RESUMO
A 32-year-old female presented with palpitations and chest discomfort. The patient had a history of pericardiotomy due to pericardial effusion. Multimodal imaging, including echocardiography, cardiac magnetic resonance (CMR), and coronary computed tomography angiography (CCTA) showed a single mass in the pericardium as the cause of the symptoms. Furthermore, its location and potential complications were accurately defined. The patient underwent a successful surgical resection of the pericardial cyst, microscopic histopathological examination was compatible with a bronchogenic cyst, a very rare congenital malformation. The article discusses the rarity of bronchogenic cysts in the pericardium and the importance of accurate diagnosis and appropriate treatment.
RESUMO
Covered stent correction of a superior sinus venosus atrial septal defect is increasingly performed as an alternative to surgical repair. While sinus node dysfunction requiring pacemaker implantation may be required after surgical repair, this has not been previously reported after covered stent implantation. We reviewed the experience in two interventional centers. Balloon inflation in the superior vena cava was used to confirm the anomalous pulmonary vein drainage would be unobstructed after stent implantation. During balloon testing in 62 consecutive patients, we assessed gradients across the pulmonary vein to left atrium while monitoring the rhythm. We observed the outcomes after covered stent correction in 51 patients. In a single patient, significant bradycardia and pauses developed on repeat balloon testing and the procedure was abandoned without stent implantation. In another patient, there was no sign of sinus node dysfunction during balloon testing but several hours after stent implantation, the patient became symptomatic from sinus bradycardia and pauses and had a pacemaker implanted 3 days later. Over a year later there are some signs of improvement in sinus node function. While sinus node dysfunction has not been described previously during balloon testing or after stent implantation, this report demonstrates for the first time that it may occur. Larger registries are therefore required to monitor for this uncommon complication.
Assuntos
Fibrilação Atrial , Comunicação Interatrial , Dispositivo para Oclusão Septal , Humanos , Dispositivo para Oclusão Septal/efeitos adversos , Resultado do Tratamento , Ecocardiografia Transesofagiana , Comunicação Interatrial/diagnóstico por imagem , Comunicação Interatrial/cirurgia , Cateterismo CardíacoRESUMO
INTRODUCTION AND OBJECTIVES: Mitral valve (MV) prolapse is highly prevalent in patients with atrial septal defect (ASD). Abnormal left ventricular geometry has been proposed as the main mechanism of MV prolapse in ASD, however, the changes in the morphology of the MV apparatus remain to be clarified. Our aim was to assess the MV geometry in patients with ASD and MV prolapse. METHODS: We evaluated 99 patients (73% female, median age 40 years) with ASD who underwent a three-dimensional transesophageal echocardiogram. Three-dimensional analysis of the MV was done using dedicated automated software. Transthoracic echocardiographic parameters were assessed post ASD closure in 28 patients. RESULTS: MV prolapse was found in 39% of patients. Although smaller left ventricular dimensions and greater interatrial shunt were found in patients with MV prolapse compared with those without prolapse, there was no difference in the subvalvular parameters. MV prolapse was associated with larger mitral anterior-posterior diameter, anterolateral-posteromedial diameter, anterior perimeter, posterior perimeter, total perimeter, and anterior leaflet area (all p < 0.05). Mitral regurgitation was more frequent in patients with MV prolapse (80 vs. 48%, p = 0.002). CONCLUSIONS: In patients with ASD, the main mechanism of MV prolapse is the presence of an organic primary process of the MV apparatus (excessive anterior mitral leaflet tissue and mitral annular enlargement).
Assuntos
Ecocardiografia Tridimensional , Comunicação Interatrial , Insuficiência da Valva Mitral , Prolapso da Valva Mitral , Adulto , Ecocardiografia , Ecocardiografia Tridimensional/métodos , Ecocardiografia Transesofagiana/métodos , Feminino , Comunicação Interatrial/complicações , Comunicação Interatrial/diagnóstico por imagem , Humanos , Masculino , Insuficiência da Valva Mitral/complicações , Prolapso da Valva Mitral/complicações , Prolapso da Valva Mitral/diagnóstico por imagem , ProlapsoRESUMO
A 56-year-old patient with rheumatic heart disease and atrial fibrillation underwent mitral valve replacement with a mechanical prosthesis. The 3D perioperative echocardiogram showed an intermittent immobile medial disk without hemodynamic repercussion in the intensive care unit. The patient was taken back to the operating room and surgeons could not identify the cause. An enlarged left atrium and the size of the prosthetic valve was thought to have precipitated this condition. The heart team decided a biological prosthetic valve replacement would be performed. This case emphasizes the important role of the perioperative 3D echocardiogram in the detection of immediate surgical complications.
Assuntos
Ecocardiografia Tridimensional , Implante de Prótese de Valva Cardíaca , Próteses Valvulares Cardíacas , Insuficiência da Valva Mitral , Estenose da Valva Mitral , Cardiopatia Reumática , Próteses Valvulares Cardíacas/efeitos adversos , Implante de Prótese de Valva Cardíaca/efeitos adversos , Implante de Prótese de Valva Cardíaca/métodos , Humanos , Pessoa de Meia-Idade , Valva Mitral/diagnóstico por imagem , Valva Mitral/cirurgia , Insuficiência da Valva Mitral/diagnóstico por imagem , Insuficiência da Valva Mitral/etiologia , Insuficiência da Valva Mitral/cirurgia , Estenose da Valva Mitral/diagnóstico por imagem , Estenose da Valva Mitral/etiologia , Estenose da Valva Mitral/cirurgia , Cardiopatia Reumática/complicações , Cardiopatia Reumática/cirurgiaRESUMO
Background: In patients with Ischemia and non-obstructive coronary artery stenosis (INOCA) wall motion is rarely abnormal during stress echocardiography (SE). Our aim was to determine if patients with INOCA and reduced coronary flow velocity reserve (CVFR) have altered cardiac mechanics using two-dimensional speckle-tracking echocardiography (2DSTE) during SE. Methods: In a prospective, multicenter, international study, we recruited 135 patients with INOCA. Overall, we performed high dose (0.84 mg/kg) dipyridamole SE with combined assessment of CVFR and 2DSTE. The population was divided in patients with normal CVFR (>2, group 1, n = 95) and abnormal CVFR (≤2, group 2, n = 35). Clinical and 2DSTE parameters were compared between groups. Results: Feasibility was high for CFVR (98%) and 2DSTE (97%). A total of 130 patients (mean age 63 ± 12 years, 67 women) had complete flow and strain data. The two groups showed similar 2DSTE values at rest. At peak SE, Group 1 patients showed lower global longitudinal strain (p < 0.007), higher mechanical dispersion (p < 0.0005), lower endocardial (p < 0.001), and epicardial (p < 0.0002) layer specific strain. Conclusions: In patients with INOCA, vasodilator SE with simultaneous assessment of CFVR and strain is highly feasible. Coronary microvascular dysfunction is accompanied by an impairment of global and layer-specific deformation indices during stress.
Assuntos
Ecocardiografia Tridimensional , Ruptura do Septo Ventricular , Ecocardiografia Tridimensional/métodos , Ecocardiografia Transesofagiana , Humanos , Tomografia Computadorizada por Raios X , Transiluminação , Ruptura do Septo Ventricular/diagnóstico por imagem , Ruptura do Septo Ventricular/etiologia , Ruptura do Septo Ventricular/cirurgiaRESUMO
ABSTRACT Background: Fukuyama congenital muscular dystrophy (FCMD) is the most common form of a group of autosomal recessive disorders characterized by altered α-dystroglycan glycosylation and caused by FKTN gene mutations. However, mutations of this gene may cause a broad range of phenotypes, including Walker-Warburg syndrome, muscle-brain-eye disease, FCMD, limb-girdle muscular dystrophy without mental retardation, and cardiomyopathy with no or minimal skeletal muscle weakness. Objective: Our purpose was to describe two siblings who died at a young age with dilated cardiomyopathy (DCM), no muscle weakness, or atrophy, and were homozygous for a FKTN missense mutation. Methods: Site-directed next-generation sequencing (NGS) was performed. Pathogenicity of variants of interest was established according to the American College of Medical Genetics (ACMG) criteria, and all available first-degree relatives were screened for mutations by Sanger sequencing. Results: NGS revealed a homozygous FKTN variant in the index case (p.Gly424Ser, rs752358445), classified as likely pathogenic by ACMG criteria. Both parents and an unaffected brother were heterozygous carriers. Since the siblings had no apparent skeletal muscle weakness or central nervous system involvement, FKTN mutations were not initially suspected. Conclusions: This is the first report demonstrating that heterozygous individuals for the FKTN p.Gly424Ser mutation were healthy, while two homozygous brothers suffered severe DCM, strongly suggesting that this FKTN mutation is a rare cause of autosomal recessive DCM.
RESUMO
OBJECTIVES: The aim of this study was to analyze whether right ventricular free wall longitudinal strain (RVFWSL) could be a predictor of low-cardiac-output syndrome (LCOS) after surgical aortic valve replacement (SAVR) in patients with left ventricular ejection fraction ≥40%. DESIGN: Prospective, observational study. SETTING: The study was conducted at a third level university hospital. PARTICIPANTS: The study comprised 75 patients with severe aortic stenosis and LVEF ≥40% who underwent SAVR. The primary outcome was the occurrence of LCOS, and secondary outcomes were in-hospital mortality, hospital stay, or vasoplegic syndrome. INTERVENTIONS: Patients were divided into two groups (LCOS and no LCOS), and RVFWSL was analyzed to determine whether it is a predictor for LCOS. In addition, a receiver operating characteristic curve also was constructed, and the best cutoff value to predict LCOS was found. Furthermore, the reproducibility of RVFWSL measurements was evaluated. MEASUREMENT AND MAIN RESULTS: The incidence of LCOS was 20% in the present study's cohort. After multivariate analysis, cross-clamp time (odds ratio 1.06, 95% confidence interval 1.02-1.11; pâ¯=â¯0.002) and RVFWSL (odds ratio 1.41, 95% confidence interval 1.07-1.87; pâ¯=â¯0.015) were the only predictors of LCOS. However, RVFWSL did not show association with secondary outcomes (p > 0.05 for all). The area under the curve of RVFWSL to predict LCOS was 0.75, and the best cutoff value was -17.3%, with a sensitivity of 86.7% and specificity of 61.7%. CONCLUSIONS: RVFWSL seems to be a predictor of LCOS in patients with severe aortic stenosis and LVEF ≥40% undergoing SAVR. RVFWSL less than -17.3% may identify patients at increased risk for LCOS.
Assuntos
Estenose da Valva Aórtica , Implante de Prótese de Valva Cardíaca , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/cirurgia , Estenose da Valva Aórtica/diagnóstico por imagem , Estenose da Valva Aórtica/cirurgia , Implante de Prótese de Valva Cardíaca/efeitos adversos , Humanos , Estudos Prospectivos , Reprodutibilidade dos Testes , Fatores de Risco , Volume Sistólico , Resultado do Tratamento , Função Ventricular EsquerdaRESUMO
An enlarged left atrial volume index (LAVI) at rest mirrors increased LA pressure and/or impairment of LA function. A cardiovascular stress may acutely modify left atrial volume (LAV) within minutes. Aim of this study was to assess the feasibility and functional correlates of LAV-stress echocardiography (SE) Out of 514 subjects referred to 10 quality-controlled labs, LAV-SE was completed in 490 (359 male, age 67 ± 12 years) with suspected or known chronic coronary syndromes (n = 462) or asymptomatic controls (n = 28). The utilized stress was exercise in 177, vasodilator in 167, dobutamine in 146. LAV was measured with the biplane disk summation method. SE was performed with the ABCDE protocol. The intra-observer and inter-observer LAV variability were 5% and 8%, respectively. ∆-LAVI changes (stress-rest) were negatively correlated with resting LAVI (r = - 0.271, p < 0.001) and heart rate reserve (r = -.239, p < 0.001). LAV-dilators were defined as those with stress-rest increase ≥ 6.8 ml/m2, a cutoff derived from a calculated reference change value above the biological, analytical and observer variability of LAVI. LAV dilation occurred in 56 patients (11%), more frequently with exercise (16%) and dipyridamole (13%) compared to dobutamine (4%, p < 0.01). At multivariable logistic regression analysis, B-lines ≥ 2 (OR: 2.586, 95% CI = 1.1293-5.169, p = 0.007) and abnormal contractile reserve (OR: 2.207, 95% CI = 1.111-4.386, p = 0.024) were associated with LAV dilation. In conclusion, LAV-SE is feasible with high success rate and low variability in patients with chronic coronary syndromes. LAV dilation is more likely with reduced left ventricular contractile reserve and pulmonary congestion.
Assuntos
Função do Átrio Esquerdo , Pressão Atrial , Doença da Artéria Coronariana/diagnóstico por imagem , Ecocardiografia Doppler de Pulso , Ecocardiografia sob Estresse , Átrios do Coração/diagnóstico por imagem , Agonistas de Receptores Adrenérgicos beta 1/administração & dosagem , Idoso , Idoso de 80 Anos ou mais , Argentina , Brasil , Doença Crônica , Doença da Artéria Coronariana/fisiopatologia , Europa (Continente) , Exercício Físico , Estudos de Viabilidade , Feminino , Átrios do Coração/fisiopatologia , Humanos , Itália , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Estudos Prospectivos , Síndrome , Vasodilatadores/administração & dosagemRESUMO
BACKGROUND: Fukuyama congenital muscular dystrophy (FCMD) is the most common form of a group of autosomal recessive disorders characterized by altered α-dystroglycan glycosylation and caused by FKTN gene mutations. However, mutations of this gene may cause a broad range of phenotypes, including Walker-Warburg syndrome, muscle-brain-eye disease, FCMD, limbgirdle muscular dystrophy without mental retardation, and cardiomyopathy with no or minimal skeletal muscle weakness. OBJECTIVE: Our purpose was to describe two siblings who died at a young age with dilated cardiomyopathy (DCM), no muscle weakness, or atrophy, and were homozygous for a FKTN missense mutation. METHODS: Site-directed next-generation sequencing (NGS) was performed. Pathogenicity of variants of interest was established according to the American College of Medical Genetics (ACMG) criteria, and all available first-degree relatives were screened for mutations by Sanger sequencing. RESULTS: NGS revealed a homozygous FKTN variant in the index case (p.Gly424Ser, rs752358445), classified as likely pathogenic by ACMG criteria. Both parents and an unaffected brother were heterozygous carriers. Since the siblings had no apparent skeletal muscle weakness or central nervous system involvement, FKTN mutations were not initially suspected. CONCLUSIONS: This is the first report demonstrating that heterozygous individuals for the FKTN p.Gly424Ser mutation were healthy, while two homozygous brothers suffered severe DCM, strongly suggesting that this FKTN mutation is a rare cause of autosomal recessive DCM.
RESUMO
BACKGROUND: OSA conveys worse clinical outcomes in patients with coronary artery disease. The STOP-BANG score is a simple tool that evaluates the risk of OSA and can be added to the large number of clinical variables and scores that are obtained during the management of patients with myocardial infarction (MI). Currently, machine learning (ML) is able to select and integrate numerous variables to optimize prediction tasks. RESEARCH QUESTION: Can the integration of STOP-BANG score with clinical data and scores through ML better identify patients who experienced an in-hospital cardiovascular event after acute MI? STUDY DESIGN AND METHODS: This is a prospective observational cohort study of 124 patients with acute MI of whom the STOP-BANG score classified 34 as low (27.4%), 30 as intermediate (24.2%), and 60 as high (48.4%) OSA-risk patients who were followed during hospitalization. ML implemented feature selection and integration across 47 variables (including STOP-BANG score, Killip class, GRACE score, and left ventricular ejection fraction) to identify those patients who experienced an in-hospital cardiovascular event (ie, death, ventricular arrhythmias, atrial fibrillation, recurrent angina, reinfarction, stroke, worsening heart failure, or cardiogenic shock) after definitive MI treatment. Receiver operating characteristic curves were used to compare ML performance against STOP-BANG score, Killip class, GRACE score, and left ventricular ejection fraction, independently. RESULTS: There were an increasing proportion of cardiovascular events across the low, intermediate, and high OSA risk groups (P = .005). ML selected 7 accessible variables (ie, Killip class, leukocytes, GRACE score, c reactive protein, oxygen saturation, STOP-BANG score, and N-terminal prohormone of B-type natriuretic peptide); their integration outperformed all comparators (area under the curve, 0.83 [95% CI, 0.74-0.90]; P < .01). INTERPRETATION: The integration of the STOP-BANG score into clinical evaluation (considering Killip class, GRACE score, and simple laboratory values) of subjects who were admitted for an acute MI because of ML can significantly optimize the identification of patients who will experience an in-hospital cardiovascular event.
Assuntos
Doenças Cardiovasculares/etiologia , Aprendizado de Máquina , Infarto do Miocárdio/complicações , Medição de Risco/métodos , Idoso , Doenças Cardiovasculares/epidemiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Apneia Obstrutiva do Sono/complicaçõesRESUMO
To investigate the role of right ventricular free wall strain (RVFWSL) to predict low functional capacity in repaired tetralogy of Fallot (rTOF). We prospectively enrolled 33 patients with rTOF with moderate to severe PR who underwent rest and peak exercise echocardiography on a semisupine cycloergometer. Conventional function and strain imaging parameters of both ventricles were measured. Patients performing < 7 METS were defined to have low functional capacity. Logistic regression was used to identify parameters associated with low functional capacity. Eleven patients (33.3%) had low functional capacity. These patients were shorter (height 155 ± 7 vs 163 ± 9 cm, p = 0.023), more frequently female (27.3 vs 72.7%, p = 0.024) and had history of Blalock-Taussig shunt (45.5 vs 9.1%, p = 0.027). On multivariate analysis RVFWSL was the only predictor of low functional capacity OR 1.39 (CI 95%, 1.06-1.83., p = 0.018) per % change. A RVFWSL < 17% (absolute value) had an AUC of 0.785, sensitivity of 81.8% and specificity of 77.3% to predict low functional capacity. Right ventricular free wall strain is an independent predictor of low functional capacity in repaired tetralogy of Fallot with moderate to severe PR. A value < 17% might be useful in deciding when to perform pulmonary valve replacement, when functional capacity cannot be objectively measured.
Assuntos
Procedimentos Cirúrgicos Cardíacos , Ecocardiografia Doppler em Cores , Ecocardiografia sob Estresse , Teste de Esforço , Insuficiência da Valva Pulmonar/diagnóstico por imagem , Tetralogia de Fallot/cirurgia , Função Ventricular Direita , Adolescente , Adulto , Ciclismo , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Tomada de Decisão Clínica , Feminino , Implante de Prótese de Valva Cardíaca , Humanos , Masculino , Seleção de Pacientes , Valor Preditivo dos Testes , Estudos Prospectivos , Insuficiência da Valva Pulmonar/etiologia , Insuficiência da Valva Pulmonar/fisiopatologia , Insuficiência da Valva Pulmonar/cirurgia , Recuperação de Função Fisiológica , Índice de Gravidade de Doença , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/fisiopatologia , Resultado do Tratamento , Adulto JovemAssuntos
Átrios do Coração/cirurgia , Ventrículos do Coração/cirurgia , Radiografia Intervencionista , Dispositivo para Oclusão Septal , Ultrassonografia de Intervenção , Adulto , Pontos de Referência Anatômicos , Ecocardiografia Doppler em Cores , Ecocardiografia Transesofagiana , Feminino , Fluoroscopia , Átrios do Coração/diagnóstico por imagem , Átrios do Coração/lesões , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/lesões , Humanos , Doença Iatrogênica , Imageamento TridimensionalRESUMO
OBJECTIVES: This study sought to evaluate the potential clinical impact of using 3-dimensional echocardiography (3DE) to measure left ventricular ejection fraction (LVEF) in patients considered for implantable cardioverter-defibrillator (ICD) implantation and to assess the predictive value of 3DE LVEF for arrhythmic events. BACKGROUND: ICD therapy is currently recommended to prevent sudden cardiac death in patients with symptomatic heart failure and LVEF ≤35%, and in asymptomatic patients with ischemic heart disease and LVEF ≤30%. Two-dimensional echocardiography (2DE) is currently used to calculate LVEF. However, 3DE has been reported to be more reproducible and accurate than 2DE to measure LVEF. METHODS: The study prospectively enrolled 172 patients with LV dysfunction (71% ischemic). Both 2DE and 3DE LVEF were obtained during the same study. The outcome was the occurrence of major arrhythmic events (sudden cardiac death, aborted cardiac arrest, appropriate ICD therapy). RESULTS: After a median follow up of 56 (range 18 to 65) months, major arrhythmic events occurred in 30% of the patients. Compared with 2DE, 3DE changed the assignment above or below the LVEF thresholds for ICD implantation in 20% of patients, most of them having 2DE LVEFs within ± 10% from threshold. By cause-specific hazard model, 3DE LVEF was the only independent predictor of the occurrence of major arrhythmic events. CONCLUSIONS: LVEF by 3DE was an independent predictor of major arrhythmic events and improved arrhythmic risk prediction in patients with LV dysfunction. When compared with 2DE LVEF, 3DE measurement of LVEF may change the decision to implant an ICD in a sizable number of patients.
Assuntos
Arritmias Cardíacas/etiologia , Ecocardiografia Tridimensional , Volume Sistólico , Disfunção Ventricular Esquerda/diagnóstico por imagem , Função Ventricular Esquerda , Idoso , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/fisiopatologia , Arritmias Cardíacas/terapia , Morte Súbita Cardíaca/etiologia , Morte Súbita Cardíaca/prevenção & controle , Desfibriladores Implantáveis , Cardioversão Elétrica/instrumentação , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prognóstico , Estudos Prospectivos , Reprodutibilidade dos Testes , Medição de Risco , Fatores de Risco , Fatores de Tempo , Disfunção Ventricular Esquerda/complicações , Disfunção Ventricular Esquerda/fisiopatologiaRESUMO
Coexisting bicuspid aortic and pulmonary valves is an extremely rare condition, and there have been few published cases. Diagnosis of bicuspid aortic valve is straightforward with 2D echocardiography; however, analysis of the morphology of the pulmonary valve is challenging. In this study, we report on a case of a 32-year-old man with bicuspid aortic and pulmonary valves diagnosed by 2D and 3D transthoracic echocardiography. The enlarged pulmonary artery without any obvious etiology led us to suspect a pulmonary valve anomaly; thus, we comprehensively evaluated it with 2D and 3D echocardiography, which confirmed the diagnosis of bicuspid pulmonary valve.