Biventricular endomyocardial fibrosis with right ventricular hypertrabeculation and rapid progression to heart transplantation: imaging and pathologic correlation.
Sofia Alves Figueiredo Faustino, Ana Clara Tude Rodrigues, Ebe Christie de Oliveira et al.
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Case in brief
A 57-year-old male amateur endurance cyclist presented with a two-year history of progressive heart failure, including fatigue, ascites, and lower limb edema. Imaging revealed severe biatrial enlargement, diastolic dysfunction, and apical obliteration of both ventricles, alongside marked right ventricular trabeculation. Despite medical therapy, he progressed to advanced heart failure, necessitating heart transplantation, which confirmed endomyocardial fibrosis with right ventricular hypertrabeculation.
What made this case unusual
This case highlights a previously unreported association between endomyocardial fibrosis and right ventricular hypertrabeculation, which is not typically seen in this condition.
Diagnostic / clinical pearl
Clinicians should consider the possibility of non-compacted myocardium in patients with endomyocardial fibrosis presenting with atypical imaging findings.
Why it matters
Recognizing this association can aid in the comprehensive assessment and management of patients with restrictive cardiomyopathies, potentially influencing treatment strategies.