Multimodality Approach Chagas Disease PDF

Exploration of Cardiology Open Access Case Report Enhancing patient care: a multimodality strategy for dilated cardiomyopathy in Chagas disease Luigy Vasquez-Yeng1 , Juan Jose Hernandez-Ruiz2 , Mauricio Garcia-Cardenas1 , Raul Velazquez-Castañeda2 , Santiago Luna-Alcala2 , Pavel Martinez-Dominguez...

Exploration of Cardiology Open Access Case Report Enhancing patient care: a multimodality strategy for dilated cardiomyopathy in Chagas disease Luigy Vasquez-Yeng1 , Juan Jose Hernandez-Ruiz2 , Mauricio Garcia-Cardenas1 , Raul Velazquez-Castañeda2 , Santiago Luna-Alcala2 , Pavel Martinez-Dominguez2 , Enrique C. Guerra2 , José Armendariz-Ferrari1 , Nilda Espinola-Zavaleta2* 1 Department of Cardiology, Hipolito Unanue National Hospital, Lima 15007, Peru 2 Department of Nuclear Cardiology, National Institute of Cardiology Ignacio Chavez, Mexico City 14080, Mexico *Correspondence: Nilda Espinola-Zavaleta, Department of Nuclear Cardiology, National Institute of Cardiology Ignacio Chavez, Juan Badiano No 1, Colonia Seccion XVI Tlalpan, Mexico City 14080, Mexico. [email protected] Academic Editor: Andrea Borghini, Institute of Clinical Physiology - National Research Council (IFC-CNR), Italy Received: March 23, 2024 Accepted: August 5, 2024 Published: September 22, 2024 Cite this article: Vasquez-Yeng L, Hernandez-Ruiz JJ, Garcia-Cardenas M, Velazquez-Castañeda R, Luna-Alcala S, Martinez- Dominguez P, et al. Enhancing patient care: a multimodality strategy for dilated cardiomyopathy in Chagas disease. Explor Cardiol. 2024;2:196–203. https://doi.org/10.37349/ec.2024.00033 Abstract Chagas disease is a systemic illness characterized by acute and chronic phases. If untreated, it can lead to dysfunction of vital organs, notably the heart, ultimately resulting in heart failure. Transmission primarily occurs through the feces of triatomine insects carrying the protozoan parasite Trypanosoma cruzi, either via a bite wound or intact mucous membranes. Diagnosis of Chagas disease involves serological tests, electrocardiographic findings, and imaging studies. A 58-year-old male patient from Peru with chronic dilated cardiomyopathy underwent evaluation at a tertiary care hospital. Given the uncertain etiology, a comprehensive diagnostic approach was adopted, emphasizing the pivotal role of cardiovascular magnetic resonance imaging and computed tomography angiography in managing chronic cardiomyopathy of Chagas disease. Leveraging these imaging modalities together could augment our ability to evaluate myocardial inflammation and tailor therapeutic strategies accordingly. Keywords Cardiac magnetic resonance imaging, Chagas disease, multimodality, cardiomyopathy Introduction Chagas disease (CD) is caused by the protozoan Trypanosoma cruzi and is transmitted by triatomine insects in endemic areas such as Mexico, Central America, and South America. In 2023, Peru was considered a high- prevalence country, with 19 confirmed cases of CD reported [1, 2]. The infection leads to an acute phase lasting 4–8 weeks, with 20–30% of cases progressing to chronic CD, characterized by cardiomyopathy and megaviscera, ultimately leading to heart failure (HF). © The Author(s) 2024. This is an Open Access article licensed under a Creative Commons Attribution 4.0 International License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, sharing, adaptation, distribution and reproduction in any medium or format, for any purpose, even commercially, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. Explor Cardiol. 2024;2:196–203 | https://doi.org/10.37349/ec.2024.00033 Page 196  Recently, cardiovascular magnetic resonance (CMR) has gained significance as a noninvasive tool for identifying myocardial inflammatory activity (edema and myocardial hyperemia) at an early stage. These changes are identifiable before irreversible lesions such as necrosis and fibrosis develop. However, other tools have also emerged, such as computed tomography (CT), for the early recognition of tissue characterization. The combined use of these modalities can further enhance our ability to assess myocardial inflammation and guide therapeutic interventions. Case report A 58-year-old male from San Martín, Peru, with a history of valvular disease diagnosed in 2020 and HF (NYHA functional class II), sought medical care after two years of persistent and worsening respiratory symptoms. He has worked in the agriculture field over the past decades. A transthoracic echocardiogram revealed severe symptomatic mitral valve insufficiency. He was evaluated at a tertiary care hospital due to exacerbated dyspnea (NYHA functional class IV), orthopnea, prominent edema, chest pain, and syncope. Cardiovascular examination revealed jugular distension, hepatojugular reflux, and a holosystolic murmur at the mitral and tricuspid foci. As part of the medical evaluation, an electrocardiogram revealed sinus rhythm with left anterior fascicular block and low QRS voltage (Figure 1); a chest X-ray showed moderate to severe cardiomegaly. Subsequently, transthoracic echocardiography revealed a significant reduction in left ventricular systolic function with a reduced left ventricular ejection fraction (LVEF 24%), global hypokinesia associated with a suggestive image of an interatrial aneurysm, four-chamber dilation, and severe mitral and tricuspid insufficiency (Figure 2). Mortality risk stratification was performed using the Rassi score, which yielded a high-risk score of 17/20, considering the NYHA functional class IV, cardiomegaly, global wall-motion abnormality, male sex, and low QRS voltage. Figure 1. 12-lead electrocardiogram (ECG). The ECG shows sinus rhythm, frontal plane axis left deviation of 60°, in lead avL qR pattern and R-peak time of 60 ms is observed (red square), QRS duration of 80 ms, and generalized low voltage Due to suspicion of chronic ischemia, patient age, and findings of mitral insufficiency on echocardiography, a coronary computed tomography angiography (CTA) was conducted, along with high- sensitivity cardiac troponin T test (hs-cTnT) and C-reactive protein (CRP) levels. The imaging test did not reveal abnormalities, and the hs-cTnT level upon admission was 6 ng/L, with no significant changes during hospitalization, ruling out the probability of obstructive subepicardial coronary artery disease (Figure 3). Additionally, the CRP levels were within the normal range at 0.55 mg/dL. Iodine contrast enhancement confirmed inferolateral and apical aneurysmatic images, revealing transmural enhancement patterns in aneurysmatic segments (Figure 4). Systematically, the most common causes of non-ischemic dilated cardiomyopathy were excluded, including toxic, endocrinological, nutritional, and autoimmune factors. Notably, given the endemic nature of Explor Cardiol. 2024;2:196–203 | https://doi.org/10.37349/ec.2024.00033 Page 197 Figure 2. Four-chamber view transthoracic echocardiography. (A) Global hypokinesia associated with a suggestive image of an interatrial aneurysm (red circle). (B) Color-Doppler echocardiography evidencing four-chamber dilation with severe tricuspid valve insufficiency (red arrow) Figure 3. Coronary computed tomography angiography. (A and B) 3D cardiac reconstruction of the RCA (green arrow) and LCA (red arrow). (C–E) Coronary computed tomography angiography showing adequate blood flow, absence of luminal abnormalities, ruling out coronary disease. RCA: right coronary artery; LCA: left coronary artery; LAD: left anterior descending artery; Cx: circumflex artery Figure 4. Computed tomography showing delayed enhancement with iodine, protocol was implemented with low 80 kV and maximum milliampere at 700 to enhance the iodine. Images were acquired at 7 min post-contrast. (A–C) Short axis view showing inferolateral (white arrows) and apical (red arrows) aneurysmatic segment with transmural enhancement. (D and E) Two-chamber view showing transmural enhancement at the level of the aneurysmatic segments (white arrows) Explor Cardiol. 2024;2:196–203 | https://doi.org/10.37349/ec.2024.00033 Page 198 Figure 5. Short axis view CMR. (A) T1 mapping CMR evidencing shortened global relaxation time (1,100 s) showed by areas with increased signal (green circle), indicative of diffuse myocardial fibrosis. (B) T1 mapping CMR evidencing prolonged global relaxation time (1,091 s) and presence of aneurysmatic segment (white arrow). (C and D) T2 mapping CMR evidencing increase signal areas (red arrows) with increased T2 values corresponding to late gadolinium enhancement demonstrating presence of edema. CMR: cardiovascular magnetic resonance Figure 6. Post-contrast enhanced short T1 mapping cardiovascular magnetic resonance. (A) Four-chamber view providing a comprehensive assessment of the heart chambers. (B–D) Basal third, middle third and apical third of the LV indicating fibrotic changes according to ECV (32%). LV: left ventricle; ECV: extracellular volume CD vectors in Peru, suspicion was raised, prompting the performance of an indirect chemiluminescent immunoassay to detect specific IgG antibodies against excretory-secretory antigens of T. cruzi, as well as an enzyme-linked immunosorbent assay for IgG, both of which returned positive results. CMR was performed to quantify myocardial fibrosis and assess disease severity. T1 mapping revealed prolonged global relaxation times (1,100 ms), indicating diffuse myocardial fibrosis (Figure 5). The examination also revealed a 32% extracellular volume (ECV) fraction in post-contrast-enhanced T1 mapping, suggesting edema (Figure 6). Furthermore, contrast-enhanced gadolinium images confirmed a Explor Cardiol. 2024;2:196–203 | https://doi.org/10.37349/ec.2024.00033 Page 199 transmural pattern of late gadolinium enhancement (LGE), revealing 16 grams of fibrosis mass in aneurysmal segments (Figure 7). Figure 7. CMR with gadolinium. (A) Short axis view CMR with aneurysmal segments in the LV free wall, evidenced with LGE (white arrow). (B) Short axis view CMR evidencing LGE transmural pattern with a fibrosis mass (white arrow) of 16 grams in the apical aneurysmatic segments located in the LV. (C) Vertical long axis view CMR evidencing basal aneurysmatic segment with presence of fibrosis (white arrow). CMR: cardiovascular magnetic resonance; LV: left ventricle; LGE: late gadolinium enhancement A 24-hour Holter study revealed sinus rhythm with a mean heart rate of 65 bpm. Additionally, it showed isolated supraventricular extrasystoles, including bigeminy and trigeminy patterns, without supraventricular tachycardia. Due to the high mortality risk associated with chronic cardiomyopathy of Chagas disease (CCCD), an implantable cardioverter-defibrillator (ICD) was recommended for the prevention of sudden cardiac death (SCD). The patient was discharged without complications following the placement of the ICD. Discussion The diagnosis of CD involves conventional and non-conventional serological tests. According to the World Health Organization, confirmation requires two positive results. Common electrocardiographic abnormalities include complete right bundle branch block and left anterior fascicular block [3, 5]. Echocardiography plays a crucial role in assessing CCCD, enabling the prediction of mortality from early changes to advanced stages with left ventricular enlargement and impaired systolic function. CCCD patients presenting with atypical pain may undergo CTA to rule out obstructive subepicardial coronary artery disease. Additionally, CT can be used for the early characterization of myocardial fibrosis through late enhancement assessment and evaluate biventricular systolic function, complementing echocardiography. Holter monitoring aids in detecting cardiac arrhythmias for routine CCCD assessment, facilitating the diagnosis of conditions such as sinus node dysfunction and supraventricular tachyarrhythmias. Long-term mortality risk stratification using the Rassi score aids in directing therapy based on individual survival probabilities determined by the existence of six clinical features: NYHA class III or IV, cardiomegaly on chest radiography, segmental or global wall-motion abnormalities on echocardiography, non-sustained ventricular tachycardia on Holter monitoring, low QRS voltage on electrocardiography, and male sex. Patients at high risk of mortality may benefit the most from aggressive therapeutic interventions, including ICD, heart transplantation, and cardiac resynchronization. Comprehensive evaluation of CCCD also involves CMR, which correlates myocardial fibrosis with disease severity, ventricular arrhythmias, and cardiovascular events. CMR assesses biventricular systolic function, T2 mapping for edema, and LGE for regional fibrosis detection. Myocardial T1 mapping measures ECV for interstitial fibrosis assessment, and global T1-weighted enhancement aids in detecting hyperemia and inflammation. ECV fraction has a prognostic role in early-stage cardiomyopathy. The late enhancement technique in CMR serves as a crucial predictor for severe cardiovascular events, independently forecasting Explor Cardiol. 2024;2:196–203 | https://doi.org/10.37349/ec.2024.00033 Page 200 Table 1. Role of cardiac imaging for the diagnosis and prognosis of CCCD Cardiac study Initial indications Repeated testing Typical features 12-lead ECG Initial diagnostic and prognostic Perform an ECG Most frequent and defined alterations are assessment of every individual with annually if there are atrioventricular conduction blocks, right positive serology for CD, enabling the multiple specific bundle branch block, left anterior fascicular clinical classification. changes or every two block, ventricular repolarization alterations, years if the ECG is and ventricular ectopic beats. normal or has Association of 2 or more abnormalities in nonspecific (isolated) the same electrocardiographic tracing, changes. characterizes severe cardiomyopathy. Chest X-ray Initial diagnostic and prognostic Perform when there is An enlarged cardiac area with minimally assessment of every individual with clinical evidence of congested pulmonary fields is often CCCD. To diagnose cardiovascular pulmonary or systemic present. In addition, right ventricle impairment. congestion. enlargement signs are common on posteroanterior and lateral views. Signs of right pleural effusion secondary to systemic congestion can also be evidenced. Echocardiography Initial assessment and follow-up of Reassess periodically: Left ventricular enlargement, segmental patients with CD. Assessment of every 3 to 5 years for and/or diffuse hypokinesia, ventricular every individual with CCCD. preserved ejection aneurysms , and left ventricle dysfunction. Suspicion of CCCD arises from fraction cases and no Alteration in myocardial relaxation is the history, clinical examination, or previous segmental first change visible, as it progresses, it can electrocardiographic changes. contractility changes. worsen to a typical restrictive pattern. Reassess every 1 to 2 years for cases with global or segmental left ventricular dysfunction. CMR When there is suspicion of concurrent Not established. Allows for morphological and functional CCCD and coronary artery disease or assessment, globally and segmentally, as other non-ischemic cardiomyopathy, well as the detection of intracardial CMR is utilized to assess the etiology thrombi, overcoming technical limitations and extent of myocardial fibrosis. of echocardiography. In non-endemic areas where initial Identification of myocardial inflammatory clinical suspicion of CD is absent, activity (edema and myocardial hyperemia) CMR can strongly indicate probable at an early stage before the development CCCD diagnosis based on LGE of irreversible lesions, such as necrosis patterns, prompting further and fibrosis. They can aid in risk serological tests for etiological stratification and therapeutic decision- diagnosis confirmation. making. T2-weighted images and T2 mapping should be included for assessment of myocardial edema in addition of LGE to detect gross regional myocardial fibrosis. T1 mapping should be included to calculate the myocardial extracellular volume (interstitial and diffuse fibrosis measure). Computed Non-invasive evaluation of coronary Not established. Characterization of normal myocardial tomography anatomy in patients with CCCD and a tissue/fibrosis using late enhancement as angiography high likelihood of obstructive an alternative to CMR. subepicardial coronary artery Assessment of biventricular systolic disease. function, along with echocardiography, for patients with contraindications to CMR. CCCD: chronic cardiomyopathy of Chagas disease; ECG: electrocardiogram; CD: Chagas disease; CMR: cardiovascular magnetic resonance; LGE: late gadolinium enhancement total death, cardiovascular death, and sustained ventricular tachyarrhythmias. The European Association of Cardiovascular Imaging and the SBC Cardiovascular Imaging Department recommend CMR for quantifying myocardial fibrosis extension, assessing SCD risk, and potentially influencing implantable ICD indications [3, 7]. With a confirmed diagnosis of non-ischemic HF, a LVEF of 24%, and NYHA class IV, the individual met class I Level B evidence criteria for an ICD according to the 2012 American Heart Association guidelines. The ICD effectively addresses potential ventricular arrhythmias, preventing SCD, and offers a promising approach to enhancing overall survival [8, 9]. Explor Cardiol. 2024;2:196–203 | https://doi.org/10.37349/ec.2024.00033 Page 201  The clinical case presented demonstrates how patients can benefit from a multimodality-based strategy in CCCD, aiding in the selection of the most appropriate therapeutic management based on specific findings (Table 1). While iodine late enhancement is an innovative technique that can assist in guiding management, it does not replace gadolinium late enhancement for the determination of fibrosis and disease severity. Abbreviations CCCD: chronic cardiomyopathy of Chagas disease CD: Chagas disease CMR: cardiovascular magnetic resonance ECV: extracellular volume HF: heart failure ICD: implantable cardioverter-defibrillator SCD: sudden cardiac death Declarations Author contributions LVY: Project administration, Resources. JJHR: Writing—original draft, Writing—review & editing, Investigation, Visualization, Supervision. MGC, PMD, ECG, and JAF: Resources. RVC: Investigation, Writing—review & editing, Validation. SLA: Resources, Writing—review & editing. NEZ: Resources, Writing—review & editing, Project administration. All authors read and approved the submitted version. Conflicts of interest The authors declare that they have no conflicts of interest. Ethical approval The research described in this case report has been conducted in accordance with the ethical guidelines established by the World Medical Association’s Declaration of Helsinki for research involving human subjects. Given the nature of this research—specifically, the retrospective analysis of anonymized clinical data—the Ethics Committee of the National Institute of Cardiology Ignacio Chavez has determined that specific ethical approval is not required. Consent to participate Informed consent to participate in the study was obtained from the participant. Consent to publication Informed consent to publication was obtained from relevant participant. Availability of data and materials The data is not publicly available due to ethical restrictions and legal constraints. But the data analyzed during the current case report is available from the corresponding author on reasonable request. Funding Not applicable. Copyright © The Author(s) 2024. 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