Chagas heart disease: Treatment and prognosis
- J Antonio Marin-Neto, MD, PhD, FACC
J Antonio Marin-Neto, MD, PhD, FACC
- Full Professor of Medicine
- Head, Interventional Cardiology Unit, Department of Internal Medicine
- Medical School of Ribeirao Preto
- University of São Paulo, Brazil
- Marcus V Simões, MD
Marcus V Simões, MD
- Associate Professor of Medicine
- Cardiology Division, Department of Internal Medicine
- Medical School of Ribeirao Preto
- University of São Paulo, Brazil
- Andre Schmidt, MD, PhD
Andre Schmidt, MD, PhD
- Associate Professor of Medicine
- Head, Cardiology Division
- Medical School of Ribeirao Preto - University of São Paulo, Brazil
- Anis Rassi, Jr, MD, PhD, FACC, FAHA, FACP
Anis Rassi, Jr, MD, PhD, FACC, FAHA, FACP
- Scientific Director
- Anis Rassi Hospital
- Goiânia, Goiás, Brazil
- Section Editors
- William J McKenna, MD
William J McKenna, MD
- Section Editor — Myopericardial Disease
- Professor of Cardiology
- University College, London
- Peter F Weller, MD, FACP
Peter F Weller, MD, FACP
- Editor-in-Chief — Infectious Diseases
- Section Editor — Tropical Medicine
- William Bosworth Castle Professor of Medicine
- Harvard Medical School
- Professor of Immunology and Infectious Diseases
- Harvard T. H. Chan School of Public Health
- Leonard I Ganz, MD, FHRS, FACC
Leonard I Ganz, MD, FHRS, FACC
- Section Editor — Cardiac Arrhythmias
- Director of Cardiac Electrophysiology
- Heritage Valley Health System
Chagas disease (CD) is caused by Trypanosoma cruzi, a protozoan parasite that can cause acute myopericarditis as well as chronic fibrosing myocarditis. Chagas myocarditis is the most common cause of non-ischemic cardiomyopathy in Latin America .
The treatment and prognosis of Chagas heart disease will be reviewed here. Other issues related to CD, including clinical manifestations and diagnosis of Chagas heart disease and noncardiac manifestations, are discussed separately. (See "Chagas heart disease: Clinical manifestations and diagnosis" and "Chagas disease: Natural history and diagnosis" and "Chagas disease: Pathology and pathogenesis" and "Chagas gastrointestinal disease" and "Chagas disease: Antitrypanosomal drug therapy" and "Chagas disease: Management of acute disease, early chronic disease, and disease in immunosuppressed hosts".)
Overview of phases — The natural history of Chagas disease (CD) has been divided into acute and chronic phases. Cardiac involvement and prognosis in the acute phase of T. cruzi is discussed separately. (See "Chagas heart disease: Clinical manifestations and diagnosis" and "Chagas disease: Natural history and diagnosis".)
In the chronic phase, two well-defined forms of disease are distinguished: indeterminate (latent, pre-clinical) and determinate (clinical), which is subdivided into cardiac, digestive, and cardiodigestive forms. The indeterminate form has been defined as a form of chronic T. cruzi infection in which anti-T. cruzi antibodies are present but no signs or symptoms of Chagas cardiomyopathy or gastrointestinal involvement are identified. Various criteria have been proposed to define the indeterminate form, as discussed separately. (See "Chagas heart disease: Clinical manifestations and diagnosis", section on 'Definition'.)
About one-third to one-half of patients with the indeterminate form develop chronic cardiomyopathy after a latent period ranging from 5 to 30 years. Chronic Chagas cardiomyopathy (CCC) is a cause of heart failure, cardiac arrhythmias and thromboembolism.
- Moncayo A, Silveira AC. Current epidemiological trends for Chagas disease in Latin America and future challenges in epidemiology, surveillance and health policy. Mem Inst Oswaldo Cruz 2009; 104 Suppl 1:17.
- Rassi A Jr, Rassi SG, Rassi A. Sudden death in Chagas' disease. Arq Bras Cardiol 2001; 76:75.
- Freitas HF, Chizzola PR, Paes AT, et al. Risk stratification in a Brazilian hospital-based cohort of 1220 outpatients with heart failure: role of Chagas' heart disease. Int J Cardiol 2005; 102:239.
- Issa VS, Amaral AF, Cruz FD, et al. Beta-blocker therapy and mortality of patients with Chagas cardiomyopathy: a subanalysis of the REMADHE prospective trial. Circ Heart Fail 2010; 3:82.
- Espinosa R, Carrasco HA, Belandria F, et al. Life expectancy analysis in patients with Chagas' disease: prognosis after one decade (1973-1983). Int J Cardiol 1985; 8:45.
- Rassi A Jr, Rassi A, Little WC, et al. Development and validation of a risk score for predicting death in Chagas' heart disease. N Engl J Med 2006; 355:799.
- Rassi A Jr, Rassi A, Rassi SG. Predictors of mortality in chronic Chagas disease: a systematic review of observational studies. Circulation 2007; 115:1101.
- Salles G, Xavier S, Sousa A, et al. Prognostic value of QT interval parameters for mortality risk stratification in Chagas' disease: results of a long-term follow-up study. Circulation 2003; 108:305.
- Mady C, Cardoso RH, Barretto AC, et al. Survival and predictors of survival in patients with congestive heart failure due to Chagas' cardiomyopathy. Circulation 1994; 90:3098.
- Acquatella H, Catalioti F, Gomez-Mancebo JR, et al. Long-term control of Chagas disease in Venezuela: effects on serologic findings, electrocardiographic abnormalities, and clinical outcome. Circulation 1987; 76:556.
- Maguire JH, Hoff R, Sherlock I, et al. Cardiac morbidity and mortality due to Chagas' disease: prospective electrocardiographic study of a Brazilian community. Circulation 1987; 75:1140.
- Ribeiro AL, Marcolino MS, Prineas RJ, Lima-Costa MF. Electrocardiographic abnormalities in elderly Chagas disease patients: 10-year follow-up of the Bambui Cohort Study of Aging. J Am Heart Assoc 2014; 3:e000632.
- Rassi A Jr, Rassi A, Marin-Neto JA. Chagas disease. Lancet 2010; 375:1388.
- Kuschnir E, Sgammini H, Castro R, et al. [Evaluation of cardiac function by radioisotopic angiography, in patients with chronic Chagas cardiopathy]. Arq Bras Cardiol 1985; 45:249.
- Ministério da Saúde. Secretaria de Vigilância em Saúde. [Brazilian Consensus on Chagas disease]. Rev Soc Bras Med Trop 2005; 38 Suppl 3:7.
- Viotti R, Vigliano C, Lococo B, et al. Long-term cardiac outcomes of treating chronic Chagas disease with benznidazole versus no treatment: a nonrandomized trial. Ann Intern Med 2006; 144:724.
- Marin-Neto JA, Rassi A Jr, Morillo CA, et al. Rationale and design of a randomized placebo-controlled trial assessing the effects of etiologic treatment in Chagas' cardiomyopathy: the BENznidazole Evaluation For Interrupting Trypanosomiasis (BENEFIT). Am Heart J 2008; 156:37.
- Morillo CA, Marin-Neto JA, Avezum A, et al. Randomized Trial of Benznidazole for Chronic Chagas' Cardiomyopathy. N Engl J Med 2015; 373:1295.
- Botoni FA, Poole-Wilson PA, Ribeiro AL, et al. A randomized trial of carvedilol after renin-angiotensin system inhibition in chronic Chagas cardiomyopathy. Am Heart J 2007; 153:544.e1.
- Martí-Carvajal AJ, Kwong JS. Pharmacological interventions for treating heart failure in patients with Chagas cardiomyopathy. Cochrane Database Syst Rev 2016; 7:CD009077.
- Bocchi EA, Bellotti G, Mocelin AO, et al. Heart transplantation for chronic Chagas' heart disease. Ann Thorac Surg 1996; 61:1727.
- Bocchi EA, Fiorelli A. The paradox of survival results after heart transplantation for cardiomyopathy caused by Trypanosoma cruzi. First Guidelines Group for Heart Transplantation of the Brazilian Society of Cardiology. Ann Thorac Surg 2001; 71:1833.
- Moreira LF, Stolf NA, Braile DM, Jatene AD. Dynamic cardiomyoplasty in South America. Ann Thorac Surg 1996; 61:408.
- Ribeiro Dos Santos R, Rassi S, Feitosa G, et al. Cell therapy in Chagas cardiomyopathy (Chagas arm of the multicenter randomized trial of cell therapy in cardiopathies study): a multicenter randomized trial. Circulation 2012; 125:2454.
- Cardinalli-Neto A, Bestetti RB, Cordeiro JA, Rodrigues VC. Predictors of all-cause mortality for patients with chronic Chagas' heart disease receiving implantable cardioverter defibrillator therapy. J Cardiovasc Electrophysiol 2007; 18:1236.
- Rassi A Jr. Implantable cardioverter-defibrillators in patients with Chagas heart disease: misperceptions, many questions and the urgent need for a randomized clinical trial. J Cardiovasc Electrophysiol 2007; 18:1241.
- di Toro D, Muratore C, Aguinaga L, et al. Predictors of all-cause 1-year mortality in implantable cardioverter defibrillator patients with chronic Chagas' cardiomyopathy. Pacing Clin Electrophysiol 2011; 34:1063.
- Martinelli M, de Siqueira SF, Sternick EB, et al. Long-term follow-up of implantable cardioverter-defibrillator for secondary prevention in chagas' heart disease. Am J Cardiol 2012; 110:1040.
- Barbosa MP, da Costa Rocha MO, de Oliveira AB, et al. Efficacy and safety of implantable cardioverter-defibrillators in patients with Chagas disease. Europace 2013; 15:957.
- Gali WL, Sarabanda AV, Baggio JM, et al. Implantable cardioverter-defibrillators for treatment of sustained ventricular arrhythmias in patients with Chagas' heart disease: comparison with a control group treated with amiodarone alone. Europace 2014; 16:674.
- Martinelli M, Rassi A Jr, Marin-Neto JA, et al. CHronic use of Amiodarone aGAinSt Implantable cardioverter-defibrillator therapy for primary prevention of death in patients with Chagas cardiomyopathy Study: rationale and design of a randomized clinical trial. Am Heart J 2013; 166:976.
- Haedo AH, Chiale PA, Bandieri JD, et al. Comparative antiarrhythmic efficacy of verapamil, 17-monochloracetylajmaline, mexiletine and amiodarone in patients with severe chagasic myocarditis: relation with the underlying arrhythmogenic mechanisms. J Am Coll Cardiol 1986; 7:1114.
- Rosenbaum M, Posse R, Sgammini H, et al. [Comparative multicenter clinical study of flecainide and amiodarone in the treatment of ventricular arrhythmias associated with chronic Chagas cardiopathy]. Arch Inst Cardiol Mex 1987; 57:325.
- Marin-Neto JA, Rassi JR A, Maciel BC, et al. Chagas heart disease. In: Evidence-Based Cardiology, 3rd ed, Yusuf S, Cairns J, Camm J, et al. (Eds), 2010. p.823.
- Benaim G, Paniz Mondolfi AE. The emerging role of amiodarone and dronedarone in Chagas disease. Nat Rev Cardiol 2012; 9:605.
- de Paola AA, Melo WD, Távora MZ, Martinez EE. Angiographic and electrophysiological substrates for ventricular tachycardia mapping through the coronary veins. Heart 1998; 79:59.
- Sosa E, Scanavacca M, D'Avila A, et al. Endocardial and epicardial ablation guided by nonsurgical transthoracic epicardial mapping to treat recurrent ventricular tachycardia. J Cardiovasc Electrophysiol 1998; 9:229.
- de Paola AA, Balbão CE, Castiglioni ML, et al. [Radioisotopic mapping of the arrhythmogenic focus in patients with chronic chagasic cardiomyopathy and sustained ventricular tachycardia]. Arq Bras Cardiol 1993; 60:373.
- Milei J, Pesce R, Valero E, et al. Electrophysiologic-structural correlations in chagasic aneurysms causing malignant arrhythmias. Int J Cardiol 1991; 32:65.
- Castagnino HE, Cicco JA, Coniglio J, et al. [Chagasic ventricular aneurysm with ventricular tachycardia operated on with good results]. Medicina (B Aires) 1975; 35:166.
- Iturralde P, Barragán R, Araya V, et al. [Surgical resection of a focus of ventricular tachycardia guided by endocardial and epicardial mapping]. Arch Inst Cardiol Mex 1992; 62:69.
- Andrade JP, Marin Neto JA, Paola AA, et al. I Latin American Guidelines for the diagnosis and treatment of Chagas' heart disease: executive summary. Arq Bras Cardiol 2011; 96:434.
- Epstein AE, DiMarco JP, Ellenbogen KA, et al. ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices): developed in collaboration with the American Association for Thoracic Surgery and Society of Thoracic Surgeons. Circulation 2008; 117:e350.
- Zipes DP, Camm AJ, Borggrefe M, et al. ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (writing committee to develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death): developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society. Circulation 2006; 114:e385.
- Sousa AS, Xavier SS, Freitas GR, Hasslocher-Moreno A. Prevention strategies of cardioembolic ischemic stroke in Chagas' disease. Arq Bras Cardiol 2008; 91:306.
- Overview of phases
- Prognosis of CCC
- - Rassi score for predicting mortality
- Prognostic value of ECG
- Acute and indeterminate disease
- Chronic disease
- - T. cruzi infection
- - Heart failure
- Medical therapy
- Other HF therapies
- - Bradyarrhythmias and heart block
- - Ventricular arrhythmia and SCA
- Our approach
- CCC-specific evidence
- - ICD therapy
- - Antiarrhythmic therapy
- - Ablation and surgery
- Recommendations of others
- - Thromboembolism
- Chagas cardioembolism risk score
- Antithrombotic prophylaxis
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS