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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 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 Supplemental Figs. S1-S5). (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.
