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



These findings conform to features described in previous



Right ventricular EMF echocardiographic features

included fibrosis of the apex, right ventricular free wall and

anterior interventricular septum with obliteration of the

ventricular cavity and dilated right atrium (Fig. 3A). The

pericardium was also found to be affected and looked fibrosed

and thickened, with mild-to-moderate effusion in the majority

of cases (87%). Severe effusion (32 mm) was noted in one case,

without evidence of cardiac tamponade, possibly due to the

chronicity of the disease.

As a result of fibrosis, the three layers forming the heart wall

were identified as separate layers, especially in the posterior

ventricular wall. This feature resulted in a form of ‘layering’

and is shown in Fig. 3 in both the short-axis (3A) and M-mode

views (3B). Layering can be seen on all left ventricular walls,

specifically the lateral and posterior walls. Although the thick

fibrosis of the three separate layers was recognised in post

mortem findings reported by Davies,


echocardiographic images

of layering have not been reported before.

New echocardiographic features

Endocardial fibrous shelf:

in countries where EMF is prevalent,

the rates of rheumatic heart disease are also high and diagnostic

difficulties arise in differentiating patients with mitral stenosis

from those with EMF. A new echocardiographic feature, the

endocardial fibrous shelf (EFS) seen in Fig. 5A–D provides

useful diagnostic help.

These echocardiographic images correlate well with a

previously recognised pathological finding first described by

Davies in 1955. He reported that the posterior mitral cusp was

completely immobilised by adherence to the endocardium of the

posterior wall of the ventricle, and the end result was a fibrous

surface running straight down from the atrium to the ventricle

where the cusp had become embedded.


Davies further added

that in other cases, the remains of the cusp projected as a short,

thick shelf. This finding is shown echocardiographically by an

immobile posterior mitral leaflet tethered to the endocardium

and appearing like a solid shelf. The anterior mitral valve leaflet,

although moderately thick, moves freely, while the whole mitral

structure becomes reduced to a single leaflet valve.

The echocardiographic endocardial fibrous shelf can be

visualised in the modified APLX in all cases of left ventricular

and biventricular EMF and provides a mark for differentiation

between EMF of the left ventricle and rheumatic mitral stenosis,

where the leaflets and subvalvular structure are fibrosed and may

be calcified but the posterior LV wall remains free.

Endomyocardiopericardial fibrosis: in three cases of advanced

EMF, a dense fibrous pericardium and pericardial calcification

were seen (Fig. 6). This entity behaved clinically like constrictive

pericarditis, as the three patients presented with tachycardia,

ascites and gross oedema of the ankles. We opted to give this type

of EMF, inwhich the pericardiumplayed a significant pathological

and clinical role, the name endomyocardiopericardial fibrosis

(EMPF), and considered it a cause of pericardial constriction.

Although EMF is among the common causes of restrictive

cardiomyopathy, its role in pericardial constriction has not been

described before. Despite the fact that an endomyocardial biopsy

from patients with both tuberculous constrictive pericarditis

and endomyocardial fibrosis revealed similar histopathological

changes of endocardial thickening and focal myofibrosis,

evidence to support pericardial constriction in EMF could not

be confirmed.


The echocardiographic and clinical presentation

of patients with EMPF lends support to pericardial constriction

in association with EMF.

Thedifferentiationof EMFfromhypertrophiccardiomyopathy

(HCM), especially the apical type, can be difficult. However our

observations are consistent with the view of Fawzy, Ziady and

Halilm in that with EMF, apical obliteration appears during

both systole and diastole, in contrast to HCM where it occurs

only in systole.


One additional observation is the characteristically huge left

atrium (91 mm in one case), which could not be seen, even in

cases of severe mitral stenosis. Among the explanations offered

were the obliteration of the ventricular cavity, and hence the

increase in filling pressure, together with the additional volume

load due to mitral regurgitation.

This study has provided high-definition images of the

main diagnostic features of EMF. Images of layering provide

additional identification of this multi-layer disease. This study

has described and shown images of a new echocardiographic

feature: the endocardial fibrous shelf, which offers an additional

feature for left ventricular EMF. We also report a new entity,

EMPF, a form of advanced EMF that clinically behaved like

constrictive pericarditis.

Fig. 6.

Endomyocardiopericardial fibrosis (EMPF). A short-

axis view from a patient with advanced EMF and

intractable heart failure. The endocardium looks dense

and bright with the myocardium clearly seen under-

neath it. The striking finding is the appearance of

densely fibrosed and calcified pericardium with effu-

sion forming an endomyocardiopericarial fibrosis.

Note the presence of pericardial effusion and the

posterior papillary muscle being engulfed by the thick-

ened endocardium.