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BACKGROUND: The Virtual Reality Headset (VRH) is a device aiming at improving patient's comfort by reducing pain and anxiety during medical interventions. Its interest during cardiac implantable electronic devices (CIED) implant procedures has not been studied. METHODS: We randomized consecutive patients admitted for pacemaker or Implantable Cardioverter Defibrillator (ICD) at our center to either standard analgesia care (STD-Group), or to VRH (VRH-Group). Patients in the STD-Group received intra-venous paracetamol (1 g) 60 min before the procedure, and local anesthesia was performed with lidocaine. For patients of the VRH-Group, VRH was used on top of standard care. We monitored patients' pain and anxiety using numeric rating scales (from 0 to 10) at the time of sub-cutaneous pocket creation, and during deep axillary vein puncture. Patient comfort during the procedure was assessed using a detailed questionnaire. Morphine consumption was also assessed. RESULTS: We randomized 61 patients to STD-Group (n = 31) or VRH-Group (n = 30). Pain and anxiety were lower in the VRH-Group during deep venous puncture (3.0 ± 2.0 vs. 4.8 ± 2.2, p = 0.002 and 2.4 ± 2.2 vs. 4.1 ± 2.4, p = 0.006) but not during pocket creation (p = 0.58 and p = 0.5). Morphine consumption was lower in the VRH-Group (1.6 ± 0.7 vs. 2.1 ± 1.1 mg; p = 0.041). Patients' overall comfort during procedure was similar in both groups. CONCLUSION: VRH use improved pain and anxiety control during deep venous puncture compared to standard analgesia care, and allowed morphine consumption reduction. However, pain and anxiety were similar at the time of sub-cutaneous pocket creation.
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Desfibriladores Implantáveis , Marca-Passo Artificial , Humanos , Ansiedade/prevenção & controle , Dor , Derivados da MorfinaRESUMO
INTRODUCTION: We describe an unusual case of atrial tachycardia (AT) emanating from the left atrial appendage body (LAA), successfully treated by chemical ablation. METHODS: A 66-year-old patient with cardiac amyloidosis and history of persistent atrial fibrillation ablation presented poorly tolerated AT with 1:1 atrioventricular nodal conduction at 135/min, despite amiodarone therapy. Three-dimensional mapping suggested a reentrant AT from the anterior aspect of the left LAA. RESULTS: The tachycardia could not be terminated with radiofrequency ablation. The LAA vein was then selectively catheterized and infused with Ethanol, resulting in immediate termination of tachycardia, without LAA isolation. No recurrence occurred at 12 months. CONCLUSION: Atrial tachycardias emanating from the LAA that are resistant to radiofrequency ablation may respond to chemical ablation of the LAA vein.
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Apêndice Atrial , Fibrilação Atrial , Ablação por Cateter , Taquicardia Supraventricular , Humanos , Idoso , Apêndice Atrial/diagnóstico por imagem , Apêndice Atrial/cirurgia , Resultado do Tratamento , Taquicardia , Fibrilação Atrial/complicações , Fibrilação Atrial/diagnóstico , Fibrilação Atrial/tratamento farmacológico , Ablação por Cateter/efeitos adversos , Ablação por Cateter/métodosRESUMO
Common atrium (CA), also called three-chambered heart, is one of the rare congenital anomalies, defined by a complete absence of the atrial septum, eventually associated with malformation of the atrioventricular (AV) valves. We report the case of a 57-year-old woman with CA complicated with Eisenmenger syndrome and inferior vena cava interruption, who suffered from symptomatic persistent atrial fibrillation (AF). She underwent an initial successful pulmonary vein isolation procedure. A repeat procedure for perivalvular atrial flutter was complicated with inadvertent complete AV block, due to unusual AV node location in this challenging anatomy.
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Fibrilação Atrial , Ablação por Cateter , Cardiopatias Congênitas , Defeitos dos Septos Cardíacos , Feminino , Humanos , Pessoa de Meia-Idade , Fibrilação Atrial/complicações , Fibrilação Atrial/cirurgia , Veia Cava Inferior/diagnóstico por imagem , Veia Cava Inferior/cirurgia , Veia Cava Inferior/anormalidades , Resultado do Tratamento , Eletrocardiografia , Cardiopatias Congênitas/cirurgia , Nó Atrioventricular , Ablação por Cateter/métodosRESUMO
Rationale: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection is associated with pulmonary endothelial dysfunction. There are limited data available on the outcomes of coronavirus disease (COVID-19) in patients with pulmonary hypertension (PH), a disease characterized by pulmonary endothelial dysfunction. Objectives: To describe characteristics and outcomes of patients with precapillary PH and COVID-19. Methods: We prospectively collected characteristics, management, and outcomes of adult patients with precapillary PH in the French PH network who had COVID-19 between February 1, 2020, and April 30, 2021. Clinical, functional, and hemodynamic characteristics of PH before COVID-19 were collected from the French PH registry. Measurements and Main Results: A total of 211 patients with PH (including 123 with pulmonary arterial hypertension, 47 with chronic thromboembolic PH, and 41 with other types of PH) experienced COVID-19, and 40.3% of them were outpatients, 32.2% were hospitalized in a conventional ward, and 27.5% were in an ICU. Among hospitalized patients (n = 126), 54.0% received corticosteroids, 37.3% high-flow oxygen, and 11.1% invasive ventilation. Right ventricular and acute renal failure occurred in 30.2% and 19.8% of patients, respectively. Fifty-two patients (all hospitalized) died from COVID-19. Overall mortality was 24.6% (95% CI [confidence interval], 18.8-30.5) and in-hospital mortality 41.3% (95% CI, 32.7-49.9). Nonsurvivors were significantly older, more frequently male and suffering comorbidities (diabetes, chronic respiratory diseases, systemic hypertension, chronic cardiac diseases, and/or chronic renal failure), and had more severe PH at their most recent evaluation preceding COVID-19 diagnosis (in terms of functional class and 6-minute-walk distance; all P < 0.05). Use of pulmonary arterial hypertension therapy was similar between survivors and nonsurvivors. Conclusions: COVID-19 in patients with precapillary PH was associated with a high in-hospital mortality. The typical risk factors for severe COVID-19 and severity of PH were associated with mortality in this population.
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COVID-19 , Hipertensão Pulmonar , Hipertensão Arterial Pulmonar , Adulto , COVID-19/complicações , Teste para COVID-19 , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/etiologia , Masculino , Estudos Prospectivos , SARS-CoV-2RESUMO
AIMS: The very early management of pulmonary embolism (PE), a part from antithrombotic treatment, has been little studied. Our aim was to compare the effects of diuretic therapy (DT) versus volume expansion (VE) in patients hospitalized for PE with RV dysfunction. METHODS AND RESULTS: We conducted a randomized open-label multicentric study including patients with intermediate high-risk PE. Patients were randomized between diuretics or saline infusion. The primary endpoint was time to troponin (Tp) normalization. Secondary endpoints were time to normalization of B-type natriuretic peptide (BNP), changes in echocardiographic RV function parameters and treatment tolerance. Sixty patients presenting intermediate high-risk PE were randomized. Thirty received DT and 30 VE. We noted no changes in Tp kinetics between the two groups. In contrast, faster normalization of BNP was obtained in the DT group: 56 [28-120] vs 108 [48-144] h: p = 0.05, with a shorter time to 50%-decrease from peak value 36 [24-48] vs 54 [41-67] h, p = 0.003 and a higher rate of patients with a lower BNP concentration within the first 12 h (42% vs 12% p < 0.001). RV echocardiographic parameters were unchanged between the groups. One dose 40 mg furosemide was well-tolerated and not associated with any serious adverse events. CONCLUSION: In the acute management of intermediate high-risk PE, initial therapy including diuretic treatment is well-tolerated and safe. Although changes in Tp kinetics and echocardiographic RV dysfunction parameters did not differ, normalization of BNP is achieved more quickly in the DT group. This finding, which need to be confirmed in trials with clinical end points, may reflects a rapid improvement in RV function using one dose 40 mg furosemide. TRIAL REGISTRY: Clinical Trial Registration NCT02531581.
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Diuréticos , Embolia Pulmonar , Disfunção Ventricular Direita , Doença Aguda , Biomarcadores , Diuréticos/uso terapêutico , Furosemida/uso terapêutico , Humanos , Peptídeo Natriurético Encefálico , Embolia Pulmonar/complicações , Embolia Pulmonar/diagnóstico por imagem , Embolia Pulmonar/tratamento farmacológico , Disfunção Ventricular Direita/diagnóstico por imagem , Disfunção Ventricular Direita/tratamento farmacológicoRESUMO
Rationale: The relationship between the initial treatment strategy and survival in pulmonary arterial hypertension (PAH) remains uncertain. Objectives: To evaluate the long-term survival of patients with PAH categorized according to the initial treatment strategy. Methods: A retrospective analysis of incident patients with idiopathic, heritable, or anorexigen-induced PAH enrolled in the French Pulmonary Hypertension Registry (January 2006 to December 2018) was conducted. Survival was assessed according to the initial strategy: monotherapy, dual therapy, or triple-combination therapy (two oral medications and a parenteral prostacyclin). Measurements and Main Results: Among 1,611 enrolled patients, 984 were initiated on monotherapy, 551 were initiated on dual therapy, and 76 were initiated on triple therapy. The triple-combination group was younger and had fewer comorbidities but had a higher mortality risk. The survival rate was higher with the use of triple therapy (91% at 5 yr) as compared with dual therapy or monotherapy (both 61% at 5 yr) (P < 0.001). Propensity score matching of age, sex, and pulmonary vascular resistance also showed significant differences between triple therapy and dual therapy (10-yr survival, 85% vs. 65%). In high-risk patients (n = 243), the survival rate was higher with triple therapy than with monotherapy or dual therapy, whereas there was no difference between monotherapy and double therapy. In intermediate-risk patients (n = 1,134), survival improved with an increasing number of therapies. In multivariable Cox regression, triple therapy was independently associated with a lower risk of death (hazard ratio, 0.29; 95% confidence interval, 0.11-0.80; P = 0.017). Among the 148 patients initiated on a parenteral prostacyclin, those on triple therapy had a higher survival rate than those on monotherapy or dual therapy. Conclusions: Initial triple-combination therapy that includes parenteral prostacyclin seems to be associated with a higher survival rate in PAH, particularly in the youngest high-risk patients.
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Anti-Hipertensivos/uso terapêutico , Hipertensão Arterial Pulmonar/tratamento farmacológico , Hipertensão Arterial Pulmonar/mortalidade , Administração Oral , Adulto , Idoso , Quimioterapia Combinada , Feminino , Seguimentos , França/epidemiologia , Humanos , Infusões Parenterais , Masculino , Pessoa de Meia-Idade , Pontuação de Propensão , Sistema de Registros , Estudos Retrospectivos , Análise de Sobrevida , Resultado do TratamentoRESUMO
BACKGROUND: Cardiac injury and myocarditis have been described in adults with coronavirus disease 2019 (COVID-19). Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in children is typically minimally symptomatic. We report a series of febrile pediatric patients with acute heart failure potentially associated with SARS-CoV-2 infection and the multisystem inflammatory syndrome in children as defined by the US Centers for Disease Control and Prevention. METHODS: Over a 2-month period, contemporary with the SARS-CoV-2 pandemic in France and Switzerland, we retrospectively collected clinical, biological, therapeutic, and early outcomes data in children who were admitted to pediatric intensive care units in 14 centers for cardiogenic shock, left ventricular dysfunction, and severe inflammatory state. RESULTS: Thirty-five children were identified and included in the study. Median age at admission was 10 years (range, 2-16 years). Comorbidities were present in 28%, including asthma and overweight. Gastrointestinal symptoms were prominent. Left ventricular ejection fraction was <30% in one-third; 80% required inotropic support with 28% treated with extracorporeal membrane oxygenation. Inflammation markers were suggestive of cytokine storm (interleukin-6 median, 135 pg/mL) and macrophage activation (D-dimer median, 5284 ng/mL). Mean BNP (B-type natriuretic peptide) was elevated (5743 pg/mL). Thirty-one of 35 patients (88%) tested positive for SARS-CoV-2 infection by polymerase chain reaction of nasopharyngeal swab or serology. All patients received intravenous immunoglobulin, with adjunctive steroid therapy used in one-third. Left ventricular function was restored in the 25 of 35 of those discharged from the intensive care unit. No patient died, and all patients treated with extracorporeal membrane oxygenation were successfully weaned. CONCLUSIONS: Children may experience an acute cardiac decompensation caused by severe inflammatory state after SARS-CoV-2 infection (multisystem inflammatory syndrome in children). Treatment with immunoglobulin appears to be associated with recovery of left ventricular systolic function.
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COVID-19/complicações , Insuficiência Cardíaca/virologia , Inflamação/virologia , Síndrome de Resposta Inflamatória Sistêmica/complicações , Adolescente , COVID-19/virologia , Criança , Feminino , Insuficiência Cardíaca/complicações , Insuficiência Cardíaca/tratamento farmacológico , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Inflamação/complicações , Inflamação/tratamento farmacológico , Masculino , Estudos Retrospectivos , Volume Sistólico/fisiologia , Síndrome de Resposta Inflamatória Sistêmica/virologia , Disfunção Ventricular Esquerda/tratamento farmacológico , Função Ventricular Esquerda/imunologiaRESUMO
The number of kidney transplant candidates with prosthetic heart valves (PHVs) is increasing. Yet, outcomes of kidney transplantation in these patients are still unclear. This is the first report of post-transplant outcomes in patients with PHVs at time of kidney transplantation. We conducted a matched cohort study among recipients from the multicentric and prospective DIVAT cohort to compare the outcomes in patients with left-sided PHVs at time of transplantation and a group of recipients without PHV matched according to age, dialysis time, initial disease, pretransplant DSA, diabetes, and cardiovascular events. Of 23 018 patients, 92 patients with PHVs were included and compared to 276 patients without PHV. Delayed graft function and postoperative bleeding occurred more frequently in patients with PHVs. Kidney graft survival was similar between groups. 5-year overall survival was 68.5% in patients with PHV vs. 87.9% in patients without PHV [HR, 2.72 (1.57-4.70), P = 0.0004]. Deaths from infection, endocarditis, and bleeding were more frequent in patients with PHV. Mechanical valves, but not bioprosthetic valves, were independent risk factors for mortality [HR, 2.89 (1.68-4.97), P = 0.0001]. Patients with PHV have high mortality rates after kidney transplantation. These data suggest that mechanical valves, but not biological valves, increase risks of post-transplant mortality.
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Transplante de Rim , Estudos de Coortes , Valvas Cardíacas , Humanos , Hemorragia Pós-Operatória , Estudos Prospectivos , Estudos RetrospectivosRESUMO
Rationale: Pulmonary hypertension (PH) associated with neurofibromatosis type 1 (NF1) is a rare and largely unknown complication of NF1.Objectives: To describe characteristics and outcomes of PH-NF1.Methods: We reported the clinical, functional, radiologic, histologic, and hemodynamic characteristics, response to pulmonary arterial hypertension (PAH)-approved drugs, and transplant-free survival of patients with PH-NF1 from the French PH registry.Measurements and Main Results: We identified 49 PH-NF1 cases, characterized by a female/male ratio of 3.9 and a median (minimum-maximum) age at diagnosis of 62 (18-82) years. At diagnosis, 92% were in New York Heart Association functional class III or IV. The 6-minute-walk distance was 211 (0-460) m. Pulmonary function tests showed low DlCO (30% [12-79%]) and severe hypoxemia (PaO2 56 [38-99] mm Hg). Right heart catheterization showed severe precapillary PH with a mean pulmonary artery pressure of 45 (10) mm Hg and a pulmonary vascular resistance of 10.7 (4.2) Wood units. High-resolution computed tomography images revealed cysts (76%), ground-glass opacities (73%), emphysema (49%), and reticulations (39%). Forty patients received PAH-approved drugs with a significant improvement in functional class and hemodynamic parameters. Transplant-free survival at 1, 3, and 5 years was 87%, 54%, and 42%, respectively, and four patients were transplanted. Pathologic assessment showed nonspecific interstitial pneumonia and major pulmonary vascular remodeling.Conclusions: PH-NF1 is characterized by a female predominance, a low DlCO, and severe functional and hemodynamic impairment. Despite a potential benefit of PAH treatment, prognosis remains poor, and double-lung transplantation is an option for eligible patients.
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Hipertensão Pulmonar/tratamento farmacológico , Hipertensão Pulmonar/fisiopatologia , Hipertensão Pulmonar/cirurgia , Neoplasias Pulmonares/fisiopatologia , Neurofibromatose 1/complicações , Neurofibromatose 1/genética , Neurofibromina 1/genética , Adolescente , Adulto , Feminino , França , Humanos , Hipertensão Pulmonar/etiologia , Transplante de Pulmão/métodos , Masculino , Pessoa de Meia-Idade , Fenótipo , Prognóstico , Adulto JovemRESUMO
BACKGROUND: Pulmonary embolism (PE) is a common life-threatening disease, with mortality related to right ventricular (RV) dysfunction. AIMS: To investigate the value of 3D global and regional RV strain in patients with acute PE and at 1 month, as compared to a control population. METHODS AND RESULTS: We conducted a longitudinal case-control prospective study, including 24 consecutive intermediate-risk PE patients. All patients underwent 2D and 3D transthoracic echocardiography within 12 hours of PE diagnosis and 1 month after hospital discharge. A control group was recruited, consisting of healthy volunteers matched on age and sex with PE patients. 3D RV echocardiographic sequences were analyzed by commercial RV-specific software and output meshes were post-processed to extract regional deformation. 3D echocardiographic 1-month follow-up was available in 18 patients. During acute PE, area strain was substantially altered in the RV free wall and within the trabecular septum. PE patients initially had RV dysfunction as assessed by 2D and 3D parameters. At follow-up, 2D parameters were restored compared to the control group, contrary to 3D RV area and circumferential strains. The McConnell's sign was identified in 83% of patients and was associated with reduced apical and global RV area strain. CONCLUSIONS: Our 3D RV strain study demonstrates an incomplete recovery of 3D strain parameters 1 month after an episode of intermediate-risk acute PE despite restored 2D parameters. Further studies are required to assess the prognostic role and implications of this residual RV strain impairment after PE.
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Embolia Pulmonar , Disfunção Ventricular Direita , Ecocardiografia , Humanos , Estudos Prospectivos , Embolia Pulmonar/diagnóstico por imagem , Disfunção Ventricular Direita/diagnóstico por imagem , Função Ventricular DireitaRESUMO
The long-term prospective multi-centre nationwide (French) observational study FRANCISCO will provide new information on perimembranous ventricular septal defect with left ventricular overload but no pulmonary hypertension in children older than 1 year. Outcomes will be compared according to treatment strategy (watchful waiting, surgical closure, or percutaneous closure) and anatomic features of the defect. The results are expected to provide additional guidance about the optimal treatment of this specific population, which is unclear at present. BACKGROUND: The management of paediatric isolated perimembranous ventricular septal defect (pmVSD) with left ventricle (LV) volume overload but no pulmonary arterial hypertension (PAH) remains controversial. Three therapeutic approaches are considered: watchful waiting, surgical closure, and percutaneous closure. We aim to investigate the long-term outcomes of these patients according to anatomic pmVSD characteristics and treatment strategy. METHODS: The Filiale de Cardiologie Pediatrique et Congénitale (FCPC) designed the FRANCISCO registry, a long-term prospective nationwide multi-centre observational cohort study sponsored by the French Society of Cardiology, which enrolled, over 2 years (20182020), patients older than 1 year who had isolated pmVSD with LV volume overload. Prevalent complications related to pmVSD at baseline were exclusion criteria. Clinical, echocardiographic, and functional data will be collected at inclusion then after 1, 5, and 10 years. A core lab will analyse all baseline echocardiographic data to depict anatomical pmVSD features. The primary outcome is the 5-year incidence of cardiovascular events (infective endocarditis, sub-aortic stenosis, aortic regurgitation, right ventricular outflow tract stenosis, tricuspid regurgitation, PAH, arrhythmia, stroke, haemolysis, heart failure, or death from a cardiovascular event). We plan to enrol 200 patients, given the 10% estimated 5-year incidence of cardiovascular events with a 95% confidence interval of ±5%. Associations linking anatomical pmVSD features and treatment strategy to the incidence of complications will be assessed. CONCLUSIONS: The FRANSCICO study will provide the long-term incidence of complications in patients older than 1 year with pmVSD and LV volume overload. The results are expected to improve guidance for treatment decisions.
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Insuficiência Cardíaca , Comunicação Interventricular , Dispositivo para Oclusão Septal , Cateterismo Cardíaco , Criança , Pré-Escolar , Comunicação Interventricular/epidemiologia , Comunicação Interventricular/cirurgia , Ventrículos do Coração/diagnóstico por imagem , Humanos , Estudos Observacionais como Assunto , Estudos Prospectivos , Resultado do TratamentoRESUMO
BACKGROUND & AIMS: Long-term outcomes in portopulmonary hypertension (PoPH) are poorly studied in the current era of pulmonary hypertension management. We analysed the effect of pulmonary arterial hypertension (PAH)-targeted therapies, survival and predictors of death in a large contemporary cohort of patients with PoPH. METHODS: Data from patients with PoPH consecutively enrolled in the French Pulmonary Hypertension Registry between 2007 and 2017 were collected. The effect of initial treatment strategies on functional class, exercise capacity and cardiopulmonary haemodynamics were analysed. Survival and its association with PAH- and hepatic-related characteristics were also examined. RESULTS: Six hundred and thirty-seven patients (mean age 55 ± 10 years; 58% male) were included. Fifty-seven percent had mild cirrhosis, i.e. Child-Pugh stage A. The median model for end-stage liver disease (MELD) score was 11 (IQR 9-15). Most patients (n = 474; 74%) were initiated on monotherapy, either with a phosphodiesterase-5 inhibitor (n = 336) or with an endothelin-receptor antagonist (n = 128); 95 (15%) were initiated on double oral combination therapy and 5 (1%) on triple therapy. After a median treatment time of 4.5 months, there were significant improvements in functional class (p <0.001), 6-minute walk distance (6MWD) (p <0.0001) and pulmonary vascular resistance (p <0.0001). Overall survival rates were 84%, 69% and 51% at 1, 3 and 5 years, respectively. Baseline 6MWD, sex, age and MELD score or Child-Pugh stage were identified as independent prognostic factors. Survival from PoPH diagnosis was significantly better in the subgroup of patients who underwent liver transplantation (92%, 83% and 81% at 1, 3 and 5 years, respectively). CONCLUSION: Survival of patients with PoPH is strongly associated with the severity of liver disease. Patients who underwent liver transplantation had the best long-term outcomes. LAY SUMMARY: Portopulmonary hypertension is defined by the presence of pulmonary arterial hypertension in the context of chronic liver disease and is characterized by progressive shortness of breath and exercise limitation. The presence of severe pulmonary arterial hypertension in liver transplant candidates represents a contraindication for such a surgery; however, treatments targeting pulmonary arterial hypertension are efficacious, allowing for safe transplantation and conferring good survival outcomes in those who undergo liver transplantation.
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Antagonistas dos Receptores de Endotelina/uso terapêutico , Hipertensão Portal , Cirrose Hepática , Inibidores da Fosfodiesterase 5/uso terapêutico , Hipertensão Arterial Pulmonar , Sistema Cardiovascular/fisiopatologia , Tolerância ao Exercício , Feminino , França/epidemiologia , Estado Funcional , Humanos , Hipertensão Portal/diagnóstico , Hipertensão Portal/mortalidade , Hipertensão Portal/fisiopatologia , Cirrose Hepática/diagnóstico , Cirrose Hepática/fisiopatologia , Cirrose Hepática/cirurgia , Transplante de Fígado/estatística & dados numéricos , Masculino , Pessoa de Meia-Idade , Avaliação de Resultados em Cuidados de Saúde , Administração dos Cuidados ao Paciente/métodos , Prognóstico , Hipertensão Arterial Pulmonar/mortalidade , Hipertensão Arterial Pulmonar/fisiopatologia , Hipertensão Arterial Pulmonar/terapia , Índice de Gravidade de Doença , Análise de SobrevidaRESUMO
In the published version of this paper the author Neus Baena's name was incorrectly given as Neus Baena Diez. This has now been corrected in both the HTML and PDF versions of the paper.
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We aimed to identify factors associated with unfavorable outcome in patients treated for infective endocarditis (IE), with a focus on departure from European guidelines. We conducted a retrospective audit of all adult patients treated for endocarditis during a 1-year period across a regional network of nine care centers in the south-east of France. Medical records were reviewed regarding patient and infection characteristics, antibiotic therapy, outcome, and compliance to the European Society of Cardiology guidelines. Antibiotic treatment appropriateness was evaluated regarding molecule, dosage, and duration, according to guidelines. Primary endpoint was the assessment of factors associated with unfavorable outcome, defined as in-hospital mortality or IE relapse at 1-year follow-up. Secondary endpoints were intensive care admission, iatrogenic events, and nosocomial infections that occurred during hospital stay. One hundred patients were included. Median age was 71 years old. Twenty-two patients died and IE relapse occurred in two patients, representing 24 patients with unfavorable outcome. Overall, antibiotic treatment was deemed appropriate in 28 cases. Thirty-three patients required intensive care, 34 iatrogenic events were found, including 19 acute kidney injuries, and 13 nosocomial infections occurred during care. Using a logistic regression, factors associated with unfavorable outcome were admission in the intensive care unit (adjusted odd ratio 7.26 [1.8-29.28]; p = 0.005), new-onset nosocomial infection (adjusted odd ratio 8.83 [1.42-54.6]; p = 0.019), and age > 71 years old (adjusted odd ratio 11.2 [2.76-46.17]; p < 0.001). Departure from guidelines was frequent but not related to unfavorable outcome in our study. Only intensive care, age, and nosocomial infections were associated with mortality and relapse. Iatrogenic events were numerous, with no impact on outcome.
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Endocardite Bacteriana , Idoso , Idoso de 80 Anos ou mais , Antibacterianos/uso terapêutico , Infecção Hospitalar , Endocardite Bacteriana/epidemiologia , Endocardite Bacteriana/mortalidade , Endocardite Bacteriana/terapia , Feminino , Humanos , Doença Iatrogênica , Masculino , Auditoria Médica , Pessoa de Meia-Idade , Recidiva , Estudos Retrospectivos , Fatores de Risco , Resultado do TratamentoRESUMO
PURPOSE: We delineate the clinical spectrum and describe the histology in arterial tortuosity syndrome (ATS), a rare connective tissue disorder characterized by tortuosity of the large and medium-sized arteries, caused by mutations in SLC2A10. METHODS: We retrospectively characterized 40 novel ATS families (50 patients) and reviewed the 52 previously reported patients. We performed histology and electron microscopy (EM) on skin and vascular biopsies and evaluated TGF-ß signaling with immunohistochemistry for pSMAD2 and CTGF. RESULTS: Stenoses, tortuosity, and aneurysm formation are widespread occurrences. Severe but rare vascular complications include early and aggressive aortic root aneurysms, neonatal intracranial bleeding, ischemic stroke, and gastric perforation. Thus far, no reports unequivocally document vascular dissections or ruptures. Of note, diaphragmatic hernia and infant respiratory distress syndrome (IRDS) are frequently observed. Skin and vascular biopsies show fragmented elastic fibers (EF) and increased collagen deposition. EM of skin EF shows a fragmented elastin core and a peripheral mantle of microfibrils of random directionality. Skin and end-stage diseased vascular tissue do not indicate increased TGF-ß signaling. CONCLUSION: Our findings warrant attention for IRDS and diaphragmatic hernia, close monitoring of the aortic root early in life, and extensive vascular imaging afterwards. EM on skin biopsies shows disease-specific abnormalities.
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Artérias/anormalidades , Proteínas Facilitadoras de Transporte de Glucose/genética , Hérnia Diafragmática/genética , Instabilidade Articular/genética , Síndrome do Desconforto Respiratório do Recém-Nascido/genética , Dermatopatias Genéticas/genética , Malformações Vasculares/genética , Adolescente , Adulto , Aorta/diagnóstico por imagem , Aorta/fisiopatologia , Artérias/diagnóstico por imagem , Artérias/fisiopatologia , Biópsia , Criança , Pré-Escolar , Fator de Crescimento do Tecido Conjuntivo/genética , Feminino , Hérnia Diafragmática/fisiopatologia , Humanos , Lactente , Instabilidade Articular/epidemiologia , Instabilidade Articular/fisiopatologia , Masculino , Mutação , Linhagem , Síndrome do Desconforto Respiratório do Recém-Nascido/fisiopatologia , Pele/patologia , Dermatopatias Genéticas/epidemiologia , Dermatopatias Genéticas/fisiopatologia , Proteína Smad2/genética , Fator de Crescimento Transformador beta/genética , Malformações Vasculares/epidemiologia , Malformações Vasculares/fisiopatologiaRESUMO
Andersen's syndrome (AS) is a rare autosomal disorder that has been defined by the triad of periodic paralysis, cardiac arrhythmia, and developmental anomalies. AS has been directly linked to over 40 different autosomal dominant negative loss-of-function mutations in the KCNJ2 gene, encoding for the tetrameric strong inward rectifying K+ channel KIR2.1. While KIR2.1 channels have been suggested to contribute to setting the resting membrane potential (RMP) and to control the duration of the action potential (AP) in skeletal and cardiac muscle, the mechanism by which AS mutations produce such complex pathophysiological symptoms is poorly understood. Thus, we use an adenoviral transduction strategy to study in vivo subcellular distribution of wild-type (WT) and AS-associated mutant KIR2.1 channels in mouse skeletal muscle. We determined that WT and D71V AS mutant KIR2.1 channels are localized to the sarcolemma and the transverse tubules (T-tubules) of skeletal muscle fibers, while the ∆314-315 AS KIR2.1 mutation prevents proper trafficking of the homo- or hetero-meric channel complexes. Whole-cell voltage-clamp recordings in individual skeletal muscle fibers confirmed the reduction of inwardly rectifying K+ current (IK1) after transduction with ∆314-315 KIR2.1 as compared to WT channels. Analysis of skeletal muscle function revealed reduced force generation during isometric contraction as well as reduced resistance to muscle fatigue in extensor digitorum longus muscles transduced with AS mutant KIR2.1. Together, these results suggest that KIR2.1 channels may be involved in the excitation-contraction coupling process required for proper skeletal muscle function. Our findings provide clues to mechanisms associated with periodic paralysis in AS.
Assuntos
Síndrome de Andersen/genética , Técnicas de Silenciamento de Genes , Músculo Esquelético/patologia , Mutação/genética , Canais de Potássio Corretores do Fluxo de Internalização/genética , Adenoviridae/metabolismo , Síndrome de Andersen/patologia , Síndrome de Andersen/fisiopatologia , Animais , Células COS , Chlorocebus aethiops , Proteínas de Fluorescência Verde/metabolismo , Humanos , Ativação do Canal Iônico , Contração Isométrica , Camundongos , Fadiga Muscular , Fibras Musculares Esqueléticas/patologia , Músculo Esquelético/fisiopatologiaRESUMO
BACKGROUND: Two-dimensional transesophageal echocardiography (2DTEE) is currently validated for left atrial appendage (LAA) thrombus assessment but has some limitations. AIMS: To evaluate the performance and interest of systematic real time three-dimensional transesophageal echocardiography (3DTEE) for LAA thrombus assessment, when performed after 2DTEE. METHODS AND RESULTS: Consecutive patients undergoing TEE were prospectively included. LAA was first evaluated using 2DTEE, and patients were classified as "2D-NT" if no thrombus was found, "2D-T" in case of clear thrombus, or "2D-EQ" if equivocal. Then, 3DTEE of the LAA was performed and patients were similarly classified as "3D-NT," "3D-T," or "3D-EQ." Additional LAA CT scan was only performed if LAA thrombus was not clearly ruled out or confirmed by TEE. Additional value of 3DTEE after 2DTEE LAA evaluation was then assessed. We included 104 patients undergoing TEE. Agreement between 2DTEE and 3DTEE was very good for thrombus diagnosis (k = 0.936), but moderate for vacant LAA (k = 0.562) due to more frequent 2D-EQ than 3D-EQ (11.5% vs 2.9%; P = .016). 3DTEE allowed to refine the LAA status in 11 of 12 (91.7%) 2D-EQ patients: 10 3D-NT, 1 3D-T, and 1 3D-EQ. Coupling 3DTEE to 2DTEE permitted a definite LAA diagnosis in 103 of 104 (99%) vs 92 of 104 (88.5%) patients when 2DTEE was used alone (P = .002). Nine (8.7%) LAA thrombi were diagnosed, and 3 CT scan were performed. CONCLUSION: 3DTEE of the LAA is more effective for thrombus assessment than 2DTEE. 3DTEE should be particularly considered in case of equivocal 2DTEE, as it allows to reach a definite LAA diagnosis in almost all of the patients.
Assuntos
Apêndice Atrial/diagnóstico por imagem , Trombose Coronária/diagnóstico por imagem , Ecocardiografia Tridimensional/métodos , Ecocardiografia Transesofagiana/métodos , Idoso , Sistemas Computacionais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Reprodutibilidade dos TestesRESUMO
AIM: Axillary vein access for pacemaker implantation is uncommon in many centres because of the lack of training in this technique. We assessed whether the introduction of the axillary vein technique was safe and efficient as compared with cephalic vein access, in a centre where no operators had any previous experience in axillary vein puncture. METHODS AND RESULTS: Patients undergoing pacemaker implantation were randomized to axillary or cephalic vein access. All three operators had no experience nor training in axillary vein puncture, and self-learned the technique by reading a published review. Axillary vein puncture was fluoroscopy-guided without contrast venography. Cephalic access was performed by dissection of delto-pectoral groove. Venous access success, venous access duration (from skin incision to guidewire or lead in superior vena cava), procedure duration, X-ray exposure, and peri-procedural (1 month) complications were recorded. results We randomized 74 consecutive patients to axillary (n = 37) or cephalic vein access (n = 37). Axillary vein was successfully accessed in 30/37 (81.1%) patients vs. 28/37 (75.7%) of cephalic veins (P = 0.57). Venous access time was shorter in axillary group than in cephalic group [5.7 (4.4-8.3) vs. 12.2 (10.5-14.8) min, P < 0.001], as well as procedure duration [34.8 (30.6-38.4) vs. 42.0 (39.1-46.6) min, P = 0.043]. X-ray exposure and peri-procedural overall complications were comparable in both groups. Axillary puncture was safe and faster than cephalic access even for the five first procedures performed by each operator. CONCLUSION: Self-taught axillary vein puncture for pacemaker implantation seems immediately safe and faster than cephalic vein access, when performed by electrophysiologists trained to pacemaker implantation but not to axillary vein puncture.