Biventricular endomyocardial fibrosis with right ventricular hypertrabeculation and rapid progression to heart transplantation: imaging and pathologic correlation.

A 57-year-old male amateur endurance cyclist presented with a two-year history of progressive heart failure symptoms including fatigue, ascites, and lower limb edema.

• Patient age was 57 years
• Symptoms had been progressive over a two-year period
• Clinical presentation included fatigue, ascites, and lower limb edema
• The patient was an amateur endurance cyclist, a detail noted as potentially relevant to the clinical context

Transthoracic echocardiography revealed severe biatrial enlargement, grade III diastolic dysfunction, valvular regurgitation, and apical obliteration of both ventricles.

• Severe biatrial enlargement was identified on echocardiography
• Diastolic dysfunction was classified as grade III
• Valvular regurgitation was present
• Apical obliteration was observed in both the right and left ventricles
• These findings are consistent with restrictive cardiomyopathy physiology

Marked right ventricular trabeculation with deep intertrabecular recesses and turbulent flow was observed, raising suspicion of non-compacted myocardium.

• Right ventricular hypertrabeculation was identified on imaging
• Deep intertrabecular recesses were noted
• Turbulent flow within the recesses was detected
• The imaging findings raised suspicion for non-compacted myocardium (left ventricular non-compaction)
• This coexistence of endomyocardial fibrosis with noncompacted myocardium had not been previously reported

Despite optimized medical therapy, the patient experienced recurrent hospitalizations and progressed to advanced restrictive heart failure requiring orthotopic heart transplantation.

• Medical therapy was optimized but failed to control disease progression
• The patient required multiple hospitalizations
• Disease course culminated in orthotopic heart transplantation
• The rapid progression to transplantation is highlighted as a notable feature of this case

Pathological examination of the explanted heart confirmed chronic-stage endomyocardial fibrosis with extensive endocardial fibrosis and prominent right ventricular trabeculation.

• Explanted heart pathology provided definitive diagnosis
• Findings confirmed chronic-stage endomyocardial fibrosis
• Extensive endocardial fibrosis was documented histopathologically
• Prominent right ventricular trabeculation was confirmed on pathological examination, correlating with imaging findings
• Imaging and pathologic correlation is emphasized as a key feature of this report

The coexistence of endomyocardial fibrosis and right ventricular hypertrabeculation represents a previously unreported association that expands the phenotypic spectrum of endomyocardial fibrosis.

• No prior reports of endomyocardial fibrosis coexisting with noncompacted myocardium were identified by the authors
• The case is described as expanding "the phenotypic spectrum of endomyocardial fibrosis"
• The authors emphasize the importance of comprehensive imaging assessment in atypical presentations
• Endomyocardial fibrosis is characterized by deposition of fibrotic tissue in the endocardium, predominantly affecting ventricular apices and atrioventricular valves
• The pathophysiology of endomyocardial fibrosis is noted to remain "incompletely understood"