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1.
Presse Med ; 48(12): 1401-1405, 2019 Dec.
Artigo em Francês | MEDLINE | ID: mdl-31699540

RESUMO

Sudden cardiac death represents a major public health issue, with up to 50% of the cardiovascular mortality. Coronary artery disease and dilated cardiomyopathy both represent almost 90% of sudden cardiac death burden. Primary prevention using implantable cardioverter defibrillator relies, in this population, on the left ventricle ejection fraction simple measurement. In this paper, we aim to discuss in which extent a better understanding of competing risk situation may help for a better patient selection and eventually for optimizing primary prevention using implantable cardioverter defibrillator.


Assuntos
Morte Súbita Cardíaca/prevenção & controle , Desfibriladores Implantáveis , Cardiomiopatia Dilatada/epidemiologia , Cardiomiopatia Dilatada/terapia , Morte Súbita Cardíaca/epidemiologia , Humanos , Seleção de Pacientes , Prevenção Primária/instrumentação , Prevenção Primária/métodos , Risco , Medição de Risco , Fatores de Risco , Disfunção Ventricular Esquerda/epidemiologia , Disfunção Ventricular Esquerda/terapia
2.
Zhonghua Xin Xue Guan Bing Za Zhi ; 47(11): 901-906, 2019 Nov 24.
Artigo em Chinês | MEDLINE | ID: mdl-31744280

RESUMO

Objective: To investigate the clinical characteristics and prognostic factors after catheter ablation of accessory pathway (AP)-induced dilated cardiomyopathy (DCM) in children. Methods: Data were collected and analyzed on 147 consecutive pediatric patients (81males and 66 females), who hospitalized in our pediatric heart center between January 2009 and August 2018 and received catheter ablation for ventricular pre-excitation (right AP). Thirty-one children were diagnosed as AP-induced DCM and 116 children with normal cardiac function served as control. Data including clinical characteristics, electrocardiogram (ECG), echocardiography, electrophysiological examination (EPS), successful ablation and follow up were analyzed. Results: The median age at first examination was 3.07 (0.09, 5.83) years. The pre-hospital misdiagnosis rate was 87.1% (27/31). The incidence of the AP-induced DCM was 21.1% (31/147) and the rate of right anterior free wall accessory pathway (AP) was the highest (50.0%, 10/20). AP-induced DCM was more common in right free wall Aps (41.9%, 13/31) and right anterior free wall (32.3%, 10/31). 48.4% (15/31) cases had no supraventricular tachycardia (SVT). EPS showed that 29% (9/31) of the AP did not have retrograde conduction. The median follow-up time after successful catheter ablation was 8 (2, 36) months of AP-induced DCM group, LVDd was significantly decreased ((41.6±12.8)mm vs. (45.6±13.9)mm, P<0.01) and LVEF ((56.2±11.8)% vs. (40.8±12.5)%, P<0.01) was significantly increased after ablation. Cox regression analysis showed that LVEF was the predictor of the duration of cardiac function recovery (HR=1.08, 95%CI 1.01-1.15, P=0.03). Conclusions: Misdiagnosis rate is high for children with AP-induced DCM, leading to the delayed treatment. All of the AP-induced DCM occurred in right APs and right anterior free wall APs is the highest. Right free wall APs and right anterior free wall are most common in AP-induced DCM. Catheter ablation is a safe and effective treatment option for these patients. The lower the LVEF, the longer the cardiac function recovery.


Assuntos
Cardiomiopatia Dilatada , Ablação por Cateter , Fascículo Atrioventricular , Cardiomiopatia Dilatada/diagnóstico , Cardiomiopatia Dilatada/terapia , Pré-Escolar , Eletrocardiografia , Feminino , Humanos , Lactente , Masculino , Prognóstico
3.
Int Heart J ; 60(5): 1201-1205, 2019 Sep 27.
Artigo em Inglês | MEDLINE | ID: mdl-31484869

RESUMO

Right-sided accessary pathways in patients with Wolff-Parkinson-White (WPW) syndrome may cause cardiac dyssynchrony and dilated cardiomyopathy, with a characteristic septal shape, irrespective of any supraventricular tachycardia episodes. We report on two infants (13 and 5 months), whose right-sided accessary pathway-induced dilated cardiomyopathy was successfully treated by flecainide for the first time. After the flecainide administration, an abnormal aneurysmal dilation of the basal interventricular septum was almost restored to normal, and the decreased ejection fraction recovered. Flecainide use may be an important therapeutic option for this entity to avoid catheter ablation during infancy.


Assuntos
Feixe Acessório Atrioventricular/diagnóstico por imagem , Feixe Acessório Atrioventricular/tratamento farmacológico , Cardiomiopatia Dilatada/diagnóstico por imagem , Flecainida/uso terapêutico , Síndrome de Wolff-Parkinson-White/diagnóstico por imagem , Feixe Acessório Atrioventricular/complicações , Cardiomiopatia Dilatada/tratamento farmacológico , Cardiomiopatia Dilatada/etiologia , Cardiomiopatia Dilatada/terapia , Ecocardiografia Doppler/métodos , Eletrocardiografia/métodos , Feminino , Seguimentos , Humanos , Lactente , Injeções Intravenosas , Masculino , Medição de Risco , Amostragem , Resultado do Tratamento , Síndrome de Wolff-Parkinson-White/complicações , Síndrome de Wolff-Parkinson-White/fisiopatologia
4.
BMJ Case Rep ; 12(8)2019 Aug 05.
Artigo em Inglês | MEDLINE | ID: mdl-31387863

RESUMO

The pathophysiology of narrow complex dilated cardiomyopathy is not defined, so therapeutic options are limited. By utilising the velocity equation, the pathophysiology of narrow complex cardiomyopathy allows above normal conduction propagation velocities. There are two pathophysiological theories that allow above normal conduction velocities and failure to capture the myocardium: (1)insulating fibres of the conduction system extending beyond the apex and (2) reduction of axon branching. A patient with narrow complex cardiomyopathy was subjected to graded increase in amplitude and pulse width pacing to overcome the failure of native conduction to capture the myocardium. Peak systolic strain maps demonstrated a progressive increase in apical contractility with increasing pulse width and amplitude. Ejection fraction improved from 17% to 31%. Understanding the pathophysiology of narrow complex cardiomyopathy leads to proposed therapies. One potential pacing therapy is multi-lead pacing at high amplitude and pulse width to capture myocardial cells not captured by native conduction.


Assuntos
Cardiomiopatia Dilatada/fisiopatologia , Sistema de Condução Cardíaco/fisiopatologia , Idoso , Estimulação Cardíaca Artificial/métodos , Cardiomiopatia Dilatada/terapia , Desfibriladores Implantáveis , Feminino , Insuficiência Cardíaca/etiologia , Frequência Cardíaca , Humanos
5.
Acta Myol ; 38(1): 1-7, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-31309174

RESUMO

Cardiomyopathy associated with dystrophinopathies - Duchenne muscular Dystrophy (DMD), Becker muscular dystrophy (BMD), X-linked dilated cardiomyopathy (XL-CM) and cardiomyopathy of Duchenne/Becker (DMD/BMD carriers - is an almost constant manifestation of these neuromuscular disorders and contribute significantly to their morbidity and mortality. Dystrophinopathic cardiomyopathy is the result of the dystrophin protein deficiency at the myocardium level, parallel to that occurring at the skeletal muscle level. Typically, cardiomyopathy begins as a "presymptomatic" stage in the first decade of life and evolves in a stepwise manner toward an end-stage dilated cardiomyopathy. Nearly complete replacement of the myocardium by fibrous and fatty connective tissue results in an irreversible cardiac failure, characterized by a further reduction of ejection fraction (EF < 30%) and frequent episodes of acute heart failure (HF). The picture of a severe dilated cardiomyopathy with intractable heart failure is typical of dystrophinopathies. Despite an appropriate pharmacological treatment, this condition is irreversible because of the extensive loss of myocites. Heart transplantation is the only curative therapy for patients with end-stage heart failure, who remain symptomatic despite an optimal medical therapy. However there is a reluctance to perform heart transplantation (HT) in these patients due to the scarcity of donors and the concerns that the accompanying myopathy will limit the benefits obtained through this therapeutic option. Therefore the only possibility to ameliorate clinical symptoms, prevent fatal arrhythmias and cardiac death in dystrophinopathic patients could be the implantation of intracardiac device (ICD) or resynchronizing devices with defibrillator (CRT-D). This overview reports the personal series of patients affected by DMD and BMD and DMD carriers who received ICD or CRT-D system, describe the clinical outcomes so far published and discuss pro and cons in the use of such devices.


Assuntos
Cardiomiopatia Dilatada/diagnóstico , Cardiomiopatia Dilatada/terapia , Desfibriladores Implantáveis , Distrofias Musculares/complicações , Adolescente , Criança , Ecocardiografia , Eletrocardiografia , Feminino , Humanos , Masculino , Estudos Retrospectivos
6.
Eur J Pediatr ; 178(8): 1229-1235, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31187263

RESUMO

Dilated cardiomyopathy is the most common form of childhood cardiomyopathy and is known to result in significant morbidity and mortality. This study aims to review the aetiology and associated outcomes of DCM. The median age at diagnosis was 6 months (0-42 months); n = 23 (43.3%) were idiopathic; n = 11 (20.9%) secondary to a viral infection; n = 12 (22.6%) genetic disorders and n = 7 (13.2%) as a result of vitamin D deficiency. There was a significant correlation between aetiology and mortality, r = 0.85, with a lower survival rate in idiopathic and genetic cohorts. Males were significantly less likely to survive to 1 year of age, p = 0.035. The age at diagnosis did not alter survival to 1 year and the predicted survival beyond 1 year was 84.3% (95% CI, 71.3 to 94.5%). Severely impaired left ventricular fractional shortening at presentation (< 15%) was an independent predictor of death, p = 0.002, (95% CI, 11.2 to 14.2%).Conclusion: Paediatric DCM is a heterogeneous disease resulting in significant morbidity. The aetiology alters the age of presentation. Identification of a specific cause is a useful for risk stratification and prognostication. The first year after diagnosis is a critical time period reflected by the significant morbidity and mortality. What is Known: •Paediatric dilated cardiomyopathy (DCM) is the commonest of the childhood cardiomyopathies, with significant associated morbidity and mortality. •DCM is most commonly idiopathic. What is New: •Identifying the aetiology of DCM in the paediatric population aids risk stratification and prognostication. •The first year after diagnosis of DCM is associated with significant mortality.


Assuntos
Cardiomiopatia Dilatada/etiologia , Cardiomiopatia Dilatada/diagnóstico , Cardiomiopatia Dilatada/mortalidade , Cardiomiopatia Dilatada/terapia , Pré-Escolar , Bases de Dados Factuais , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Prognóstico , Estudos Retrospectivos , Reino Unido/epidemiologia
7.
J Cardiothorac Surg ; 14(1): 121, 2019 Jun 28.
Artigo em Inglês | MEDLINE | ID: mdl-31253165

RESUMO

BACKGROUND: Pacemaker implantation techniques using thoracoscopy have been described since about 25 years. However, the published reports concerning types of electrodes refer mostly to monopolar screw-in leads. We report our experience of thoracoscopic implantation of a bipolar suture-on epicardial electrode with monofilamentous sutures tightened by automated fasteners to avoid hand-tied knots. CASE PRESENTATION: A 69-year-old Caucasian female patient with a cardiac resynchronization therapy - defibrillator (CRT-D) due to dilated cardiomyopathy required the implantation of a supplementary left ventricluar resynchronization electrode. Because of unfavorable venous access, we chose a thoracoscopic approach. A bipolar suture-on epicardial electrode, was implanted by means of polypropylene monofilament 2-0 threads and automated titanium fasteners (Cor-Knot®). The intervention was uneventful. The correct function of the device was confirmed postoperatively and the patient was dismissed within 3 days from hospital. Six months after implantation the cardiologic control asserted regular device function and restitution of normal ejection fraction (EF 60%). CONCLUSION: This case demonstrates the feasibility, safety and effectiveness of automated fasteners in the setting of thoracoscopic implantation of epicardial bipolar suture-on leads.


Assuntos
Terapia de Ressincronização Cardíaca/métodos , Cardiomiopatia Dilatada/terapia , Ventrículos do Coração/fisiopatologia , Marca-Passo Artificial , Técnicas de Sutura/instrumentação , Toracoscopia/métodos , Titânio , Idoso , Feminino , Humanos , Suturas
8.
Med Hypotheses ; 128: 91-93, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31203918

RESUMO

Dilated cardiomyopathy (DCM) is a disease with high incidence and mortality rates. Its therapies have one primary goal, which is to minimize symptoms and it has only one effective approach to healing, the heart transplantation. As it is widely associated with genetic causes, the use of gene therapies, such as the CRISPR/Cas9 system, is a promising alternative to treat DCM. For this purpose, it is necessary to analyze possible target genes for this approach and what would be the implications of their use. Here, we hypothesized that cardiac troponin I type 3 interacting kinase (TNI3K), involved with superoxide production in DCM patients, besides other factors, could be a good target for the use of gene editing.


Assuntos
Sistemas CRISPR-Cas , Cardiomiopatia Dilatada/genética , Cardiomiopatia Dilatada/terapia , Edição de Genes , Sistema de Sinalização das MAP Quinases , DNA/análise , Genoma Humano , Transplante de Coração , Humanos , Modelos Teóricos , Superóxidos/metabolismo , Troponina I/metabolismo
9.
Nat Rev Dis Primers ; 5(1): 32, 2019 05 09.
Artigo em Inglês | MEDLINE | ID: mdl-31073128

RESUMO

Dilated cardiomyopathy (DCM) is a clinical diagnosis characterized by left ventricular or biventricular dilation and impaired contraction that is not explained by abnormal loading conditions (for example, hypertension and valvular heart disease) or coronary artery disease. Mutations in several genes can cause DCM, including genes encoding structural components of the sarcomere and desmosome. Nongenetic forms of DCM can result from different aetiologies, including inflammation of the myocardium due to an infection (mostly viral); exposure to drugs, toxins or allergens; and systemic endocrine or autoimmune diseases. The heterogeneous aetiology and clinical presentation of DCM make a correct and timely diagnosis challenging. Echocardiography and other imaging techniques are required to assess ventricular dysfunction and adverse myocardial remodelling, and immunological and histological analyses of an endomyocardial biopsy sample are indicated when inflammation or infection is suspected. As DCM eventually leads to impaired contractility, standard approaches to prevent or treat heart failure are the first-line treatment for patients with DCM. Cardiac resynchronization therapy and implantable cardioverter-defibrillators may be required to prevent life-threatening arrhythmias. In addition, identifying the probable cause of DCM helps tailor specific therapies to improve prognosis. An improved aetiology-driven personalized approach to clinical care will benefit patients with DCM, as will new diagnostic tools, such as serum biomarkers, that enable early diagnosis and treatment.


Assuntos
Cardiomiopatia Dilatada/diagnóstico , Cardiomiopatia Dilatada/terapia , Autoimunidade/genética , Autoimunidade/fisiologia , Terapia de Ressincronização Cardíaca/métodos , Cardiomiopatia Dilatada/fisiopatologia , Ecocardiografia/métodos , Eletrocardiografia/métodos , Insuficiência Cardíaca/etiologia , Humanos , Inflamação/complicações , Inflamação/fisiopatologia , Imagem por Ressonância Magnética/métodos , Prognóstico , Qualidade de Vida/psicologia , Fatores Sexuais
11.
Intern Med ; 58(17): 2545-2549, 2019 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-31118394

RESUMO

Becker muscular dystrophy (BMD) carriers are at risk to developing cardiac dysfunction. The prevalence of female BMD carriers remains underestimated, and the disease progression varies. We herein report the case of a young female BMD carrier who developed dilated cardiomyopathy (DCM) and heart failure without any skeletal muscle signs. Her cardiac dysfunction progressed over a mere two months, resulting in the need for left ventricular assist device implantation. Her case demonstrates that progressive cardiomyopathy can be the only clinical manifestation in some BMD carriers, suggesting the need for a more aggressive implementation of genetic testing in female DCM patients.


Assuntos
Cardiomiopatia Dilatada/etiologia , Insuficiência Cardíaca/etiologia , Distrofia Muscular de Duchenne/complicações , Adulto , Cardiomiopatia Dilatada/diagnóstico , Cardiomiopatia Dilatada/terapia , Progressão da Doença , Feminino , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/terapia , Coração Auxiliar , Heterozigoto , Humanos , Distrofia Muscular de Duchenne/genética
12.
Kardiologiia ; 59(4S): 59-64, 2019 May 24.
Artigo em Russo | MEDLINE | ID: mdl-31131761

RESUMO

We present a 13-years follow-up results in patient with dilated cardiomyopathy. We performed intracoronary infusion of bone marrow mononuclear fraction in patient with 4th heart failure functional class in 2005. We observed an improvement in symptoms (patient had 1st functional class of heart failure) during 10-years follow-up. In 2015 due to clinical worsening we performed 2nd and 3rd bone marrow mononuclear cells infusion with 9-month interval. We observed a signifcant improvement in symptoms, EF and LV sizes. We continue the follow-up.


Assuntos
Cardiomiopatia Dilatada , Insuficiência Cardíaca , Transplante de Medula Óssea , Cardiomiopatia Dilatada/terapia , Terapia Baseada em Transplante de Células e Tecidos , Humanos , Resultado do Tratamento
13.
Prog Cardiovasc Dis ; 62(3): 249-255, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31004606

RESUMO

The implantable cardioverter-defibrillator (ICD) has been successfully treating patients with lethal ventricular arrhythmias for decades. The main acute and chronic complications of this therapy modality are related to the use of a transvenous lead. An entirely extravascular ICD concept was developed over the last 20 years, with emergence of the subcutaneous ICD (S-ICD). This device was approved for clinical use seven years ago, and accumulating real-life experience confirms its safety and efficacy. The main limitations related to this system include the lack of pacing capabilities for bradycardia, tachycardia or resynchronization therapy, a large size, and relatively high energy requirements for effective defibrillation. This review article summarizes current knowledge and potential future developments of the extravascular ICD technologies.


Assuntos
Desfibriladores Implantáveis , Algoritmos , Cardiomiopatia Dilatada/diagnóstico por imagem , Cardiomiopatia Dilatada/terapia , Morte Súbita Cardíaca/prevenção & controle , Feminino , Humanos , Masculino , Desenho de Prótese , Radiografia
15.
Int J Cardiol ; 280: 124-129, 2019 04 01.
Artigo em Inglês | MEDLINE | ID: mdl-30679073

RESUMO

BACKGROUND: Presence of myocardial fibrosis in well-established non-ischaemic dilated cardiomyopathy (NIDCM) is associated with adverse clinical outcomes. However, the impact of myocardial fibrosis at first presentation in NIDCM, and its long-term association with left ventricular (LV) dysfunction, heart failure (HF) and ventricular arrhythmia (VA) remains unclear. We investigated whether the presence of myocardial fibrosis quantified by late gadolinium enhancement cardiovascular magnetic resonance (LGE-CMR) at presentation, is independently associated with long-term major adverse cardiovascular events (MACE) in patients with first presentation NIDCM. METHODS: Consecutive patients with a first diagnosis of NIDCM were recruited. Patients underwent LGE-CMR at baseline. Replacement myocardial fibrosis by LGE-CMR was quantified by experienced observers blinded to patient outcome. MACE was defined as a composite end-point including cardiac death, HF rehospitalisation and the occurrence of sustained VA. RESULTS: Fifty-one patients with first presentation NIDCM were included, of which 49 (96%) had follow up and outcome data. Median follow up was 8.2 years. Both the LGE positive and LGE negative groups had similar clinical characteristics at follow up. In univariate Cox regression analysis, positive LGE was associated with MACE (HR:3.44; 95% CI:1.89 to 6.24, p-value < 0.001) and HF rehospitalisation (HR:2.89; 95% CI:1.42 to 5.85, p-value = 0.003). In multivariate Cox regression, positive LGE-CMR was independently associated with MACE (HR:3.53; 95% CI:1.51 to 8.27, p-value = 0.004) and HF rehospitalisation (HR:3.07; 95% CI:1.24 to 7.59, p-value = 0.015). CONCLUSIONS: The presence of myocardial fibrosis in first presentation NIDCM is independently associated with an increased risk of HF rehospitalisation, at long term follow-up.


Assuntos
Cardiomiopatia Dilatada/diagnóstico por imagem , Cardiomiopatia Dilatada/terapia , Gadolínio , Imagem Cinética por Ressonância Magnética/métodos , Idoso , Cardiomiopatia Dilatada/fisiopatologia , Feminino , Seguimentos , Humanos , Imagem Cinética por Ressonância Magnética/tendências , Masculino , Pessoa de Meia-Idade , Readmissão do Paciente/tendências , Prognóstico , Fatores de Tempo
16.
Int J Artif Organs ; 42(6): 321-322, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30630389

RESUMO

Congenitally corrected transposition of the great arteries and situs inversus totalis are rare congenital anomalies. While congenital heart diseases affect about 0.75%-0.9% of newborns, less than 1% of them have congenitally corrected transposition of the great arteries. Meanwhile, the incidence of situs inversus totalis is about 0.01%. This is a case report of a patient with congenitally corrected transposition of the great arteries and situs inversus totalis who was supported with a ventricular assist device, resulting in a challenging clinical scenario.


Assuntos
Cardiomiopatia Dilatada , Oxigenação por Membrana Extracorpórea/métodos , Coração Auxiliar , Situs Inversus/complicações , Transposição dos Grandes Vasos/complicações , Adulto , Insuficiência da Valva Aórtica/diagnóstico , Insuficiência da Valva Aórtica/fisiopatologia , Cardiomiopatia Dilatada/etiologia , Cardiomiopatia Dilatada/terapia , Evolução Fatal , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/etiologia , Masculino , Insuficiência de Múltiplos Órgãos/diagnóstico , Insuficiência de Múltiplos Órgãos/etiologia , Choque Séptico/diagnóstico , Choque Séptico/etiologia , Volume Sistólico
17.
Pacing Clin Electrophysiol ; 42(2): 153-160, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30569458

RESUMO

BACKGROUND: A left ventricular (LV) endocardial lead implant to achieve cardiac resynchronization therapy (CRT) is feasible when a conventional implant failed due to anatomical or technical issues or when the venous implant was performed but the patient did not respond to the therapy. METHODS: Data about the implantation procedure (age, sex, clinical characteristics, anticoagulant use, and previous devices), patient characteristics (indication, technique used, lead model, complications), and follow-up (clinical and echocardiographic outcome, LV lead electrical measurements) were analyzed for all CRT systems implanted using LV endocardial lead, due to failed conventional implant or nonresponse, between April 2011 and November 2016. RESULTS: Thirty-five patients were implanted with an active fixation LV endocardial lead during the study period, without significant complications. There were no dislodgements or severe complications related to the implant procedure in the follow-up period (36 ± 20 months) and a high percentage of patients responded to therapy, as assessed by several indicators. CONCLUSIONS: An LV endocardial lead implant was feasible when the conventional technique had previously failed or was not effective. A high rate of response was achieved without any significant complications.


Assuntos
Terapia de Ressincronização Cardíaca , Cardiomiopatia Dilatada/terapia , Desfibriladores Implantáveis , Marca-Passo Artificial , Idoso , Feminino , Seguimentos , Ventrículos do Coração , Humanos , Masculino , Fatores de Tempo , Resultado do Tratamento
18.
Front Biosci (Elite Ed) ; 11: 102-108, 2019 01 01.
Artigo em Inglês | MEDLINE | ID: mdl-30468641

RESUMO

Autoantibodies to beta-1 adrenergic receptor have been reported in adult patients with dilated cardiomyopathy (DCM). Removal of these antibodies has a positive hemodynamic effect. Our aim was to investigate whether these antibodies are present in children with DCM and explore the potential hemodynamic benefit of immunoadsorption (IA). Seventeen children with DCM  were tested for these antibodies. The etiology of DCM was genetic (n=5), myocarditis (n=4), DCM and congenital heart block (n=3), DCM associated to maternal lupus (n=1), DCM and Wolff Parkinson White Syndrome (n=1), and idiopathic (n=3). All patients evidenced ventricular dysfunction. Antibody testing was positive in 8 patients, 7  received IA.  Three patients with high titers had a poor clinical outcome and needed transplantation. Two patients with low titers exhibited a full recovery of heart function. One patient with multiple myocarditis episodes was treated with immunoglobulin IgG and IA ; after 5 years this patient presented a LVEF of 40 percent. Beta-1 adrenergic receptors autoantibodies are present in children with DCM. Immunoadsorption therapy may help improve heart failure in this context.


Assuntos
Autoanticorpos/imunologia , Cardiomiopatia Dilatada/imunologia , Hemodinâmica/genética , Receptores Adrenérgicos/imunologia , Cardiomiopatia Dilatada/terapia , Pré-Escolar , Humanos , Técnicas de Imunoadsorção , Lactente , Recém-Nascido , Resultado do Tratamento
19.
J Interv Card Electrophysiol ; 54(2): 161-170, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30471050

RESUMO

PURPOSE: To compare lead failure manifestation and lead performance of the Biotronik Linox/Sorin Vigila defibrillator lead (Linox group) with the St. Jude Medical Riata/Riata ST (Riata group) and Medtronic Sprint Fidelis defibrillator leads (Fidelis group). METHODS: We assessed the performance of all aforementioned leads implanted at our center and investigated the manifestation of lead failures. RESULTS: Of 93 Linox, 86 Riata, and 81 Fidelis leads implanted at our center, 11 (12%), 22 (26%), and 25 (31%) leads failed during a median follow-up of 46, 61, and 84 months, respectively. Inappropriate shocks were delivered in 64% (Linox), 5% (Riata), and 32% (Fidelis) of lead failures; a device alert was noted in none (Linox), 5% (Riata), and 52% (Fidelis); and lead failure was a coincidental finding in 36% (Linox), 91% (Riata), and 16% (Fidelis) of cases (p < 0.001). Non-physiological high rate signals were observed in 73% (Linox), 27% (Riata), and 80% (Fidelis) of lead failures (p = 0.001) and damaged lead integrity was found in 36% (Linox), 73% (Riata), and 24% (Fidelis) of cases (p = 0.064). Lead survival at 5 years was 88%, 92%, and 71% for Linox, Riata, and Fidelis group, respectively. CONCLUSIONS: The most frequent clinical manifestation of lead failure was inappropriate shocks for Linox, coincidental finding for Riata and device alert for Fidelis leads. Non-physiological high rate signals were frequently observed in Linox and Fidelis lead failures whereas in Riata lead failures, a damaged lead integrity was the predominant finding.


Assuntos
Desfibriladores Implantáveis/efeitos adversos , Eletrodos Implantados/efeitos adversos , Desenho de Equipamento/métodos , Análise de Falha de Equipamento , Segurança de Equipamentos/estatística & dados numéricos , Adulto , Fatores Etários , Idoso , Terapia de Ressincronização Cardíaca/efeitos adversos , Terapia de Ressincronização Cardíaca/métodos , Cardiomiopatia Dilatada/terapia , Estudos de Coortes , Feminino , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Isquemia Miocárdica/terapia , Estudos Retrospectivos , Medição de Risco , Fatores Sexuais , Estados Unidos
20.
ESC Heart Fail ; 6(1): 182-193, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30299600

RESUMO

AIMS: The clinical effectiveness of primary prevention implantable cardioverter defibrillator (ICD) therapy is under debate. The EUropean Comparative Effectiveness Research to Assess the Use of Primary ProphylacTic Implantable Cardioverter Defibrillators (EU-CERT-ICD) aims to assess its current clinical value. METHODS AND RESULTS: The EU-CERT-ICD is a prospective investigator-initiated non-randomized, controlled, multicentre observational cohort study performed in 44 centres across 15 European Union countries. We will recruit 2250 patients with ischaemic or dilated cardiomyopathy and a guideline indication for primary prophylactic ICD implantation. This sample will include 1500 patients at their first ICD implantation and 750 patients who did not receive a primary prevention ICD despite having an indication for it (non-randomized control group). The primary endpoint is all-cause mortality; the co-primary endpoint in ICD patients is time to first appropriate shock. Secondary endpoints include sudden cardiac death, first inappropriate shock, any ICD shock, arrhythmogenic syncope, revision procedures, quality of life, and cost-effectiveness. At baseline (and prior to ICD implantation if applicable), all patients undergo 12-lead electrocardiogram (ECG) and Holter ECG analysis using multiple advanced methods for risk stratification as well as detailed documentation of clinical characteristics and laboratory values. Genetic biobanking is also organized. As of August 2018, baseline data of 2265 patients are complete. All subjects will be followed for up to 4.5 years. CONCLUSIONS: The EU-CERT-ICD study will provide a necessary update about clinical effectiveness of primary prophylactic ICD implantation. This study also aims for improved risk stratification and patient selection using clinical and ECG risk markers.


Assuntos
Cardiomiopatia Dilatada/terapia , Morte Súbita Cardíaca/prevenção & controle , Desfibriladores Implantáveis , Prevenção Primária/métodos , Medição de Risco , Cardiomiopatia Dilatada/complicações , Cardiomiopatia Dilatada/mortalidade , Morte Súbita Cardíaca/epidemiologia , Morte Súbita Cardíaca/etiologia , Eletrocardiografia , Europa (Continente)/epidemiologia , Seguimentos , Humanos , Seleção de Pacientes , Estudos Prospectivos , Qualidade de Vida , Taxa de Sobrevida/tendências , Resultado do Tratamento
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