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1.
J Med Syst ; 48(1): 28, 2024 Mar 05.
Artículo en Inglés | MEDLINE | ID: mdl-38441786

RESUMEN

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.


Asunto(s)
Desfibriladores Implantables , Marcapaso Artificial , Humanos , Ansiedad/prevención & control , Dolor , Derivados de la Morfina
2.
J Cardiovasc Electrophysiol ; 34(7): 1577-1581, 2023 07.
Artículo en Inglés | MEDLINE | ID: mdl-37293820

RESUMEN

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.


Asunto(s)
Apéndice Atrial , Fibrilación Atrial , Ablación por Catéter , Taquicardia Supraventricular , Humanos , Anciano , Apéndice Atrial/diagnóstico por imagen , Apéndice Atrial/cirugía , Resultado del Tratamiento , Taquicardia , Fibrilación Atrial/complicaciones , Fibrilación Atrial/diagnóstico , Fibrilación Atrial/tratamiento farmacológico , Ablación por Catéter/efectos adversos , Ablación por Catéter/métodos
3.
Ann Noninvasive Electrocardiol ; 28(4): e13057, 2023 07.
Artículo en Inglés | MEDLINE | ID: mdl-37002650

RESUMEN

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.


Asunto(s)
Fibrilación Atrial , Ablación por Catéter , Cardiopatías Congénitas , Defectos de los Tabiques Cardíacos , Femenino , Humanos , Persona de Mediana Edad , Fibrilación Atrial/complicaciones , Fibrilación Atrial/cirugía , Vena Cava Inferior/diagnóstico por imagen , Vena Cava Inferior/cirugía , Vena Cava Inferior/anomalías , Resultado del Tratamiento , Electrocardiografía , Cardiopatías Congénitas/cirugía , Nodo Atrioventricular , Ablación por Catéter/métodos
4.
Am J Respir Crit Care Med ; 206(5): 573-583, 2022 09 01.
Artículo en Inglés | MEDLINE | ID: mdl-35549842

RESUMEN

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.


Asunto(s)
COVID-19 , Hipertensión Pulmonar , Hipertensión Arterial Pulmonar , Adulto , COVID-19/complicaciones , Prueba de COVID-19 , Humanos , Hipertensión Pulmonar/diagnóstico , Hipertensión Pulmonar/epidemiología , Hipertensión Pulmonar/etiología , Masculino , Estudios Prospectivos , SARS-CoV-2
5.
Lung ; 200(2): 179-185, 2022 04.
Artículo en Inglés | MEDLINE | ID: mdl-35381867

RESUMEN

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.


Asunto(s)
Diuréticos , Embolia Pulmonar , Disfunción Ventricular Derecha , Enfermedad Aguda , Biomarcadores , Diuréticos/uso terapéutico , Furosemida/uso terapéutico , Humanos , Péptido Natriurético Encefálico , Embolia Pulmonar/complicaciones , Embolia Pulmonar/diagnóstico por imagen , Embolia Pulmonar/tratamiento farmacológico , Disfunción Ventricular Derecha/diagnóstico por imagen , Disfunción Ventricular Derecha/tratamiento farmacológico
6.
Am J Respir Crit Care Med ; 204(7): 842-854, 2021 10 01.
Artículo en Inglés | MEDLINE | ID: mdl-34185620

RESUMEN

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.


Asunto(s)
Antihipertensivos/uso terapéutico , Hipertensión Arterial Pulmonar/tratamiento farmacológico , Hipertensión Arterial Pulmonar/mortalidad , Administración Oral , Adulto , Anciano , Quimioterapia Combinada , Femenino , Estudios de Seguimiento , Francia/epidemiología , Humanos , Infusiones Parenterales , Masculino , Persona de Mediana Edad , Puntaje de Propensión , Sistema de Registros , Estudios Retrospectivos , Análisis de Supervivencia , Resultado del Tratamiento
7.
Circulation ; 142(5): 429-436, 2020 08 04.
Artículo en Inglés | MEDLINE | ID: mdl-32418446

RESUMEN

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.


Asunto(s)
COVID-19/complicaciones , Insuficiencia Cardíaca/virología , Inflamación/virología , Síndrome de Respuesta Inflamatoria Sistémica/complicaciones , Adolescente , COVID-19/virología , Niño , Femenino , Insuficiencia Cardíaca/complicaciones , Insuficiencia Cardíaca/tratamiento farmacológico , Humanos , Inmunoglobulinas Intravenosas/uso terapéutico , Inflamación/complicaciones , Inflamación/tratamiento farmacológico , Masculino , Estudios Retrospectivos , Volumen Sistólico/fisiología , Síndrome de Respuesta Inflamatoria Sistémica/virología , Disfunción Ventricular Izquierda/tratamiento farmacológico , Función Ventricular Izquierda/inmunología
8.
Transpl Int ; 34(11): 2297-2304, 2021 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-34425020

RESUMEN

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.


Asunto(s)
Trasplante de Riñón , Estudios de Cohortes , Válvulas Cardíacas , Humanos , Hemorragia Posoperatoria , Estudios Prospectivos , Estudios Retrospectivos
9.
Am J Respir Crit Care Med ; 202(6): 843-852, 2020 09 15.
Artículo en Inglés | MEDLINE | ID: mdl-32437637

RESUMEN

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.


Asunto(s)
Hipertensión Pulmonar/tratamiento farmacológico , Hipertensión Pulmonar/fisiopatología , Hipertensión Pulmonar/cirugía , Neoplasias Pulmonares/fisiopatología , Neurofibromatosis 1/complicaciones , Neurofibromatosis 1/genética , Neurofibromina 1/genética , Adolescente , Adulto , Femenino , Francia , Humanos , Hipertensión Pulmonar/etiología , Trasplante de Pulmón/métodos , Masculino , Persona de Mediana Edad , Fenotipo , Pronóstico , Adulto Joven
10.
Echocardiography ; 38(10): 1694-1701, 2021 10.
Artículo en Inglés | MEDLINE | ID: mdl-34672394

RESUMEN

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.


Asunto(s)
Embolia Pulmonar , Disfunción Ventricular Derecha , Ecocardiografía , Humanos , Estudios Prospectivos , Embolia Pulmonar/diagnóstico por imagen , Disfunción Ventricular Derecha/diagnóstico por imagen , Función Ventricular Derecha
11.
Cardiol Young ; 31(10): 1557-1562, 2021 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-34551835

RESUMEN

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.


Asunto(s)
Insuficiencia Cardíaca , Defectos del Tabique Interventricular , Dispositivo Oclusor Septal , Cateterismo Cardíaco , Niño , Preescolar , Defectos del Tabique Interventricular/epidemiología , Defectos del Tabique Interventricular/cirugía , Ventrículos Cardíacos/diagnóstico por imagen , Humanos , Estudios Observacionales como Asunto , Estudios Prospectivos , Resultado del Tratamiento
12.
J Hepatol ; 73(1): 130-139, 2020 07.
Artículo en Inglés | MEDLINE | ID: mdl-32145258

RESUMEN

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.


Asunto(s)
Antagonistas de los Receptores de Endotelina/uso terapéutico , Hipertensión Portal , Cirrosis Hepática , Inhibidores de Fosfodiesterasa 5/uso terapéutico , Hipertensión Arterial Pulmonar , Sistema Cardiovascular/fisiopatología , Tolerancia al Ejercicio , Femenino , Francia/epidemiología , Estado Funcional , Humanos , Hipertensión Portal/diagnóstico , Hipertensión Portal/mortalidad , Hipertensión Portal/fisiopatología , Cirrosis Hepática/diagnóstico , Cirrosis Hepática/fisiopatología , Cirrosis Hepática/cirugía , Trasplante de Hígado/estadística & datos numéricos , Masculino , Persona de Mediana Edad , Evaluación de Resultado en la Atención de Salud , Manejo de Atención al Paciente/métodos , Pronóstico , Hipertensión Arterial Pulmonar/mortalidad , Hipertensión Arterial Pulmonar/fisiopatología , Hipertensión Arterial Pulmonar/terapia , Índice de Severidad de la Enfermedad , Análisis de Supervivencia
14.
Eur J Clin Microbiol Infect Dis ; 38(1): 109-115, 2019 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-30324540

RESUMEN

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.


Asunto(s)
Endocarditis Bacteriana , Anciano , Anciano de 80 o más Años , Antibacterianos/uso terapéutico , Infección Hospitalaria , Endocarditis Bacteriana/epidemiología , Endocarditis Bacteriana/mortalidad , Endocarditis Bacteriana/terapia , Femenino , Humanos , Enfermedad Iatrogénica , Masculino , Auditoría Médica , Persona de Mediana Edad , Recurrencia , Estudios Retrospectivos , Factores de Riesgo , Resultado del Tratamiento
15.
Genet Med ; 20(10): 1236-1245, 2018 10.
Artículo en Inglés | MEDLINE | ID: mdl-29323665

RESUMEN

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.


Asunto(s)
Arterias/anomalías , Proteínas Facilitadoras del Transporte de la Glucosa/genética , Hernia Diafragmática/genética , Inestabilidad de la Articulación/genética , Síndrome de Dificultad Respiratoria del Recién Nacido/genética , Enfermedades Cutáneas Genéticas/genética , Malformaciones Vasculares/genética , Adolescente , Adulto , Aorta/diagnóstico por imagen , Aorta/fisiopatología , Arterias/diagnóstico por imagen , Arterias/fisiopatología , Biopsia , Niño , Preescolar , Factor de Crecimiento del Tejido Conjuntivo/genética , Femenino , Hernia Diafragmática/fisiopatología , Humanos , Lactante , Inestabilidad de la Articulación/epidemiología , Inestabilidad de la Articulación/fisiopatología , Masculino , Mutación , Linaje , Síndrome de Dificultad Respiratoria del Recién Nacido/fisiopatología , Piel/patología , Enfermedades Cutáneas Genéticas/epidemiología , Enfermedades Cutáneas Genéticas/fisiopatología , Proteína Smad2/genética , Factor de Crecimiento Transformador beta/genética , Malformaciones Vasculares/epidemiología , Malformaciones Vasculares/fisiopatología
16.
Cell Tissue Res ; 371(2): 309-323, 2018 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-29018970

RESUMEN

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.


Asunto(s)
Síndrome de Andersen/genética , Técnicas de Silenciamiento del Gen , Músculo Esquelético/patología , Mutación/genética , Canales de Potasio de Rectificación Interna/genética , Adenoviridae/metabolismo , Síndrome de Andersen/patología , Síndrome de Andersen/fisiopatología , Animales , Células COS , Chlorocebus aethiops , Proteínas Fluorescentes Verdes/metabolismo , Humanos , Activación del Canal Iónico , Contracción Isométrica , Ratones , Fatiga Muscular , Fibras Musculares Esqueléticas/patología , Músculo Esquelético/fisiopatología
17.
Echocardiography ; 35(4): 474-480, 2018 04.
Artículo en Inglés | MEDLINE | ID: mdl-29399868

RESUMEN

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.


Asunto(s)
Apéndice Atrial/diagnóstico por imagen , Trombosis Coronaria/diagnóstico por imagen , Ecocardiografía Tridimensional/métodos , Ecocardiografía Transesofágica/métodos , Anciano , Sistemas de Computación , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Reproducibilidad de los Resultados
18.
Europace ; 19(12): 2001-2006, 2017 Dec 01.
Artículo en Inglés | MEDLINE | ID: mdl-28064251

RESUMEN

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.


Asunto(s)
Vena Axilar , Estimulación Cardíaca Artificial , Cateterismo Periférico/métodos , Competencia Clínica , Curva de Aprendizaje , Marcapaso Artificial , Anciano , Anciano de 80 o más Años , Vena Axilar/diagnóstico por imagen , Cateterismo Periférico/efectos adversos , Femenino , Francia , Humanos , Masculino , Flebografía , Valor Predictivo de las Pruebas , Estudios Prospectivos , Punciones , Exposición a la Radiación , Radiografía Intervencional , Factores de Riesgo , Factores de Tiempo , Resultado del Tratamiento
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