Diffuse myocardial fibrosis in hypertrophic cardiomyopathy can be identified by cardiovascular magnetic resonance, and i
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RESEARCH
Open Access
Diffuse myocardial fibrosis in hypertrophic cardiomyopathy can be identified by cardiovascular magnetic resonance, and is associated with left ventricular diastolic dysfunction Andris H Ellims1,2, Leah M Iles1,2, Liang-han Ling1,2, James L Hare1,2, David M Kaye1,2 and Andrew J Taylor1,2*
Abstract Background: The presence of myocardial fibrosis is associated with worse clinical outcomes in hypertrophic cardiomyopathy (HCM). Cardiovascular magnetic resonance (CMR) with late gadolinium enhancement (LGE) sequences can detect regional, but not diffuse myocardial fibrosis. Post-contrast T1 mapping is an emerging CMR technique that may enable the non-invasive evaluation of diffuse myocardial fibrosis in HCM. The purpose of this study was to non-invasively detect and quantify diffuse myocardial fibrosis in HCM with CMR and examine its relationship to diastolic performance. Methods: We performed CMR on 76 patients - 51 with asymmetric septal hypertrophy due to HCM and 25 healthy controls. Left ventricular (LV) morphology, function and distribution of regional myocardial fibrosis were evaluated with cine imaging and LGE. A CMR T1 mapping sequence determined the post-contrast myocardial T1 time as an index of diffuse myocardial fibrosis. Diastolic function was assessed by transthoracic echocardiography. Results: Regional myocardial fibrosis was observed in 84% of the HCM group. Post-contrast myocardial T1 time was significantly shorter in patients with HCM compared to controls, consistent with diffuse myocardial fibrosis (498 ± 80 ms vs. 561 ± 47 ms, p < 0.001). In HCM patients, post-contrast myocardial T1 time correlated with mean E/e’ (r = −0.48, p < 0.001). Conclusions: Patients with HCM have shorter post-contrast myocardial T1 times, consistent with diffuse myocardial fibrosis, which correlate with estimated LV filling pressure, suggesting a mechanistic link between diffuse myocardial fibrosis and abnormal diastolic function in HCM. Keywords: Hypertrophic cardiomyopathy, Magnetic resonance imaging, Myocardial fibrosis, T1 mapping
Background Hypertrophic cardiomyopathy (HCM) is a relatively common inherited cardiac disease defined by the presence of otherwise unexplained left ventricular (LV) hypertrophy associated with non-dilated ventricular chambers [1,2]. Inheritance is autosomal dominant and * Correspondence: [email protected] 1 Heart Centre, Alfred Hospital, Melbourne, Australia 2 Baker IDI Heart and Diabetes Research Institute Melbourne, Australia
mutations generally involve sarcomeric genes [3,4]. Patients may develop symptoms from LV outflow tract (LVOT) obstruction [2] and, in the presence of certain high-risk features, an implantable cardioverter defibrillator is recommended to reduce the risk of sudden cardiac death [5]. Many patients, however, develop symptoms of breathlessness due to diastolic dysfunction which is largely independent of the severity of LVOT obstruction [6]. As patients with HCM are known to develop diffuse, as well as regional myocardial fibrosis, this ma
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