Pure left-sided EMF (Fig. 25.13) is less frequent clinically than right- sided disease (10% versus 47%). A chest radiograph reveals little if any cardiac enlargement although the left atrium may cast a double shadow. Elevated pulmonary venous pressure and sometimes a degree of pulmonary arterial hypertension will cause enlargement of the pulmonary conus and infundibulum; the main pulmonary arteries become dilated and pulsating. The peripheral arteries do not change and there is no pulmonary edema. The aorta appears small. In about 5% of patients with EMF, curvilinear calcification can be detected where the former apex of the ventricle has been obliterated (Fig. 25.6). Apart from this calcification it can be difficult to differentiate between EMF and rheumatic heart disease on plain radiographs, but MRI or angiography in EMF will show two abnormalities deforming the apex of the left ventricle. These remain constant between systole and diastole; there may be mitral incompetence. The left atrium empties slowly, presumably because the small scarred left ventricle cannot take a full volume.

Fig. 25.13 A,B Left ventricular EMF with typical obliteration of ventricular apex in two views and opacification of left atrium because of mitral incompetence (angiogram).

Ultrasound clearly demonstrates the deformed ventricle with reduced volume and with adherent papillary muscles (Fig. 25.14).

Fig. 25.14. Echocardiogram showing fibrosing EMF (FIB) at the left ventricular apex and involvement of the posterior papillary muscle. LV Left ventricle; RV right ventricle; LA left atrium; AO aorta. (Courtesy of Professor Bertrand, Abidjan).

Biventricular disease is dominated clinically and radiologically by the right side, because the low cardiac output tends to protect and mask the left-sided changes. Pericardial effusions are frequent and the general features are those of right-sided EMF, with a greatly enlarged globular transverse cardiac diameter and relatively oligemic lung fields. The angiographic, echocardiographic, and MRI features are also dominated by the right side, showing a dilated right atrium, obliterated right ventricle, and a small deformed left ventricle (Fig. 25.15). The enlarged right atrium displaces the root of the aorta to the left, and the right coronary artery is displaced anteriorly in a wide arc as it lies in the atrioventricular (AV) groove and courses to the crux.

Fig. 25.15. Biventricular EMF with left-sided opacification showing a small deformed left ventricle with loss of the apical portion and reflux into the left atrium from mitral incompetence. The elevation and curvature of the right coronary artery is consequent on displacement of the atrioventricular groove from the giant right atrium.

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