RESUMO
Wilson disease (WD) is a rare genetic condition that results from a build-up of copper in the body. It requires life-long treatment and is mainly characterized by hepatic and neurological features. Copper accumulation has been reported to be related to the occurrence of heart disease, although little is known regarding this association. We have conducted a systematic review of the literature to document the association between WD and cardiac involvement. Thirty-two articles were retained. We also described three cases of sudden death. Cardiac manifestations in WD include cardiomyopathy (mainly left ventricular (LV) remodeling, hypertrophy, and LV diastolic dysfunction, and less frequently LV systolic dysfunction), increased levels of troponin, and/or brain natriuretic peptide, electrocardiogram (ECG) abnormalities, and rhythm or conduction abnormalities, which can be life-threatening. Dysautonomia has also been reported. The mechanism of cardiac damage in WD has not been elucidated. It may be the result of copper accumulation in the heart, and/or it could be due to a toxic effect of copper, resulting in the release of free oxygen radicals. Patients with signs and/or symptoms of cardiac involvement or who have cardiovascular risk factors should be examined by a cardiologist in addition to being assessed by their interdisciplinary treating team. Furthermore, ECG, cardiac biomarkers, echocardiography, and 24-hours or more of Holter monitoring at the diagnosis and/or during the follow-up of patients with WD need to be evaluated. Cardiac magnetic resonance imaging, although not always available, could also be a useful diagnostic tool, allowing assessment of the risk of ventricular arrhythmias and further guidance of the cardiac workup.
Assuntos
Arritmias Cardíacas/etiologia , Cardiomiopatias/etiologia , Morte Súbita Cardíaca/etiologia , Degeneração Hepatolenticular/complicações , Disautonomias Primárias/etiologia , Adulto , Arritmias Cardíacas/fisiopatologia , Autopsia , Cardiomiopatias/fisiopatologia , Cobre/sangue , Cobre/metabolismo , Ecocardiografia , Eletrocardiografia Ambulatorial , Feminino , Degeneração Hepatolenticular/genética , Degeneração Hepatolenticular/fisiopatologia , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Disautonomias Primárias/fisiopatologiaRESUMO
BACKGROUND: Randomized studies have reported low rates of atrial fibrillation (AF) after patent foramen ovale (PFO) closure (<6%) but have relied on patient-reported symptomatic episodes, so the true incidence and timing of AF after PFO closure remain unknown. OBJECTIVES: The aim of this study was to prospectively determine the incidence, timing, and determinants of supraventricular arrhythmia following PFO closure on the basis of loop recorder monitoring. METHODS: Cardiac monitoring was proposed to all patients after PFO closure from June 2018 to October 2021 at a single center by means of implantable loop recorder monitoring in patients considered at higher risk for AF (age ≥ 55 years, associated cardiovascular risk factors, prior palpitations, or documented supraventricular ectopic activity) or 4-week external loop recorder monitoring in other patients. The primary endpoint was the incidence of AF, atrial flutter, or supraventricular tachycardia lasting >30 seconds within 28 days of the procedure. Determinants of the primary endpoint were assessed using a stepwise logistic regression model. RESULTS: A total of 225 patients were included. The primary endpoint occurred in 47 patients (20.9%), including 13 (9.9%) and 24 (28.9%) among patients monitored with external loop recorders and implantable loop recorders, respectively. Overall, the median delay from procedure to arrhythmia was 14.0 days (IQR: 6.5-19.0 days), and one-half of these patients reported symptomatic episodes. Determinants of the primary endpoint were older age (adjusted OR: 1.67 per 10-year increase; 95% CI: 1.18-2.36), device left disc diameter ≥25 mm (adjusted OR: 2.67; 95% CI: 1.19-5.98) and male sex (adjusted OR: 4.78; 95% CI: 1.96-11.66). CONCLUSIONS: Using loop recorder monitoring for ≥28 days, supraventricular arrhythmia was diagnosed in 1 in 5 patients, with a median delay of 14 days, suggesting that this postprocedural event has so far been underestimated.
Assuntos
Fibrilação Atrial , Flutter Atrial , Forame Oval Patente , Dispositivo para Oclusão Septal , Acidente Vascular Cerebral , Humanos , Masculino , Pessoa de Meia-Idade , Forame Oval Patente/diagnóstico por imagem , Forame Oval Patente/terapia , Forame Oval Patente/complicações , Acidente Vascular Cerebral/etiologia , Resultado do Tratamento , Fibrilação Atrial/diagnóstico , Fibrilação Atrial/epidemiologia , Fibrilação Atrial/terapia , Cateterismo Cardíaco/efeitos adversos , Cateterismo Cardíaco/métodos , Dispositivo para Oclusão Septal/efeitos adversosRESUMO
BACKGROUND: Left ventricular ejection fraction (LVEF) and end diastolic volume (EDV) are measured using Simpson's biplane (SB), 3-dimensional method (3DE), and speckle tracking (STE). Comparisons between methods in routine practice are limited. Our purpose was to compare and to determine the correlations between these three methods in clinical setting. METHODS: LVEF and EDV were measured by three methods in 474 consecutive patients and compared using multiple Bland-Altman (BA) plots. The correlations (R) between methods were calculated. RESULTS: Median (IQR) LVEF_SB, LVEF_STE, and LVEF_3DE were 63.0% (60-69)%, 61% (57-65)%, and 62% (57-68)%. Median (IQR) EDV_SB, EDV_STE, and EDV_3DE were 85 ml (71-106) ml, 82 ml (69-100) ml, and 73 ml (59-89) ml. R between LVEF_SB and LVEF_3DE was 0.65 when echogenicity was good and 0.43 when poor. R for EDV_SB and EDV_3DE was 0.75 when echogenicity was good and 0.45 when poor. On BA analysis, biases were acceptable (<3.5% for LVEF) but limits of agreement (LOA) were large: 95% of the differences were between -15.4% and +18.8% for LVEF as evaluated by SB in comparison with 3DE, with a bias of 1.7%. In the comparison EDV_SB and EDV_3DE, the bias was 14 ml and the LOA were between -24 ml and +53 ml. On linear regressions, LVEF_3DE = 17.92 + 0.69 LVEF_SB and EDV_3DE = 18.94 + 0.63 EDV_SB. CONCLUSIONS: The three methods were feasible and led to acceptable bias but large LOA. Although these methods are not interchangeable, our results allow 3DE value prediction from SB, the most commonly used method.
RESUMO
BACKGROUND: COVID-19 interacts at multiple levels with the cardiovascular system. The prognosis of COVID-19 infection is known to be worse for patients with underlying cardiovascular diseases. Furthermore, the virus is responsible for many cardiovascular complications. Myocardial injury may affect up to 20% of the critically ill patients. However, echocardiography's impact on the management of patients affected by COVID-19 remains unknown. OBJECTIVES: To explore echocardiography's impact on the management of COVID-19 patients. METHODS: This study was conducted from March 24th to April 14th, 2020, in a single center at Adolphe de Rothschild Foundation Hospital, Paris, France. All consecutive inpatients with laboratory and/or CT COVID-19 diagnosis were included in this study. Patients' characteristics (clinical, biological, and imaging) and treatment change induced by echocardiography were collected and analyzed. Patients with and without treatment change induced by echocardiography were compared. RESULTS: A total of 56 echocardiographies in 42 patients with highly suspected or confirmed COVID-19 were included in the final analyses. The median age was 66 (IQR 60.5-74). Echocardiography induced a treatment change in 9 cases (16%). The analyzed clinical data were not associated with any treatment change induced by echocardiography. D-dimer and Troponin levels were the only biological predictors of the induced treatment change. On echocardiography, higher systolic pulmonary arterial pressure and documented cardiac thrombi were associated with treatment changes in these patients. CONCLUSIONS: Echocardiography may be useful for the management of selected COVID-19 patients, especially those with elevated D-Dimer and Troponin levels, in up to 16% of patients.
Assuntos
Coartação Aórtica/diagnóstico por imagem , Hemorragia Subaracnóidea/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Angiografia , Coartação Aórtica/complicações , Coartação Aórtica/patologia , Diagnóstico Diferencial , Cefaleia/etiologia , Cefaleia/fisiopatologia , Humanos , Masculino , Hemorragia Subaracnóidea/etiologia , Hemorragia Subaracnóidea/patologia , Hemorragia Subaracnóidea/fisiopatologia , Adulto JovemRESUMO
BACKGROUND: Transthoracic echocardiography is the most commonly used tool for the detection of left ventricular wall motion (LVWM) abnormalities using "naked eye evaluation". This subjective and operator-dependent technique requires a high level of clinical training and experience. Two-dimensional speckle-tracking echocardiography (2D-STE), which is less operator-dependent, has been proposed for this purpose. However, the role of on-line segmental longitudinal peak systolic strain (LPSS) values in the prediction of LVWM has not been fully evaluated. AIM: To test segmental LPSS for predicting LVWM abnormalities in routine echocardiography laboratory practice. METHODS: LVWM was evaluated by an experienced cardiologist, during routine practice, in 620 patients; segmental LPSS values were then calculated. RESULTS: In this work, reflecting real life, 99.6% of segments were successfully tracked. Mean (95% confidence interval [CI]) segmental LPSS values for normal basal (n=3409), mid (n=3468) and apical (n=3466) segments were -16.7% (-16.9% to -16.5%), -18.2% (-18.3% to -18.0%) and -21.1% (-21.3% to -20.9%), respectively. Mean (95% CI) segmental LPSS values for hypokinetic basal (n=114), mid (n=116) and apical (n=90) segments were -7.7% (-9.0% to -6.3%), -10.1% (-11.1% to -9.0%) and -9.3% (-10.5% to -8.1%), respectively. Mean (95% CI) segmental LPSS values for akinetic basal (n=128), mid (n=95) and apical (n=91) segments were -6.6% (-8.0% to -5.1%), -6.1% (-7.7% to -4.6%) and -4.2% (-5.4% to -3.0%), respectively. LPSS allowed the differentiation between normal and abnormal segments at basal, mid and apical levels. An LPSS value≥-12% detected abnormal segmental motion with a sensitivity of 78% for basal, 70% for mid and 82% for apical segments. CONCLUSIONS: Segmental LPSS values may help to differentiate between normal and abnormal left ventricular segments.
Assuntos
Ecocardiografia/métodos , Ventrículos do Coração/diagnóstico por imagem , Sístole/fisiologia , Disfunção Ventricular Esquerda/diagnóstico , Função Ventricular Esquerda/fisiologia , Feminino , Seguimentos , Ventrículos do Coração/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Disfunção Ventricular Esquerda/fisiopatologiaRESUMO
Although transesophageal echocardiographically derived parameters, notably spontaneous echocardiographic contrast (SEC) in the left atrium or left atrial appendage (LAA), are known predictors of embolism in atrial fibrillation, their value is less well known in patients who have lone atrial fibrillation (LAF). This study describes transesophageal echocardiographic findings and identifies clinical predictors of SEC in the left atrium or LAA in a cohort of patients who had LAF. Eighty-two patients who had LAF and 289 patients who had non-LAF and underwent transesophageal echocardiography were enrolled from July 1998 to March 2002. LAA abnormality was defined as the presence of an LAA area >5 cm(2), emptying or filling LAA velocities <25 cm/s, or the presence of SEC or thrombus in the left atrium or LAA; LAA abnormalities were significantly less frequent in patients who had LAF than in those who had non-LAF. SEC in the left atrium or LAA was significantly less frequent in patients who had LAF than in those who had non-LAF (29.3% vs 49.8%, respectively, p <0.001). In patients who had LAF, SEC in the left atrium or LAA was significantly (p <0.05) less frequent in patients who were =60 years old (17.9%) than in patients who were >60 years old (39.5%) and in patients who had paroxysmal LAF (5.9%) than in those who had persistent LAF (45.8%). On multivariate analysis, age and persistent LAF were the only clinical variables independently associated with SEC. Thus, transesophageal echocardiography detects thromboembolism risk markers in patients who have LAF. These abnormalities are less frequent in patients who have LAF than in those who are at low risk and have non-LAF; however, in patients who have LAF, older age and persistent atrial fibrillation are associated with these risk markers.
Assuntos
Fibrilação Atrial/diagnóstico por imagem , Ecocardiografia Transesofagiana , Embolia e Trombose Intracraniana/fisiopatologia , Idoso , Fibrilação Atrial/complicações , Distribuição de Qui-Quadrado , Feminino , Humanos , Embolia e Trombose Intracraniana/etiologia , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Estudos Prospectivos , Fatores de RiscoRESUMO
BACKGROUND: Transthoracic echocardiography (TTE) is the most commonly used method for measuring left ventricular ejection fraction (LVEF), but its reproducibility remains a matter of controversy. Speckle tracking echocardiography assesses myocardial deformation and left ventricular systolic function by measuring global longitudinal strain (GLS), which is more reproducible, but is not used routinely in hospital practice. AIM: To investigate the feasibility of on-line two-dimensional GLS in predicting LVEF during routine echocardiographic practice. METHODS: The analysis involved 507 unselected consecutive patients undergoing TTE between August 2012 and November 2013. Echocardiograms were performed by a single sonographer. Echogenicity was noted as good, moderate or poor. Simple linear regression was used to assess the relationship between LVEF and GLS, overall and according to quality of echogenicity. Receiver operating curve (ROC) analysis was used to identify the threshold GLS that predicts LVEF≤40%. RESULTS: Mean LVEF was 64±11% and GLS was -18.0±4.0%. A reasonable correlation was found between LVEF and GLS (r=-0.53; P<0.001), which was improved when echogenicity was good (r=-0.60; P<0.001). GLS explained 28.1% of the variation in LVEF, and for one unit decrease in GLS, a 1.45 unit increase in LVEF was expected. Correlations between LVEF and GLS were -0.51 for patients in sinus rhythm (n=490) and -0.86 in atrial fibrillation (n=17). Based on ROC analysis, the area under the curve was 0.97 for GLS≥-14%, allowing detection of LVEF≤40% with a sensitivity of 95% and specificity of 86%. CONCLUSION: Two-dimensional GLS is easy to obtain and accurately detects LVEF≤40% in unselected patients. GLS may be especially helpful when a suboptimal acoustic window makes LVEF measurement by Simpson's biplane method difficult and in atrial fibrillation patients with low heart rate variability.
Assuntos
Ecocardiografia Doppler , Volume Sistólico , Disfunção Ventricular Esquerda/diagnóstico por imagem , Disfunção Ventricular Esquerda/fisiopatologia , Função Ventricular Esquerda , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Área Sob a Curva , Fibrilação Atrial/diagnóstico por imagem , Fibrilação Atrial/fisiopatologia , Fenômenos Biomecânicos , Criança , Estudos de Viabilidade , Feminino , Frequência Cardíaca , Humanos , Interpretação de Imagem Assistida por Computador , Modelos Lineares , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Curva ROC , Reprodutibilidade dos Testes , Estresse MecânicoRESUMO
Rhabdomyomas are the most common benign cardiac tumours. They are often associated with tuberous sclerosis and can be diagnosed antenatally and postnatally by echocardiography. Rhabdomyomas tend to regress spontaneously and are not usually operated upon, unless they become obstructive or cause severe arrhythmias. We describe the case of a child with tuberous sclerosis who was admitted for the resection of a subependymal giant cell astrocytoma, in whom cardiac rhabdomyomas in the right ventricular outflow tract were diagnosed. These two kinds of tumours are well known in the setting of tuberous sclerosis.
Assuntos
Neoplasias Cardíacas/diagnóstico , Rabdomioma/diagnóstico , Esclerose Tuberosa/complicações , Criança , Ecocardiografia , Feminino , Neoplasias Cardíacas/complicações , Humanos , Rabdomioma/complicaçõesRESUMO
Transient left ventricular apical ballooning also called Takotsubo cardiomyopathy is a recently described cardiac syndrome. It often affects postmenopausal women having an acute physical or emotional stress, but it have also been described after convulsive status epilepticus. Although this association is rare and left ventricular function often recovers, it would be useful to the neurologist to be aware of the signs leading to this diagnosis, to improve the cardiac later care. Here we report a patient with partial status epilepticus in whom the diagnosis of transient left ventricular apical ballooning was made. We describe the diagnostic criteria and the potential complications that should be monitored.