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

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.

Keywords: apical hcm; cardiovascular magnetic resonance imaging (cmr); endomyocardial calcification; myocardial perfusion reserve; myocardial strain.

Figure 1. A 12-lead electrocardiogram showing T-wave inversion in leads I, II, III, aVF, and V2-V6.

Figure 2. Transthoracic echocardiography.

(A) Three-chamber view. (B) Short-axis view. Both panels show apical endomyocardial calcification.

Figure 3. Coronary computed tomography angiography (CCTA) showing no significant stenosis in the right (A) and left (B) coronary arteries. Apical hypertrophy with endomyocardial calcification is evident in the left ventricle (C, D), with no evidence of thrombus.

Figure 4. Cine cardiac magnetic resonance imaging (four-chamber view).

(A) End-diastolic frame. (B) End-systolic frame. Both panels show a spade-shaped configuration and marked apical hypertrophy of the left ventricle.

Figure 5. Stress perfusion cardiac magnetic resonance imaging.

(A) Stress phase showing a circumferential stress-induced perfusion defect predominantly in the apical region. (B) Rest phase showing no corresponding perfusion defect.

Figure 6. Late gadolinium enhancement images.

(A) Short-axis view. (B) Four-chamber view. Both panels show apical subepicardial and transmural enhancement, with a low-signal endocardial area suggestive of thrombus or calcification.

Figure 7. Global circumferential strain (GCS) analysis based on cardiac magnetic resonance imaging.

(A) Bull’s eye plot demonstrating reduced strain in the apical segments. Strain values are color-coded from dark red (more negative, indicating preserved contraction) to light red and white (less negative, indicating impaired contraction). (B) Corresponding segmental strain curves for the basal, mid, and apical levels (top to bottom), confirming predominant apical dysfunction.