Canadian Journal of Cardiology

Isolated Endomyocardial Fibrosis of the Right Ventricle

      In a 38-year-old African woman, cardiomegaly was incidentally diagnosed on a computed tomography (CT) scan. Transthoracic echocardiography (TTE) showed dilated right cardiac chambers with obliteration of the right ventricular (RV) cavity. A borderline blood eosinophilia was initially found, related to a parasitic infection, which spontaneously normalized. The patient never reported typical symptoms of carcinoid syndrome and presented with normal blood levels of chromogranine A, vasoactive intestinal polypeptide, and pancreatic polypeptide. Only urine 5-hydroxyindoleacetic acid was slightly elevated, which was attributed to a tryptophane-rich regimen. Repeated liver ultrasound and abdominal CT scan did not identify any neoplastic lesion, and TTE did not show any valvular abnormality typically associated with carcinoid heart. Her medical history was negative for thromboembolic events, and coagulation tests and platelet count were normal. We proceeded to an endomyocardial biopsy, which demonstrated important subendocardial and interstitial fibrosis without any inflammation, eosinophilic infiltration or granuloma (Fig. 1, A, B). Therefore, a tropical form of endomyocardial fibrosis (EMF) was diagnosed.
      Figure thumbnail gr1
      Figure 1(A) Endomyocardial biopsy containing endocardium and myocardium with interstitial and subendocardial fibrosis (arrows) stained with hematoxylin and eosin and (B) Masson trichrome (additional stains/magnifications in ). (C, E-G) Cardiac magnetic resonance imaging study, demonstrating localized obliteration of the right ventricular apex (red arrows) with associated fibrosis of the subendocardium (yellow arrows). A thrombus in the right atrium is seen (yellow asterisk). (D) TTE pulsed-wave Doppler on the tricuspid valve showing restrictive inflow pattern, and continuous-wave Doppler through the tricuspid regurgitation showing a triangle-shaped profile.
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        • Mocumbi A.O.
        • Freers J.
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        Tropical endomyocardial fibrosis—natural history, challenges, and perspectives.
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