Cardiac Sarcoidosis Presenting as Sustained Ventricular Tachycardia.

Introduction: Sarcoidosis has many possible clinical presentations since it can affect any organ, most commonly the lungs. The hallmark of the disease consists of the formation of non-necrotising granulomas. Pathogenesis is thought to rely on the interplay of genetic, environmental and epigenetic factors. This case highlights the importance of a thorough clinical history and physical examination, and the correlation with imaging findings in the diagnostic work-up of the non-ischaemic cardiomyopathy. Case description: A 57-year-old woman was admitted due to the sudden onset of malaise, dizziness, and chest discomfort. Sustained monomorphic ventricular tachycardia was evidenced and the patient rapidly evolved with haemodynamic instability; she underwent successful electrical cardioversion. The electrocardiogram afterwards showed a high-risk electrocardiographic pattern. Invasive coronary angiography excluded obstructive epicardial coronary lesions. Physical examination revealed skin lesions on the lower limbs which raised suspicion for erythema nodosum and therefore a biopsy was performed. Transthoracic echocardiography and cardiac magnetic resonance imaging revealed features consistent with an inflammatory cardiomyopathy, and an implantable cardioverter-defibrillator was placed. The histologic examination of the cutaneous lesions showed a non-necrotising granulomatous inflammatory process. Radionuclide imaging was inconclusive. The patient underwent an endomyocardial biopsy, which confirmed the diagnosis of systemic sarcoidosis with cardiac involvement. Conclusions: Systemic sarcoidosis with cardiac involvement is a challenging diagnosis. The role of imaging techniques such as transthoracic echocardiography, cardiac magnetic resonance imaging and radionuclide imaging is essential in raising suspicion and diagnosing this pathology. Endomyocardial biopsy is the 'gold standard' for its diagnosis; however, it has a low diagnostic yield. LEARNING POINTS: Systemic sarcoidosis with cardiac involvement is a challenging diagnosis as it may present in many different ways.The case presented highlights the importance of a thorough clinical history and physical examination, and the correlation with imaging findings.Imaging techniques such as transthoracic echocardiogram, cardiac magnetic resonance and radionuclide imaging are essential in raising suspicion and diagnosing cardiac sarcoidosis.

Three-dimensional echocardiography for the evaluation of hypertrophic cardiomyopathy patients: relation to symptoms and exercise capacity.

Patients with hypertrophic cardiomyopathy may exhibit impaired functional capacity, associated with increased morbidity and mortality. Systolic function is one of the determinants of functional capacity. Early identification of systolic disfunction may identify patients at risk for adverse outcomes. Myocardial deformation parameters, derived from three-dimensional (3D) speckle-tracking echocardiography (3DSTE) are useful tools to assess left ventricular systolic function, and are often abnormal before a decline in ejection fraction is seen. The aim of this study was to evaluate the correlation between myocardial deformation parameters obtained by 3DSTE and functional capacity in patients with hypertrophic cardiomyopathy. Seventy-four hypertrophic cardiomyopathy adult patients were prospectively evaluated. All patients underwent a dedicated 2D and 3D echocardiographic examination and cardiopulmonary exercise testing (CPET). Values of 3D global radial (GRS), longitudinal (3DGLS) and circumferential strain (GCS) were overall reduced in our population: 99% (n = 73) of the patients had reduced GLS, 82% (n = 61) had reduced GRS and all patients had reduced GCS obtain by 3DSTE. Average peak VO2 was 21.01 (6.08) ml/Kg/min; 58% (n = 39) of the patients showed reduced exercise tolerance (predicted peak VO2 < 80%). The average VE/VCO2 slope was 29.0 (5.3) and 16% (n = 11) of the patients had impaired ventilatory efficiency (VE/VCO2 > 34). In multivariable analysis, 3D GLS (ß1 = 0.10, 95%CI: 0.03;0.23, p = 0.014), age (ß1 = -0.15, 95%CI: -0.23; -0.05, p = 0.002) and female gender (ß1 = -5.10, 95%CI: -7.7; -2.6, p < 0.01) were independently associated with peak VO2. No association was found between left ventricle ejection fraction obtain and peak VO2 (r = 0.161, p = 0.5). Impaired myocardial deformation parameters evaluated by 3DSTE were associated with worse functional capacity assessed by peak VO2.

Microvascular Dysfunction Is Associated With Impaired Myocardial Work in Obstructive and Nonobstructive Hypertrophic Cardiomyopathy: A Multimodality Study.

Background Two-dimensional speckle tracking echocardiography has been shown to correlate with microvascular dysfunction, a hallmark of hypertrophic cardiomyopathy (HCM). We hypothesized that there is an association between myocardial work and left ventricular ischemia, with incremental value to global longitudinal strain, in patients with HCM. Methods and Results We performed a prospective assessment of patients with HCM, undergoing 2-dimensional speckle tracking echocardiography and stress perfusion cardiac magnetic resonance. Results were stratified according to obstructive or nonobstructive HCM and the presence of significant replacement fibrosis (late gadolinium enhancement ≥15% of left ventricular mass). Seventy-five patients with HCM (63% men, age 55±15 years) were evaluated, 28% with obstructive HCM (mean gradient 89±60 mm Hg). Perfusion defects were found in 90.7%, involving 22.5±16.9% of left ventricular mass, and 38.7% had late gadolinium enhancement ≥15%. In a multivariable analysis, a lower global work index (r=-0.519, ß-estimate -10.822; P=0.001), lower global work efficiency (r=-0.379, ß-estimate -0.123; P=0.041), and impaired global constructive work (r=-0.532, ß-estimate -13.788; P<0.001) significantly correlated with ischemia. A segmental analysis supported these findings, albeit with lower correlation coefficients. A global work index cutoff ≤1755 mm Hg% was associated with hypoperfusion with a sensitivity of 88% and a specificity of 71%, while the best cutoff for global longitudinal strain (>-15.5%) had a sensitivity of 64% and a specificity of 57%. The association between myocardial work and perfusion defects was significant independently of late gadolinium enhancement ≥15% and obstructive HCM. Conclusions Impaired myocardial work was significantly correlated with the extent of ischemia in cardiac magnetic resonance, independently of the degree of left ventricular hypertrophy or fibrosis, with a higher predictive power than global longitudinal strain.

Dilated Cardiomyopathy: A Comprehensive Approach to Diagnosis and Risk Stratification.

Dilated cardiomyopathy (DCM) represents one of the most common causes of non-ischemic heart failure, characterised by ventricular dilation alongside systolic dysfunction. Despite advances in therapy, DCM mortality rates remain high, and it is one of the leading causes of heart transplantation. It was recently recognised that many patients present minor structural cardiac abnormalities and express different arrhythmogenic phenotypes before overt heart-failure symptoms. This has raised several diagnostic and management challenges, including the differential diagnosis with other phenotypically similar conditions, the identification of patients at increased risk of malignant arrhythmias, and of those who will have a worse response to medical therapy. Recent developments in complementary diagnostic procedures, namely cardiac magnetic resonance and genetic testing, have shed new light on DCM understanding and management. The present review proposes a comprehensive and systematic approach to evaluating DCM, focusing on an improved diagnostic pathway and a structured stratification of arrhythmic risk that incorporates novel imaging modalities and genetic test results, which are critical for guiding clinical decision-making and improving outcomes.

Electrical storm in hypertrophic cardiomyopathy: Cardiac magnetic resonance and sudden cardiac death risk stratification: a case report.

Background: Risk stratification for sudden cardiac death (SCD) is a key factor in the management of patients with hypertrophic cardiomyopathy (HCM). Cardiac magnetic resonance (CMR) has a unique role in the evaluation of HCM and offers superior diagnostic and prognostic information to assess the indication for a prophylactic implantable cardioverter-defibrillator (ICD). Case summary: A 39-year-old patient with non-obstructive HCM with a low ESC HCM Risk-SCD score underwent a CMR revealing a left ventricular apical aneurysm and extensive late gadolinium enhancement; a prophylactic ICD was thus implanted. A month later, the patient was admitted in refractory electrical storm with over 50 appropriate ICD shocks due to sustained ventricular tachycardia. Despite anti-arrhythmic therapy and mechanical ventilation, the evolution was unfavourable with haemodynamic instability; veno-arterial extracorporeal membrane oxygenation was implanted. The patient was submitted to CMR-guided epicardial VT catheter ablation with complications of LV thrombus and severe pericardial effusion. Discussion: This case details the complex risk stratification for SCD in patients with HCM, highlighting the important role of CMR in the integrated approach to risk stratification.

Late gadolinium enhancement in the left ventricular wall is associated with atrial fibrillation in patients with hypertrophic cardiomyopathy.

PURPOSE: Atrial fibrillation (AF) is the most common arrhythmia in patients with hypertrophic cardiomyopathy (HCM). The aim of this study was to evaluate the relation between AF and left ventricular (LV) late gadolinium enhancement (LGE). METHODS: 55 patients with HCM were retrospectively included. Patients were divided in HCM with AF and HCM without AF. Baseline clinical, echocardiographic and cardiovascular magnetic resonance (CMR) characteristics were collected and compared between groups. RESULTS: In univariable analysis, the factors related to AF development were HCM risk score for sudden cardiac death (SCD) > 2.29% (p = 0.002), left atrium (LA) diameter > 42.5 mm (p = 0.014) and LGE in the mid anterior interventricular septum (IVS) (p = 0.021), basal inferior IVS (p = 0.012) and mid inferior IVS (p = 0.012). There were no differences in LV diastolic function and LA strain between groups. Independent predictors of AF were LA diameter (p = 0.022, HR 5.933) and LGE in mid inferior IVS (p = 0.45, HR 3.280). Combining LA diameter (> 42.5 mm or < 42.5 mm) and LGE in mid inferior IVS (present or absent) in a model with four groups showed a statistically significant difference between groups (p = 0.013 for the model). CONCLUSIONS: LGE in mid inferior IVS is an independent predictor for AF occurrence in patients with HCM. Combining both LGE in mid inferior IVS and enlarged LA improves prediction of AF and may be preferred for risk stratification.

Multimodality imaging in cardiac amyloidosis: State-of-the-art review.

Amyloidosis is a systemic disease, characterized by deposition of amyloid fibrils in various organs, including the heart. For the diagnosis of cardiac amyloidosis (CA) it is required a high level of clinical suspicion and in the presence of clinical, laboratorial, and electrocardiographic red flags, a comprehensive multimodality imaging evaluation is warranted, including echocardiography, magnetic resonance, scintigraphy, and computed tomography, that will confirm diagnosis and define the CA subtype, which is of the utmost importance to plan a treatment strategy. We will review the use of multimodality imaging in the evaluation of CA, including the latest applications, and a practical flow-chart will sum-up this evidence.

Myocardial work is associated with significant left ventricular myocardial fibrosis in patients with hypertrophic cardiomyopathy.

Late gadolinium enhancement on cardiac magnetic resonance adds prognostic information in patients with hypertrophic cardiomyopathy. Whether Myocardial work, a new parameter on transthoracic echocardiographic, can be associated with significant fibrosis in hypertrophic cardiomyopathy patients is unknown. In a single-centre prospective evaluation of hypertrophic cardiomyopathy patients in whom transthoracic echocardiographic and cardiac magnetic resonance were performed, Myocardial work and related indices were calculated from global longitudinal strain and from estimated left ventricular pressure curves. The extent of late gadolinium enhancement was quantitatively assessed. Late gadolinium enhancement ≥ 15% was chosen to define significant fibrosis. Logistic regression analysis was used to find the variables associated with late gadolinium enhancement ≥ 15% and cut-off values were determined. Among the forty-six patients analysed mean age was 56 ± 15 years, 28 (61%) were male patients and the mean left ventricular ejection fraction by transthoracic echocardiographic was 67 ± 8%. Global constructive work and global work index were significantly related to late gadolinium enhancement ≥ 15%, while global longitudinal strain nearly reached statistical significance. A cut-off ≤ 1550 mmHg% of global constructive work was associated with significant fibrosis with a sensitivity of 91% and a specificity of 84%, while the best cut-off for global longitudinal strain (> - 15%) had a sensitivity of 67% and a specificity of 76%. In our study cohort, global constructive work was associated with significant left ventricular myocardial fibrosis in cardiac magnetic resonance, suggesting its utility in patients who may not be able to have a cardiac magnetic resonance study.

Bicuspid aortic valve aortopathies: An hemodynamics characterization in dilated aortas.

Bicuspid aortic valve (BAV) aortopathy remains of difficult clinical management due to its heterogeneity and further assessment of related aortic hemodynamics is necessary. The aim of this study was to assess systolic hemodynamic indexes and wall stresses in patients with diverse BAV phenotypes and dilated ascending aortas. The aortic geometry was reconstructed from patient-specific images while the aortic valve was generated based on patient-specific measurements. Physiologic material properties and boundary conditions were applied and fully coupled fluid-structure interaction (FSI) analysis were conducted. Our dilated aortic models were characterized by the presence of abnormal hemodynamics with elevated degrees of flow skewness and eccentricity, regardless of BAV morphotype. Retrograde flow was also present. Both features, predicted by flow angle and flow reversal ratios, were consistently higher than those reported for non-dilated aortas. Right-handed helical flow was present, as well as elevated wall shear stress (WSS) on the outer ascending aortic wall. Our results suggest that the abnormal flow associated with BAV may play a role in aortic enlargement and progress it further on already dilated aortas.

What is the role of beta-blockers in a contemporary treatment cohort of patients with acute coronary syndrome? A propensity-score matching analysis.

INTRODUCTION: The evidence for beta-blocker use in patients after acute coronary syndrome (ACS), particularly in those with left ventricular (LV) dysfunction, dates from the late 1990s. We aimed to assess the role of beta-blockers in a contemporary population of patients with ACS. METHODS: Propensity-score matching (1:2) was performed for the use of beta-blockers in a population of consecutive patients admitted to our department with ACS. After matching, 1520 patients were analyzed. Cox regression analysis was used to assess the impact of beta-blocker use on the primary outcome (one-year all-cause mortality). RESULTS: Patients who did not receive beta-blockers were less aggressively treated with other pharmacological and invasive interventions and had higher one-year mortality (20.3% vs. 7.5%). Beta-blocker use was an independent predictor of mortality, with a significant relative risk reduction of 56%. The other independent predictors were age, diabetes, LV dysfunction, heart rate, systolic blood pressure and creatinine on admission. The impact of beta-blockers was significant for all classes of LV function, including patients with normal or mildly reduced ejection fraction. CONCLUSIONS: In a contemporary ACS population, we confirmed the benefits of beta-blocker use after ACS, including in patients with normal or mildly to moderately impaired LV function.

Risk factor paradox in the occurrence of cardiac arrest in acute coronary syndrome patients. / Paradoxo dos fatores de risco na ocorrência de parada cardiorrespiratória em pacientes com síndrome coronária aguda.

OBJECTIVE:: To compare patients without previously diagnosed cardiovascular risk factors) and patients with one or more risk factors admitted with acute coronary syndrome. METHODS:: This was a retrospective analysis of patients admitted with first episode of acute coronary syndrome without previous heart disease, who were included in a national acute coronary syndrome registry. The patients were divided according to the number of risk factors, as follows: 0 risk factor (G0), 1 or 2 risk factors (G1 - 2) and 3 or more risk factors (G ≥ 3). Comparative analysis was performed between the three groups, and independent predictors of cardiac arrest and death were studied. RESULTS:: A total of 5,518 patients were studied, of which 72.2% were male and the mean age was 64 ± 14 years. G0 had a greater incidence of ST-segment elevation myocardial infarction, with the left anterior descending artery being the most frequently involved vessel, and a lower prevalence of multivessel disease. Even though G0 had a lower Killip class (96% in Killip I; p < 0.001) and higher ejection fraction (G0 56 ± 10% versus G1 - 2 and G ≥ 3 53 ± 12%; p = 0.024) on admission, there was a significant higher incidence of cardiac arrest. Multivariate analysis identified the absence of risk factors as an independent predictor of cardiac arrest (OR 2.78; p = 0.019). Hospital mortality was slightly higher in G0, although this difference was not significant. By Cox regression analysis, the number of risk factors was found not to be associated with mortality. Predictors of death at 1 year follow up included age (OR 1.05; p < 0.001), ST-segment elevation myocardial infarction (OR 1.94; p = 0.003) and ejection fraction < 50% (OR 2.34; p < 0.001). CONCLUSION:: Even though the group without risk factors was composed of younger patients with fewer comorbidities, better left ventricular function and less extensive coronary disease, the absence of risk factors was an independent predictor of cardiac arrest.

Paradoxo dos fatores de risco na ocorrência de parada cardiorrespiratória em pacientes com síndrome coronária aguda / Risk factor paradox in the occurrence of cardiac arrest in acute coronary syndrome patients

RESUMO Objetivo: Comparar pacientes admitidos com síndrome coronariana aguda sem prévia identificação de fatores de risco cardiovascular com pacientes que portavam um ou mais fatores de risco. Métodos: Análise retrospectiva dos pacientes admitidos com o primeiro episódio de síndrome coronariana aguda sem cardiopatia prévia, incluídos em um registro nacional de síndrome coronariana aguda. Os pacientes foram divididos segundo o número de fatores de risco: nenhum fator de risco (G0), um ou dois fatores de risco (G1 - 2) e três ou mais fatores de risco (G ≥ 3). Realizou-se uma análise comparativa entre os três grupos e se estudaram os preditores independentes de parada cardíaca e óbito. Resultados: O total apurado foi de 5.518 pacientes, 72,2% deles do sexo masculino, com média de idade de 64 ± 14 anos. O G0 teve uma incidência maior de infarto do miocárdio com elevação do segmento ST, sendo o vaso mais frequentemente envolvido a artéria descendente anterior esquerda, e menor prevalência de envolvimento de múltiplos vasos. Embora o G0 tivesse uma classe Killip mais baixa (96% Killip I; p < 0,001) e maior fração de ejeção (G0: 56 ± 10% versus G1 - 2 e G ≥ 3: 53 ± 12%; p = 0,024) na admissão, houve incidência significantemente maior de parada cardíaca. A análise multivariada identificou ausência de fatores de risco como um fator independente para parada cardíaca (OR 2,78; p = 0,019). A mortalidade hospitalar foi ligeiramente maior no G0, embora sem significância estatística. Segundo a análise de regressão de Cox, o número de fatores de risco não se associou com mortalidade. Os preditores de óbito em 1 ano de seguimento foram infarto do miocárdio com elevação do segmento ST (OR 1,05; p < 0,001) e fração de ejeção inferior a 50% (OR 2,34; p < 0,001). Conclusão: Embora o grupo sem fatores de risco fosse composto de pacientes mais jovens e com menos comorbidades, melhor função ventricular esquerda e coronariopatia menos extensa, a ausência de fatores de risco foi um preditor independente de parada cardíaca.

ABSTRACT Objective: To compare patients without previously diagnosed cardiovascular risk factors) and patients with one or more risk factors admitted with acute coronary syndrome. Methods: This was a retrospective analysis of patients admitted with first episode of acute coronary syndrome without previous heart disease, who were included in a national acute coronary syndrome registry. The patients were divided according to the number of risk factors, as follows: 0 risk factor (G0), 1 or 2 risk factors (G1 - 2) and 3 or more risk factors (G ≥ 3). Comparative analysis was performed between the three groups, and independent predictors of cardiac arrest and death were studied. Results: A total of 5,518 patients were studied, of which 72.2% were male and the mean age was 64 ± 14 years. G0 had a greater incidence of ST-segment elevation myocardial infarction, with the left anterior descending artery being the most frequently involved vessel, and a lower prevalence of multivessel disease. Even though G0 had a lower Killip class (96% in Killip I; p < 0.001) and higher ejection fraction (G0 56 ± 10% versus G1 - 2 and G ≥ 3 53 ± 12%; p = 0.024) on admission, there was a significant higher incidence of cardiac arrest. Multivariate analysis identified the absence of risk factors as an independent predictor of cardiac arrest (OR 2.78; p = 0.019). Hospital mortality was slightly higher in G0, although this difference was not significant. By Cox regression analysis, the number of risk factors was found not to be associated with mortality. Predictors of death at 1 year follow up included age (OR 1.05; p < 0.001), ST-segment elevation myocardial infarction (OR 1.94; p = 0.003) and ejection fraction < 50% (OR 2.34; p < 0.001). Conclusion: Even though the group without risk factors was composed of younger patients with fewer comorbidities, better left ventricular function and less extensive coronary disease, the absence of risk factors was an independent predictor of cardiac arrest.