6SD, >5SD, 4SD and FWHM techniques systematically underestimated total LGE volume. Slice based analysis of selected SI thresholds similarly showed the STRM >3SD threshold to most closely approximate manually adjusted SI thresholds (ICC = 0.88). For reproducibility, the intra- and inter-observer reproducibility of the >3SD threshold demonstrated an acceptable mean difference and 95% limits of agreement of −0.5 ± 6.8 g and −0.9 ± 5.6 g, respectively.ConclusionsFWHM segmentation provides superior reproducibility, however systematically underestimates total LGE volume compared to manual segmentation in patients with HCM. The STRM >3SD technique provides the greatest accuracy while retaining acceptable reproducibility and may therefore be a preferred approach for LGE quantification in this population.

Background-Late gadolinium enhancement-cardiac magnetic resonance is increasingly performed in patients with systolic dysfunction. Numerous patterns of fibrosis are commonly reported among this population. However, the relative prevalence and prognostic significance of these findings remains uncertain. Methods and Results-Three hundred eighteen consecutive patients referred for late gadolinium enhancement-cardiac magnetic resonance and a left ventricular ejection fraction <55% were followed up for the primary end point of sudden cardiac arrest or appropriate implantable cardiac defibrillator therapy. Late gadolinium enhancement images were blindly interpreted for the presence of 6 distinct pattern(s) of myocardial fibrosis in addition to signal threshold-based quantification of total fibrosis volume. The mean age and left ventricular ejection fraction of participants were 62.0±12.9 years and 32.6±11.9%, respectively. Any pattern of myocardial fibrosis was seen in 248 patients (78%) with ≥2 patterns present in 25% of patients. During follow-up (median of 467 days), 49 patients (15%) had a primary outcome. After adjustment for left ventricular ejection fraction, cardiomyopathy pathogenesis, and total fibrosis volume, the presence of a midwall striae pattern of fibrosis was an independent predictor of sudden cardiac arrest or appropriate implantable cardiac defibrillator therapy with a hazard ratio of 2.4 (95% confidence interval, 1.2-4.6; P=0.01); this finding is present in 30% of patients with nonischemic and 15% of patients with ischemic cardiomyopathy. Cumulative event rate was significantly higher among those with midwall striae, particularly among those with a left ventricular ejection fraction >35% (40% versus 6%; P=0.005). Conclusions-Patients with systolic dysfunction frequently demonstrate multiple patterns of myocardial fibrosis. Of these, a midwall striae pattern of fibrosis is the strongest independent predictor of sudden cardiac arrest or appropriate implantable cardiac defibrillator therapy. (Circ Cardiovasc Imaging. 2014;7:593-600.)