Guideline sources The following summarized guidelines for the evaluation and management of chagas disease are prepared by our editorial team based on guidelines from the Infectious Diseases Society of America (IDSA/CDC/NIH/HIVMA 2023), the European Society of Cardiology (ESC 2022), the Center for Disease Control (CDC 2021), the Pan American Health Organization (PAHO/WHO 2019), the International Society for Diseases of the Esophagus (ISDE 2018), the American Heart Association (AHA 2016), and the American Heart Association (AHA/HRS/ACC 2013). 1 2 3 4 5 6 7 8 8 8 8 9 Definition Chagas disease is a tropical parasitic disease caused by the protozoan Trypanosoma cruzi, and associated with cardiomyopathy, arrhythmias, as well as megaviscera. 8 Epidemiology Chagas disease is caused by the protozoan Trypanosoma cruzi. 8 Pathophysiology The total estimated number of people with Chagas disease in the United States is 238,091. 9 Disease course https://web.pathway.md/diseases/rec55LUcYfgxzmpPu 1/7 6/29/23, 3:50 AM Chagas disease Pathway Disease course The transmission of the Trypanosoma cruzi parasite in the humans causes tissue damage and acute inflammatory response; with the persistence of infection, the chronic inflammatory response is elicited resulting in Romana sign, lymphadenopathy, hepatosplenomegaly, myocarditis, pericardial effusion, meningoencephalitis, cardiomyopathy, megaviscera, polyneuropathy, stroke, and death. 8 Prognosis and risk of recurrence The risk of mortality in advanced disease is as high as 85% at 10 years. 8 Guidelines 1. Screening and diagnosis Indications for screening (in pregnancy): Obtain screening for Trypanosoma cruzi infection before or during pregnancy in females at risk for Chagas disease. Recognize that females lived in Mexico, Central America, and South America are at greatest risk for Chagas disease. E Do not obtain routine screening for Trypanosoma cruzi infection in pregnant individuals living in the United States unless there is concern for exposure to triatomines. D Indications for screening (in infants): Obtain screening for congenital Chagas disease in infants if: the mother has been diagnosed with Chagas disease the mother has not been screened but is from an endemic area where she may have acquired the infection the infant has signs suggestive of Chagas disease. E Indications for screening (in family relatives): obtain screening for Trypanosoma cruzi infection in family members of a patient with Chagas disease if they have a similar history of possible exposure to the parasite in endemic settings. E Show 2 more 2. Diagnostic investigations Blood smear: As per CDC 2021 guidelines, obtain parasitologic tests, including identification of trypomastigotes in blood by microscopy, for the diagnosis of acute Trypanosoma cruzi infection. Recognize that circulating parasite levels decrease rapidly within a few months and are undetectable by most methods during the chronic phase. E Obtain direct parasitological tests (microhematocrit and direct observation) and any subsequent serological follow-up (acute congenital infection, starting at 8 months of age; seroconversion for https://web.pathway.md/diseases/rec55LUcYfgxzmpPu 2/7 6/29/23, 3:50 AM Chagas disease Pathway other transmission modes) in patients with suspected acute Trypanosoma cruzi infection. B Serology: As per CDC 2021 guidelines, obtain serologic tests for antibody to the parasite for the diagnosis of chronic Chagas disease. Obtain 2 tests using different techniques (such as ELISA and immunofluorescence assy) and detecting antibodies to different antigens, because a single test is not sufficiently sensitive and specific to make the diagnosis. E Consider obtaining a combination of 2 serological tests with antigens that detect different antibodies against Trypanosoma cruzi (ELISA, HI assay, or IDIF assay) and a third test if there are conflicting results as a diagnostic gold standard for making a definitive diagnosis in patients with suspected chronic Trypanosoma cruzi infection. B Show 2 more Work-up: Include the following in the baseline initial evaluation of patients with newly diagnosed Chagas disease: a complete medical history, including possible exposure to the vector in endemic settings, history of blood transfusions, mother's history, and a review of systems a complete physical examination a resting 12-lead ECG with a 30-second lead II rhythm strip. E Show 2 more 3. Medical management Antitrypanosomal therapy, indications: As per CDC 2021 guidelines, initiate antitrypanosomal therapy in all patients with acute or reactivated Chagas disease and in pediatric patients < 18 years old with chronic Trypanosoma cruzi infection. View the diagnosis of congenital Chagas disease as a form of acute Trypanosoma cruzi infection. E Show 2 more As per PAHO/WHO 2019 guidelines, consider initiating trypanocidal therapy in adult patients with chronic Trypanosoma cruzi infection and no specific organ damage. C Show 3 more Antitrypanosomal therapy, choice of agent: As per CDC 2021 guidelines, administer antitrypanosomal therapy with one of the following regimens: benznidazole 5-8 mg/kg/day PO in 2 divided doses for 60 days in pediatric patients 2-12 years old nifurtimox 10-20 mg/kg/day for patients < 40 kg (at least 2.5 kg), or 8-10 mg/kg/day for patients 40 kg, PO in 3 divided doses for 60 days in pediatric patients from birth to < 18 years old). E Show 3 more https://web.pathway.md/diseases/rec55LUcYfgxzmpPu 3/7 6/29/23, 3:50 AM Chagas disease Pathway As per PAHO/WHO 2019 guidelines, consider administering either benznidazole or nifurtimox for trypanocidal therapy in patients with acute or chronic Chagas disease. C 4. Specific circumstances Pregnant patients: obtain screening for Trypanosoma cruzi infection before or during pregnancy in females at risk for Chagas disease. Recognize that females lived in Mexico, Central America, and South America are at greatest risk for Chagas disease. E Show 2 more Patients with congenital Chagas disease: Obtain screening for congenital Chagas disease in infants if: the mother has been diagnosed with Chagas disease the mother has not been screened but is from an endemic area where she may have acquired the infection the infant has signs suggestive of Chagas disease. E Show 6 more Patients with Chagas cardiomyopathy (evaluation): screen for Chagas disease in patients with dilated cardiomyopathy and epidemiological risk factors for Chagas disease, such as origin from Central or South America or exposure to infected vectors. B Patients with Chagas cardiomyopathy (amiodarone): consider initiating amiodarone to reduce the arrhythmia burden in patients with Chagas cardiomyopathy presenting with symptomatic premature ventricular complexes or VT. C Patients with Chagas cardiomyopathy, ICD placement: As per ESC 2022 guidelines, consider placing an ICD in patients with Chagas cardiomyopathy and symptomatic VT, if antiarrhythmic drugs (amiodarone and -blockers) are ineffective or not tolerated. C As per HRS/AHA/ACC 2013 guidelines, consider placing an ICD in patients with Chagas cardiomyopathy. C Patients with Chagas cardiomyopathy (catheter ablation): consider performing catheter ablation in specialized centers in patients with Chagas cardiomyopathy and recurrent, symptomatic sustained monomorphic VT or ICD shocks for sustained monomorphic VT, if antiarrhythmic drugs are ineffective, contraindicated, or not tolerated. C Patients with Chagas disease esophagopathy: Consider obtaining the same work-up in patients with Chagas disease esophagopathy as used for idiopathic achalasia, due to the similarities in manometric and clinical features. C Consider administering the treatment options employed for idiopathic achalasia for symptom relief in patients with Chagas disease esophagopathy. C Patients with HIV: consider administering a single course of benznidazole or nifurtimox (doses and duration same as for treatment of disease) in patients with human immunodeficiency virus https://web.pathway.md/diseases/rec55LUcYfgxzmpPu 4/7 6/29/23, 3:50 AM Chagas disease Pathway infection with epidemic risk factors for Chagas disease and tested positive for antibodies to Trypanosoma cruzi, not treated previously treated, and not having advanced Chagas cardiomyopathy. C Show 3 more 5. Patient education General counseling: advise patients with Chagas disease to refrain from donating blood. E Clinical findings Symptoms Past medical history Chest pain Dilated cardiomyopathy Cough HF Dizziness Human immunodeficiency virus infection Dysphagia Hypertrophic cardiomyopathy Fever Immunocompromising condition Food regurgitation Myocarditis Hiccups Solid organ transplantation Malaise Stroke Odynophagia Palpitations Vital signs Syncope Weight loss Bradycardia Tachycardia Social history Ocular exam Living in or travel to Latin America Conjunctivitis Eyelid swelling Vascular exam Roma a sign Peripheral edema Cardiac exam Head and neck exam Split S2 heart sound Parotid gland enlargement Abdominal exam Respiratory exam Hepatomegaly Rales Palpable abdominal mass Splenomegaly Lymphatic exam Integument exam Lymphadenopathy Panniculitis https://web.pathway.md/diseases/rec55LUcYfgxzmpPu 5/7 6/29/23, 3:50 AM Chagas disease Pathway Skin nodule Lab findings Serological findings Trypanosoma cruzi deoxyRNA anti-Trypanosoma cruzi antibodies ECG findings AV block Imaging findings Ventricular arrhythmia Cardiomegaly Esophageal dilation MR Megacolon Megaesophagus Pericardial effusion Tricuspid regurgitation LVEF References 1. Centers for Disease Control and Prevention CDC . Parasites American Trypanosomiasis (also known as Chagas Disease). CDC. 2021. Open 2. Zaninotto G, Bennett C, Boeckxstaens G et al. The 2018 ISDE achalasia guidelines. Dis Esophagus. 2018 Sep 1;31 9 . Open 3. Ana Cristina Pereiro. Guidelines for the diagnosis and treatment of Chagas disease. Lancet. 2019 Apr 13;393 10180 1486 1487. Open 4. Biykem Bozkurt, Monica Colvin, Jennifer Cook et al. Current Diagnostic and Treatment Strategies for Specific Dilated Cardiomyopathies: A Scientific Statement From the American Heart Association. Circulation. 2016 Dec 6;134 23):e579-e646. Open 5. Epstein AE, DiMarco JP, Ellenbogen KA et al. 2012 ACCF/AHA/HRS focused update incorporated into the ACCF/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines and the Heart Rhythm Society. J Am Coll Cardiol. 2013 Jan 22;61 3):e6 75. Open 6. Katja Zeppenfeld, Jacob Tfelt-Hansen, Marta de Riva et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Aug 26;ehac262. Open 7. Panel on Opportunistic Infections in Adults and Adolescents with HIV. Guidelines for the Prevention and Treatment of Opportunistic Infections in Adults and Adolescents with HIV. Department of Health and Human Services. 2023. Open 8. Perez-Molina JA, Molina I. Chagas disease. Lancet. 2018 Jan 6;391 10115 82 94. Open 9. Manne-Goehler J, Umeh CA, Montgomery SP et al. Estimating the Burden of Chagas Disease in the United States. PLoS Negl Trop Dis. 2016 Nov 7;10 11):e0005033. Open https://web.pathway.md/diseases/rec55LUcYfgxzmpPu 6/7 6/29/23, 3:50 AM Chagas disease Pathway 10. Harry Acquatella, Federico M Asch, Marcia M Barbosa et al. Recommendations for Multimodality Cardiac Imaging in Patients with Chagas Disease: A Report from the American Society of Echocardiography in Collaboration With the InterAmerican Association of Echocardiography ECOSIAC and the Cardiovascular Imaging Department of the Brazilian Society of Cardiology DIC SBC . J Am Soc Echocardiogr. 2018 Jan;31 1 3 25. Open 11. Jadelson Pinheiro de Andrade, Jose Antonio Marin Neto, Angelo Amato Vincenzo de Paola et al. I Latin American Guidelines for the diagnosis and treatment of Chagas' heart disease: executive summary. Arq Bras Cardiol. 2011 Jun;96 6 434 42. Open https://web.pathway.md/diseases/rec55LUcYfgxzmpPu 7/7