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



Despite the lack of evidence to pinpoint the aetiology of

EMF, some progress has been made in the diagnosis as well as in

medical and surgical treatment. In earlier reports, the diagnosis

of EMF was based on post mortem findings and clinical

correlations but presently echocardiography is the standard

diagnostic tool. Recently, cardiac magnetic resonance imaging

(CMR) emerged as an additional tool to define the primary

and secondary structural and functional abnormalities of EMF.

CMR with gadolinium enhancement seems ideally suited to

diagnose this condition and monitor response to medical and/

or surgical therapy.


Medical treatment of EMF entails reduction of fluid overload

with diuretics and preload with nitrates. Rate and rhythm control

may help in patients presenting with tachyarrhythmias, including

atrial fibrillation. Surgical treatment of EMF was practiced

for many years and consisted of removal of fibrosis from both

ventricles, and atrioventricular valve replacement. Mitral or

tricuspid annuloplasty has had only limited success.


In Sudan, EMF was first described by O’Brien in 1954 and

later by El Hassan, who had carried out post mortem studies

in 133 cases of cardiovascular deaths. Among these, six out of

13 cases of cardiomyopathy had EMF affecting both left and

right ventricles, with fibrosis that involved the endocardium and

subendocardial tissues and covering the apex, papillary muscles

and posterior ventricular wall, leading to atrioventricular valve



The objective of our study was to document the current

frequency of EMF in Sudan by identifying and selecting cases

from patients attending our echocardiography clinics in four

different hospitals, and creating an EMF registry. However

during the process of acquisition and analysis of images, it

emerged that there were new echocardiographic features that

had not been reported before. These findings are presented and

discussed below.


This study is a prospective, descriptive study, which started

in January 2007 and is on-going. Patients attending our

echocardiography sessions at Amal National Hospital from

2007 to 2009, and the Academy Teaching and Yastabshiroun

Hospitals from 2010 to 2011 were included; however the majority

of the selected patients were enrolled from the Heart Clinic in

Khartoum, Sudan. Permission for the study was obtained from

the ethics committees in the four centres and informed verbal

consent was obtained from each patient.

Transthoracic echocardiography using Mylab30 (Esaote,

Italy) was performed by SIK on all selected patients, using

the American Society of Echocardiography (ASE) standards.


Additional information was obtained from the

Echo Manual


Oh, Seward and Tajik.


The following standard echocardiographic views were used:

parasternal long-axis (PLAX), short-axis (SAX), M-mode, apical

two-, four- and five-chamber views (AP2, AP4, AP5) and the

apical long-axis view (APLX). Additionally, a modified APLX

view, obtained by angulating the probe medially and rotating

counter-clockwise to focus on the recess between the posterior

papillary muscle and the posterior mitral valve leaflet was used.

The diagnosis of EMF was made on cases that fulfilled the

following echocardiographic features and definitions:

Generalised endomyocardial thickening and fibrosis of the

apex, ventricular walls and papillary muscles, and atrioven-

tricular (AV) valve incompetence.

Obliteration of the ventricular cavity by fibrous tissue,

defined as left ventricular (LV) diastolic volume less than

40 ml, measured by the modified Simpson’s rule in AP4 view

and right ventricular (RV) size less than 20 mm, measured by

the mid-RV diameter in the AP4 view.

Left atrial (LA) volume was measured with the biplane

method of disks (modified Simpson’s rule) from AP4 and

AP2 views at ventricular end-diastole. Huge left atrium was

defined as atrial volume of more than 70 ml.

Right atrium (RA) was quantified from the apical four-cham-

ber view. The minor-axis diameter was measured according to

ASE recommendations.


Right atrial dilatation was assumed

when the mid-axis diameter was more than 5.0 cm.

Diagnosis was made from a detailed initial examination and

ascertained by two review examinations, carried out during the

first four weeks after the case was identified.

Additional information was derived from targeted history,

clinical examination, 12-lead electrocardiogram (ECG), chest

X-ray and complete blood count, including total blood count,

differential white cell count and absolute eosinophilia (







Out of 4 332 cases studied, 23 (0.5%) were found to have features

of EMF. Of these 23 cases identified, two were from Amal

Hospital, two from Yastabshiroun Hospital, five from Academy

Teaching Hospital and 14 cases were from the Heart Clinic in

Khartoum. Females constituted 52% and the age range was 24

to 67 years. All patients presented with dyspnoea grades III–IV,

and advanced heart failure with gross fluid overload was seen in

54% of cases and ascites was seen in 30%.

ECG findings were non-specific; sinus tachycardia was found

in 22% of patients, atrial abnormality in 43%, first-degree heart

block in 39% and atrial fibrillation in 13% of patients. Chest

X-ray findings were also non-specific and showed cardiomegaly

in 92% of patients.

Haematological findings included absolute eosinophilia in

three patients and five cases had iron deficiency. EMF was

biventricular in 53%, left ventricular in 29% and right ventricular

in 18% of cases. Echocardiographic features of EMF are

summarised in Table 1.

Table 1. Frequencies of echocardiographic

features of EMF (


= 23)

Echocardiographic features

Percentage of patients

Apical fibrosis


Ventricular wall fibrosis


Atrial enlargement


Atrioventricular valve regurgitation


Ventricular cavity obliteration


Pericardial effusion


Endocardium fibrous shelf formation


Restrictive flow pattern




Thrombus formation