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7.1.4.3 Chagas’ cardiomyopathy

Chagas’ disease, a myocardial disease caused by the parasite Trypanosoma cruzi, is the most common cause of non-ischaemic cardiomyopathy in Latin America. After the incubation period, the vast majority of infected persons have minor or no symptoms, and very few (,1%) develop acute myocarditis.845 However, people are infected for life, and years to decades after the initial infection 20– 30% of them will develop cardiomyopathy,846 which can lead to heart failure, heart block and atrial and ventricular arrhythmias. SCD, particularly due to VF, is the most common cause of death in patients with Chagas’ cardiomyopathy.847,848 A risk score developed by Rassi et al.849 as well as the presence of myocardial fibrosis at LGE-CMR850 are useful in assessing the risk of death in Chagas’ disease patients. However, whether such risk stratification can translate into clinical benefit through ICD treatment needs to be assessed in prospective trials, also considering the high annual mortality in Chagas patients with ICDs.851 Although ICD implantation may seem reasonable for the secondary prevention of SCD in patients with Chagas’ cardiomyopathy, there are controversial studies851–856 about the benefits and risks

N

Patients with ca r diac sa r coidosis

Amioda r one (Class IIa)

Catheter ablation

(Class IIb)

Indication f or permanent

f f ca r diac pacing

N

Significant LGE a

N

N

N L VEF 35-50%

L L and minor LGE

PES (Class IIa)

ICD implantation

(Class IIa)

F oll o w-up Abo r ted ca r diac a r r est or

sustained VT or L VEF

L L ≤ 35%

SMVT inducible

Symptomatic r ecu r r ent V A

V V

Y

Y ICD implantation

(Class I)

Y

Y

Y

Figure 24 Algorithm for sudden cardiac death prevention and treatment of ventricular arrhythmia in patients with cardiac sarcoidosis. ICD, implantable cardioverter defibrillator; LGE, late gadolinium enhancement; LVEF, left ventricular ejection fraction; N, No; PES, programmed electrical stimulation; SMVT, sustained monomorphic ventricular tachycardia; VA, ventricular arrhythmia; VT, ventricular tachycardia; Y, Yes. aLGE affecting ≥9/22 segments or ≥22% of the LV mass has been associated with arrhythmic endpoints.

Secondary prevention of SCD and treatment of VAs ICD implantation is recommended in patients with cardiac sarcoidosis who (1) have documented sustained VT, or (2) aborted CA.812,828–830,832 I B In patients with cardiac sarcoidosis and recurrent, symptomatic VA, AAD treatment should be considered. IIa C Catheter ablation, in specialized centres, may be considered in cardiac sarcoidosis ICD-recipients with recurrent, symptomatic SMVT or ICD shocks for SMVT, in whom AADs are ineffective, contraindicated, or not tolerated.839,841,842 IIb C © ESC 2022

AAD, anti-arrhythmic drug; AV, atrio-ventricular; CA, cardiac arrest; CMR, cardiac magnetic resonance; ICD, implantable cardioverter defibrillator; LGE, late gadolinium enhancement; LVEF, left ventricular ejection fraction; PES, programmed electrical stimulation; SCD, sudden cardiac death; SMVT, sustained monomorphic VT; AV, ventricular arrhythmia; VT, ventricular tachycardia. aClass of recommendation. bLevel of evidence. Downloaded from academic.oup.com/eurheartj/article/43/40/3997/6675633 by guest on 07 October 2026