Apical hypertrophic cardiomyopathy with apical endomyocardial fibrosis and calcification

Abstract

Rationale: Apical hypertrophic cardiomyopathy (AHCM) is a rare form of hypertrophic cardiomyopathy which affects predominantly the apex of the left ventricle. Generally, left ventricular enlargement is not present in AHCM; additionally, endomyocardial fibrosis, and calcification are also rare. Patient concerns: A 61-year-old female (Case 1) and a 60-year-old female (Case 2) both presented with the symptoms of atypical chest pain, dyspnoea, exercise intolerance, palpitations. Diagnosis: Magnetic resonance and single-photon emission computed tomography (SPECT) revealed apical hypertrophic cardiomyopathy. Furthermore, 2D-transthoracic echocardiogram showed left atrium and ventricular enlargement, as well as endomyocardial fibrosis and calcification. Based on these findings, the patients were diagnosed with AHCM. Interventions: Both the patients were treated with ACEI, metoprolol, and aspirin. Additionally, both these patient underwent genetic test. Outcomes: The results of the genetic test of the 2 cases for hypertrophic cardiomyopathy (HCM) were negative. However, the gene mutation for dilated cardiomyopathy (TMPO) was detected in one of the cases. No change in condition during follow-up. Lessons: In past reports, Apical hypertrophic cardiomyopathy has been shown to have a benign prognosis. But in this case report, the imaging studies of the 2 patients suggest a poor prognosis. Furthermore, diagnosing cardiomyopathy should require multimodality imaging examinations to rule out differential diagnoses.

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

“…[ 10 ] Secondary EMF usually exists in patients with underlying ischemic heart disease and primary myocardial disease. [ 12 ] CMR can accurately identify EMF with the typical “double V” sign consisting of normal myocardium, fibrotic endocardial myocardium and covered thrombus. [ 13 ] This patient did not involve any above conditions related to tropical EMF including infection and eosinophilia.…”