Heterogeneity of Intramural Function in Hypertrophic Cardiomyopathy

Abstract

Background— In hypertrophic cardiomyopathy (HCM), myocardial abnormalities are commonly heterogeneous. Two patterns of late gadolinium enhancement (LGE) have been reported: a bright “confluent” and an intermediate intensity abnormality termed “diffuse,” each representing different degrees of myocardial scarring. We used MRI to study the relation between intramural cardiac function and the extent of fibrosis in HCM. The aim of this study was to determine whether excess collagen or myocardial scarring, as determined by LGE MRI, are the primary mechanisms leading to heterogeneous regional contractile function in patients with HCM. Methods and Results— Intramural left ventricular strain, transmural left ventricular function, and regions of myocardial fibrosis/scarring were imaged in 22 patients with HCM, using displacement encoding with stimulated echoes (DENSE), cine MRI, and LGE. DENSE systolic strain maps were qualitatively and quantitatively compared with LGE images. Intramural systolic strain by DENSE was significantly depressed within areas of confluent and diffuse LGE but also in the core of the most hypertrophic nonenhanced segment (all P <0.001 versus nonhypertrophied segments). DENSE demonstrated an unexpected inner rim of largely preserved contractile function and a noncontracting outer wall within hypertrophic segments in 91% of patients. Conclusions— LGE predicted some but not all of the heterogeneity of intramural contractile abnormalities. This indicates that myocardial scarring or excess interstitial collagen deposition does not fully explain the observed contractile heterogeneity in HCM. Thus, myofibril disarray or other nonfibrotic processes affect systolic function in a large number of patients with HCM.