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CARDIOVASCULAR JOURNAL OF AFRICA • Volume 28, No 4, July/August 2017



Cardiovascular Topics

Endomyocardial fibrosis in Sudan: clinical and

echocardiographic features

Siddiq Ibrahim Khalil, Suha Khalil, Salma El Tigani, Hanan A Saad



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.


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-



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



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.


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



: 208–214

DOI: 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,


and in Africa in 1946 by Bedford

and Konstam,


but its clinicopathological features were first

recognised byDavies inUganda in 1948.


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.



has also been reported from other subtropical countries such as



and Nigeria, and some sub-Saharan countries,



and Kerala in India,


It is exceedingly rare in Europe and North

America; however a few cases have been reported in China





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.



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.


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.


Department of Medicine, University of Medical Sciences

and Technology, Khartoum, Sudan

Siddiq Ibrahim Khalil, MD, FRCP, FESC, FACC, psiddiq@gmail.


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