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CARDIOVASCULAR JOURNAL OF AFRICA • Volume 27, No 5, September/October 2016
e4
AFRICA
Active schistosomiasis, severe hypereosinophilia and
rapid progression of chronic endomyocardial fibrosis
AO Mocumbi, C Goncalves, A Damasceno, C Carrilho
Abstract
Endomyocardial fibrosis (EMF) is a neglected restrictive
cardiomyopathy of unknown aetiology and unclear natural
history, which causes premature deaths in endemic areas. We
present the case of a 13-year-old boy from a highly endemic
area, presenting with concurrent signs of chronic EMF and
severe hypereosinophilia associated with active schistosomal
cystitis. We discuss the possible role of this parasitic infection
in determining the progression of EMF in endemic areas for
both conditions.
Keywords:
endomyocardial fibrosis, schistosomiasis, pathogen-
esis, management
Submitted 20/10/15, accepted 11/3/16
Cardiovasc J Afr
2016;
27
: e4–e6
www.cvja.co.zaDOI: 10.5830/CVJA-2016-030
Endomyocardial fibrosis (EMF) is a poorly understood
restrictive cardiomyopathy that affects mainly children and
adolescents in endemic areas of Africa, Asia and Latin America.
1
The suggested pathogenesis is that of succession of necrosis,
thrombosis and fibrosis, but this has been difficult to prove
because most patients are seen in late stages of the disease. We
describe a case of EMF with severe fibrosis associated with
active schistosomiasis, hypereosinophilia and a fatal outcome.
Case report
A 13-year-old boy of black ethnicity from an endemic zone
of EMF was referred to hospital in congestive heart failure.
He reported a three-month history of progressive exertional
dyspnoea without orthopnoea or paroxysmal nocturnal
dyspnoea, as well as central, crushing and constant chest pain,
which was exacerbated by exercise and alleviated on rest. He also
complained of progressive painless abdominal distension, but
denied having palpitations, wheeze, cough, night sweats, fever,
or any gastrointestinal or urinary symptoms. His past medical
history was uneventful, and he was not on medication prior to
his first admission, two weeks before he was transferred.
On examination he was alert, apyretic, had no neurological
signs of disease or disorientation and presented a good general
status. His heart rate was 108 beats/minute with a regular
rhythm, blood pressure was 90/60 mmHg, and respiratory rate
was 16 breaths/minute.
Cardiac examination revealed raised jugular venous
pressure, a visibly pulsating, palpable, non-displaced apex beat,
and a mild holosystolic murmur on auscultation. Besides a
bilateral inspiratory wheeze, the respiratory examination was
unremarkable.
The abdomen was soft, non-tender and mildly distended,
with a 3-cm hepatomegaly and no other organomegaly. He was
not jaundiced and there was no shifting dullness on abdominal
examination.
Blood examinations for malaria, human immunodeficiency
virus, recent streptococcal infection, rheumatoid factor and
syphilis were all negative. Erythrosedimentation rate was raised
at 55 mm/h. White blood cell count was normal with marked
eosinophilia. Stool examination for helminths was negative.
The chest X-ray showed prominence of the pulmonary artery.
The ECG revealed sinus rhythm, signs of right ventricular
overload and non-specific repolarisation abnormalities.
On transthoracic echocardiography, the right ventricular
cavity was reduced and areas of endocardial thickening suggested
fibrosis; the overall right ventricular function was preserved. The
right atrium was dilated and moderate tricuspid regurgitation
was present, allowing estimation of systolic pulmonary pressure
at 85 mmHg. No images suggesting thrombi were detected on the
right side of the heart. The left ventricle had marked thickening
of the mural endocardium and a homogeneous mass occupying
its apical third, suggesting a thrombus that did not interfere with
the mitral valve function. Left ventricular systolic and mitral
valve function were preserved; mild circumferential pericardial
effusion was present.
The results of rectal biopsy were inconclusive for schistosomal
infection.
A diagnosis of bilateral EMF with hypereosinophilia was
made and the patient was managed with daily oral furosemide 40
Instituto Nacional de Saúde, Maputo, Mozambique
AO Mocumbi, MD,
amocumbi@gmail.comEduardo Mondlane University, Maputo, Mozambique
AO Mocumbi, MD
A Damasceno, MD
C Carrilho, MD
Maputo Central Hospital, Mozambique
C Goncalves, MD
A Damasceno, MD
C Carrilho, MD
Case Report