RESUMO
AIMS: To describe the incidence and identify predictors of sudden death (SD), major conduction defects and sustained ventricular tachyarrhythmias (VTA) in myotonic dystrophy type 1 (DM1). METHODS AND RESULTS: We retrospectively enrolled 1388 adults with DM1 referred to six French medical centres between January 2000 and October 2013. We confirmed their vital status, classified all deaths, and determined the incidence of major conduction defects requiring permanent pacing and sustained VTA. We searched for predictors of overall survival, SD, major conduction defects, and sustained VTA by Cox regression analysis. Over a median 10-year follow-up, 253 (18.2%) patients died, 39 (3.6%) suddenly. Analysis of the cardiac rhythm at the time of the 39 SD revealed sustained VTA in 9, asystole in 5, complete atrioventricular block in 1 and electromechanical dissociation in two patients. Non-cardiac causes were identified in the five patients with SD who underwent autopsies. Major conduction defects developed in 143 (19.3%) and sustained VTA in 26 (2.3%) patients. By Cox regression analysis, age, family history of SD and left bundle branch block were independent predictors of SD, while age, male sex, electrocardiographic conduction abnormalities, syncope, and atrial fibrillation were independent predictors of major conduction defects; non-sustained VTA was the only predictor of sustained VTA. CONCLUSIONS: SD was a frequent mode of death in DM1, with multiple mechanisms involved. Major conduction defects were by far more frequent than sustained VTA, whose only independent predictor was a personal history of non-sustained VTA. ClinicalTrials.gov no: NCT01136330.
Assuntos
Doença do Sistema de Condução Cardíaco/etiologia , Morte Súbita Cardíaca/etiologia , Distrofia Miotônica/complicações , Adulto , Fatores Etários , Bloqueio Atrioventricular/etiologia , Bloqueio Atrioventricular/mortalidade , Bloqueio de Ramo/etiologia , Bloqueio de Ramo/mortalidade , Doença do Sistema de Condução Cardíaco/mortalidade , Estimulação Cardíaca Artificial , Desfibriladores Implantáveis , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Distrofia Miotônica/mortalidade , Linhagem , Estudos Retrospectivos , Taquicardia Ventricular/etiologia , Taquicardia Ventricular/mortalidadeAssuntos
Desfibriladores Implantáveis , Remoção de Dispositivo/métodos , Cardioversão Elétrica/instrumentação , Terapia a Laser , Falha de Prótese , Malformações Vasculares/complicações , Veia Cava Superior/anormalidades , Angiografia por Tomografia Computadorizada , Feminino , Humanos , Pessoa de Meia-Idade , Flebografia/métodos , Desenho de Prótese , Fatores de Risco , Falha de Tratamento , Malformações Vasculares/diagnóstico por imagem , Veia Cava Superior/diagnóstico por imagemRESUMO
We report the case of a 92-year old woman with congenitally corrected transposition of the great arteries (CCTGA). The case is remarkable as the CCTGA had remained undiagnosed until the diagnosis was made when the patient was aged 70 years, and had 10 pregnancies without heart failure.
Assuntos
Transposição dos Grandes Vasos , Idoso de 80 Anos ou mais , Transposição das Grandes Artérias Corrigida Congenitamente , Diagnóstico Tardio , Ecocardiografia Tridimensional , Feminino , Humanos , Transposição dos Grandes Vasos/diagnóstico , Transposição dos Grandes Vasos/diagnóstico por imagem , Transposição dos Grandes Vasos/fisiopatologiaRESUMO
AIMS: Lead fractures in implantable cardioverter-defibrillator (ICD) patients may cause inappropriate shocks (ISs). An early diagnosis is essential to prevent adverse clinical events. Implantable cardioverter-defibrillator remote monitoring (RM) permits prompt detection of lead fracture. Limited data define the impact of RM on ISs specifically related to lead fracture. We sought to compare the number of ISs related to lead fracture in patients with vs. without RM follow-up. METHODS AND RESULTS: We checked the registry of our institution and collected, between July 2007 and June 2014, 115 cases of right ventricular lead fractures. All relevant data were documented from patients' files, device-interrogation printouts and electronic records, and remote transmissions databases when applicable. We assessed the ISs that were related to lead fracture. The first study endpoint was the number of ISs per shocked patient. Among the 82 patients with conventional follow-up (CFU) and the 33 patients with RM, a first IS occurred to 32.9% (n = 27) and 30.3% (n = 10, P = 0.83) of the patients, respectively. Shocked patients in the RM group underwent significantly fewer ISs with a mean of 6 ± 2 shocks per patient [median of 3.5 shocks (2-8)] than those in the CFU group with a mean of 18 ± 5 shocks per patient [median of 10 shocks (5-22), P = 0.03]. CONCLUSION: Remote monitoring helps to reduce the burden of ISs related to ICD lead fractures.
Assuntos
Desfibriladores Implantáveis/efeitos adversos , Cardioversão Elétrica/efeitos adversos , Falha de Equipamento , Tecnologia de Sensoriamento Remoto , Feminino , Seguimentos , França , Humanos , Masculino , Pessoa de Meia-Idade , Sistema de RegistrosRESUMO
BACKGROUND: Implantable cardioverter-defibrillators (ICDs) are a standard means of sudden cardiac death prevention. Compared with ambulatory visits, remote monitoring (RM) of ICD recipients has improved the quality of health care and spared its resources. Few studies have addressed the organization of RM. We optimized and validated our institutional model of RM organization for ICD recipients. METHODS AND RESULTS: This observational study of 562 ICD recipients compared 2 RM periods consisting of iterative, qualitative, and quantitative (1) device diagnostic evaluations by nurses and cardiologists; and (2) selected decisional trees. The main study end points were the professional interventions prompted by, and times allocated to, RM alerts. During the first period, 1134 alerts occurred in 427 patients (286 patient-year), of which 376 (33%) were submitted to cardiologists' reviews, compared with, 1522 alerts in 562 patients (458 patient-year), of which 273 (18%) were submitted to cardiologists' reviews during the second period (P<0.001). An intervention was prompted by 73 of 376 (19.4%) alerts in the first versus 77 of 273 (28.2%) in the second period (P=0.009). The mean time to manage an alert was 4 minutes 31 s in the first versus 2 minutes 10 s in the second period (P<0.001). The annual numbers of alert-related hospitalizations were 10.8 versus 8.1 per 100-patient-year (P=0.230), and annual numbers of alert-related visits were 9.8 and 6.1 per 100-patient-year (P=0.081), respectively. CONCLUSIONS: An optimized RM organization based on automated alerts and decisional trees enabled a focus on clinically relevant events and a decrease in the consumption of resources without compromising the quality of ICD recipients' care.
Assuntos
Morte Súbita Cardíaca/prevenção & controle , Desfibriladores Implantáveis , Gerenciamento Clínico , Insuficiência Cardíaca/terapia , Modelos Organizacionais , Monitorização Fisiológica/métodos , Telemedicina/métodos , Feminino , Seguimentos , Insuficiência Cardíaca/fisiopatologia , Hospitalização/tendências , Humanos , Masculino , Pessoa de Meia-Idade , Qualidade de Vida , Reprodutibilidade dos Testes , Estudos Retrospectivos , Fatores de TempoAssuntos
Arritmias Cardíacas/diagnóstico por imagem , Sistema de Condução Cardíaco/anormalidades , Distrofia Miotônica/diagnóstico por imagem , Adulto , Arritmias Cardíacas/fisiopatologia , Síndrome de Brugada , Doença do Sistema de Condução Cardíaco , Ecocardiografia/métodos , Feminino , Sistema de Condução Cardíaco/diagnóstico por imagem , Sistema de Condução Cardíaco/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Distrofia Miotônica/fisiopatologia , Disfunção Ventricular Esquerda/diagnóstico por imagem , Disfunção Ventricular Esquerda/fisiopatologiaRESUMO
BACKGROUND: Remote monitoring (RM) is increasingly used to follow up patients with implantable cardioverter-defibrillators (ICDs). Randomized control trials provide evidence for the benefit of this intervention, but data for RM in daily clinical practice with multiple-brands and unselected patients is lacking. AIMS: To assess the effect of RM on patient management and clinical outcome for recipients of ICDs in daily practice. METHODS: We reviewed ICD recipients followed up at our institution in 2009 with RM or with traditional hospital only (HO) follow-up. We looked at the effect of RM on the number of scheduled ambulatory follow-ups and urgent unscheduled consultations, the time between onset of asymptomatic events to clinical intervention and the clinical effectiveness of all consultations. We also evaluated the proportion of RM notifications representing clinically relevant situations. RESULTS: We included 355 patients retrospectively (RM: n=144, HO: n=211, 76.9% male, 60.3±15.2 years old, 50.1% with ICDs for primary prevention and mean left ventricular ejection fraction 35.5±14.5%). Average follow-up was 13.5 months. The RM group required less scheduled ambulatory follow-up consultations (1.8 vs. 2.1/patient/year; P<0.0001) and a far lower median time between the onset of asymptomatic events and clinical intervention (7 vs. 76 days; P=0.016). Of the 784 scheduled ambulatory follow-up consultations carried out, only 152 (19.4%) resulted in therapeutic intervention or ICD reprogramming. We also found that the vast majority of RM notifications (61.9%) were of no clinical relevance. CONCLUSION: RM allows early management of asymptomatic events and a reduction in scheduled ambulatory follow-up consultations in daily clinical practice, without compromising safety, endorsing RM as the new standard of care for ICD recipients.
Assuntos
Arritmias Cardíacas/terapia , Morte Súbita Cardíaca/prevenção & controle , Desfibriladores Implantáveis , Cardioversão Elétrica/instrumentação , Prevenção Primária/instrumentação , Ensaios Clínicos Controlados Aleatórios como Assunto , Telemedicina/métodos , Telemetria , Adulto , Idoso , Assistência Ambulatorial , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/mortalidade , Arritmias Cardíacas/fisiopatologia , Medicina Baseada em Evidências , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Encaminhamento e Consulta , Estudos Retrospectivos , Volume Sistólico , Fatores de Tempo , Resultado do Tratamento , Função Ventricular EsquerdaRESUMO
BACKGROUND: For patients presenting with syncope and bundle branch block (BBB), results during electrophysiological studies (EPS) might depend on the electrocardiographic pattern of conduction disturbances. We sought to identify predictors of advanced His-Purkinje conduction disturbances (HPCDs) in these patients. METHODS: In this retrospective multicentre study, patients were included who: (1) presented with unexplained syncope; (2) had BBB (QRS duration ≥ 120 ms); and (3) were investigated with EPS. HPCD was diagnosed if the baseline His-ventricular interval was ≥ 70 ms or if second- or third-degree His-Purkinje block was observed during atrial pacing or pharmacological challenge. RESULTS: Of the 171 patients studied (72 ± 13 years, 64% male sex, mean left ventricular ejection fraction 57 ± 9%), advanced HPCD was found in 73 patients (43%). The following electrocardiographic features were associated with HPCD (P = 0.01): isolated right BBB (34.4%), right BBB with left anterior fascicular block (36.4%), left BBB (46.2%), and right BBB with left posterior fascicular block (LPFB, 78.6%). Multivariate analysis identified first-degree atrioventricular block (odds ratio, 2.4; 95% confidence interval, 1.2-4.7; P = 0.01) and LPFB (odds ratio, 4.8; 95% confidence interval, 1.3-18.5; P = 0.02) as the only 2 independent predictors of advanced HPCD. CONCLUSIONS: For patients presenting with syncope and BBB, first-degree atrioventricular block and LPFB increased the likelihood of finding HPCDs during EPS. However, no single electrocardiographic feature could consistently predict the outcome of EPS, so this investigation is still necessary in assessing the need for pacemaker implantation, irrespective of the precise appearance of abnormalities on ECG.
Assuntos
Fascículo Atrioventricular/fisiopatologia , Bloqueio de Ramo/fisiopatologia , Técnicas Eletrofisiológicas Cardíacas , Ramos Subendocárdicos/fisiopatologia , Síncope/fisiopatologia , Idoso , Idoso de 80 Anos ou mais , Bloqueio Atrioventricular/classificação , Bloqueio Atrioventricular/fisiopatologia , Bloqueio Atrioventricular/cirurgia , Bloqueio de Ramo/diagnóstico , Bloqueio de Ramo/cirurgia , Desfibriladores Implantáveis , Eletrocardiografia , Feminino , Humanos , Masculino , Análise Multivariada , Marca-Passo Artificial , Estudos Retrospectivos , Volume Sistólico/fisiologiaRESUMO
AIMS: To describe obstetric/neonatal and cardiac outcomes for a cohort of women carrying implantable cardioverter-defibrillators (ICDs) during pregnancy. METHODS AND RESULTS: All women in routine follow-up at our institution for ICD implantation who became pregnant between 2006 and 2013 were included in this study. All ICDs were pre-pectoral devices with bipolar endocardial leads. Obstetric/neonatal and cardiac outcomes were assessed during pregnancy and post-partum. Twenty pregnancies were conceived by 12 women carrying ICD devices, 14 of which resulted in live births and none in maternal death. Seven of these women had structural cardiomyopathies and five had channelopathies. No device-related complications were recorded. Twelve shocks (nine transthoracic and three from ICDs) were experienced during pregnancy by two women, one of whom miscarried shortly afterwards at 4 weeks gestation. One stillbirth, three miscarriages and one termination were recorded for women with long QT syndrome, repaired tetralogy of Fallot and repaired Laubry-Pezzi syndrome, respectively. Intrauterine growth restriction, low birth weight, and neonatal hypoglycaemia were recorded in four, three, and five pregnancies, respectively. CONCLUSIONS: Pregnancy had no effect on ICD operation and no evidence was found to link ICD carriage with adverse pregnancy outcomes, although one miscarriage may have been induced by ICD shock therapy. A worsening of cardiac condition occurs in specific cardiac diseases and ß-blocker therapy should be continued for all women carrying ICDs in pregnancy as the benefits outweigh the risks of taking this medication.
Assuntos
Cardiomiopatias/terapia , Desfibriladores Implantáveis , Cardioversão Elétrica/instrumentação , Cardiopatias Congênitas/terapia , Complicações Cardiovasculares na Gravidez/terapia , Aborto Espontâneo/etiologia , Antagonistas Adrenérgicos beta/uso terapêutico , Adulto , Cardiomiopatias/congênito , Cardiomiopatias/diagnóstico , Cardiomiopatias/fisiopatologia , Progressão da Doença , Cardioversão Elétrica/efeitos adversos , Eletrocardiografia , Feminino , França , Idade Gestacional , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/fisiopatologia , Humanos , Nascido Vivo , Segurança do Paciente , Gravidez , Complicações Cardiovasculares na Gravidez/diagnóstico , Complicações Cardiovasculares na Gravidez/fisiopatologia , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Adulto JovemRESUMO
PURPOSE: This study aims to study the clinical implications of the concomitant use of a left ventricular assist device (LVAD) and an implantable cardioverter-defibrillator (ICD). METHODS: In this retrospective study, all patients who underwent LVAD (Heart Mate II) implantation with concomitant ICD therapy at our institution between June 2007 and August 2012 were included. We sought to investigate (1) the electromagnetic interference between LVAD and ICD telemetry, (2) the effect of LVAD implantation on right ventricular (RV) lead parameters and (3) the ventricular tachyarrhythmias (VAs) that occur post-LVAD implantation. RESULTS: Of the 23 patients (53 ± 9 years, 73 % male, LVEF 19 ± 9 %) included, ICD telemetry was lost in four patients post-LVAD implantation (Saint-Jude-Medical Atlas V-193, V-240, V-243, and Sorin CRT-8750), prompting either use of a metal shield (n = 1), a change in position of the programmer head (n = 1) or ICD replacement (n = 2). LVAD implantation was associated with a decrease in both RV signal amplitude (p = 0.04) and RV impedance (p < 0.01), and a trend towards an increased RV pacing threshold (p = 0.08), without affecting clinical outcome. Eleven patients (47.8 %) experienced VAs after LVAD implantation, which on the whole were well tolerated. Their occurrence was strongly linked to a history of VAs before device implantation (p < 0.01). CONCLUSIONS: Electromagnetic interference between LVADs and ICD telemetry may necessitate ICD replacement. LVAD placement is associated with significant changes in RV lead parameters that have minimal clinical significance. VAs occur in approximately half of LVAD patients seen and their occurrence is strongly related to a history of VAs prior to LVAD implantation.
Assuntos
Desfibriladores Implantáveis/efeitos adversos , Falha de Equipamento , Insuficiência Cardíaca/prevenção & controle , Coração Auxiliar/efeitos adversos , Fibrilação Ventricular/etiologia , Fibrilação Ventricular/prevenção & controle , Adulto , Idoso , Terapia Combinada/efeitos adversos , Terapia Combinada/instrumentação , Feminino , Insuficiência Cardíaca/complicações , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do TratamentoRESUMO
BACKGROUND: Mechanical dyssynchrony associated with rapid pacing induces cardiac cell stress and myocardial apoptotic pathway activation that has been implicated in the pathophysiology of left ventricular (LV) dysfunction. Effects of dyssynchrony per se are not fully understood. The objective of our study was to test whether ventricular dyssynchrony would elicit myocardial alterations in LV calcium handling regulation and cell survival or apoptosis signalling in right ventricular-paced swine. METHODS: Implantation of pacemaker was performed under anaesthesia. Endocardial bipolar screw lead was inserted into the right jugular vein and positioned either in the right atrium or at the right ventricular (RV) apex. Swine were paced at 150 beats per minute for 3 weeks. RESULTS: Compared with right atrial pacing, RV pacing led to abnormal LV sarcoplasmic reticulum calcium uptake (315 ± 65 vs 155 ± 55 nmol/min/mg, P < 0.05) and LV calcium-handling protein expression, ie, 35% reduction in ryanodine receptor 2, 25% decline in sarcoplasmic reticulum Ca(2+) ATPase, 70% increase in Na(+)/Ca(2+) exchanger, and 10% increase in phospholamban. RV pacing also elicited activation of LV apoptotic cascades without nuclear apoptosis. So-called interrupted apoptosis was the result of increased expression of X-linked inhibitor of apoptosis protein. Apoptosis and calcium mishandling were documented in absence of depressed heart function (ejection fraction 62 ± 8% vs 57 ± 12%, in right atrial- and RV-paced hearts, respectively, P > 0.05). CONCLUSIONS: Slow rate RV pacing causes mechanical dyssynchrony and profound LV alterations in both apoptotic pathways and calcium handling in the early stages of pacing-induced cardiomyopathy.
Assuntos
Apoptose , Cálcio/metabolismo , Estimulação Cardíaca Artificial/efeitos adversos , Cardiomiopatias/etiologia , Frequência Cardíaca , Ventrículos do Coração , Miócitos Cardíacos/metabolismo , Animais , Western Blotting , Proteínas de Ligação ao Cálcio/metabolismo , ATPases Transportadoras de Cálcio/metabolismo , Cardiomiopatias/metabolismo , Caspases/metabolismo , Sobrevivência Celular , Ecocardiografia , Ensaio de Imunoadsorção Enzimática , Feminino , Regulação da Expressão Gênica , Ventrículos do Coração/metabolismo , Ventrículos do Coração/fisiopatologia , Reação em Cadeia da Polimerase em Tempo Real , Reação em Cadeia da Polimerase Via Transcriptase Reversa , Canal de Liberação de Cálcio do Receptor de Rianodina/metabolismo , Retículo Sarcoplasmático/metabolismo , Suínos , Fator de Necrose Tumoral alfa/análiseRESUMO
BACKGROUND: Little is known about predictors of antitachycardia pacing (ATP) failure in implantable cardioverter defibrillator (ICD) recipients. Distance between the stimulation site and the ventricular tachycardia (VT) site of origin may critically affect ATP effectiveness. We hypothesized that ATP may be less effective in ICD patients who had basal VT than in those who had apical VT. METHODS: We reviewed data from 52 patients with sustained monomorphic VT and left ventricular disease referred for ICD implantation. ATP was delivered exclusively at the right ventricular apex. The clinical VTs site of origin (basal, midventricular, or apical) was determined in each patient, using 12-lead electrocardiogram. VTs episodes treated with ATP during the 1-year follow-up were studied. ATP success rate (%), defined as the ratio between the number of successful ATP sequences and the number of delivered ATP sequences, was determined in each patient. RESULTS: VT exit site was apical in 19 patients (36%), basal in 18 patients (35%), and midventricular in 15 patients (29%). In those 52 patients, 1,393 ATP sequences, delivered to treat 761 VT episodes, were analyzed. ATP success rate was found to be associated with the VT site of origin (median [interquartile range]): basal (33%[11-67]), midventricular (50%[37-100]), apical (100%[41-100]) (P = 0.027). Multivariate analysis identified basal VT site of origin as an independent predictor of ATP failure (P = 0.023). CONCLUSION: ATP is less effective in ICD patients who had basal VT than in those who had apical VT before ICD implantation.