2025;38(1) – ABC CARDIOL

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Original Article

Mitral Annulus Diameter's Relevance in the Diagnosis of Atrial Etiology in Mitral Regurgitation: A Comparative Analysis

Alexandre Costa Souza et al. Bruna de Mattos Ivo Junqueira Stephanie de Azevedo Drubi Laila Caroline Gomes … Marcus Vinicius Freire Priscila Pinheiro Alessandra Ghattas Basile Marco André Moraes Sales Ricardo André Sales Pereira Guedes Carolina Thé Macêdo

Abstract

Background:

Atrial Functional Mitral Regurgitation (FMR) is defined by mitral annular dilation and functional alterations without structural impairment of the leaflets or subvalvular apparatus. It is associated with higher cardiovascular mortality, being often characterized as an exclusion diagnosis due to the absence of more precise diagnostic criteria. Therefore, it is crucial to identify markers that distinguish it from other Mitral Regurgitation (MR) types.

Objective:

To assess the accuracy of the intercommissural diameter of the mitral annulus and its value indexed to body surface area in differentiating atrial etiology in patients with MR.

Methods:

This is an observational cross-sectional study with 109 patients diagnosed with moderate or severe MR. Data were obtained between October 2022 and January 2024, from transesophageal echocardiograms performed at a referral hospital in the city of Salvador-BA.

Results:

The mean age was 69 ± 15, with 67 males and 28 cases of atrial etiology. The comparison between patients with MR of Atrial etiology versus Non-atrial etiology revealed significantly increased diameters of the intercommissural mitral annulus and its indexed value, with considerably larger diameters in the Atrial group (p = 0.009; 95% CI: 0.501 to 3.507). The receiver operating characteristic (ROC) curve analysis identified an optimal cut-off value of 20.8750, in which the sensitivity and specificity were 67.9%.

Conclusion:

Indexing the mitral annular diameter to the body surface area improves diagnostic accuracy in identifying the atrial etiology of MR compared to diameter alone, supporting its potential role in more comprehensive diagnostic algorithms.

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Case Report

Late Evolution of Takotsubo Cardiomyopathy Following Transcatheter Aortic Valve Implantation

Merve Akbulut Çakır et al. Emirhan Çakır Çağlar Kaya Kenan Yalta

Introduction

Transcatheter aortic valve implantation (TAVI) is recognized as a first-line treatment for symptomatic patients with advanced aortic stenosis who are at high surgical risk. In inoperable patients with poor prognosis, it carries a risk for complications, such as myocardial infarction due to coronary occlusion, stroke, annular rupture, etc.¹ Takotsubo Cardiomyopathy (TTC) is a syndrome that usually develops in the absence of overt coronary occlusion and signs of alternative diagnosis, including myocarditis. It presents with electrocardiographic changes, increased cardiac biomarkers, and typical echocardiographic findings.^, In this article, we describe a patient with TTC who previously underwent TAVI and subsequent implantable cardiac defibrillator (ICD) implantation.

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Review Article

Permissive Cardiotoxicity: When the Optimal Is the Enemy of the Good

Carolina Maria Pinto Domingues Carvalho Silva et al. Juliane Dantas Seabra-Garcez Ana Palmira Lima Neves Ariane Vieira Scarlatelli Macedo

Abstract

Cancer and cardiovascular diseases are the leading causes of mortality worldwide. As these diseases share common metabolic pathways, patients with cancer are currently seen as having subclinical alterations with great potential for developing cardiovascular diseases (and vice versa). This tenuous interrelationship has led to the emergence of cardio-oncology (CO) as an area of study and discussion, with a major impact on the management of these cases.

In clinical practice, CO is dominated by the dilemma of how to best manage cardiovascular toxicity during cancer treatment. The management of these cases focuses not only on restoring cardiovascular balance, but also on ensuring that the best oncological treatment will be offered, without suspensions or interruptions.

Within this context, the concept of permissive cardiotoxicity (PC) has emerged, proposing the idea of maintaining antitumor treatment in the event that ventricular dysfunction is detected, with no pauses or interruptions, provided that certain premises are respected.

This review aims to examine the concept of PC in depth, contextualizing its emergence, reviewing definitions, and analyzing the evidence from experimental clinical studies. Furthermore, management strategies are detailed, discussing the central role of cardiovascular imaging in guiding this management. Finally, limitations and gaps in the literature in the area are discussed.

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Original Article

Misinterpretation of Indeterminate Diastolic Function: Awareness and Insights from a Survey among Brazilian Cardiologists

Daniela do Carmo Rassi et al. Salvador Rassi Jorge Eduardo Assef Alex dos Santos Felix … Adenalva Lima de Souza Beck Cecília Beatriz Bittencourt Viana Cruz Silvio Henrique Barberato

Abstract

Background:

Diastolic dysfunction (DD) is highly prevalent and is associated with significant morbidity and mortality. More recent guidelines have introduced the classification of Indeterminate Diastolic Function or Indeterminate Diastolic Dysfunction (IDF or IDD). Nevertheless, both diagnoses are still poorly understood in clinical practice, with variable prevalence.

Objectives:

Assess the understanding of IDF and IDD among non-echocardiographer cardiologists in Brazil.

Methods:

A nationwide online survey was conducted among non-echocardiography readers cardiologists via mobile messaging platforms. A brief and anonymous questionnaire on knowledge and interpretation of Diastolic Function.

Results:

A total of 570 cardiologists from all regions of Brazil participated in the study. Most participants (64.21%) had more than ten years of clinical experience. While 71% correctly identified grades 2 or 3 as indicative of elevated filling pressures, only 34.21% accurately understood the diagnostic criteria for IDF or IDD. Among the mistakes associated with the indeterminate classification, the following stand out: attribution of this status to conditions that interfere with the assessment of diastole (49.12%), technical limitations of the echocardiography equipment (3.33%), and alleged lack of knowledge by echocardiographers (4.91%). In addition, 46.7% reported finding this diagnosis rarely in the reports, 33.5% believed that this classification influenced clinical conduct, and 43.5% considered that the examination could have been better performed.

Conclusion:

Despite knowledge about diastolic function, misinterpretation of IDF or IDD is still common among non-echocardiographer cardiologists. Investment in education, sharing these concepts with clinicians, and clear guidelines are essential to optimize the use and diagnostic accuracy of echocardiography.

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Case Report

Successful Occlusion of a Giant Subclavian Artery Aneurysm Sac with a Vascular Plug: A Case Report

Fuat Polat et al. Osman Eren Karpuzoğlu

Abstract

Subclavian artery aneurysms are rare vascular anomalies that carry significant risks, including thromboembolism, ischemia, and rupture. This case report describes a 71-year-old female patient presenting with left arm ischemia, including pain, numbness, and weakness, caused by a left subclavian artery aneurysm complicated by distal thrombosis. Imaging revealed a 65 × 55 mm aneurysm with a 2.5 cm thrombotic occlusion. The aneurysm was treated with an endovascular approach, utilizing a vascular plug deployed via femoral access. This was preceded by failed attempts to access the lesion through the radial approach due to the presence of thrombotic occlusion and complex vascular anatomy. After the procedure, the patient experienced significant symptom relief, and follow-up imaging confirmed thrombosis of the aneurysm sac. This case highlights the effectiveness of endovascular techniques in managing complex subclavian artery aneurysms and emphasizes the importance of individualized treatment strategies and close follow-up.

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Editorial

The Role of Objective Assessment of the Mitral Annulus

Minna Moreira Dias Romano

The mitral annulus is a complex and dynamic structure responsive to both structural and functional changes involving the mitral valve, as well as those affecting the geometry of the left ventricle (LV) and left atrium (LA). Its bean-shaped or “D”-shaped three-dimensional structure is similar to a horse’s saddle, in which the nadir (height) varies throughout the cardiac cycle. The malleability of the mitral annulus is important for valve competence. The posterior portion of the annulus, which is less fibrotic, moves during left ventricular systole, increasing the height of the saddle and reducing its circumferential area. In situations of left atrial dilation and dysfunction, mainly accompanied by atrial fibrillation (AF) and commonly associated with various causes of LV diastolic dysfunction, mitral annulus’ dynamics are often compromised. This results in reduced mobility of the posterior leaflet, annulus stiffness, and diminished oscillation of the saddle height, in addition to valve retraction (tethering), which contribute to mitral coaptation failure.

The mitral annulus can be studied using echocardiography, both two-dimensional and three-dimensional, with transesophageal imaging being the preferred approach. Three-dimensional imaging allows for various quantitative measurements facilitated by computational reconstruction software. These measurements can also be dynamic, capturing structural modifications throughout the cardiac cycle in both systole and diastole (). Key measurements include annular circumference and area, anteroposterior and inter-commissural diameters, and saddle height. The three-dimensional approach offers significant advantages, particularly when segmentation of valve anatomy is required, such as in cases of mitral valve prolapse that necessitate detailed anatomical analysis of valve bulges. Different etiologies of mitral regurgitation (MR) are accompanied by different degrees of mitral annulus deformation, with the most subtle ones observed in ischemic MR and the most significant in the most advanced spectra of mitral degenerative diseases, such as Barlow’s disease. For example, in rheumatic disease—still largely common in Brazil despite its low prevalence in more developed countries—mitral annulus changes are characterized by a “flattening of the saddle shape” and an increased area without a significant change in the perimeter.

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Original Article

First Percutaneous Orthotopic Tricuspid Valve Implantations using the LuX-Valve Plus Device in Latin America: Report of the First Three Cases

Fernanda Esteves et al. Vinícius Esteves Marcio Mendes Pereira Frederico José Neves Mancuso … Marden Tebet Sergio Kreimer Edmur Carlos de Araújo Francisco Monteiro de Almeida Magalhães

Abstract

Background:

Severe tricuspid regurgitation (TR) is associated with high morbidity and mortality, especially in patients with contraindications for surgery. Transcatheter tricuspid valve replacement has emerged as a minimally invasive alternative for this population, with the LuX-Valve Plus device designed for orthotopic implantation via the transjugular route.

Objective:

To report the first 3 cases of transcatheter tricuspid valve replacement using the LuX-Valve Plus device in Latin America, assessing the feasibility, safety, and immediate clinical outcomes.

Methods:

The study included 3 female patients with torrential TR and high surgical risk. Pre-procedure assessment included transesophageal echocardiography, 3-dimensional transthoracic echocardiography (3D TEE), computed tomography, and cardiac catheterization. The procedure was performed via transjugular access, under general anesthesia, with 3D TEE and fluoroscopy guidance. Implant efficacy was assessed as resolution of TR, transprosthetic gradient, and post-procedure complications.

Results:

The implants were successful in all cases, with complete resolution of TR, absence of paravalvular leaks, and adequate transprosthetic gradient. Right ventricular function was preserved, and all patients were discharged 3 to 4 days after the procedure with no significant complications.

Conclusion:

Transcatheter tricuspid valve replacement using the LuX-Valve Plus was shown to be a viable and safe alternative for patients with severe TR and high surgical risk. The use of 3D TEE was essential to the planning and execution of the implant. Further studies are needed to assess durability and long-term clinical outcomes.

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Case Report

Asymptomatic Non-Bacterial Thrombotic Endocarditis Leading to a Diagnosis of Pancreatic Cancer

Diego Chemello et al. Patricia Chagas Daniel Marques Barreiro Camila Sales Fagundes … Rafael Coelho Marcelo Dantas Tavares de Melo

Introduction

Non-bacterial thrombotic endocarditis (NBTE) is a phenomenon associated with sterile vegetations composed of fibrin and platelets aggregating on healthy heart valves. Most cases are diagnosed after death or in patients experiencing systemic embolization. Diagnosis relies on clinical suspicion and a compatible echocardiographic image. The correct treatment is long-term systemic anticoagulation to avoid arterial embolization, which often presents as multiple and recurrent ischemic strokes.

The first antemortem report of NBTE as the initial presentation of underlying pancreatic cancer was published in 2008. However, incidental diagnosis of uncomplicated NBTE that leads to a subsequent discovery of carcinoma is rare.

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Review Article

Pericardial Agenesis: When Should This Diagnosis Be Considered?

Bruna Barbosa Chagas de Almeida et al. Bruna Zanforlin Jácome Bernardo Nascimento Lourenço Alessandro Zakhia de Seixas … Juliana Serafim da Silveira Eliza de Almeida Gripp

Abstract

Pericardial agenesis (PA) is a rare condition characterized by the partial or complete absence of the pericardium. The consequences associated with this condition can be quite serious. Therefore, understanding the peculiarities of this disease is essential for accurate diagnosis and treatment.

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Case Report

ATTRwt Cardiac Amyloidosis and Aortic Regurgitation in a Patient with Acute Myelomonocytic Leukemia: an Unusual Combination

Diego Chemello et al. Patricia Chagas Camila Sales Fagundes Juliana Chiodelli Salvador Marcelo Tavares

Introduction

Systemic amyloidosis is a relatively rare multisystem disease caused by the deposition of misfolded protein in various tissues and organs. Cardiac amyloidosis (CA) is recognized as an underlying cause of left ventricular wall thickening, heart failure, and arrhythmia with variable clinical presentation. The clinical diagnosis of cardiac involvement in amyloidosis is challenging, requiring a patient-centered diagnostic work-up that ensures an appropriate diagnosis.^,

Although amyloidosis has been associated with multiple malignant disorders, its association with leukemia is rather uncommon.^– Also, there is a relationship between CA and valvular heart disease, particularly aortic stenosis (AS). The association of ATTRwt CA and aortic regurgitation (AR) in a patient with acute myelomonocytic leukemia (MML) is unique and has not been described so far.

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Review Article

My Approach to HFpEF Considering Ventricular-Arterial Coupling and Ventricular Interdependence

João Carlos Moron Saes Braga et al. Jorge Eduardo Assef Fabio Villaça Guimarães Filho Raphael Aparecido Barreto da Silva

Abstract

Heart failure (HF) is a serious public health issue that impacts the mortality and morbidity of the global population. The prevalence of heart failure with preserved ejection fraction (HFpEF) is clearly increasing, associated with population aging, obesity, sedentary lifestyle, and cardiometabolic diseases. It is one of the most urgent diagnostic and therapeutic challenges nowadays. Additionally, the obesity phenotype of HFpEF may be associated with HF and occurs as a result of several mechanisms that are deleterious to cardiac function, including the direct action of epicardial adipose tissue (EAT), causing direct restraint of the pericardium and ventricular interdependence, with significant hemodynamic repercussions, decline in functional capacity, and a worse prognosis. The estimation of ventricular-arterial coupling (VAC) in a non-invasive manner by echocardiography allows a better understanding of the interaction between the heart and the arterial system by monitoring hemodynamic changes, which can guide medical therapy and have an impact on prognosis. Although these tools and parameters are promising, new technologies, such as artificial intelligence and machine learning, must be used to enable the applicability of VAC in clinical practice. Further studies are key to standardizing new methods and values in this context.

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Case Report

A Common Primary Cardiac Tumor With an Uncommon Presentation

Juan Felipe Vasquez-Rodriguez et al. Carolina Paola Idrovo-Turbay Jaime Alberto Rodríguez Lorena Montes Villalobos

Abstract

Background

Cardiac tumors are rare, and most are secondary (metastatic) lesions. Primary tumors, though uncommon, are typically benign, with myxomas being the most frequent and occasionally displaying unique features. Diagnosis often requires multimodality imaging.

Case summary

A 41-year-old man with a history of ischemic stroke and resection of an apical left ventricular myxoma presented for evaluation after a follow-up transthoracic echocardiogram (TTE) revealed a mobile, ovoid apical lesion with high embolic risk, despite being asymptomatic. Cardiac magnetic resonance (CMR) imaging suggested recurrence of the ventricular myxoma. Surgical resection was performed, and histopathology confirmed the diagnosis of cardiac myxoma (CM).

Discussion

CMs are the most common primary benign cardiac tumors, typically located in the left atrium and attached to the interatrial septum. They are less frequently found in the ventricular cavities. About half of cases are asymptomatic, and recurrence is possible after resection. This case highlights a rare instance of late recurrence in an atypical location.

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Original Article

Association of Noonan Syndrome, Noncompaction of the Myocardium, Hypertrophic Cardiomyopathy, and Long QT Syndrome: A Case Series

Anna Luisa de Melo Lula Lins Pimentel et al. Érika Alves da Silva Marcelo Dantas Tavares de Melo Vera Maria Cury Salemi

Abstract

Background:

The Noonan syndrome (NS) is a rare genetic disorder characterized by manifestations such as short stature, a webbed neck, micrognathia, and hypertelorism. Although NS predisposes to cardiac disorders such as hypertrophic cardiomyopathy (HCM), it has only rarely been associated with noncompaction cardiomyopathy (NCCM), an embryonic anomaly defined by excessive trabeculation and formation of deep myocardial recesses. The clinical presentation of NCCM ranges from asymptomatic to severe thromboembolic events. This study aims to describe cardiovascular changes and outcomes in patients with NS and NCCM followed at the cardiology department of a Brazilian hospital.

Methods:

Observational, longitudinal, prospective follow-up of three adult patients with the NS phenotype. AII underwent echocardiography and cardiac magnetic resonance imaging (CMR). Data such as age, sex, family history, symptom onset, and outcomes of interest, such as thromboembolic events, heart transplantation (HTx), and death, were analyzed.

Results:

All three patients followed (one male-female sibling pair and one unrelated male) had NCCM with ventricular dysfunction. The female patient also had the long QT syndrome (LQTS). All developed apical thrombi, in the left ventricle in two cases. The female patient underwent successful HTx, while the two males died after decompensation of heart failure (HF) progressing to cardiogenic shock.

Conclusion:

This case series highlights the need for early diagnosis and family screening of patients with NS to optimize treatment and improve prognosis. There is no prior record in the literature of the association of all conditions described herein in the same patient.

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Case Report

Persistent Cephalgia Secondary to Carotid Web

José Aldo Ribeiro Teodoro et al. Pedro Bution Teodoro Lucas Bution Teodoro Marco Antonio Zandoni Junior Antônio Pio Maria Jemma

Introduction

The Carotid Web (CW) consists of an intraluminal projection of the carotid artery’s intima, forming either as a membrane or tissue fold, and may cause arterial lumen stenosis, disrupt blood flow patterns, leading to vascular embolic events such as a Cerebrovascular Accident (CVA). The Carotid Web can be asymptomatic or symptomatic, with symptoms most often presenting as persistent cephalgia (headache). CW is a congenital anomaly of embryological development at the carotid bifurcation level. Currently, due to technological advances, CW is more easily diagnosed through the following diagnostic methods: Color Doppler Echocardiography (CDE), Computed Tomography Angiography (CTA), Magnetic Resonance Angiography (MRA) and Angiography (AGP). CDE is a highly accurate exam, demonstrating high sensitivity (94%) and specificity (92%). However, its positive and negative predictive values can be influenced by the disease’s low prevalence, making the exam highly reliant on a qualified, experienced examiner.,,,,,,

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Review Article

My Approach To Myocardial Extracellular Volume Quantification Using Cardiac Computed Tomography

Gabriel C. Camargo et al. Letícia R. Sabioni

Abstract

The quantification of myocardial extracellular volume (ECV) has demonstrated both diagnostic and prognostic value in various heart diseases. While ECV is typically assessed using cardiac magnetic resonance imaging (CMR), it can also be measured through cardiac computed tomography (CCT) imaging. This article discusses the application of CCT for myocardial ECV calculation, detailing the technique, its advantages and disadvantages, and its potential clinical applications.

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Case Report

Prosthetic Valve Endocarditis: A Multidisciplinary Challenge

Ana Beatriz Aisemann Goulart Paiva et al. Ana Carolina de Azevedo Pastori Gabriel Silva Vaz Martins Lucas Norambuena Aulicino Marcelo Goulart Paiva

Clinical case

We report the case of a 55-year-old male patient who was admitted to the hospital in July 2019 with sudden left hemiplegia, mitral systolic murmur 4+, and aortic systolic and diastolic murmurs 2+. He had a history of aortic valve replacement in 2015, with a mechanical prosthetic aortic valve (PAV) due to infective endocarditis (IE). Cranial computed tomography (CT) revealed a right frontoparietal hematoma (), and blood tests revealed hyperglycemia and elevated C-reactive protein. His electrocardiogram (ECG) showed sinus rhythm with repolarization abnormalities (), and a chest X-ray showed cardiomegaly +/4 (). Transthoracic echocardiography (TTE) showed preserved function, thickening of the mobile elements of the mechanical PAV, mild double aortic lesion, pseudoaneurysm of the mitral-aortic intervalvular fibrosa, and important eccentric mitral regurgitation (). Blood cultures were requested, and ceftriaxone 4 g/d and teicoplanin 800 mg/d were started. Transesophageal echocardiography (TEE) showed vegetations on the ventricular side of the mechanical PAV leaflets and periprosthetic abscess (). Group B Streptococcus agalactiae was identified, and surgical treatment was indicated after 4 weeks due to cardiovascular stability and hemorrhagic stroke.

On day 22 of hospitalization, the patient presented pulmonary congestion and respiratory failure. Repeated TTE revealed partial loosening of the mechanical PAV and severe periprosthetic leak. Emergency surgery was performed to clean and replace the mechanical PAV with a biological PAV, without mitral valve approach. Culture of the explanted prosthesis revealed no bacterial growth.

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Editorial

Metformin in Combination with Immune Checkpoint Inhibitors: Myocardial FDG Uptake Can Predict Prognosis?

Claudio Tinoco Mesquita et al. Ana Beatriz Couto Marina Schmid Nunes Fernanda Azevedo Silva

In Brazil, the estimate for 2023-2025 indicates that there will be 704,000 new cancer cases in the country, 221,000 of which will be non-melanoma skin cancers, cited as the most common type. After this type of cancer, breast cancer has the highest incidence, with 74,000 cases, followed by prostate cancer, with 72,000 cases, colorectum cancer, with 46,000 cases, and lung cancer, with 32,000 new cases. The cancer epidemiological profile in Brazil is like countries with a high income; however, it differs when analyzed by region, indicating significant regional inequalities. While in the country’s more developed regions (South and Southeast), the age-adjusted incidence rates range between 180 and 190 cases per 100,000 inhabitants, in less developed regions (North and Northeast), these rates vary from 157 to 164 cases per 100,000 inhabitants. The highest cancer mortality in Brazil is due to lung cancer (13.7% of cancer deaths), followed by colorectum cancer (10.4% of cancer deaths), and then breast cancer (8% of cancer deaths).

Immune checkpoints, like PD-1, PD-L1, and CTLA-4, regulate the immune system and help to equilibrate immune activation and suppression. They act by avoiding a strong immune response that could attack normal cells. Tumor cells can use these mechanisms to elude the immune system, decreasing its response. The mechanisms used for immune system evasion in lung cancer are linked to PD-1 inhibition, which reduces the activity of tumor immunity cells. Additionally, there is a decrease in T-lymphocyte proliferation and cytokine production (IL-2 and IFN-γ), allowing neoplastic cells to proliferate.

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Case Report

Percutaneous Closure of the Aortopulmonary Window in an Infant Using an Amplatzer Duct Occluder II Prosthesis

Edmundo Clarindo Oliveira et al. José Augusto Almeida Barbosa Marco Antonio Gonçalves Moura Marcelo Frederigue de Castro … Renata Fonseca Mendoza Maria Carmo Pereira Nunes

Introduction

The Aortopulmonary Window (APW) is a rare congenital malformation, representing approximately 0.2% of all congenital heart diseases. It is typically nonrestrictive and requires early surgical intervention to prevent complications such as pulmonary congestion, respiratory infections, and obstructive pulmonary vascular disease. In cases like ours, where the surgical risk is exceedingly high, percutaneous closure may present a viable alternative.

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Image

A Prosthetic Dysfunction Earlier Than Expected

Mariana Laranjeira et al. Maria Inês Bertão Rodolfo Pereira Olga Sousa

Prosthetic Heart Valves, despite their improvements over the last few decades, can be subject to complications and dysfunction. Mechanical prosthetic valves have an expected lifespan of approximately 10 to 20 years, with many lasting longer. For biological valves, the lifespan is generally shorter (5 to 15 years). However, the durability varies according to individual factors, valve type, and patient health. Although their greater durability, Mechanical Heart Valves are more pro-thrombotic than biological valves, and their dysfunction may be associated, for example, with thrombotic events and the formation of pannus. Pannus is an inflammatory tissue composed of inflammatory cells, fibroblasts, and blood vessels, and its presence can lead to dysfunction or other complications such as valve obstruction or endocarditis. The prevalence of pannus in cardiac valve prostheses varies depending on several factors, such as the type of prosthesis (biological or mechanical) and the patients’ clinical conditions. It is estimated that the formation of pannus occurs in about 1% to 5% of patients with valve prostheses, although it may be more common in biological ones. The annual rate of thrombus formation can reach 5.7-8%, being higher in tricuspid and mitral valve prostheses.^, A multimodality-based approach can provide useful data for differential diagnosis, complementing clinical assessment and offering an appropriate and optimized therapeutic approach. Valve dysfunction, in case of delayed diagnosis, can progress to cardiogenic shock. Valvular dysfunctions can be structural (valve dysfunction due to changes related to the valve itself) or non-structural (dysfunctions not related to the valve resulting in stenosis or regurgitation, which include the formation of pannus), which arises from the accumulation of fibrous tissue in the valve, causing its dysfunction. It forms less frequently than thrombi and its risk is higher in the mitral position compared to the tricuspid.

The authors present a case of mechanical mitral valve prosthesis dysfunction due to pannus. A Caucasian 43 years-old woman, with dyslipidaemia and hypertension with a history of infectious endocarditis of the mitral valve in 04/2022, presented with signs of infectious infiltration of both leaflets and severe mitral regurgitation. At this point, a cycle of antibiotic therapy was performed with ampicillin and flucloxacillin, with no agent being isolated (neither in blood cultures nor microbiological examination of the explanted valve). A double-disc mechanical valve prosthesis was implanted in the mitral position. The patients were hypocoagulated with a vitamin K antagonist (warfarin, with target INR levels of 2.5-3.5) and discharged from the hospital for outpatient follow-up.

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Original Article

Clinical and Echocardiographic Characterization of Patients with ATTR VAL142IIe

Tonnison de Oliveira Silva et al. Tonnison de Oliveira Silva Filho Lara Tavares Cardoso dos Santos Maria Luisa Fialho … Gabriel de Souza Santos Pereira Ellen Beatriz Menezes Enzo Galeffi Rodrigues Marcelo Tavares Marcela Câmara Machado Costa Luiz Eduardo Fonteles Ritt

Abstract

Background:

Transthyretin (TTR) amyloid cardiomyopathy associated with the VAL142Ile variant typically presents in patients in their sixth or seventh decades of life. It is among the most common hereditary forms of cardiac amyloidosis. This study aims to characterize the clinical, laboratory, and echocardiographic features observed in patients with the VAL142Ile variant, contributing to a deeper understanding of the phenotypic expression of this genetic variant.

Method:

Cross-sectional study of 31 patients diagnosed with HF due to transthyretin amyloidosis (ATTR) VAL142IIe. Functional class, presence of atrial fibrillation (AF) and carpal tunnel syndrome (CTS), and NT-proBNP value were assessed. Diastolic and systolic dimensions, septum, posterior wall (PW), left atrial volume, ejection fraction (EF), diastolic function and global longitudinal strain (GLS) were quantified. Statistical analysis was performed using SPSS software version 26.0.

Results:

Mean age at symptoms onset was 74.3 years (±5.9 years), with 61% being male. A total of 14 (45%) were identified with AF and 17 (55%) with CTS. Regarding New York Heart Association (NYHA) classification, 19 (61%) were in FC II and 8 (25.8%) in FC III. Measurements were diastolic diameter (DD) of 50.6 mm (±8.5), systolic diameter (SD) of 35.2 mm (±7.8), pw = 13.5 mm (±2.1) and ivs = 14.8 mm (±2.4). The left atrial volume was 54.3 mL/m² (±12) and the EF was 47.3% (±15.1). A total of 8 (25%) underwent strain echocardiography, and 7 (87.5%) showed apical sparing. Diastolic dysfunction grade II was observed in 8 (47%), and 14 (45%) had AF. The mean NT-proBNP was 3026 pg/mL (±1941 pg/mL).

Conclusion:

Our findings underscore the importance of characterizing this population, highlighting the potential for early disease onset even with the VAL142Ile variant. Elevated NT-proBNP levels, along with the presence of AF and CTS, further elucidate the phenotypic patterns of patients with the ATTR VAL142Ile variant.

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Original Article

Left Ventricular Global Longitudinal Strain: an Early Marker of Diabetic Cardiomyopathy

Ana Carolina Saran et al. Stephanie Corradini Tormin Jennifer Vogel Rogério Krakauer João Eduardo Nunes Salles

Abstract

Background:

Diabetic cardiomyopathy (DCM) leads to abnormal myocardial structure and function in the absence of cardiac risk factors. Screening in the pre-clinical phase is not well established. Global longitudinal strain (GLS) has been measured as an important echocardiography parameter in asymptomatic patients.

Objective:

To describe the presence of early parameters of DCM in patients with type 2 diabetes (T2DM) without cardiovascular disease and to compare results against 2 control groups (CT groups).

Methods:

A total of 58 patients were divided into the following 3 groups: T2DM (n = 20); heart failure (HF) with preserved ejection fraction (HFpEF) without T2DM (n = 19); and control without T2DM or HFpEF (n = 19). Patients with cardiovascular disease and those using SGLT2 inhibitors, pioglitazone or saxagliptin were excluded.

Results:

The mean overall prevalence of GLS was 16% (standard deviation [SD] ± 2.9), and 41% of participants had abnormal values, comprising 10 (50%) patients from the T2DM, 11 (58%) from the HFpEF, and 3 (16%) from the CT groups (p = 0.019). Mean GLS values in the T2DM, HFpEF, and CT groups were 16.1%, 14.8%, and 17.5%, respectively (p = 0.015). There was a negative moderate association between HbA1c levels and GLS values in the T2DM group (p = 0.043).

Conclusions:

GLS proved to be a potential early marker of left ventricular (LV) cardiac changes in patients with T2DM, given the similarity between this patient group and the HFpEF group studied.

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Original Article

Variations in Myocardial FDG Uptake and Metformin Use: Implications for Survival during Immunotherapy

Matheus Coelho Torres et al. Juliana Goes Martins Fagundes Luís Fábio Barbosa Botelho Alinne Fernanda Amaral Verçosa Marcelo Dantas Tavares de Melo

Abstract

Background:

The rise of immune checkpoint inhibitors (ICIs) has significantly improved lung cancer outcomes; however, there is a lack of response prediction protocols. Furthermore, preclinical studies have indicated a promising association between metformin, β-blockers (BBs), and improved cancer patient outcomes.

Objectives:

The primary objective of this study was to investigate metformin’s impact on survival outcomes. The secondary objectives included assessing myocardial FDG uptake variation (change in standardized uptake value [ΔSUV]) during ICI treatment and evaluating the effects of smoking, diabetes, hypertension, and BB usage on survival outcomes.

Methods:

This single-arm, unicentric retrospective cohort study evaluated lung cancer patients who started using ICIs from July 2016 to December 2021. Inclusion criteria were age 18 years or above, lung cancer treated with ICIs (CTLA-4, PD-1, and PD-L1 inhibitors), and having undergone at least two positron emission tomography-computed tomography (PET-CT) scans.

Results:

Fifty-eight patients fulfilled all the inclusion criteria. Metformin users presented a 759-day increase in overall survival (OS) (p = 0.015). A trend of a 161-day increase in progression-free survival was observed in patients with positive myocardial ΔSUV compared to the negative ΔSUV group (p = 0.066), along with a trend of a 285-day increase in favor of BB users (p=0.886).

Conclusion:

The significant association between metformin use and increased OS suggests metformin as a promising adjuvant for ICI therapy. A trend of positive myocardial ΔSUV and improved outcomes may suggest a potential role of PET-CT in response prediction; however, larger studies are necessary to solidify this hypothesis.

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Case Report

Atrial Myxoma as a Rare Cause of Stroke: Case Report

Maurício Ângelo de Almeida Junior et al. Ana Flavia Parreira de Morais Jamil Alli Murad Junior

Introduction

Atrial myxoma (AM) is the most common primary benign tumor affecting the heart. Its prevalence is rare, estimated at 0.03% in the general population. Surgical intervention should be introduced as soon as diagnosed due to the increased risk of cardiac complications, such as intracardiac obstructions or systemic embolizations.

Cardiac myxomas are commonly observed in the left atrium in >75% of cases, predominantly attached with a stalk to the fossa ovalis (>90%). However, they can be seen in other locations (left ventricle, right atrium, and, though quite rarely, the right ventricle).

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Crawford 2 Thoracoabdominal Aneurysm Associated with Bicarotid Trunk and Aberrant Right Subclavian Artery

Milton Sérgio Bohatch Junior et al. Fernando Reis Neto Daniel Gustavo Miquelin Andre Rodrigo Miquelin … Henrique Amorim Santos Amanda Fernandes Vidal da Silva Luiz Fernando Reis Jose Maria Pereira de Godoy

A 73-year-old woman with a history of hypertension was referred to the vascular surgery department for severe chest pain and signs of shock, including tachycardia and a tendency toward hypotension. An angiotomography revealed a thoracoabdominal aneurysm (Crawford Classification type 2 – ) with rupture signs ( and ) and anatomical variations in the supra-aortic trunks, including an aberrant right subclavian artery (ARSA) ( and ) and a bicarotid trunk (). She underwent emergency surgery with a hybrid approach, which included visceral branching via laparotomy and endovascular repair of the aneurysm. Unfortunately, she developed hemodynamic instability and refractory shock during the procedure, resulting in death. Her family authorized the publication and signed the consent form.

An ARSA is a rare anomaly with a 0.2% to 13.3% prevalence.^, It is more common among females, individuals with congenital anomalies (up to 3%), and those with Down syndrome (up to 35%). ARSA may be associated with a bicarotid trunk in 0.8% to 20% of cases, where common carotid arteries originate from the same point on the aortic arch. Thoracoabdominal Aortic Aneurysms (TAAAs) represent 10% of all aortic aneurysms. These aneurysms are potentially fatal if not treated early, as they may progress to rupture or dissection. In a São Paulo epidemiological study, the mortality rate for emergency TAAA repairs was nearly double that of elective repairs (42.10% vs. 26.78%). This difference is often attributed to the hemodynamic instability of ruptured aneurysms and the lack of timely preoperative preparation in urgent cases. Anatomical assessment is essential for treating TAAAs, whether through open surgery, endovascular surgery, or hybrid techniques. Anatomical variations in the supra-aortic trunks increase surgical complexity, regardless of the chosen technique.

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