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CARDIOVASCULAR JOURNAL OF AFRICA • Volume 28, No 4, July/August 2017
208
AFRICA
Cardiovascular Topics
Endomyocardial fibrosis in Sudan: clinical and
echocardiographic features
Siddiq Ibrahim Khalil, Suha Khalil, Salma El Tigani, Hanan A Saad
Abstract
Objective:
Endomyocardial fibrosis (EMF) is a rare disease
and is often an underdiagnosed and forgotten cardiomyopa-
thy. The objective of this study was to document the current
frequency of EMF in Sudan by defining and selecting cases
from patients attending the echocardiography laboratory.
Additionally we aimed to create an EMF registry for Sudan.
Methods:
The study started in January 2007 and is on-going.
All the patients attending our echocardiography clinics in
four different hospitals in Khartoum, Sudan, were included.
Transthoracic echocardiography was used as the main diag-
nostic and selection tool. The diagnosis of EMF was based on
predefined criteria and definitions, and was further supported
by additional clinical, ECG, laboratory and chest X-ray find-
ings.
Results:
Out of 4 332 cases studied, 23 (0.5%) were found to
have features of EMF. Females constituted 52% and the age
range was 24 to 67 years. All patients presented with dysp-
noea grades III–IV. Advanced heart failure with gross fluid
overload was seen in 54% of cases and ascites was seen in
30%. EMF was biventricular in 53%, left ventricular in 29%
and right ventricular in 18% of cases. Apical and ventricular
wall fibrosis was found in all cases, followed by atrial enlarge-
ment, atrioventricular valve incompetence, ventricular cavity
obliteration, restrictive flow pattern and pericardial effu-
sion. Additional echocardiographic features are defined and
discussed.
Conclusion:
Although a rare disease, cases of EMF can be
identified in Sudan if a high index of suspicion is observed.
New echocardiographic features of ventricular wall layering,
endocardial fibrous shelf and endomyocardiopericarial fibro-
sis were identified and are discussed.
Keywords:
endomyocardial fibrosis in Sudan, apical fibrosis,
ventricular cavity obliteration, endocardial fibrous shelf, endo-
myocardiopericarial fibrosis
Submitted 23/5/16, accepted 2/8/16
Cardiovasc J Afr
2017;
28
: 208–214
www.cvja.co.zaDOI: 10.5830/CVJA-2016-079
Endomyocardial fibrosis (EMF) is a form of cardiomyopathy
characterised by fibrosis and thickening that distinctly involves
the ventricular apex and walls. It is one of the common causes
of restrictive cardiomyopathy and is frequently underdiagnosed
and occasionally mislabelled as rheumatic valvular disease or
hypertrophic cardiomyopathy.
EMF was described by Loffler in 1936 in a patient with
associated eosinophilia,
1
and in Africa in 1946 by Bedford
and Konstam,
2
but its clinicopathological features were first
recognised byDavies inUganda in 1948.
3
Although sporadic cases
with similar clinical and pathological features have since been
reported from other parts of the world, the majority of reported
cases have come from West and Central Africa. In Uganda, it
accounted for 25% of cases reported for echocardiography, and
for 20% in a random population sample in Mozambique.
4,5
EMF
has also been reported from other subtropical countries such as
Egypt
6
and Nigeria, and some sub-Saharan countries,
7,8
Brazil,
and Kerala in India,
9,10
It is exceedingly rare in Europe and North
America; however a few cases have been reported in China
11
and
Japan.
12
The aetiology of EMF is unknown, however on an
epidemiological basis, it behaves like a vector-transmitted disease.
In addition to geography, several factors have been associated
with the pathogenesis of EMF in Africa, including ethnicity,
poverty, diet, age and gender, infection and eosinophilia.
3
There
is now evidence that the initial heart lesion in EMF may be
associated with abnormalities of the eosinophils, although
eosinophilia is common in many tropical regions where EMF
does not appear to be prevalent. This suggests that other
factors, possibly immunological in nature, are also necessary to
determine the prevalence of the disease in a particular location.
3,6
There is agreement among researchers that African
endomyocardial fibrosis is a distinct entity and, despite the
similarity in pathological features with Loffler’s endocarditis
and the cardiac lesions seen in eosinophilic leukaemia or reactive
eosinophilia, there is no hard evidence to suggest that African
endomyocardial fibrosis is a variant of Loffler’s disease.
13-15
Department of Medicine, University of Medical Sciences
and Technology, Khartoum, Sudan
Siddiq Ibrahim Khalil, MD, FRCP, FESC, FACC, psiddiq@gmail.
com
The Heart Clinic, Khartoum, Sudan
Suha Khalil, MB BS, MRCGP
Amal National Hospital, Khartoum, Sudan
Salma El Tigani, MB BS, MRCP
Academy Teaching Hospital, Khartoum, Sudan
Hanan A Saad, MD