Clinical Characteristics

Endomyocardial fibrosis is often a chance autopsy finding, totally unexpected. The disease is most common in children and young adults, but has been reported as early as the age of 2 years and as late as 50 years.

In some patients there is an acute febrile illness with malaise and anorexia; the fever may persist for months and continue until heart failure develops. Recurrent pyrexia is usually accompanied by myocarditis and fresh endomyocardial necrosis. EMF seldom occurs as a single clinical episode, but rather as a progressive disease.

The clinical presentation will depend largely on the laterality of the disease, and the dominant side varies in different series; right-sided EMF is probably equally distributed between males and females, whereas left-sided EMF seems to be more frequent in females.

Patients with right-sided EMF present with a grossly swollen abdomen. The neck veins are distended but there is a striking absence of peripheral edema. The main problem is the ascitic fluid within the abdomen, which may result in a bizarre appearance because of the underlying generalized wasting. Surprisingly, dyspnea is not a problem, and many of the patients are able to lie flat. When cardiac failure occurs, peripheral edema follows. Clinical examination is usually nonspecific. The peripheral pulse is of small volume and the ECG will show atrial fibrillation or premature ventricular beats.

Left ventricular EMF usually presents as mitral insufficiency, associated with pulmonary venous hypertension. The patient complains of dyspnea or pain in the chest and a cough or pulmonary congestion develops.

When both ventricles are affected it is often impossible to be certain which ventricle is more severely diseased. In either variety pericardial fluid may be detected but seldom causes tamponade unless cardiac failure has occurred. There is usually more pericardial fluid in patients with right-sided EMF.

The complications are those of heart failure and peripheral emboli, particularly in the lungs. Death may be dramatic, due to massive pulmonary infarction. In other cases, cardiac decompensation occurs more slowly but inevitably.

Imaging Diagosis

Right-sided EMF results in raised pulmonary venous pressure with tricuspid incompetence, an enlarged liver, ascites, and a hyperkinetic right ventricular outflow tract. On chest radiographs, the heart is always enlarged in the transverse diameter, and often it is enormous. This may be due to a coexisting pericardial effusion, but is usually due to a dilated, almost aneurysmal right atrium. If pericardial fluid is scanty there will be an outflow tract convexity, which on fluoroscopy or ultrasound is seen to be very active. In late cases there may be an oblique, linear calcification at the elevated apex of the right ventricle or base of the pulmonary conus (Fig. 25.6). The lung fields are strikingly oligemic (Figs. 25.7, 25.8) and, because of low cardiac output, the superior vena cava and azygos veins are very prominent.

Fig. 25.6. The patterns of calcification in EMF. The top row shows the typical linear oblique pattern of right-sided disease. The lower row displays the curvilinear left ventricular form.

Fig. 25.7. Typical right-sided EMF with grossly enlarged cardiac contours and oligemic lung fields. It can be difficult without ultrasonography or angiography to know whether pericardial fluid or a large right atrium is mainly responsible for the cardiac enlargement.

Fig. 25.8. The chest radiograph of a West African woman with right-sided EMF. There is marked pulmonary oligemia, which is in contrast to the cardiac size. This penetrated film, with the patient supine, shows calcification near the base of the pulmonary conus.