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Published on: August 28, 2018
Apical Hypertrophic Cardiomyopathy With Endomyocardial Calcification: A Multimodality Imaging-Based Case Report
Sho Tanabe1, Chisato Takamura1, Masahiro Terashima1
1Cardiology, Cardiovascular Imaging Clinic Iidabashi, Tokyo, JPN.
Insights
Apical hypertrophic cardiomyopathy (ApHCM) with endomyocardial calcification is rare. Multimodality imaging revealed apical hypertrophy and calcification, suggesting potential chronic ischemia and guiding clinical management.
Area of Science:
- Cardiology
- Medical Imaging
- Pathophysiology
Background:
- Apical hypertrophic cardiomyopathy (ApHCM) with endomyocardial calcification is infrequently reported, leading to poorly defined imaging characteristics.
- Early detection of ApHCM is crucial for appropriate patient management and prognosis.
Observation:
- A 53-year-old woman presented with abnormal electrocardiographic findings.
- Multimodality imaging, including echocardiography, coronary CT angiography, and cardiac MRI, diagnosed ApHCM with endomyocardial calcification.
Findings:
- Adenosine stress perfusion cardiac MRI revealed a circumferential perfusion defect in the apical myocardium.
- Myocardial strain analysis showed reduced apical global circumferential strain, suggesting possible chronic subendocardial ischemia.
- The presence of calcification was distinguished from thrombus, impacting treatment decisions.
Implications:
- This case highlights the utility of a multimodality imaging approach for diagnosing atypical ApHCM phenotypes.
- Distinguishing calcification from thrombus is critical for preventing unnecessary anticoagulation and optimizing patient care.
- Further research is needed to validate the association between ApHCM, calcification, and chronic ischemia.
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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