Apical Hypertrophic Cardiomyopathy with Apical Endomyocardial Fibrosis and Calcification: Two Case Reports
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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.
Radano I, Mabritto B, Luceri S, Bongioanni S, Maiellaro F, Zappia L ESC Heart Fail. 2024; 11(4):2415-2420.
PMID: 38634252 PMC: 11287301. DOI: 10.1002/ehf2.14775.