Abstract

BackgroundHypertrophic cardiomyopathy (HCM) is one of the commonest inheritable cardiac disorders. Being a global disease with diffuse myocardial fibrosis, it has a wide range of adverse outcomes ending with sudden cardiac death. Cardiac magnetic resonance (CMR) with late gadolinium enhancement (LGE) has become a reference standard for visualization of focal myocardial fibrosis. In the setting of less severe or more diffuse fibrosis, LGE is unlikely to reveal the presence of abnormal tissue given the lack of normal myocardium as a reference. Direct measurement of myocardial T1 time (T1 mapping) may improve these methodologic problems of LGE CMR in the setting of diffuse retention of gadolinium-based contrast material. So, we aim at this study to evaluate the clinical application of CMRI native and post-contrast T1 relaxation in assessing diffuse myocardial fibrosis non-invasively in hypertrophic cardiomyopathy.ResultsThere was a significant difference between the percent of fibrosis detected by measuring the extracellular volume percent compared to that detected by LGE, with the former detecting fibrosis in 45.1% of the examined cardiac segments while the latter showed fibrosis in 20.9% of the cardiac segments. Also, measuring the native T1 values showed evidence of fibrosis in about 32.2% of the cardiac segments superseding the percent of fibrosis detected using the LGE alone. The ejection fraction percent showed a negative correlation with the left ventricular mass with a correlation coefficient value of − 0.139 where both interstitial and replacement fibrosis play an important role in the pathophysiology of diastolic dysfunction as well as impairing the myocardial contractility. Also, in cases of obstruction, the extracellular volume (ECV) is more likely to increase in the basal anterior and antero-septal segments as well as the basal inferior segment with P values 0.015, 0.013, and 0.045, respectively.ConclusionDiffuse fibrosis was found to be difficult to be distinguished using LGE. The unique ability of CMR to use proton relaxation times provides a quantitative measurement to detect increased interstitial volume in diffuse myocardial fibrosis. Moreover, it showed that in cases of obstruction, the segments exposed to the highest pressure are more vulnerable to the fibrotic process denoting a relationship between the pressure gradient and the adverse myocardial remodeling.

Highlights

  • Hypertrophic cardiomyopathy (HCM) is one of the commonest inheritable cardiac disorders

  • Magnetic resonance imaging (MRI) was done to diagnose the presence of HCM, detect its phenotype, assess the presence of obstruction, and measure the gradient as well as assessing the presence of fibrosis using the routine late gadolinium enhancement (LGE) method and adding T1 mapping sequence to evaluate its clinical application in assessing diffuse myocardial fibrosis non-invasively

  • Correlating the extracellular volume (ECV) to the presence of obstruction, the results were statistically significant in the basal anterior and antero-septal segments

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Summary

Introduction

Hypertrophic cardiomyopathy (HCM) is one of the commonest inheritable cardiac disorders. Being a global disease with diffuse myocardial fibrosis, it has a wide range of adverse outcomes ending with sudden cardiac death. We aim at this study to evaluate the clinical application of CMRI native and post-contrast T1 relaxation in assessing diffuse myocardial fibrosis non-invasively in hypertrophic cardiomyopathy. Hypertrophic cardiomyopathy (HCM) is the commonest inheritable cardiac disorder, with an estimated prevalence of 1:500 in the general population. Its clinical manifestations are wide-ranging, including sudden cardiac death [1, 2]. It is an extremely heterogeneous disease having several phenotypes with the most common of them is the asymmetric septal form [1, 3–6]. Systolic anterior motion (SAM) of the mitral valve is common and is caused by the pressure drop in the narrowed left ventricular outflow tract (LVOT) leading to partial or complete outflow obstruction with moderate to severe mitral regurgitation [3]

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