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Chagas Cardiomyopathy


Epidemiology of Chagas Disease

First described by the Brazilian physician Carlos Chagas in 1909, Chagas cardiomyopathy is the result of a chronic myocarditis caused by infection with the parasite Trypanosoma cruzi. The chief mode of transmission is via the bite of a Triatomine species insect (kissing bug), although other modes of transmission such as congenital, transfusion-related, and oral have become more common. The disease is typically referred to as an illness of non-island nations of Latin America. There are an estimated 6 million individuals with Chagas disease. Both domestic rural to urban migration and international migration from Latin America have led to a broader distribution of infected individuals. There are an estimated 300,000 seropositive individuals in the United States, 80,000 in Europe, and smaller numbers in Japan, Australia, and Canada.

Chagas Disease Stages

Chagas disease is divided into three stages: acute, indeterminate, and chronic.

  • Initial phase. Most commonly, after an insect bite and hematogenous spread of parasite, an acute febrile illness follows, which is accompanied by nonspecific symptoms and is therefore frequently undiagnosed. In a small number of patients (<5%), a more serious illness occurs, including myopericarditis with pericardial effusion and meningoencephalitis.

  • Intermediate phase. Virtually all patients then pass into the “indeterminate phase” of the illness, characterized by two distinct positive serologic assays and the absence of end-organ manifestation of the disease.

  • Chronic phase. After 15 to 30 years, 20% to 30% of individuals develop cardiac manifestations of the illness, which can lead to complications such as stroke, arrhythmias, and heart failure.

Chagas Cardiomyopathy Stages

Chagas cardiomyopathy is stratified into stages A through D ( Table 157.1 ): stage A (abnormal serology with normal electrocardiogram [ECG]), stage B1 (abnormal serology, cardiac disease such as abnormal ECG but with normal ejection fraction), stage B2 (abnormal EF, no symptoms), stage C (abnormal echocardiography with clinical heart failure), and stage D (refractory heart failure). Individuals with normal ECGs are thought to progress to Chagas cardiomyopathy at a rate of 1.8% to 5% per year.

TABLE 157.1
Stages of Chagas Heart Disease
Reproduced with permission from Acquatella, H, et al: Recommendations for multimodality cardiac imaging in patients with Chagas disease, J Am Soc Echocardiogr 31:3–25, 2018; Filho P, et al: Left ventricular global performance and diastolic function in indeterminate and cardiac forms of Chagas’ disease, J Am Soc Echocardiogr 20:1338–1343, 2007.
Chronic Phase
Acute Phase Indeterminate Form Chagas Cardiomyopathy
A B1 B2 C D

  • Infected with Trypanosoma cruzi

  • Findings of acute Chagas disease

  • Positive serology

  • Normal ECG

  • No heart disease

  • No heart failure

  • Structural abnormalities

  • Abnormal laboratory results (abnormal ECG or echocardiograph)

  • Normal LV function

  • No heart failure

  • LV dysfunction

  • No heart failure

  • LV dysfunction

  • Current or prior heart failure

  • Refractory heart failure despite optimal medical management

LV, Left ventricular.

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