Endomyocardial fibrosis and apical calcification: A case report with unusual presentations of apical hypertrophic cardiomyopathy

• Published in: Medicine (Baltimore) Vol. 102; no. 45; p. e35823
• Main Authors: Gao, Man, Zhang, Feifei, Xie, Yuetao, Li, Jialin, Hao, Xiao, Liu, Huiliang, Qi, Xiaoyong, Dang, Yi
• Format: Journal Article
• Language: English
• Published: United States Lippincott Williams & Wilkins 10-11-2023
• Subjects: Apical Hypertrophic Cardiomyopathy; Calcinosis - complications; Calcinosis - diagnostic imaging; Cardiomyopathy, Hypertrophic - diagnosis; Cardiomyopathy, Hypertrophic - diagnostic imaging; Clinical Case Report; Echocardiography - methods; Endomyocardial Fibrosis - diagnosis; Endomyocardial Fibrosis - diagnostic imaging; Heart Failure; Humans
• ISSN: 0025-7974; 1536-5964
• DOI: 10.1097/MD.0000000000035823

Apical hypertrophic cardiomyopathy (ApHCM) is a phenotypic variant of hypertrophic cardiomyopathy. Endomyocardial fibrosis and endocardial calcification are especially rare in ApHCM.
The main symptoms was chest tightness, palpitation, shortness of breath, and fatigue. Echocardiography and imaging examinations found apical hypertrophy along with endocardial calcification and endomyocardial fibrosis. Abnormal structural changes led to thrombosis and made the left ventricle a flat shape resembling an "apple."
The typical presentations, hypertrophic apex on echocardiography and an elevated N-terminal pro-brain natriuretic peptide level indicated the diagnosis of ApHCM and heart failure with preserved ejection fraction.
Optimal medical therapy including the administration of ApHCM, heart failure and atrial fibrillation to improve symptoms and life quality.
Since discharge, the patient could perform normal daily activities and had no discomfort based on the optimal medical therapy.
We report a ApHCM patients with unusual presentations of endomyocardial fibrosis and apical calcification. This case highlights the importance of understanding the specific pathological changes of ApHCM for treatment and prognosis.