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Chronic Chagas' Myocardiopathy (CCM)

Imaging Diagnosis

In 20% of patients with severe chronic Chagas' myocardiopathy, the cardiac size is normal or only slightly enlarged. Equally, some patients who have severe heart disease clinically with cardiomegaly and advanced atrioventricular block have few or no symptoms. There is good correlation between the intensity of the microscopic changes and the degree of cardiac enlargement. The size of the heart depends on the severity and location of myocardial lesions and varies from normal to enormous dilatation and hypertrophy.

In the early stages of heart failure, isolated dilatation of the left ventricle may be present, since damage there is usually more severe than in other chambers and it has a heavier work load. Loss of distensibility of the left ventricular wall due to marked fibrosis may cause annular dilatation and functional incompetence of the mitral valve, and restricted diastolic filling of the left ventricle with resultant pulmonary venous congestion. Dyspnea is then more marked and on chest x-rays the cardiac contour, lung fields and hila resemble the radiographic appearance of mitral and aortic valvular disease rather than a myocardiopathy (Fig. 4.29). At times, the left atrium may be quite prominent and an apical systolic murmur may be present, imitating mitral insufficiency.

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Fig. 4.29 Chronic Chagas' myocardiopathy in a 55-year-old black Brazilian man. PA (A), right anterior oblique with barium in the esophagus (B), and left anterior oblique views of the chest (C) show marked generalized cardiac enlargement with prominence especially of the left ventricle and left atrium. The latter causes a prominent indentation upon the barium-filled esophagus in the RAO view, and also elevation of the left main stem bronchus and straightening of the left heart border on the PA view. There is minimal pulmonary vascular congestion with some cephalization of venous blood flow. There is also dilatation of the superior vena cava and a small right pleural effusion. In such patients, loss of distensibility of the left ventricular wall from fibrosis may cause functional mitral valve incompetence and restricted diastolic filling of the left ventricle, with resultant pulmonary venous congestion. The cardiac silhouette, pulmonary vasculature and hilar areas may resemble the radiographic appearance of mitral and/or aortic valvular disease rather than a myocardiopathy. Clinically, this patient had shortness of breath, easy fatiguability, an enlarged liver, and weight gain for the past year. The heart sounds were weak with a few premature beats. No murmurs were heard. EKG showed slight sinus tachycardia, complete right bundle branch block, left ventricular hypertrophy, a wide QT interval and isolated premature ventricular contractions. Serologic reaction for Chagas' disease (Machado-Guerreiro test) was positive. Necropsy showed Chagas' cardiopathy with a heart weight of 650 gm, generalized cardiac enlargement, right ventricular infarction, chronic passive congestion of the viscera, hydrothorax and ascites, and partial denervation of the esophagus and Auerbach plexi of the sigmoid colon. (Courtesy of Dr. Clovis Simao, Sao Paulo).

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