Apical Hypertrophic Cardiomyopathy With Endomyocardial Calcification: A Multimodality Imaging-Based Case Report

TL;DR

A case report describes a rare heart condition with apical hypertrophy and calcification, using multiple imaging techniques to better understand its features and implications.

Contribution

This case report provides new insights into the multimodality imaging features of apical hypertrophic cardiomyopathy with endomyocardial calcification.

Findings

Multimodality imaging confirmed apical myocardial hypertrophy and calcification in a 53-year-old woman.

Adenosine stress perfusion CMR showed a perfusion defect and reduced apical strain, suggesting chronic subendocardial ischemia.

Calcification was differentiated from thrombus to guide appropriate clinical management and avoid unnecessary anticoagulation.

Abstract

Apical hypertrophic cardiomyopathy (ApHCM) with endomyocardial calcification has been reported in only a small number of cases, making its imaging features less well established. We describe a 53-year-old woman with no significant medical history who was referred for further evaluation following abnormal electrocardiographic findings identified during a routine medical check-up. Multimodality imaging, including transthoracic echocardiography, coronary computed tomography angiography, and cardiac magnetic resonance imaging (CMR), demonstrated apical myocardial hypertrophy and endomyocardial calcification, establishing the diagnosis of ApHCM with calcific involvement. The pathophysiological mechanism underlying myocardial calcification in ApHCM remains poorly defined. In this case, adenosine stress perfusion CMR and myocardial strain analysis were performed for further characterization. These investigations revealed a circumferential stress-induced perfusion defect predominantly involving the apical myocardium, along with markedly reduced apical global circumferential strain (GCS), suggesting a potential association with chronic subendocardial ischemia; however, this remains a hypothesis based on a single case and requires further validation. The use of adenosine stress perfusion CMR in this case was intended to evaluate for coexisting ischemia, given the presence of calcification and impaired apical strain. Clinically, identifying calcification rather than thrombus is critical, as it may prevent unnecessary anticoagulation and guide appropriate follow-up strategies. This case underscores the importance of a multimodality imaging approach in assessing both structural and functional alterations in atypical phenotypes of hypertrophic cardiomyopathy.

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