RESUMO
Arrhythmogenic cardiomyopathy with right dominant form (ACR) is a rare heritable cardiac cardiomyopathy disorder associated with sudden cardiac death. Pathogenic variants (PVs) in desmosomal genes have been causally related to ACR in 40% of cases. Other genes encoding nondesmosomal proteins have been described in ACR, but their contribution in this pathology is still debated. A panel of 71 genes associated with inherited cardiopathies was screened in an ACR population of 172 probands and 856 individuals from the general population. PVs and uncertain significance variants (VUS) have been identified in 36% and 18.6% of patients, respectively. Among the cardiopathy-associated genes, burden tests show a significant enrichment in PV and VUS only for desmosomal genes PKP2 (plakophilin-2), DSP (desmoplakin), DSC2 (desmocollin-2), and DSG2 (desmoglein-2). Importantly, VUS may account for 15% of ACR cases and should then be considered for molecular diagnosis. Among the other genes, no evidence of enrichment was detected, suggesting an extreme caution in the interpretation of these genetic variations without associated functional or segregation data. Genotype-phenotype correlation points to (1) a more severe and earlier onset of the disease in PV and VUS carriers, underlying the importance to carry out presymptomatic diagnosis in relatives and (2) to a more prevalent left ventricular dysfunction in DSP variant carriers.
Assuntos
Displasia Arritmogênica Ventricular Direita , Displasia Arritmogênica Ventricular Direita/diagnóstico , Displasia Arritmogênica Ventricular Direita/genética , Displasia Arritmogênica Ventricular Direita/metabolismo , Desmossomos/genética , Desmossomos/metabolismo , Estudos de Associação Genética , Heterozigoto , Humanos , Placofilinas/genética , Placofilinas/metabolismoRESUMO
AIMS: We aimed to provide contemporary real-world data on wearable cardioverter-defibrillator (WCD) use, not only in terms of effectiveness and safety but also compliance and acceptability. METHODS AND RESULTS: Across 88 French centres, the WEARIT-France study enrolled retrospectively patients who used the WCD between May 2014 and December 2016, and prospectively all patients equipped for WCD therapy between January 2017 and March 2018. All patients received systematic education session through a standardized programme across France at the time of initiation of WCD therapy and were systematically enrolled in the LifeVest Network remote services. Overall, 1157 patients were included (mean age 60 ± 12 years, 16% women; 46% prospectively): 82.1% with ischaemic cardiomyopathy, 10.3% after implantable cardioverter-defibrillator explant, and 7.6% before heart transplantation. Median WCD usage period was 62 (37-97) days. Median daily wear time of WCD was 23.4 (22.2-23.8) h. In multivariate analysis, younger age was associated with lower compliance [adjusted odds ratio (OR) 0.97, 95% confidence interval (CI) 0.95-0.99, P < 0.01]. A total of 18 participants (1.6%) received at least one appropriate shock, giving an incidence of appropriate therapy of 7.2 per 100 patient-years. Patient-response button allowed the shock to be aborted in 35.7% of well-tolerated sustained ventricular arrhythmias and in 95.4% of inappropriate ventricular arrhythmia detection, finally resulting in an inappropriate therapy in eight patients (0.7%). CONCLUSION: Our real-life findings reinforce previous studies on the efficacy and safety of the WCD in the setting of transient high-risk group in selected patients. Moreover, they emphasize the fact that when prescribed appropriately, in concert with adequate patient education and dedicated follow-up using specific remote monitoring system, compliance with WCD is high and the device well-tolerated by the patient.
Assuntos
Desfibriladores Implantáveis , Dispositivos Eletrônicos Vestíveis , Idoso , Estudos de Coortes , Morte Súbita Cardíaca/epidemiologia , Morte Súbita Cardíaca/prevenção & controle , Desfibriladores , Cardioversão Elétrica , Feminino , França/epidemiologia , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do TratamentoRESUMO
OBJECTIVES: Inflammation is a hallmark of heart failure (HF) and among inflammatory biomarkers, the most studied remains the C-reactive protein (CRP). In recent years several biomarkers have emerged, such as sST2 and soluble urokinase-type plasminogen activator receptor (suPAR). This study set out to examine the relative importance of long-time prognostic strength of suPAR and the potential additive information on patient risk with chronic HF in comparison with pronostic value of CRP and sST2. METHODS: Demographics, clinical and biological variables were assessed in a total of 182 patients with chronic HF over median follow-up period of 80 months. Inflammatory biomarkers (i.e., CRP, sST2, and suPAR) were performed. RESULTS: In univariate Cox regression analysis age, NYHA class, MAGGIC score and the five biomarkers (N-terminal pro brain natriuretic peptide [NT-proBNP], high-sensitive cardiac troponin T [hs-cTnT], CRP, sST2, and suPAR) were associated with both all-cause and cardiovascular mortality. In the multivariate model, only NT-proBNP, suPAR, and MAGGIC score remained independent predictors of all-cause mortality as well as of cardiovascular mortality. Risk classification analysis was significantly improved with the addition of suPAR particularly for all-cause short- and long-term mortality. Using a classification tree approach, the same three variables could be considered as significant classifier variables to predict all-cause or cardiovascular mortality and an algorithm were reported. We demonstrated the favorable outcome associated with patients with a low MAGGIC score and a low suPAR level by comparison to patients with low MAGGIC score but high suPAR values. CONCLUSIONS: The main findings of our study are (1) that among the three inflammatory biomarkers, only suPAR levels were independently associated with 96-month mortality for patients with chronic HF and (2) that an algorithm based on clinical score, a cardiomyocyte stress biomarker and an inflammatory biomarker could help to a more reliable long term risk stratification in heart failure.
Assuntos
Insuficiência Cardíaca , Receptores de Ativador de Plasminogênio Tipo Uroquinase , Biomarcadores , Proteína C-Reativa/análise , Doença Crônica , Insuficiência Cardíaca/diagnóstico , Humanos , Peptídeo Natriurético Encefálico , Fragmentos de Peptídeos , Prognóstico , Troponina TRESUMO
BACKGROUND: Advances in paediatric cardiology have improved the prognosis of children with inherited cardiac disorders. However, health-related quality of life (QoL) and physical activity have been scarcely analysed in children with inherited cardiac arrhythmia or inherited cardiomyopathy. Moreover, current guidelines on the eligibility of young athletes with inherited cardiac disorders for sports participation mainly rely on expert opinions and remain controversial. METHODS: The QUALIMYORYTHM trial is a multicentre observational controlled study. The main objective is to compare the QoL of children aged 6 to 17 years old with inherited cardiac arrhythmia (long QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, or arrhythmogenic right ventricular dysplasia), or inherited cardiomyopathy (hypertrophic, dilated, or restrictive cardiomyopathy), to that of age and gender-matched healthy subjects. The secondary objective is to assess their QoL according to the disease's clinical and genetic characteristics, the level of physical activity and motivation for sports, the exercise capacity, and the socio-demographic data. Participants will wear a fitness tracker (ActiGraph GT3X accelerometer) for 2 weeks. A total of 214 children are required to observe a significant difference of 7 ± 15 points in the PedsQL, with a power of 90% and an alpha risk of 5%. DISCUSSION: After focusing on the survival in children with inherited cardiac disorders, current research is expanding to patient-reported outcomes and secondary prevention. The QUALIMYORYTHM trial intends to improve the level of evidence for future guidelines on sports eligibility in this population. Trial registration ClinicalTrials.gov Identifier: NCT04712136, registered on January 15th, 2021 ( https://clinicaltrials.gov/ct2/show/NCT04712136 ).
Assuntos
Arritmias Cardíacas/genética , Cardiomiopatias/genética , Exercício Físico , Qualidade de Vida/psicologia , Adolescente , Arritmias Cardíacas/psicologia , Cardiomiopatias/psicologia , Criança , Morte Súbita Cardíaca , Exercício Físico/fisiologia , Exercício Físico/psicologia , Feminino , Humanos , Masculino , Oxigênio , Consumo de Oxigênio , Estudos ProspectivosRESUMO
BACKGROUND: Percutaneous left atrial appendage (LAA) closure is an alternative to oral anticoagulation (OAC) for atrial fibrillation (AF) patients with high thromboembolism risk, particularly with contraindications to OAC. The LAA itself could possess proarrhythmogenic properties. As patients undergoing LAA closure could be candidates for cardioversion or ablation, we aimed to evaluate AF disease progression following LAA closure and the outcome of patients undergoing a rhythm control strategy after the procedure. METHODS: The prospective multicenter French Nationwide Observational LAA Closure Registry (FLAAC) comprises 33 French interventional cardiology departments. Patients were included if they fulfilled the following criteria: history of non-valvular AF, successful LAA closure and long-term ECG follow-up. RESULTS: A total of 331 patients with successful LAA closure were enrolled in the study. Patients mean age was 75.4 ± 0.5 years. The study population was characterized by a high thromboembolic risk (CHA2DS2-VASc score: 4.5 ± 0.1) and frequent comorbidities. The median follow-up was 11.9 months. One hundred and nineteen (36.0%) patients were in sinus rhythm (SR) at baseline. Among SR patients, documented AF was observed in 16 (13.4%) patients whereas 15 (7.1%) patients in AF at baseline restored SR, at the end of follow up. Finally, only 13 patients (4%) underwent procedures to restore SR without complications during the follow-up. CONCLUSIONS: The vast majority of patients undergoing LAA closure have the same AF status at baseline and one year after the index procedure. During the follow-up, a very small proportion (4%) of our population underwent procedures to restore SR without complications whatever the post-procedural antithrombotic strategy was.
Assuntos
Apêndice Atrial/fisiopatologia , Fibrilação Atrial/terapia , Função do Átrio Esquerdo , Frequência Cardíaca , Potenciais de Ação , Idoso , Idoso de 80 Anos ou mais , Antiarrítmicos/uso terapêutico , Fibrilação Atrial/diagnóstico por imagem , Fibrilação Atrial/fisiopatologia , Ablação por Cateter , Cardioversão Elétrica , Eletrocardiografia , Feminino , França , Frequência Cardíaca/efeitos dos fármacos , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Recidiva , Sistema de Registros , Retratamento , Fatores de Tempo , Resultado do TratamentoRESUMO
Background and objectives: Renal failure is a contraindication for some glucose-lowering drugs and requires dosage adjustment for others, particularly biguanides, sulfonylureas, and inhibitors of dipeptidyl peptidase 4. In this study, we assessed adherence to prescription recommendations for glucose-lowering drugs according to renal function in hospitalized diabetic subjects. Materials and Methods: This prospective cohort study was carried out over a 2-year period in a university hospital. Glomerular filtration rate (GFR) was determined by averaging all measurements performed during hospitalization. Glucose-lowering drug dosages were analyzed according to the recommendations of the relevant medical societies. Results: In total, 2071 diabetic patients (53% hospitalized in cardiology units) were examined. GFR was <30 mL/min/1.73 m2 in 13.4% of these patients, 30-44 in 15.1%, 45-60 in 18.3%, and >60 in 53.3%. Inappropriate oral glucose-lowering treatments were administered to 273 (13.2%) patients, including 53 (2.6%) with a contraindication. In cardiology units, 53.1% and 14.3% of patients had GFRs of <60 and <30 mL/min/1.73 m2, respectively, and 179 (15.4%) patients had a contraindication or were prescribed an excessive dose of glucose-lowering drugs. Conclusions: We showed that the burden of inappropriate prescriptions is high in diabetic patients. Given the high number of patients receiving these medications, particularly in cardiology units, a search for potential adverse effects related to these drugs should be performed.
Assuntos
Diabetes Mellitus Tipo 2 , Hipoglicemiantes , Prescrição Inadequada , Cardiologia , Diabetes Mellitus Tipo 2/complicações , Diabetes Mellitus Tipo 2/tratamento farmacológico , Endocrinologia , Glucose , Fidelidade a Diretrizes , Humanos , Hipoglicemiantes/efeitos adversos , Pacientes Internados , Medicina Interna , Rim/fisiologia , Rim/fisiopatologia , Estudos ProspectivosRESUMO
BACKGROUND: Implantable cardioverter-defibrillator (ICD) lead dysfunction has been reported after left ventricular assist device (LVAD) implantation in limited single-center studies. We aimed at describing and characterizing the incidence of ICD lead parameters dysfunction after LVAD implantation. METHODS: Among the 652 patients enrolled in the ASSIST-ICD study, only patients with an ICD prior to LVAD were included (n = 401). ICD lead parameters dysfunction following LVAD implantation is defined as follows: (a) >50% decrease in sensing threshold, (b) pacing lead impedance increase/decrease by >100Ω, and (c) >50% increase in pacing threshold. RESULTS: One hundred twenty-two patients with an ICD prior to LVAD had available ICD interrogation reports prior and after LVAD. A total of 67 (55%) patients exhibited at least one significant lead dysfunction: 17 (15%) exhibited >50% decrease in right ventricular (RV) sensing, 51 (42%) had >100 Ω increase/decrease in RV pacing impedance, and 24 (20%) experienced >50% increase in RV pacing threshold. A total of 52 patients experienced ventricular arrhythmia during follow-up and all were successfully detected and treated by the device. All lead dysfunction could be managed conservatively. CONCLUSION: More than 50% of LVAD-recipients may experience >1 significant change in lead parameters but none had severe clinical consequences.
Assuntos
Desfibriladores Implantáveis/efeitos adversos , Eletrodos Implantados/efeitos adversos , Coração Auxiliar , Idoso , França , Humanos , Masculino , Pessoa de Meia-Idade , Falha de PróteseRESUMO
OBJECTIVES: The aim of this study was to determine the effect of pharmacists' interventions (PI) on the potential clinical impact of medication errors, including the lack of therapeutic optimisation of patients with cardiologic diseases, such as heart failure and acute coronary syndrome). METHODS: This was an observational, prospective study conducted in the cardiology department of a French university hospital centre for a duration of 9 months. All prescriptions were analysed and PI were registered for clinical rating by pharmacists and cardiologist. RESULTS: A total of 532 PI cases were recorded in 339 patients, with a mean of 1.57 (±1.04) PI. The PI acceptance rate was 98.1%. "Dose adjustment" and "introduction therapy" were the most common interventions and represented 38.0% and 32.9%, respectively, of all PI. Statins were the most frequently involved drugs (18.1%), followed by ACE (Angiotensin Converting Enzyme) inhibitors (10.9%) and antiplatelet agents (9.3%). Moreover, 13.8% of PI potentially avoided a severe or very severe clinical impact (n = 71) and 38.6% had a significant impact altering the quality of life (n = 198). There was no significant difference between the average score performed by the clinical pharmacist included in the cardiology team and the one obtained by the cardiologist (P = .797). In contrast, a significant difference was observed for the average score established by the pharmacist localised in central pharmacy versus the rating of the cardiologist (P < .001). CONCLUSIONS: The collaboration between clinical pharmacists and cardiologists in the medical units seems to be beneficial to the quality of prescriptions, including the implementation of recommendations. The good rate of PI acceptance and the similar rating with the cardiologist show that there is a change in perspective of the pharmacist, being closer to the clinical reality.
Assuntos
Síndrome Coronariana Aguda/tratamento farmacológico , Prescrições de Medicamentos/estatística & dados numéricos , Erros de Medicação/prevenção & controle , Adulto , Inibidores da Enzima Conversora de Angiotensina/uso terapêutico , Cardiologistas/estatística & dados numéricos , Feminino , Humanos , Inibidores de Hidroximetilglutaril-CoA Redutases/uso terapêutico , Masculino , Pessoa de Meia-Idade , Farmacêuticos/estatística & dados numéricos , Serviço de Farmácia Hospitalar , Estudos ProspectivosRESUMO
INTRODUCTION: Leadless pacemakers may provide a safe and attractive pacing option to patients with cardiac implantable electronic device (CIED) infection. We describe the characteristics and outcomes of patients with a recent CIED infection undergoing Micra implant attempt. METHODS AND RESULTS: Patients with prior CIED infection and device explant with Micra implant within 30 days, were identified from the Micra post approval registry. Procedure characteristics and outcomes were summarized. A total of 105 patients with prior CIED infection underwent Micra implant attempt ≤30 days from prior system explant (84 [80%] pacemakers and 13 [12%] ICD/CRT-D). All system components were explanted in 93% of patients and explant occurred a median of 6 days before Micra implant, with 37% occurring on the day of Micra implant. Micra was successfully implanted in 99% patients, mean follow-up duration was 8.5 ± 7.1 months (range 0-28.5). The majority of patients (91%) received IV antibiotics preimplant, while 42% of patients received IV antibiotics postprocedure. The median length of hospitalization following Micra implant was 2 days (IQR, 1-7). During follow-up, two patients died from sepsis and four patients required system upgrade, of which two patients received Micra to provide temporary pacing support. There were no Micra devices explanted due to infection. CONCLUSION: Implantation of the Micra transcatheter pacemaker is safe and feasible in patients with a recent CIED infection. No recurrent infections that required Micra device removal were seen. Leadless pacemakers appear to be a safe pacing alternative for patients with CIED infection who undergo extraction.
Assuntos
Estimulação Cardíaca Artificial/efeitos adversos , Remoção de Dispositivo , Marca-Passo Artificial/efeitos adversos , Infecções Relacionadas à Prótese/terapia , Administração Intravenosa , Idoso , Idoso de 80 Anos ou mais , Antibacterianos/administração & dosagem , Estimulação Cardíaca Artificial/mortalidade , Remoção de Dispositivo/efeitos adversos , Remoção de Dispositivo/mortalidade , Desenho de Equipamento , Feminino , Humanos , Tempo de Internação , Masculino , Pessoa de Meia-Idade , Infecções Relacionadas à Prótese/diagnóstico , Infecções Relacionadas à Prótese/microbiologia , Infecções Relacionadas à Prótese/mortalidade , Sistema de Registros , Fatores de Risco , Fatores de Tempo , Resultado do TratamentoRESUMO
AIMS: We aimed at describing outcomes and predictors of cardiac avulsion or tear (CA/T) with tamponade and vascular avulsion or tear (VA/T) after transvenous lead extraction (TLE) in the ESC-EHRA European Lead Extraction ConTRolled (ELECTRa) registry. METHODS AND RESULTS: A total of 3555 consecutive patients of whom 3510 underwent TLE at 73 centres in 19 European countries were enrolled. Among 58 patients (1.7%) with procedure-related major complications, 49 (84.5%) patients (30 CA/T and 19 VA/T) presented cardiovascular complications requiring pericardiocentesis, chest tube positioning and/or surgical repair. The mortality was 20% in patients with tamponade due to CA/T and 31.6% in patients with VA/T. Pericardiocentesis as first manoeuvre followed by rescue surgical repair was highly effective in case of CA/T (93.8%). At multivariate analysis, CA/T with tamponade was more common in RIATA lead extraction, female patients, leads with a mean dwelling time more than 10 years, and when ≥3 leads were extracted or multiple sheaths required. Occlusion or critical stenosis of superior venous access and the leads mean dwelling time more than 10 years were independent predictors for VA/T, while mechanical dilatation was an independent predictor of a lower incidence of this complication as compared to the use of powered sheaths. CONCLUSIONS: In the ELECTRa registry, RIATA lead extraction and superior venous access occlusion/thrombosis are two new independent predictors for cardiac tamponade and major vascular complications, respectively. The use of mechanical sheaths seems to be associated with a lower incidence of VA/T. A strategy of pericardiocentesis followed by a rescue surgical approach seems to be reasonable in order to treat a CA/T with tamponade.
Assuntos
Tamponamento Cardíaco , Desfibriladores Implantáveis/efeitos adversos , Remoção de Dispositivo , Marca-Passo Artificial/efeitos adversos , Pericardiocentese , Complicações Pós-Operatórias , Tamponamento Cardíaco/diagnóstico , Tamponamento Cardíaco/mortalidade , Tamponamento Cardíaco/cirurgia , Remoção de Dispositivo/efeitos adversos , Remoção de Dispositivo/instrumentação , Remoção de Dispositivo/métodos , Falha de Equipamento , Europa (Continente)/epidemiologia , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Avaliação de Processos e Resultados em Cuidados de Saúde , Pericardiocentese/métodos , Pericardiocentese/estatística & dados numéricos , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/cirurgia , Prognóstico , Sistema de Registros/estatística & dados numéricos , Fatores de Risco , Trombose/diagnóstico , Trombose/epidemiologia , Lesões do Sistema Vascular/diagnóstico , Lesões do Sistema Vascular/epidemiologiaAssuntos
Fibrilação Atrial , Ablação por Cateter , Osteófito , Veias Pulmonares , Fibrilação Atrial/diagnóstico , Fibrilação Atrial/cirurgia , Átrios do Coração/diagnóstico por imagem , Átrios do Coração/cirurgia , Humanos , Osteófito/diagnóstico por imagem , Osteófito/cirurgia , Veias Pulmonares/cirurgia , Resultado do TratamentoRESUMO
BACKGROUND: Cardiac biomarkers are the cornerstone of the biological definition of acute myocardial infarction (AMI). The key role of troponins in diagnosis of AMI is well established. Moreover, kinetics of troponin I (cTnI) and creatine kinase (CK) after AMI are correlated to the prognosis. New technical assessment like high-sensitivity cardiac troponin T (hs-cTnT) raises concerns because of its unclear kinetic following the peak. This study aims to compare kinetics of cTnI and hs-cTnT to CK in patients with large AMI successfully treated by percutaneous coronary intervention (PCI). METHODS: We prospectively studied 62 patients with anterior AMI successfully reperfused with primary angioplasty. We evaluated two consecutive groups: the first one regularly assessed by both CK and cTnI methods and the second group by CK and hs-cTnT. Modeling of kinetics was realized using mixed effects with cubic splines. RESULTS: Kinetics of markers showed a peak at 7.9 h for CK, at 10.9 h (6.9-12.75) for cTnI and at 12 h for hs-cTnT. This peak was followed by a nearly log linear decrease for cTnI and CK by contrast to hs-cTnT which appeared with a biphasic shape curve marked by a second peak at 82 h. There was no significant difference between the decrease of cTnI and CK (p=0.63). CK fell by 79.5% (76.1-99.9) vs. cTnI by 86.8% (76.6-92.7). In the hs-cTnT group there was a significant difference in the decrease by 26.5% (9-42.9) when compared with CK that fell by 79.5% (64.3-90.7). CONCLUSIONS: Kinetic of hs-cTnT and not cTnI differs from CK. The role of hs-cTnT in prognosis has to be investigated.
Assuntos
Creatina Quinase/sangue , Infarto do Miocárdio/sangue , Infarto do Miocárdio/cirurgia , Revascularização Miocárdica , Miocárdio/metabolismo , Troponina I/sangue , Troponina T/sangue , Doença Aguda , Biomarcadores/sangue , Feminino , Humanos , Cinética , Masculino , Pessoa de Meia-Idade , Infarto do Miocárdio/enzimologia , Resultado do TratamentoAssuntos
Morte Súbita Cardíaca/etiologia , Prolapso da Valva Mitral/complicações , Síncope/etiologia , Fibrilação Ventricular/etiologia , Complexos Ventriculares Prematuros/etiologia , Adulto , Morte Súbita Cardíaca/prevenção & controle , Desfibriladores Implantáveis , Cardioversão Elétrica/instrumentação , Feminino , França , Humanos , Masculino , Pessoa de Meia-Idade , Prolapso da Valva Mitral/mortalidade , Prolapso da Valva Mitral/fisiopatologia , Prolapso da Valva Mitral/terapia , Fenótipo , Medição de Risco , Fatores de Risco , Síncope/mortalidade , Síncope/fisiopatologia , Síncope/prevenção & controle , Resultado do Tratamento , Estados Unidos , Fibrilação Ventricular/mortalidade , Fibrilação Ventricular/fisiopatologia , Fibrilação Ventricular/prevenção & controle , Complexos Ventriculares Prematuros/mortalidade , Complexos Ventriculares Prematuros/fisiopatologia , Complexos Ventriculares Prematuros/prevenção & controle , Adulto JovemRESUMO
BACKGROUND: Implantable cardioverter-defibrillator indications in Brugada syndrome remain controversial, especially in asymptomatic patients. Previous outcome data are limited by relatively small numbers of patients or short follow-up durations. We report the outcome of patients with Brugada syndrome implanted with an implantable cardioverter-defibrillator in a large multicenter registry. METHODS AND RESULTS: A total of 378 patients (310 male; age, 46±13 years) with a type 1 Brugada ECG pattern implanted with an implantable cardioverter-defibrillator (31 for aborted sudden cardiac arrest, 181 for syncope, and 166 asymptomatic) were included. Fifteen patients (4%) were lost to follow-up. During a mean follow-up of 77±42 months, 7 patients (2%) died (1 as a result of an inappropriate shock), and 46 patients (12%) had appropriate device therapy (5±5 shocks per patient). Appropriate device therapy rates at 10 years were 48% for patients whose implantable cardioverter-defibrillator indication was aborted sudden cardiac arrest, 19% for those whose indication was syncope, and 12% for the patients who were asymptomatic at implantation. At 10 years, rates of inappropriate shock and lead failure were 37% and 29%, respectively. Inappropriate shock occurred in 91 patients (24%; 4±4 shocks per patient) because of lead failure (n=38), supraventricular tachycardia (n=20), T-wave oversensing (n=14), or sinus tachycardia (n=12). Importantly, introduction of remote monitoring, programming a high single ventricular fibrillation zone (>210-220 bpm), and a long detection time were associated with a reduced risk of inappropriate shock. CONCLUSIONS: Appropriate therapies are more prevalent in symptomatic Brugada syndrome patients but are not insignificant in asymptomatic patients (1%/y). Optimal implantable cardioverter-defibrillator programming and follow-up dramatically reduce inappropriate shock. However, lead failure remains a major problem in this population.
Assuntos
Síndrome de Brugada/mortalidade , Síndrome de Brugada/terapia , Morte Súbita Cardíaca/epidemiologia , Morte Súbita Cardíaca/prevenção & controle , Desfibriladores Implantáveis/estatística & dados numéricos , Adulto , Idoso , Síndrome de Brugada/diagnóstico , Desfibriladores Implantáveis/efeitos adversos , Eletrocardiografia , Feminino , Seguimentos , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Prevalência , Fatores de Risco , Resultado do TratamentoRESUMO
BACKGROUND: The aim of the Endocardial T-Wave Alternans Study was to prospectively assess the presence of T-wave alternans (TWA) or beat-to-beat repolarization changes on implantable cardioverter-defibrillator (ICD)-stored electrograms (EGMs) immediately preceding the onset of spontaneous ventricular tachycardia (VT) or fibrillation (VF). METHODS: Thirty-seven VT/VF episodes were compared to 116 baseline reference EGMs from the same 57 patients. A Bayesian model was used to estimate the T-wave waveform in each cardiac beat and a set of 10 parameters was selected to segment each detected T wave. Beat-by-beat differences in each T-wave parameter were computed using the absolute value of the difference between each beat and the following one. Fisher criterion was used for determining the most discriminant T-wave parameters, then top-M ranked parameters yielding a normalized cumulative Fisher score > 95% were selected, and analysis was applied on these selected parameters. Simulated TWA EGMs were used to validate the algorithm. RESULTS: In the simulation study, TWA was detectable even in the case of the smallest simulated alternans of 25 µV. In 13 of the 37 episodes (35%) occurring in nine of 16 patients, significant larger beat-to-beat variations before arrhythmia onset were detected compared to their respective references (median one positive episode per patient). Parameters including the T-wave apex amplitude seem the more discriminant parameters. CONCLUSIONS: Detection of beat-by-beat repolarization variations in ICD-stored EGMs is feasible in a significant subset of cases and may be used for predicting the onset of ventricular arrhythmias.
Assuntos
Desfibriladores Implantáveis , Técnicas Eletrofisiológicas Cardíacas , Taquicardia Ventricular/fisiopatologia , Taquicardia Ventricular/terapia , Adulto , Idoso , Idoso de 80 Anos ou mais , Arritmias Cardíacas , Síndrome de Brugada , Doença do Sistema de Condução Cardíaco , Feminino , Sistema de Condução Cardíaco/anormalidades , Humanos , Masculino , Pessoa de Meia-Idade , Estudos ProspectivosRESUMO
BACKGROUND: To assess the feasibility, acceptability, safety, and short-term benefits of a tailored cardiac rehabilitation program for children and adolescents with long QT syndrome (LQTS). METHODS: Eight participants, aged between 6 and 18, with a positive LQTS genotype and impaired cardiorespiratory fitness, were enrolled in a 12-week centre-based cardiac rehabilitation program. The program included supervised exercise training group sessions (aerobic, resistance, and outdoor activities) and patient education workshops. Feasibility, acceptability, and safety of the program were prospectively monitored. Feedback from the parents, children, and professionals involved was collected from qualitative interviews. Short-term effects on cardiorespiratory fitness, muscle fitness, physical activity, and health-related quality of life (HRQoL) were measured between baseline and the end of the program. RESULTS: Retention (88% with one participant dropping out) and adherence (79%) rates were good, and no cardiac events occurred during the 12-week intervention period. Participants, parents, and healthcare professionals expressed a high level of satisfaction with the program. A significant increase between the beginning and the end of the program was observed for ventilatory anaerobic threshold (21.7±5.2 vs. 28.7±5.1 mL/kg/min, P=0.01, effect size=0.89), grip strength, (18±5.3 Kg vs. 20±4.7 Kg, P=0.02, effect size=0.90), lower limb explosive strength (142±36.5 cm vs. 148±24 cm, P=0.02, effect size=0.90), and parent-reported physical health dimension of HRQoL (65.6±9.75 vs. 84.4±20.35, P=0.03, effect size=0.87). CONCLUSIONS: A 12-week tailored centre-based cardiac rehabilitation program was feasible, acceptable, and safe for children with LQTS. Cardiac rehabilitation for children with LQTS presents a new approach aligned with secondary prevention in youth with cardiac diseases. TRIAL REGISTRATION: The trial was registered at Clinicaltrials.gov (NCT05964322, registration date: 27/07/2023).
RESUMO
Background: Acute heart failure (AHF) represents a leading cause of unscheduled hospital stays, frequent rehospitalisations, and mortality worldwide. The aim of our study was to develop a bedside prognostic tool, a multivariable predictive risk score, that is useful in daily practice, thus providing an early prognostic evaluation at admission and an accurate risk stratification after discharge in patients with AHF. Methods: This study is a subanalysis of the STADE HF study, which is a single-centre, prospective, randomised controlled trial enrolling 123 patients admitted to hospital for AHF. Here, 117 patients were included in the analysis, due to data exhaustivity. Regression analysis was performed to determine predictive variables for one-year mortality and/or rehospitalisation after discharge. Results: During the first year after discharge, 23 patients died. After modellisation, the variables considered to be of prognostic relevance in terms of mortality were (1) non-ischaemic aetiology of HF, (2) elevated creatinine levels at admission, (3) moderate/severe mitral regurgitation, and (4) prior HF hospitalisation. We designed a linear model based on these four independent predictive variables, and it showed a good ability to score and predict patient mortality with an AUC of 0.84 (95%CI: 0.76-0.92), thus denoting a high discriminative ability. A risk score equation was developed. During the first year after discharge, we observed as well that 41 patients died or were rehospitalised; hence, while searching for a model that could predict worsening health conditions (i.e., death and/or rehospitalisation), only two predictive variables were identified: non-ischaemic HF aetiology and previous HF hospitalisation (also included in the one-year mortality model). This second modellisation showed a more discrete discriminative ability with an AUC of 0.67 (95% C.I. 0.59-0.77). Conclusions: The proposed risk score and model, based on readily available predictive variables, are promising and useful tools to assess, respectively, the one-year mortality risk and the one-year mortality and/or rehospitalisations in patients hospitalised for AHF and to assist clinicians in the management of patients with HF aiming at improving their prognosis.
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BACKGROUND: In patients with congenital long QT syndrome (LQTS), the risk of ventricular arrhythmia is correlated with the duration of the corrected QT interval and the changes in the ST-T wave pattern on the 12-lead surface electrocardiogram (12L-ECG). Remote monitoring of these variables could be useful. AIM: To evaluate the abilities of two wearable electrocardiogram devices (Apple Watch and KardiaMobile 6L) to provide reliable electrocardiograms in terms of corrected QT interval and ST-T wave patterns in patients with LQTS. METHODS: In a prospective multicentre study (ClinicalTrials.gov identifier: NCT04728100), a 12L-ECG, a 6-lead KardiaMobile 6L electrocardiogram and two single-lead Apple Watch electrocardiograms were recorded in patients with LQTS. The corrected QT interval and ST-T wave patterns were evaluated manually. RESULTS: Overall, 98 patients with LQTS were included; 12.2% were children and 92.8% had a pathogenic variant in an LQTS gene. The main genotypes were LQTS type 1 (40.8%), LQTS type 2 (36.7%) and LQTS type 3 (7.1%); rarer genotypes were also represented. When comparing the ST-T wave patterns obtained with the 12L-ECG, the level of agreement was moderate with the Apple Watch (k=0.593) and substantial with the KardiaMobile 6L (k=0.651). Regarding the corrected QT interval, the correlation with 12L-ECG was strong for the Apple Watch (r=0.703 in lead II) and moderate for the KardiaMobile 6L (r=0.593). There was a slight overestimation of corrected QT interval with the Apple Watch and a subtle underestimation with the KardiaMobile 6L. CONCLUSIONS: In patients with LQTS, the corrected QT interval and ST-T wave patterns obtained with the Apple Watch and the KardiaMobile 6L correlated with the 12L-ECG. Although wearable electrocardiogram devices cannot replace the 12L-ECG for the follow-up of these patients, they could be interesting additional monitoring tools.
Assuntos
Frequência Cardíaca , Síndrome do QT Longo , Valor Preditivo dos Testes , Dispositivos Eletrônicos Vestíveis , Humanos , Síndrome do QT Longo/fisiopatologia , Síndrome do QT Longo/diagnóstico , Síndrome do QT Longo/congênito , Síndrome do QT Longo/genética , Feminino , Masculino , Estudos Prospectivos , Criança , Adolescente , Adulto , Reprodutibilidade dos Testes , Adulto Jovem , Eletrocardiografia Ambulatorial/instrumentação , Potenciais de Ação , Pré-Escolar , Desenho de Equipamento , Fatores de Tempo , Pessoa de Meia-Idade , Eletrocardiografia/instrumentação , Sistema de Condução Cardíaco/fisiopatologiaRESUMO
BACKGROUND: The PRADO-IC (Programme de Retour à Domicile après une Insuffisance Cardiaque) is a transition care program designed to improve the coordination of care between hospital and home that was generalized in France in 2014. The PRADO-IC consists of an administrative assistant who visits patients during hospitalization to schedule follow-up visits. The aim of the present study was to evaluate the PRADO-IC program based on the hypotheses provided by health authorities. METHODS AND RESULTS: The PRADOC study is a multicenter, controlled, randomized, open-label, mixed-method trial of the transition program PRADO-IC versus usual management in patients hospitalized with heart failure (standard of care group; NCT03396081). A total of 404 patients were recruited between April 2018 and May 2021. The mean patient age was 75 years (±12 years) in both groups. The 2 groups were well balanced regarding severity indices. At discharge, patients homogeneously received the recommended drugs. There was no difference between groups regarding hospitalizations for acute heart failure at 1 year, with 24.60% in the standard of care group and 25.40% in the PRADO-IC group during the year following the index hospitalization (hazard ratio, 1.04 [95% CI, 0.69-1.56]; P=0.85) or cardiovascular mortality (hazard ratio, 0.67 [95% CI, 0.34-1.31]; P=0.24). CONCLUSIONS: The PRADO-IC has not significantly improved clinical outcomes, though a trend toward reduced cardiovascular mortality is evident. These results will help in understanding how transitional care programs remain to be integrated in pathways of current patients, including telemonitoring, and to better tailor individualized approaches. REGISTRATION: URL: https://www.clinicaltrials.gov; Unique Identifier: NCT03396081.
Assuntos
Insuficiência Cardíaca , Humanos , Insuficiência Cardíaca/terapia , Insuficiência Cardíaca/mortalidade , Insuficiência Cardíaca/diagnóstico , Feminino , Masculino , França , Idoso , Idoso de 80 Anos ou mais , Cuidado Transicional/organização & administração , Hospitalização/estatística & dados numéricos , Pessoa de Meia-Idade , Alta do Paciente , Fatores de TempoRESUMO
BACKGROUND: SCN5A variants are associated with a spectrum of cardiac electrical disorders with clear phenotypes. However, they may also be associated with complex phenotypic traits like overlap syndromes, or pleiotropy, which have not been systematically described. Additionally, the involvement of SCN5A in dilated cardiomyopathies (DCM) remains controversial. OBJECTIVE: We aimed to (1) evaluate the different phenotypes associated with pathogenic (P)/likely pathogenic (LP) SCN5A variants and (2) determine the prevalence of pleiotropy in a large multicentric cohort of P/LP SCN5A variant carriers. METHODS: The DNA of 13,510 consecutive probands (9960 with cardiomyopathies) was sequenced using a custom panel of genes. Individuals carrying a heterozygous single P/LP SCN5A variant were selected and phenotyped. RESULTS: The study included 170 P/LP variants found in 495 patients. Among them, 119 (70%) were exclusively associated with a single well-established phenotype: 91 with Brugada syndrome, 15 with type 3 long QT syndrome, six with progressive cardiac conduction disease, four with multifocal ectopic Purkinje-related premature contraction, and three with sick sinus syndrome. Thirty-two variants (19%) were associated with overlap syndromes and/or pleiotropy. The 19 remaining variants (11%) were associated with atypical or unclear phenotypes. Among those, eight were carried by eight patients presenting with DCM with a debatable causative genotype/phenotype link. CONCLUSION: Most P/LP SCN5A variants were found in patients with primary electrical disorders, mainly Brugada syndrome. Nearly 20% were associated with overlap syndromes or pleiotropy, underscoring the need for comprehensive phenotypic evaluation. The concept of SCN5A variants causing DCM is extremely rare (8/9960), if not questionable.