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Article title

Implantable cardioverter-defibrillator prevents sudden death in autochthonous patient

with Chagas cardiomyopathy in the Brazilian Amazon

Katia do Nascimento Couceiro [1],[2], Jessica Vanina Ortiz [2],[3], Monica Regina Hosannah da

Silva e Silva[1][4], Debora Raysa Teixeira de Sousa[1],[2], Susan Smith Doria[1],[2], Karina

Lopez Rodriguez[1],[2], José Alejandro Lazo Diéguez[2], Paula Rita Leite da Silva [1],[2], Maria

das Graças Vale Barbosa Guerra[1],[2], João Marcos Bemfica Barbosa Ferreira*[1],[2],[5], Jorge

Augusto de Oliveira Guerra[1],[2]

[1] Programa de Pós-Graduação em Medicina Tropical, Escola de Ciências da Saúde,

Universidade do Estado do Amazonas, Manaus, AM. [2]. Fundação de Medicina Tropical Dr.
Heitor Vieira Dourado, Manaus, AM. [3]. Universidade Federal do Amazonas, Manaus, AM. [4].
Fundação Hospitalar de Hematologia e Hemoterapia do Amazonas, Manaus, AM. [5]. Hospital
Francisca Mendes, Manaus, AM.

-----------------------------
Corresponding author: Dr. João Marcos Bemfica Barbosa Ferreira. Hospital Universitário
Francisca Mendes, UFAM. Av. Camapuã, Manaus, AM, Brazil.

E-mail: jmbemfica@hotmail.com

The authors declare that there are no relevant conflicts of interest.

"This author takes responsibility for all aspects of the reliability and freedom from bias of the
data presented and their discussed interpretation".
Abstract

Chagas disease (CD) has become a worldwide health problem with approximately ten thousand
deaths a year. About 35% of the cases may evolve to cardiac manifestations such as arrhythmias
and/or conduction disorders, heart failure, thromboembolic accidents and sudden death. The
Amazon region has long been considered a non-endemic area for CD, however, in the last
decades, with the increase in the number of acute and chronic cases. Here we present a report of
the successful implementation a cardioverter-defibrillator in the prevention of sudden death in a
patient with autochthonous Chagas cardiomyopathy in the Brazilian Amazon.

Keywords: Chagas cardiomyopathy; Implantable cardioverter-defibrillator; Amazonia.

Introduction

Chagas disease (CD), caused by Trypanosoma cruzi, a hemoflagellate parasite with heteroxene
cycle (1), is endemic in Latin America and has become a worldwide public health problem due
to the increase in human migrations. Considered a neglected disease in 2005, 110 years after its
discovery (2). The World Health Organization estimates that 70 million people are at risk of
acquiring the disease, 7 million people are infected and that it causes approximately 10,000
deaths a year (3.4).

This disease can manifest itself in two stages, an acute one – in asymptomatic or symptomatic
forms; and another chronic one - in indeterminate, cardiac or digestive forms. Approximately
60% of infected individuals will remain in indeterminate form; 25% to 35% will develop heart
disease, and of these, 10% may develop severe heart disease. In endemic regions (5), it is an
important cause of sudden death. Chagas cardiomyopathy (CCM) manifests itself in the form of
arrhythmias and/or conduction disorders, heart failure, thromboembolic accidents and sudden
death (6). Chronic inflammation, usually caused by persistence of the parasite, can result in
fibrosis and consequent sinus node dysfunction, atrioventricular and intraventricular conduction
abnormalities, and ventricular tachyarrhythmias (7).

The Amazon region has long been considered a non-endemic area for CD, however, in the last
decades, with the increase in the number of acute and chronic cases, this profile has changed. In
the state of Amazonas, the first record of serologically positive chronic patients occurred in 1977
(8). Since then, surveillance programs and serological surveys have been carried out and new
cases have been detected (9-11). Previous studies suggest lower morbidity levels as a result of
CD in the Amazon region, probably due to the presence of T. cruzi strains which are different
from those found in traditionally endemic areas (9, 11).

Despite this, few cases of CCM in the Amazon have been reported in scientific literature. The
first reports of dilated cardiomyopathy of chagasic etiology in this region date from 2003, with
two fatal cases (12). In 2006, three new cases appeared (13), and in 2009, three more
autochthonous cases were diagnosed (14). The presence of apical aneurysm, as well as the
occurrence of stroke, have also been reported in the state of Amazonas (15).

In this study, we present a successful case report of cardioverter-defibrillator implantation for the
prevention of sudden death in a patient with autochthonous Chagas cardiomyopathy in the
Brazilian Amazon.

Case Report

L.C.E., 60 years old, male, single, painter, born in Autazes, in the interior of Amazonas, where
he lived until the age of 18 and currently lives in the state capital, Manaus. The patient was
diagnosed with Chagas disease in 2015, after an attempt at blood donation. The reagent
serological methods for anti-Trypanosoma cruzi antibodies were ELISA and indirect
hemagglutination. He evolved to the cardiac form of the disease, and presented dilated
cardiomyopathy with ventricular dysfunction - left ventricular ejection fraction (LVEF) of 44%
using the Simpson method, with apical aneurysm, baseline ECG with sinus rhythm and changes
in ventricular repolarization in the lower-lateral wall. On the 24-hour Holter, he already
presented numerous isolated, bigeminated and polymorphic ventricular arrhythmias.

In 2018, cardiac magnetic resonance imaging was performed, which depicted increased cavity
volumes and significant left ventricular dysfunction, in addition to the presence of late
transmural enhancement in the inferior-medial-basal wall (Figure 1). A further echocardiogram
was performed which showed a global longitudinal strain of -10.5%, and LVEF already quite
reduced, 29% (Figure 2), positive high-resolution electrocardiogram for the presence of late
potentials and when submitted to the Selvester score, 12% of myocardial fibrosis was detected.

In August of 2019, he went to the emergency department with tachycardic palpitations, cold
sweats and syncope. In the emergency room, a monomorphic ventricular tachycardia without
pulse was monitored and recorded on the 12-lead electrocardiogram (ECG) (Figure 3), and
showed cardiopulmonary arrest (CA). He was immediately submitted to cardiac resuscitation
maneuvers and reverted to tachyarrhythmia after a 200 J biphasic shock, for sinus rhythm, with
isolated ventricular tachycardia (Figure 4).

Since the diagnosis of CD and CCM, he has been using medications optimized for heart failure
(HF): enalapril 20mg/day, carvedilol 50mg/day, spironolactone 25mg/day. During the
cardiorespiratory arrest episode with pulseless monomorphic ventricular tachycardia and after its
reversal with defibrillation, the patient was referred for implantation of an implantable
cardioverter-defibrillator (ICD) as a secondary prophylaxis for sudden death by the
arrhythmogenic heart disease as a result of CD.

After hospital discharge, he was referred for outpatient follow-up, and instructed to take his
medications for HF in combination with amiodarone in order to avoid arrhythmic storms. A new
24-hour Holter was performed, which demonstrated the absence of ventricular arrhythmias.
Approximately three months after the ICD implantation, the patient presented with an ICD shock
episode, documented by the intracavitary electrogram of the electronic device, during the
telemetry evaluation (Figure 5).

Discussion

In this study, we present a case of ICD implantation after cardiopulmonary arrest in a patient
with autochthonous Chagas cardiomyopathy in the Amazon, who has been undergoing outpatient
follow-up at the FMT-HVD for four years.

There are few published records of chagasic cardiomyopathy to date in the Amazon regian
(12-14). These few reports describe i) the evolution of an acute case of CMM over a five-
year follow-up period (16), ii) ventricular aneurysms (15) and iii) ventricular
tachyarrthythmias (17). Patients with acquired arrhythmogenic cardiac form in the
Amazon evolves as severely as those in regions traditionally endemic for Chagas disease.

In 2012, Marques et al. (17) reported the first known case of ventricular tachycardia in an
autochthonous patient in the Amazon. This alert for the importance of continuous
monitoring of patients with CD in the region, since the earlier the predictors of
arrhythmias are identified, the lower is the severity and permanent damage, better the
prognosis, the lower the cost for the public health system and better it is the patient's social
and economic quality of life.

It is important to note that myocardial fibrosis identified by cardiac magnetic resonance, with
late myocardial enhancement, serves as an important predictor of arrhythmias and sudden death
in several ischemic and non-ischemic cardiomyopathies, such as hypertrophic cardiomyopathy
and Chagas disease. and should be considered for patient monitoring and assessment of severity
and interventions (18).

In the Amazon region, diagnostic resources are scarce and the occurrence of acute and chronic
cases of CD have increased significantly in recent decades. Therefore, the case description of
chronic chagasic cardiomyopathy evolving to sudden death and the need for an ICD reinforces
the need to intensify disease prevention measures and improve the diagnostic and therapeutic
approach in regards to the most severe cases.
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Figures

Figure 1 – Cardiac magnetic resonance imaging with the presence of late transmural
enhancement in the lower-middle-basal wall

Figure 2 – Transthoracic echocardiogram with global longitudinal strain (-10.5%).

Figure 3 – Baseline ECG with monomorphic ventricular tachycardia without pulse.

Figure 4 – ECG after reversal showing the presence of isolated ventricular tachycardia.
Figure 5 – (A) Sustained ventricular tachycardia detected by the intracavitary electrogram.
(B) Intracavitary electrogram stimulating atrial-ventricle after ICD shock.