Abstract

The mechanisms responsible for the development of apical aneurysms in cases of hypertrophic cardiomyopathy (HCM) are currently unclear but likely involve multiple factors. Here, we present a case of HCM with marked subendocardial fibrosis involving the apical and proximal portions of the left ventricle. A 71-year-old man with left ventricular hypertrophy presented with signs and symptoms of heart failure. The presence of asymmetrical left ventricular hypertrophy and bilateral, thickened ventricular walls with an apical aneurysm on transthoracic echocardiography suggested a diagnosis of HCM with ventricular dysfunction. No intraventricular pressure gradients with obstruction were identified. Late gadolinium enhancement (LGE) with cardiac magnetic resonance imaging and endomyocardial biopsies showed subendocardial fibrosis involving the apical aneurysm and proximal portion. Whereas LGE in a transmural pattern is commonly observed in HCM apical aneurysms, subendocardial LGE, as noted in the present case, is a relatively rare occurrence. Thus, the present case may provide unique insights into the adverse remodeling process and formation of apical aneurysms in cases of HCM.

Highlights

  • The life-long process of left ventricular remodeling and progressive dysfunction occur in a substantial proportion of patients with hypertrophic cardiomyopathy (HCM), of which a subset presents with extreme fibrosis and may progress to heart failure and sudden cardiac death [1]

  • Adverse left ventricular remodeling in HCM is characterized by variable patterns of myocardial fibrosis, visualized by cardiac magnetic resonance (CMR) imaging as late gadolinium enhancement (LGE)

  • We present a rare case of global subendocardial fibrosis in an apical aneurysm and the proximal portion of the left ventricle (LV), identified by CMR imaging and endomyocardial biopsy

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Summary

Introduction

The life-long process of left ventricular remodeling and progressive dysfunction occur in a substantial proportion of patients with hypertrophic cardiomyopathy (HCM), of which a subset presents with extreme fibrosis and may progress to heart failure and sudden cardiac death [1]. Adverse left ventricular remodeling in HCM is characterized by variable patterns of myocardial fibrosis, visualized by cardiac magnetic resonance (CMR) imaging as late gadolinium enhancement (LGE). We present a rare case of global subendocardial fibrosis in an apical aneurysm and the proximal portion of the left ventricle (LV), identified by CMR imaging and endomyocardial biopsy.

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