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The histologic diagnosis of [[cardiomyopathy|Chronic Chagas cardiomyopathy]] (CCM) consists of a diffuse and patchy chronic myocarditis, interstitial [[mononuclear cell]] infiltrates, and myocardial fiber destruction with fibrotic replacement.  Grossly enlarged hearts have been found in autopsy studies in subjects with end stage of Chagas disease. Left ventricular apical aneurysms are also frequently found on autopsy.
The histologic diagnosis of [[cardiomyopathy|Chronic Chagas cardiomyopathy]] (CCM) consists of a diffuse and patchy chronic myocarditis, interstitial [[mononuclear cell]] infiltrates, and myocardial fiber destruction with fibrotic replacement.  Grossly enlarged hearts have been found in autopsy studies in subjects with end stage of Chagas disease. Left ventricular apical aneurysms are also frequently found on autopsy.
Based upon EKG changes, the presence and grade of cardiomegaly, and clinical findings, patients with Chagas disease can be divided into 4 groups (The American College of Cardiology / American Heart Association staging of disease progression);
*A. High risk of [[heart failure]] without presence of structural heart disease
*B. Structural heart disease without [[heart failure]]
*C. Structural heart disease with prior or present [[heart failure]] 
*D. [[Heart failure]] that is refractory to treatment
===Stage A===
75% of patients with positive serologic test results are asymptomatic. Those individuals with normal ECG findings are referred as being in the indeterminate phase of the Chagas disease.
===Stage B===
The appearance of ECG abnormalities implies disease progression. By definition, no [[cardiomegaly]] is present, and left ventricular systolic function is normal. Fewer than 10% of these patients have left ventricular segmental wall motion abnormalities.
===Stage C===
Symptomatic patients with mild to moderate cardiac damage, and are in New York Heart Association (NYHA) functional class II or III. [[Arrhythmias]] may present. Embolism and [[sudden  cardiac death]] may occur. [[Heart failure]] is reversible.
===Stage D===
The ECG is abnormal in almost all. The heart is dilated, and left ventricular systolic and diastolic function are usually abnormal. About half of these patients may have left ventricular apical and other segmental wall abnormalities. Mitral and tricuspid valve regurgitation may be present. Survival at 10 years is approximately 75% to 85%. If left untreated, a patient with NYHA functional class IV heart failure (e.g., stage D) has a very high mortality rate (50%) at 2 years.


The degree of myocardial fibrosis increases progressively from the mildest to the most severe disease stages. Additionally, myocardial fibrosis correlates inversely with left ventricular ejection fraction and clinical status.  
The degree of myocardial fibrosis increases progressively from the mildest to the most severe disease stages. Additionally, myocardial fibrosis correlates inversely with left ventricular ejection fraction and clinical status.  

Revision as of 19:00, 23 April 2009

The heart in Chagas' disease
This is a higher-power photomicrograph of an H & E stained heart biopsy from this patient. Note the T. cruzi amastigotes (arrows) within this longitudinal section of a myocyte.
Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology

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Overview

Chronic Chagas' disease leads to congestive heart failure. These patients commonly have right bundle branch block and/or other arrhythmias. These hearts are dilated and hypertrophied, have areas of fibrosis especially in the apex, and often contain mural thrombi. The myocardium is infiltrated with lymphocytes and macrophages and there is interstitial edema and fibrosis. This inflammatory reaction is most severe around the area of the right bundle branch. Patients may also develop megaesophagus and/or megacolon. [1] [2] [3]

Acute form of Chagas Disease

In a patient with diagnosed Chagas disease, cardiac damage is suspected if one or more following ECG findings occurs:

Myocarditis is infrequent, appearing in only 1-5% of patients whose having the acute phase of Chagas Disease (1-5 of every 10,000 infected subjects).

Chronic form of Chagas Disease

The histologic diagnosis of Chronic Chagas cardiomyopathy (CCM) consists of a diffuse and patchy chronic myocarditis, interstitial mononuclear cell infiltrates, and myocardial fiber destruction with fibrotic replacement. Grossly enlarged hearts have been found in autopsy studies in subjects with end stage of Chagas disease. Left ventricular apical aneurysms are also frequently found on autopsy.

The degree of myocardial fibrosis increases progressively from the mildest to the most severe disease stages. Additionally, myocardial fibrosis correlates inversely with left ventricular ejection fraction and clinical status.

Cardiac MRI may demonstrate myocardial involvement (hyperenhancement) among seropositive patients without clinical symptoms or left ventricular wall motion abnormalities.

Across groups A-D, coronary angiography is usually normal or shows minimally obstructive disease.

Case Example: Heart Involvement in Chagas Disease

Clinical Summary

A 12-year-old boy, whose family had recently emigrated from Brazil, presented to the emergency room with a three-day history of malaise, fever, anorexia, and edema of the face and upper extremities.

On physical examination the patient had generalized lymphadenopathy and hepatosplenomegaly.

The patient was tachycardic and dysgenic with signs of congestive heart failure.

A cardiac biopsy was performed which revealed an active myocarditis with leishmanial forms of parasitic organisms within cardiac myocytes.

Close examination of peripheral blood smears revealed occasional circulating trypomastigotes. A complement fixation test for antibodies to Trypanosoma cruzi was strongly positive.

Images courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology



Additional Images

Microscopic Images

Images courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology














Gross Images

Images courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology

References

  1. Acquatella H, Echocardiography in Chagas Heart Disease, Circulation. 2007;115:1124-1131
  2. Mady C, Ianni BM, Arteaga E, et al. Relation between interstitial myocardial collagen and the degree of clinical impairment in Chagas’ disease. Am J Cardiol 1999;84:354–6.
  3. Rochitte C. E., Oliveira P. F., Andrade J. M. et. al. Myocardial Delayed Enhancement by Magnetic Resonance Imaging in Patients with Chagas Disease; a Marker of Disease Severity. J Am Coll Cardiol 2005; 46: 1553-58

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