Cardiomyopathies – ABC CARDIOL

ABCIC

Editorial

A Nova Fronteira na Prevenção Cardiovascular: Além da Proibição, o Manejo Clínico do Usuário de Esteroides

Fabio Roston et al. Alexandre Aby Azar Ribeiro Naiara Caroline Makiniks Luiz Felipe Branco Ribeiro Liliana Ludwing Ziegler

Diagnóstico Subclínico: O Papel do Ecocardiograma Avançado

A avaliação cardíaca convencional muitas vezes falha em detectar danos precoces, pois a Fração de Ejeção do Ventrículo Esquerdo (FEVE) pode permanecer normal mesmo na presença de lesão miocárdica instalada. As fontes destacam que usuários de EAA apresentam alterações na geometria e função cardíaca, incluindo hipertrofia patológica, disfunção diastólica e comprometimento biventricular.^,

Nesse cenário, o uso do ecocardiograma com medidas avançadas, como o strain miocárdico, surge como uma ferramenta essencial. Embora a FEVE possa estar preservada em estágios iniciais, a análise da deformação miocárdica (strain) permite identificar a disfunção sistólica incipiente decorrente da fibrose e da destruição de miofibrilas causadas pela toxicidade direta dos andrógenos. Estudos recentes já demonstram que tanto usuários atuais quanto antigos apresentam cardiomiopatia biventricular persistente, reforçando a necessidade de monitoramento contínuo.

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Review Article

Genetic Testing in Cardiomyopathy: Decoding Molecular Underpinnings and Influencing Treatment Decisions

Silas Ramos Furquim et al. Layara Fernanda Vicente Pereira Lipari Fernanda Almeida Andrade Vinícius Machado Correia … Natália Quintella Sangiorgi Olivetti Lucas Vieira Lacerda Pires Luciana Sacilotto Fábio Fernandes José Eduardo Krieger

Abstract

Cardiomyopathy is characterized by structural and functional abnormalities in the heart muscle that are not influenced by coronary disease, hypertension, valve disease, or congenital heart defects. Genetic testing, which helps diagnose the molecular bases of cardiomyopathy, can affect prognostication and clinical decisions. Recent guidelines advocate classifying cardiomyopathies based on phenotypic categories, which encompass a range of clinical presentations, underlying causes, and outcomes. Beyond deepening our grasp of the disease, genetic testing can distinguish between diseases with similar presentations, known as genocopies, which can profoundly influence treatment decisions. Genetic testing is also essential for reproductive counseling and risk evaluation among family members. Combined with advanced imaging techniques such as magnetic resonance imaging, genetic investigation offers a comprehensive understanding of the disease. Thus, as the nature of cardiomyopathies continues to be unraveled, cardiologists must stay updated on advances in genetic testing and their practical applications.

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Review Article

The Role of Strain in the Prognosis of Transthyretin Amyloidosis

Ana Karolina Bento Bento da Silva et al. Leonardo Torreão Bezerra Cavalcanti Silvio Henrique Barberato Alex dos Santos Felix Marcelo Dantas Tavares de Melo

Abstract

Transthyretin amyloidosis (TTRA) is a systemic infiltrative disease where cardiac involvement is paramount for prognosis. The strain technique plays a crucial role in non-invasive cardiac screening for this condition. Research on the prognostic utility of cardiac deformation in TTRA indicates the significance of right ventricular (RV) free wall strain, global longitudinal strain (GLS), left and right atrium reservoir strain, in descending order of publication, respectively. The GLS has emerged as a promising tool for therapeutic monitoring. Analyzing myocardial deformation is vital due to its incremental value over other clinical, laboratory, and imaging parameters. However, caution is warranted due to limited data on its prognostic and therapeutic monitoring use in TTRA. Despite the strong rationale for employing myocardial deformation in patient stratification, further robust studies are necessary to establish it as a new prognostic marker in TTRA.

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Review Article

Heart Failure Management of Patients with Amyloid Cardiomyopathy

Vanessa Simioni Faria et al. Ciro Mancilha Murad Fabiana G. Marcondes-Braga

Abstract

Cardiac amyloidosis (CA) can lead to progressive heart failure (HF) by depositing insoluble amyloid fibrils within the myocardial extracellular space, resulting in an infiltrative and restrictive cardiomyopathy. Although CA was previously perceived as rare and incurable, recent advances in diagnostics and emerging therapies have been changing this outlook. It is crucial to spread awareness about CA to facilitate earlier diagnosis and proper therapeutic interventions, enhancing patient prognosis and survival. Currently, there is an estimated delay of 2 years from symptom onset to diagnosis, typically involving consultation with an average of 5 different professionals. Advances in cardiovascular imaging have facilitated earlier and more accurate diagnosis, reducing the necessity for invasive procedures, such as endomyocardial biopsy. Presently, tafamidis is the only drug that has been shown to offer prognostic benefits in ATTR-CA. Tafamidis is a highly specific medication targeting the circulating TTR protein, stabilizing the TTR tetramer to prevent its dissociation into amyloidogenic monomers that deposit in the myocardium. Alongside specific amyloidosis therapy, supportive HF treatment may be required; however, managing CA with medications typically used for HF with reduced ejection fraction (HFrEF) can be challenging due to potential intolerance. The effectiveness of guideline-directed medical therapy (GDMT) remains undetermined and still requires evaluation through randomized controlled clinical trials (RCCTs). Thus, the treatment cornerstone remains the judicious use of loop diuretics and mineralocorticoid receptor antagonists to control volume overload. Due to the safety profile, not adversely affecting hemodynamics or renal function, sodium-glucose transport protein 2 (SGLT2) inhibitors may be an effective treatment for CA, but they also still require evaluation through RCCTs.

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Editorial

Advanced Echocardiographic Techniques for the Diagnosis of Subclinical Right Ventricular Dysfunction in Chronic Obstructive Pulmonary Disease

Silvio Henrique Barberato

Chronic obstructive pulmonary disease (COPD) is a common and treatable disease in the general population, accounting for high morbidity and mortality rates. In patients with severe COPD, hypoxemia is the main cause of complications such as pulmonary hypertension (PH) and right ventricle (RV) dysfunction. However, the presence of RV vascular structural and functional changes may occur in the early stages of COPD, even before significant hypoxemia or PH. Subclinical RV dysfunction often precedes the development of signs and symptoms of RV failure; thus, there is an evident need to detect it from the onset of the disease.

The importance of the RV in cardiovascular physiology has been underestimated for decades. Previously considered a mere conduit, the chamber is now known to play a major role in maintaining global cardiac function intact. Impairment of RV systolic function is an essential determinant of clinical outcomes in different scenarios and should thus be considered in the individualized management of patients.^,

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Original Article

Chronic Obstructive Pulmonary Disease: The Role of Myocardial Deformation Indices and Right Ventricle Three-Dimensional Echocardiography

Claudia Myriam Amaral Botelho et al. José Luiz Barros Pena Bruno Rezende Passos Paulo Roberto Latorre Fortes Maria da Consolação Vieira Moreira

Abstract

Background:

Right ventricular dysfunction is a major complication in chronic obstructive pulmonary disease (COPD) and, despite its prognostic implications, is rarely considered in routine clinical analysis.

Objectives:

To compare RV function variables with standard and advanced echocardiographic techniques in patients with stable COPD and controls.

Methods:

Twenty COPD patients classified as GOLD ≥ II (13 men aged 68.4 ± 8.3 years) and 20 matched controls were compared. Myocardial strain/strain rate indices were obtained by tissue Doppler and two-dimensional speckle tracking echocardiography. Right ventricular ejection fraction was obtained with three-dimensional software. Free wall myocardial thickness (FWMT) and tricuspid annular systolic excursion (TAPSE) were also measured. Numerical variables were compared between groups with Student’s t-test or the Mann-Whitney test. Associations between categorical variables were determined with Fisher’s exact test. P-values < 0.05 were considered statistically significant.

Results:

All myocardial deformation indices, particularly global longitudinal strain (-17.2 ± 4.4 vs -21.2: ± 4.4 = 0.001) and 3D right ventricular ejection fraction (40.8 ± 9.3% vs 51.1 ± 6.4% p <0.001) were reduced in COPD patients. These patients presented higher right ventricular FWMT and lower TAPSE values than controls.

Conclusion:

Myocardial deformation indices, either tissue Doppler or speckle tracking echocardiography and 3D right ventricular ejection fraction, are robust markers of right ventricular dysfunction in patients with stable COPD. Assessing global longitudinal strain by speckle tracking echocardiography is a more practical and reproducible method.

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Editorial

What have we Learned about the Different COVID-19 Phenotypes in the Pediatric Population so Far?

Andressa Mussi Soares et al. Bernardo Mussi Soares

The understanding of SARS-CoV-2 related syndromes in the pediatric population needs to grow worldwide. In its severe presentation, COVID-19 is a systemic disease characterized by hyperinflammation, cytokine storm and increased myocardial injury markers. Cardiac involvement in seen in 20-30% of hospitalized adult patients and accounts for about 40% of deaths.

An intriguing aspect of the COVID-19 is the fact that most children and adolescents have mild or asymptomatic forms of the disease, perhaps related to a lower expression of virus receptors, recent exposure to other coronaviruses generating cross-protection, more developed innate immunity, among others. In fact, it is known that 40% of individuals under the age of 18 exposed to the virus get infected and that only 20-30% of children and adolescents infected with SARS-Cov-2 have symptoms.

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Original Article

Association between Mean Platelet Volume-to-Lymphocyte Ratio and the Presence of Apical Mural Thrombus in Post-Myocardial Infarction Patients

Cemal Koseoglu et al. Ozge Kurmus

Abstract

Background

Left ventricular apical thrombus (AT) is generally associated with ischemic and non-ischemic cardiomyopathies. The thrombo-inflammatory process plays an important role in the pathophysiology of acute coronary syndromes and post-myocardial thromboembolic complications. Mean platelet volume (MPV) has been linked to poor prognosis following myocardial infarction. Recently, platelet-to-lymphocyte ratio (PLR) has emerged as a new marker of worse outcomes linking inflammation and thrombosis.

Objective

We aimed to investigate the prognostic significance of the marker – mean platelet volume to lymphocyte ratio (MPVLR) in patients with AT.

Methods

Fifty-six patients with left ventricular AT after an anterior myocardial infarction and 51 patients without left ventricular AT after an anterior myocardial infarction were enrolled in this study retrospectively. Admission MPVLR was compared between the two groups. Logistic regression analysis was carried out to identify whether MPVLR is an independent predictor of AT. The receiver operating curve (ROC) analysis was used to show the optimal cut-off for MPVLR to predict AT. P values less than 0.05 were considered statistically significant.

Results

Age, gender, frequency of diabetes mellitus, hypertension and atrial fibrillation, and ejection fraction values did not differ between the groups. MPVLR was higher in patients with AT than patients without AT (7.91±2.5 vs 5.1±2.1, p 4.75 (82.1% sensivity and 70.2% specifity (area under the curve=0.811, 95% confidence interval [CI]: 0.731-0.891, p<0.001). MPVLR was found to be an independent risk factor for the formation of AT (B:0.441, p.0.001).

Conclusion

MPVLR is a simple, cheap and easily accessible test that can predict left ventricular AT formation. (Int J Cardiovasc Sci. 2020; [online].ahead print, PP.0-0)

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Review Article

The Role of 18F-FDG PET/CT in Cardiac Sarcoidosis

Christiane Wiefels et al. Olabimpe Lamai Riina Kandolin David Birnie … Eugene Leung Claudio Tinoco Mesquita Rob Beanlands

Introduction

Systemic sarcoidosis (SS) was first described more than a century ago by the Norwegian dermatologist Caesar Boeck. He correlated skin nodules with epithelioid cells with large nuclei and giant cells as “multiple benign sarcoid of the skin”, for their resemblance with sarcoma. Today, the cause of the disease remains unknown, but evidence points toward immunological response to an unidentified antigenic trigger in individuals with genetic susceptibility. The estimation of the prevalence of SS in the population varies in the literature also depending on the type of study: epidemiologic, autopsy or imaging.

Some previous data showed prevalence as high as 100-330 cases per 100,000 inhabitants.^, Environmental factors have been attributed to those differences, including sex, age and ethnicity, showing a predisposition for environmental and genetic factors. The reported incidence is similar across sex in North American population but greater in females in Scandinavian and Japanese populations,^, but more likely to be chronic and fatal in black Americans. The disease usually develops before the age of 50 years, with a peak incidence at 20 to 39 years old,^, and is very uncommon under the age or 15 or older than 70. Symptomatic cardiac sarcoidosis (CS) has been reported in 2-5% of the patients with the systemic form.^– However, with the advance of new cardiac imaging techniques, cases of asymptomatic (clinically silent) cardiac involvement have been diagnosed. The disease has been reported in at least 20% of the autopsies and imaging report in the United States to as much as 50% in Japan.^,

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Case Report

Iatrogenic Complications During the Diagnostic Work-Up of an Inflammatory Cardiomyopathy

Hugo Antunes et al. Júlio Gil Bruno Marmelo Maria Luísa Gonçalves … Maria Inês Pires João Miguel Santos Miguel Correia José Costa Cabral

Abstract

A 72-year-old woman was admitted for acute heart failure. The echocardiography revealed moderate depression of the left ventricular ejection fraction. Coronary disease was excluded by coronarography. Cardiac magnetic resonance showed predominantly left ventricular septal hypertrophy and severe depression of the left ventricular systolic function. There was also a bright, multifocal and patchy late gadolinium enhancement with subendocardial, mesocardial and subepicardial involvement, suggestive of sarcoidosis. Biochemical study, thoracic computed tomography and positron emission tomography were inconclusive for extra-cardiac sarcoidosis. Therefore, an endomyocardial biopsy was performed. The procedure was complicated by the development of complete atrioventricular block, requiring implantation of a cardiac resynchronization pacing device. A few days after device implantation, the patient developed fever. The echocardiography revealed extensive vegetations, and thus the diagnosis of a device-associated infective endocarditis was made. Even though antibiotic therapy was promptly started, the patient ended up dying. Biopsy results revealed lymphocytic myocarditis.

This case is paradigmatic because it shows how the etiologic diagnosis of dilated cardiomyopathy can be challenging. Non-invasive diagnostic exams may not provide a definite diagnosis, requiring an endomyocardial biopsy. However, the benefits versus risks of such procedure must always be carefully weighted.

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Case Report

Biventricular Arrhythmogenic Cardiomyopathy: A New Paradigm?

João Augusto et al. João Abecasis Victor Gil

Abstract

Arrhythmogenic right ventricular dysplasia is a classic form of chronic myocardial disease with a broad phenotypical spectrum. We report an atypical case of a patient with biventricular arrhythmogenic cardiomyopathy. Although the current diagnosis criteria are the most widely accepted ones, they focus solely on the right ventricular phenotype. The use of late gadolinium enhancement in cardiac magnetic resonance in this patient was essential for the diagnosis and assessment of the left ventricular involvement extent. This tool allows a broader use of current diagnosis criteria for this disease.

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