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1.
Eur J Radiol ; 117: 178-183, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31307645

RESUMO

PURPOSE: Dilated cardiomyopathy (DCM) is a common form of cardiomyopathy and it is associated with poor outcomes. A poor prognosis of DCM patients with low ejection fraction has been noted in the short-term follow-up. Machine learning (ML) could aid clinicians in risk stratification and patient management after considering the correlation between numerous features and the outcomes. The present study aimed to predict the 1-year cardiovascular events in patients with severe DCM using ML, and aid clinicians in risk stratification and patient management. MATERIALS AND METHODS: The dataset used to establish the ML model was obtained from 98 patients with severe DCM (LVEF < 35%) from two centres. Totally 32 features from clinical data were input to the ML algorithm, and the significant features highly relevant to the cardiovascular events were selected by Information gain (IG). A naive Bayes classifier was built, and its predictive performance was evaluated using the area under the curve (AUC) of the receiver operating characteristics by 10-fold cross-validation. RESULTS: During the 1-year follow-up, a total of 22 patients met the criterion of the study end-point. The top features with IG > 0.01 were selected for ML model, including left atrial size (IG = 0.240), QRS duration (IG = 0.200), and systolic blood pressure (IG = 0.151). ML performed well in predicting cardiovascular events in patients with severe DCM (AUC, 0.887 [95% confidence interval, 0.813-0.961]). CONCLUSIONS: ML effectively predicted risk in patients with severe DCM in 1-year follow-up, and this may direct risk stratification and patient management in the future.


Assuntos
Cardiomiopatia Dilatada/fisiopatologia , Aprendizado de Máquina , Adulto , Idoso , Algoritmos , Teorema de Bayes , Cardiomiopatia Dilatada/mortalidade , Feminino , Humanos , Aprendizado de Máquina/tendências , Masculino , Pessoa de Meia-Idade , Prognóstico , Curva ROC
2.
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
3.
Rev Assoc Med Bras (1992) ; 65(4): 524-529, 2019 May 02.
Artigo em Inglês | MEDLINE | ID: mdl-31066804

RESUMO

The Guidelines Project, an initiative of the Brazilian Medical Association, aims to combine information from the medical field in order to standardize producers to assist the reasoning and decision-making of doctors. The information provided through this project must be assessed and criticized by the physician responsible for the conduct that will be adopted, depending on the conditions and the clinical status of each patient.


Assuntos
Cardiomiopatia Dilatada/tratamento farmacológico , Cardiotônicos/uso terapêutico , Insuficiência Cardíaca/tratamento farmacológico , Simendana/uso terapêutico , Brasil , Cardiomiopatia Dilatada/mortalidade , Tomada de Decisão Clínica , Insuficiência Cardíaca/mortalidade , Humanos , Reprodutibilidade dos Testes , Fatores de Risco , Resultado do Tratamento
4.
Pediatr Cardiol ; 40(6): 1159-1164, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31087144

RESUMO

Pediatric ventricular assist device (VAD) implantation outcomes are increasingly promising for children with dilated cardiomyopathy and advanced decompensated heart failure (ADHF). VAD placement in patients with clinical features such as complex congenital cardiac anatomy, small body size, or major comorbidities remains problematic. These comorbidities have been traditionally prohibitive for VAD consideration leaving these children as a treatment-orphaned population. Here we describe the quality bundle surrounding these patients with ADHF considered high risk for VAD implantation at our institution. Over a 7-year period, a quality bundle aimed at the peri-operative care for children with high-risk features undergoing VAD implantation was incrementally implemented at a tertiary children's hospital. Patients were considered high risk if they were neonates (< 30 days), had single-ventricle physiology, non-dilated cardiomyopathy, biventricular dysfunction, or significant comorbidities. The quality improvement bundle evolved to include (1) structured team-based peri-operative evaluation, (2) weekly VAD rounds addressing post-operative device performance, (3) standardized anticoagulation strategies, and (4) a multidisciplinary system for management challenges. These measures aimed to improve communication, standardize management, allow for ongoing process improvement, and incorporate principles of a high-reliability organization. Between January 2010 and December 2017, 98 patients underwent VAD implantation, 48 (49%) of which had high-risk comorbidities and a resultant cohort survival-to-transplant rate of 65%. We report on the evolution of a quality improvement program to expand the scope of VAD implantation to patients with high-risk clinical profiles. This quality bundle can serve as a template for future large-scale collaborations to improve outcomes in these treatment-orphaned subgroups.


Assuntos
Cardiomiopatia Dilatada/cirurgia , Insuficiência Cardíaca/cirurgia , Coração Auxiliar/efeitos adversos , Adolescente , Anticoagulantes/uso terapêutico , Cardiomiopatia Dilatada/complicações , Cardiomiopatia Dilatada/mortalidade , Criança , Pré-Escolar , Estudos de Coortes , Comorbidade , Feminino , Insuficiência Cardíaca/complicações , Insuficiência Cardíaca/mortalidade , Humanos , Lactente , Masculino , Período Pós-Operatório , Melhoria de Qualidade , Reprodutibilidade dos Testes , Estudos Retrospectivos , Fatores de Risco , Resultado do Tratamento
5.
J Cardiovasc Med (Hagerstown) ; 20(7): 450-458, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-30985353

RESUMO

OBJECTIVE: The objective was to provide an exhaustive characterization of ECG features in a large cohort of dilated cardiomyopathies (DCMs) and then investigate their possible prognostic role in the long term. BACKGROUND: ECG is an accessible, reproducible, low-cost diagnostic and prognostic tool. However, an extensive description of ECG features and their long-term prognostic role in a large cohort of DCM is lacking. METHODS: All available baseline ECGs of DCM patients enrolled from 1992 to 2013 were systematically analysed. Patients underwent to a complete clinical-laboratory evaluation. The study outcome measures were death or heart transplant (D/HT) and sudden death or malignant ventricular arrhythmias (SD/MVA). RESULTS: Four hundred and fourteen DCM patients were enrolled. During a median follow-up of 125 months, 55 and 57 patients experienced D/HT and SD/MVA, respectively. At multivariate analysis, left ventricular hypertrophy (P = 0.017), heart rate (HR, P = 0.005) and anterolateral T-wave inversion (P = 0.041) predicted D/HT. Regarding SD/MVA, S wave amplitude in V2 (P = 0.008), R wave amplitude in DIII (P = 0.007), anterolateral T-wave inversion (P = 0.017) emerged as predictors. At receiver-operating curve analyses, the addition of ECG models to the clinical-laboratory evaluation significantly increased the area under the curve both for D/HT (from 0.68 to 0.74, P = 0.042) and SD/MVA (from 0.70 to 0.77, P = 0.048). CONCLUSION: The exhaustive systematic evaluation of ECG has an incremental impact in the prognostication of a large cohort of DCM patients, also regarding the arrhythmic stratification.


Assuntos
Cardiomiopatia Dilatada/diagnóstico , Eletrocardiografia , Frequência Cardíaca , Adulto , Arritmias Cardíacas/diagnóstico por imagem , Arritmias Cardíacas/mortalidade , Arritmias Cardíacas/fisiopatologia , Cardiomiopatia Dilatada/mortalidade , Cardiomiopatia Dilatada/fisiopatologia , Cardiomiopatia Dilatada/cirurgia , Morte Súbita Cardíaca/epidemiologia , Feminino , Transplante de Coração , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo
6.
Turk Kardiyol Dern Ars ; 47(3): 207-215, 2019 Apr.
Artigo em Turco | MEDLINE | ID: mdl-30982820

RESUMO

OBJECTIVE: Dilated cardiomyopathy (DCM) is a disorder featuring left ventricular dysfunction, heart failure, and a poor prognosis. The etiology is still unclear, despite diagnostic and therapeutic developments. This study was an evaluation of factors affecting the life span of a group of idiopathic DCM patients. METHODS: A total of 79 patients from between October 2005 and October 2017 with a diagnosis of idiopathic DCM were evaluated retrospectively. Demographic characteristics, clinical information, left ventricular function, treatment, and follow-up of the patients were reviewed based on hospital records. Age, gender, parental consanguinity, cardiomegaly on telecardiography, reduced ejection fraction (EF) and shortening fraction (SF), degree of mitral regurgitation, and intracardiac thrombosis were determined to affect prognosis. RESULTS: The patients were aged 20+-60 months, and the male/female ratio was 1.02/1. The patients most frequently presented with heart failure signs and symptoms (n=59, 74.7%). The most common physical examination findings were a murmur (n=53, 67.1%) and tachycardia (n=48, 60.8%). Cardiomegaly was observed on telecardiography in 73.4% of the patients. The EF and SF values were 35.7+-12.6% and 17.3+-6.5%, respectively. In all, 42 (53.2%) patients had mitral regurgitation of grade 2 or higher. The duration of follow-up was between 1 and 156 months (20+-34.9 months). Intracardiac thrombosis was detected in 4 (5.1%) patients. The mortality rate was 36.7%. When the prognostic factors were compared according to survival time, it was determined that survival was reduced in cases of parental consanguinity, low EF, and cardiomegaly. CONCLUSION: The most important negative markers affecting the length of survival of DCM patients were parental consanguinity, cardiomegaly detected on telecardiography, and a reduced EF level.


Assuntos
Cardiomiopatia Dilatada/epidemiologia , Disfunção Ventricular Esquerda/fisiopatologia , Adolescente , Cardiomegalia/diagnóstico por imagem , Cardiomiopatia Dilatada/mortalidade , Cardiomiopatia Dilatada/fisiopatologia , Criança , Serviços de Saúde da Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Prognóstico , Estudos Retrospectivos , Análise de Sobrevida , Turquia/epidemiologia
7.
J Cardiovasc Med (Hagerstown) ; 20(5): 343-350, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30921270

RESUMO

AIMS: Arrhythmic risk stratification is a challenging issue in patients with dilated cardiomyopathy (DCM), particularly when left ventricular ejection fraction (LVEF) is more than 35%. We studied the prevalence and predictors of sudden cardiac death or malignant ventricular arrhythmias (SCD/MVAs) in DCM patients categorized at low arrhythmic risk because of intermediate left ventricular dysfunction under optimal medical treatment (OMT). METHODS: DCM patients considered at low arrhythmic risk (LVEF >35% and New York Heart Association class I-III after 6 ±â€Š3 months of OMT) were analysed. An arrhythmogenic profile was defined as the presence of at least one among a history of syncope, nonsustained ventricular tachycardia, at least 1000 premature ventricular contractions/24 h, at least 50 ventricular couplets/24 h at Holter ECG monitoring. SCD/MVAs was considered as the study end-point. RESULTS: During a median follow-up of 152 months (interquartile range 100-234), 30 out of 360 (8.3%) patients at low arrhythmic risk (LVEF 47 ±â€Š7%) experienced the study end-point [14 (3.9%) SCD and 16 (4.4%) MVA]. Compared with survivors, patients who experienced SCD/MVAs had more frequently an arrhythmogenic profile and a larger left atrium. Their LVEF at the last available evaluation before the arrhythmic event was 36 ±â€Š12%. At multivariable analysis, left atrial end-systolic area [hazard ratio 1.107; 95% confidence interval (95% CI) 1.039-1.179, P = 0.002 for 1 mm increase] and arrhythmogenic profile (hazard ratio 3.667; 95% CI 1.762-7.632, P = 0.001) emerged as predictors of SCD/MVAs during follow-up. CONCLUSION: A consistent quota of DCM patients with intermediate left ventricular dysfunction receiving OMT experienced SCD/MVA during follow-up. Left atrial dilatation and arrhythmogenic pattern were associated with a higher risk of SCD/MVA.


Assuntos
Arritmias Cardíacas/epidemiologia , Cardiomiopatia Dilatada/epidemiologia , Morte Súbita Cardíaca/epidemiologia , Volume Sistólico , Disfunção Ventricular Esquerda/epidemiologia , Função Ventricular Esquerda , Adulto , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/mortalidade , Cardiomiopatia Dilatada/diagnóstico , Cardiomiopatia Dilatada/tratamento farmacológico , Cardiomiopatia Dilatada/mortalidade , Fármacos Cardiovasculares/uso terapêutico , Feminino , Humanos , Incidência , Itália/epidemiologia , Masculino , Pessoa de Meia-Idade , Prevalência , Prognóstico , Sistema de Registros , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Volume Sistólico/efeitos dos fármacos , Fatores de Tempo , Disfunção Ventricular Esquerda/diagnóstico , Disfunção Ventricular Esquerda/tratamento farmacológico , Disfunção Ventricular Esquerda/mortalidade , Função Ventricular Esquerda/efeitos dos fármacos
8.
Vet J ; 245: 15-21, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30819421

RESUMO

Dilated cardiomyopathy (DCM) is an important cause of morbidity in Irish Wolfhounds (IW), a breed also predisposed to neoplastic and orthopedic diseases that shorten life expectancy. The objective of this study was to investigate survival and causes of death in IW with DCM and to characterise the clinical findings of DCM over time. Data from cardiovascular examinations performed in 1591 IW, including echocardiography and electrocardiography, were retrospectively evaluated. IW with DCM on medical therapy with long term longitudinal follow-up were included in this study (n=151; 95 males, 56 females). Based on their clinical status at initial diagnosis, IW were classified into one of three groups: preclinical DCM with sinus rhythm (PC-DCM-SR, n=35), preclinical DCM with atrial fibrillation (PC-DCM-AF, n=87), and congestive heart failure with DCM and AF (CHF-DCM-AF, n=29). Survival data were analyzed using cumulative incidence functions, Kaplan-Meier and Cox regression. CHF was predominantly characterized by chylous pleural and mild pericardial effusions. Causes of death were cardiac (CD) in 73/151 and non-cardiac (non-CD) in 62/151; 16 dogs remained alive at study end. The majority of deaths in both preclinical DCM groups were non-CD (PC-DCM-AF=51.9% non-CD, 48.1% CD; PC-DCM-SR, 65.5% non-CD, 34.5% CD). In the CHF-DCM-AF group most dogs (89.6%) experienced a CD. Median survival of the CHF-DCM-AF group (7.3 months) was significantly shorter than in the PC-DCM-AF group (21.9 months) or PC-DCM-SR group (29.1 months, P=0.001). CHF-DCM-AF in IW was associated with reduced life expectancy and CD, while most IW with preclinical DCM died from non-cardiac causes.


Assuntos
Cardiomiopatia Dilatada/veterinária , Doenças do Cão/diagnóstico , Doenças do Cão/mortalidade , Animais , Cardiomiopatia Dilatada/diagnóstico , Cardiomiopatia Dilatada/mortalidade , Causas de Morte , Doenças do Cão/fisiopatologia , Cães , Ecocardiografia/veterinária , Eletrocardiografia/veterinária , Feminino , Expectativa de Vida , Masculino , Estudos Retrospectivos
9.
PLoS One ; 14(2): e0212017, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30811446

RESUMO

Adult heart size is determined predominantly by the cardiomyocyte number and size. The cardiomyocyte number is determined primarily in the embryonic and perinatal period, as adult cardiomyocyte proliferation is restricted in comparison to that seen during the perinatal period. Recent evidence has implicated the mammalian Hippo kinase pathway as being critical in cardiomyocyte proliferation. Though the transcription factor, Tead1, is the canonical downstream transcriptional factor of the hippo kinase pathway in cardiomyocytes, the specific role of Tead1 in cardiomyocyte proliferation in the perinatal period has not been determined. Here, we report the generation of a cardiomyocyte specific perinatal deletion of Tead1, using Myh6-Cre deletor mice (Tead1-cKO). Perinatal Tead1 deletion was lethal by postnatal day 9 in Tead1-cKO mice due to dilated cardiomyopathy. Tead1-deficient cardiomyocytes have significantly decreased proliferation during the immediate postnatal period, when proliferation rate is normally high. Deletion of Tead1 in HL-1 cardiac cell line confirmed that cell-autonomous Tead1 function is required for normal cardiomyocyte proliferation. This was secondary to significant decrease in levels of many proteins, in vivo, that normally promote cell cycle in cardiomyocytes. Taken together this demonstrates the non-redundant critical requirement for Tead1 in regulating cell cycle proteins and proliferation in cardiomyocytes in the perinatal heart.


Assuntos
Cardiomiopatia Dilatada/mortalidade , Proteínas de Ligação a DNA/genética , Proteínas de Ligação a DNA/metabolismo , Miócitos Cardíacos/citologia , Fatores de Transcrição/genética , Fatores de Transcrição/metabolismo , Animais , Animais Recém-Nascidos , Cardiomiopatia Dilatada/genética , Proliferação de Células , Células Cultivadas , Feminino , Deleção de Genes , Genes Letais , Camundongos , Miócitos Cardíacos/metabolismo , Tamanho do Órgão , Gravidez , Transdução de Sinais
10.
Pediatr Cardiol ; 40(2): 330-338, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30415380

RESUMO

In adult heart failure (HF) patients, a higher ventricular arterial (VA) coupling ratio measured non-invasively is associated with worse HF prognosis and response to treatment. There are no data regarding the relationship of VA coupling to outcome in pediatric dilated cardiomyopathy (DCM) patients. We investigated the association of VA coupling ratio with worse outcome (mechanical circulatory support, transplant, or death) in 48 children with DCM and 97 age-gender matched controls. Mean age at presentation was 9 ± 7 years; DCM patients had a higher arterial elastance (3.8 ± 1.7 vs 2.7 ± 0.7 respectively p = 0.001), a lower LV elastance (1.1 ± 0.65 vs 4.5 ± 1.4, respectively p = 0.001) and higher VA coupling ratio (5.0 ± 3.9 vs 0.34 ± 0.14, respectively p = 0.001). Outcome events occurred in 27/48 (56%) patients. Patients with an outcome event had a higher NYHA class (p = 0.001), lower LV elastance (0.8 ± 0.47 vs 1.6 ± 0.57, respectively p = 0.001), higher arterial elastance (4.5 ± 1.8 vs 2.9 ± 1.1, respectively p = 0.002), and a higher VA coupling ratio (7.1 ± 3.8 vs 2.2 ± 1.5, respectively p = 0.001) compared to those without. In a multivariate CART analysis, VA coupling was the top and only discriminator of poor outcome. In conclusion, a higher VA coupling ratio is associated with worse outcome in pediatric patients with DCM. VA coupling is promising as a bedside analysis tool that may provide insight into the mechanisms of HF in pediatric DCM and identify potential targets for therapy.


Assuntos
Cardiomiopatia Dilatada/diagnóstico por imagem , Ecocardiografia/métodos , Ventrículos do Coração/diagnóstico por imagem , Adolescente , Cardiomiopatia Dilatada/mortalidade , Cardiomiopatia Dilatada/fisiopatologia , Criança , Pré-Escolar , Estudos de Coortes , Progressão da Doença , Feminino , Insuficiência Cardíaca/epidemiologia , Insuficiência Cardíaca/etiologia , Transplante de Coração/estatística & dados numéricos , Ventrículos do Coração/fisiopatologia , Humanos , Lactente , Recém-Nascido , Masculino , Variações Dependentes do Observador , Curva ROC , Estudos Retrospectivos , Fatores de Risco , Análise de Sobrevida , Adulto Jovem
11.
Int J Cardiol ; 278: 180-185, 2019 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-30416030

RESUMO

BACKGROUND: Left bundle branch block (LBBB) negatively affects prognosis in heart failure patients with a reduced left ventricular ejection fraction (LVEF). Less is known about the prognostic role of LBBB in dilated cardiomyopathy (DCM) with intermediate LVEF (between 36% and 50%). We sought to assess the role of LBBB in optimally treated DCM patients with mildly to moderately reduced LVEF and to determine the possible variables associated with subsequent LVEF reduction. METHODS: We retrospectively analyzed DCM patients with LVEF >35% after 3-to-9 months of optimal medical treatment (OMT) consecutively evaluated from 1990 to 2010. All-cause mortality or heart transplantation (D/HTx) and sudden cardiac death (SCD) or major ventricular arrhythmias (MVA) were considered as outcome measures. LVEF deterioration during follow-up was also considered. RESULTS: Among 280 (49%) patients that met the study criteria, 76 had LBBB (27%). During a mean follow-up of 151 months, the rates of D/HTx and SCD/MVA were similar between LBBB and not LBBB patients (p value = 0.52 and p = 0.39, respectively). Twenty-six out of 76 (34%) patients with LBBB experienced LVEF deterioration below 36%. The persistence of moderate-severe mitral regurgitation (MR), left atrial end-systolic area index and LV end-diastolic volume index emerged as independent predictors of LVEF deterioration and were associated with an increased risk of D/HTx during follow-up. CONCLUSIONS: LBBB does not affect mortality in DCM patients with intermediate LVEF after OMT. However, among these patients those with persistent significant MR, left atrial and LV remodeling carries a higher risk of LVEF deterioration during follow-up.


Assuntos
Bloqueio de Ramo/diagnóstico por imagem , Cardiomiopatia Dilatada/diagnóstico por imagem , Fenótipo , Disfunção Ventricular Esquerda/diagnóstico por imagem , Adulto , Bloqueio de Ramo/mortalidade , Bloqueio de Ramo/fisiopatologia , Cardiomiopatia Dilatada/mortalidade , Cardiomiopatia Dilatada/fisiopatologia , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Sistema de Registros , Estudos Retrospectivos , Resultado do Tratamento , Disfunção Ventricular Esquerda/mortalidade , Disfunção Ventricular Esquerda/fisiopatologia
12.
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
13.
Int J Cardiovasc Imaging ; 35(1): 171-178, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30132161

RESUMO

To evaluate and compare the prognostic value of T1 mapping with feature tracking cardiovascular magnetic resonance (FT-CMR) imaging in patients with severe dilated cardiomyopathy (DCM) during short-term follow-up. A total of 46 patients with severe DCM (LVEF < 35%) underwent 3.0-T CMR with T1 mapping and FT-CMR analysis. The study end-point was defined as a combination of cardiac death, heart transplantation, and hospitalization due to cardiovascular events. The significance of the risk factors was mainly evaluated by univariate and multivariate Cox model analyses. During the median follow-up of 13 months (interquartile range 7-17 months), two patients died of heart failure, one received a heart transplantation, and six were hospitalized for heart failure. In the univariate analysis, extracellular volume fraction (ECV) showed significant predictive association with cardiovascular events (hazard ratio [HR] 1.35; 95% confidence interval [CI] 1.13-1.62; P = 0.001). No strain parameters in FT-CMR differed significantly between patients with or without events (all P > 0.05). In the multivariate analyses, ECV was the sole independent predictor of cardiovascular events (HR, 1.48; 95% CI 1.13-1.94; P = 0.005). The area under the curve of the time-dependent receiver operating characteristic in leave-one-out cross-validation (all > 0.70) further confirmed the predictive significance of ECV. In patients with severe DCM, ECV was not only a strong independent predictor of adverse cardiovascular events but also provided prognostic value prior to strain parameters of the FT-CMR in the short term.


Assuntos
Cardiomiopatia Dilatada/diagnóstico por imagem , Interpretação de Imagem Assistida por Computador/métodos , Imagem Cinética por Ressonância Magnética/métodos , Adulto , Cardiomiopatia Dilatada/mortalidade , Cardiomiopatia Dilatada/fisiopatologia , Cardiomiopatia Dilatada/cirurgia , Causas de Morte , Progressão da Doença , Feminino , Transplante de Coração , Humanos , Masculino , Pessoa de Meia-Idade , Admissão do Paciente , Valor Preditivo dos Testes , Prognóstico , Fatores de Risco , Índice de Gravidade de Doença , Fatores de Tempo
14.
PLoS One ; 13(11): e0208100, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30485353

RESUMO

INTRODUCTION: The presence of late gadolinium enhancement (LGE) at the right ventricular insertion point (RVIP) on cardiac magnetic resonance (CMR) is generally believed to be nonspecific, but the clinical implication of this unique LGE pattern in patients with non-ischemic dilated cardiomyopathy (NICM) has not been elucidated. OBJECTIVES: We investigated the prognostic significance of RVIP-LGE in NICM patients. METHODS: A total of 360 consecutive NICM patients referred for CMR (102 with no LGE, 50 with RVIP-LGE, 121 with left ventricular [LV]-LGE, and 87 with both an LV and RVIP-LGE) were studied. The primary endpoint was a composite of the all-cause death, hospitalization due to worsening of heart failure, and major arrhythmic events. RESULTS: During a mean follow-up of 45.2 ± 36.5 months, 149 (41.4%) patients (22 [21.6%] no LGE vs. 16 [32.0%] RVIP-LGE vs. 62 [51.2%] LV-LGE vs. 49 [56.3%] both LV and RVIP-LGE, P < 0.0001) reached the primary endpoint. A Kaplan Meier curve demonstrated that RVIP-LGE patients had an intermediate trend of an event free survival rate for the composite endpoint (log-rank P < 0.0001). In a multivariable Cox regression model, LV-LGE (P = 0.008) and both LV and RVIP-LGE (P = 0.003) were significantly associated with a worse outcome, whereas RVIP-LGE was not (P = 0.101). In addition, RVIP-LGE patients (n = 32) had a more favorable outcome compared to LV-LGE patients (n = 32) even after matching the extent of the LGE (median 3.4% [interquartile range, 3.1-3.8], 8 [25.0%] RVIP-LGE vs. 20 [62.5%] LV-LGE, P = 0.002). CONCLUSIONS: LGE confined to the RVIP among NICM patients did not significantly increase the risk of adverse cardiac events, and also showed a better outcome than the same extent of LGE located in the LV. Identification of this unique LGE distribution may help refine the current risk stratification.


Assuntos
Cardiomiopatia Dilatada/diagnóstico por imagem , Meios de Contraste , Gadolínio , Ventrículos do Coração/diagnóstico por imagem , Cardiomiopatia Dilatada/mortalidade , Ecocardiografia , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Análise de Sobrevida
15.
Int J Cardiol ; 270: 278-286, 2018 Nov 01.
Artigo em Inglês | MEDLINE | ID: mdl-30082120

RESUMO

BACKGROUND: Myeloid differentiation factor-2 (MD-2) has been shown to be an important modulator of the innate immune system, but its role in cardiac diseases is unknown. We investigated whether MD-2 plays a role as risk predictor and contributor in dilated cardiomyopathy (DCM). METHODS AND RESULTS: We included 174 patients with reduced left ventricular (LV) ejection fraction (LVEF <45%) due to DCM. Coronary artery disease and severe valvular diseases were excluded in all patients by angiography or echocardiography. Cardiac inflammation, viral infection and MD-2 expression were analyzed from right ventricular endomyocardial biopsies. MD-2 was quantified by ELISA in serum upon first hospital admission. Myocyte contractility and inflammatory response after stimulation with recombinant MD-2 protein were analyzed in isolated rat cardiomyocytes. Median follow-up of the patients was 3.51 years (2.73; 4.48) with 34 deaths. Absolute mortality risk increases in patients displaying a MD-2 serum concentration greater than the median (302 ng/ml) was 23% (P < 0.0001). Age- and sex-adjusted Cox regression analyses demonstrated that mortality risk was highly related to MD-2 concentrations (P < 0.001), but not to age or sex. An increase of 100 ng/ml in the MD-2 level was associated with an absolute mortality risk increase of 50.4%. Receiver operating characteristic (ROC) analyses showed no difference between MD-2 and nterminal-pro brain natriuretic peptide (NT-pro-BNP), while the combination of both MD-2 and NT-pro-BNP resulted in a significantly increased capability of risk prediction when compared to NT-pro-BNP alone (P = 0.014). In-vitro, recombinant MD-2 decreases cell shortening and modulates cytokine activation in isolated cardiomyocytes. CONCLUSION: MD-2 predicts long-term outcome in DCM patients and improves mortality risk prediction capability compared to NT-pro-BNP alone. In addition, MD-2 exerts direct negative inotropic effects on isolated cardiomyocytes in-vitro. Further randomized trials should confirm MD-2 as a diagnostic and therapeutic target.


Assuntos
Cardiomiopatia Dilatada/diagnóstico , Cardiomiopatia Dilatada/metabolismo , Antígeno 96 de Linfócito/metabolismo , Miócitos Cardíacos/metabolismo , Animais , Biomarcadores/metabolismo , Cardiomiopatia Dilatada/mortalidade , Estudos de Casos e Controles , Células Cultivadas , Estudos de Coortes , Relação Dose-Resposta a Droga , Feminino , Humanos , Antígeno 96 de Linfócito/farmacologia , Masculino , Pessoa de Meia-Idade , Miócitos Cardíacos/efeitos dos fármacos , Miócitos Cardíacos/patologia , Valor Preditivo dos Testes , Ratos , Sistema de Registros , Estudos Retrospectivos
16.
Kyobu Geka ; 71(7): 484-487, 2018 Jul.
Artigo em Japonês | MEDLINE | ID: mdl-30042251

RESUMO

Surgical strategy for non-ischemic dilated cardiomyopathy (NIDCM) is currently controversial. Subjects were 20 patients who underwent left ventriculoplasty(LVP) from 2006 to 2013 and 6 patients who underwent papillary muscle tugging approximation (PMTA) after 2015. PMTA is a new trans-mitral approach combined with valve replacement without left ventriculotomy. Another group of patients( n=14)who were registered for heart transplantation( HTx) after 2013 was also analyzed for left ventricular assist device(LVAD) free survival. Mw( slope in the preload recruitable stroke work relationship) calculated by single beat technique using echocardiography was employed as a load-independent cardiac functional parameter. The baseline characteristics and Mw were not different between the LVP and PMTA groups. One-year survival was significantly lower in the LVP group(53%)than in the PMTA group(100%)[log-rank:p=0.024]. In the HTx group, early LVAD implantation was necessary in the patients who had low Mw(<20)at the time of registration. In conclusion, PMTA would be one option for NIDCM patients( non-HTx candidates) with severe mitral regurgitation. Early LVAD implantation might be predicted in HTx candidates with low Mw(<20).


Assuntos
Cardiomiopatia Dilatada/cirurgia , Insuficiência Cardíaca/cirurgia , Transplante de Coração , Coração Auxiliar , Insuficiência da Valva Mitral/cirurgia , Cardiomiopatia Dilatada/complicações , Cardiomiopatia Dilatada/mortalidade , Insuficiência Cardíaca/complicações , Insuficiência Cardíaca/mortalidade , Ventrículos do Coração/cirurgia , Humanos , Insuficiência da Valva Mitral/complicações , Músculos Papilares/cirurgia , Resultado do Tratamento
17.
Kyobu Geka ; 71(7): 488-493, 2018 Jul.
Artigo em Japonês | MEDLINE | ID: mdl-30042252

RESUMO

Surgery for functional mitral regurgitation (FMR) and dilated cardiomyopathy (DCM) remains a challenge. We reviewed our efforts;anterior (i.e. physiological) relocation of papillary heads and muscles(PHO) in 54 patients with FMR and simplified ventricular restoration of uni-directional Dor or Frozen-Apex restoration in 22 patients with DCM. Actuarial survival 4 years after the PHO was 92±6% with improved left ventricular (LV) function and well controlled FMR. Results of the simplified restoration was also promising with no hospital death and just 1 cardiac death late postoperatively, with good symptom relieve and maintained LV function. Surgical outcome for FMR or DCM may be further improved.


Assuntos
Cardiomiopatia Dilatada/cirurgia , Insuficiência da Valva Mitral/cirurgia , Cardiomiopatia Dilatada/mortalidade , Ventrículos do Coração , Humanos , Valva Mitral , Insuficiência da Valva Mitral/mortalidade , Músculos Papilares/cirurgia , Resultado do Tratamento , Função Ventricular Esquerda
18.
Europace ; 20(12): 2003-2013, 2018 12 01.
Artigo em Inglês | MEDLINE | ID: mdl-29947763

RESUMO

Aims: Characterization of the cardiac phenotype associated with the novel LMNA nonsense mutation c.544C>T, p.Q182*, which we have identified in a large five-generation family. Methods and results: A family tree was constructed. Clinical data [arrhythmia, syncope, sudden cardiac death (SCD), New York Heart Association (NYHA) class] were collected from living and deceased family members. DNA of 23 living family members was analysed for mutations in LMNA. Additionally, dilated cardiomyopathy multi-gene-panel testing and whole exome sequencing were performed in some family members to identify potential phenotype-modifiers. In this five-generation family (n = 65), 17 SCDs occurred at 49.3 ± 10.0 years. Furthermore, we identified eight additional mutation-carriers, seven symptomatic (44 ± 13 years), and one asymptomatic (44 years). First signs of disease [sinus bradycardia with atrioventricular (AV)-block I°] occurred at 36.5 ± 8.1 years. Paroxysmal atrial fibrillation (AF) (onset at 41.8 ± 5.7 years) rapidly progressed to permanent AF (46.2 ± 9.8 years). Subsequently, AV-conduction worsened, syncope, pacemaker-dependence, and non-sustained ventricular tachycardia (43.3 ± 8.2 years) followed. Ventricular arrhythmia caused SCD in patients without implantable cardioverter-defibrillator (ICD). Patients protected by ICD developed rapidly progressive heart failure (45.2 ± 10.6 years). A different phenotype was seen in a sub-family in three patients with early onset of rapidly decompensating heart failure and only minor prior arrhythmia-related symptoms. One patient received high-urgency heart transplantation (HTX) at 32 years, while two died prior to HTX. One of them developed lethal peripartum-associated heart failure. Possible disease-modifiers were identified in this 'heart failure sub-family'. Conclusion: The novel LMNA nonsense mutation c.544C>T causes a severe arrhythmogenic phenotype manifesting with high incidence of SCD in most patients; and in one sub-family, a distinct phenotype with fast progressing heart failure, indicating the need for early consideration of ICD-implantation and listing for heart-transplantation.


Assuntos
Arritmias Cardíacas/genética , Cardiomiopatia Dilatada/genética , Códon sem Sentido , Morte Súbita Cardíaca/etiologia , Lamina Tipo A/genética , Adulto , Arritmias Cardíacas/mortalidade , Arritmias Cardíacas/fisiopatologia , Arritmias Cardíacas/terapia , Cardiomiopatia Dilatada/mortalidade , Cardiomiopatia Dilatada/fisiopatologia , Cardiomiopatia Dilatada/terapia , Morte Súbita Cardíaca/prevenção & controle , Desfibriladores Implantáveis , Progressão da Doença , Cardioversão Elétrica/instrumentação , Feminino , Predisposição Genética para Doença , Transplante de Coração , Hereditariedade , Humanos , Masculino , Pessoa de Meia-Idade , Linhagem , Fenótipo , Prognóstico , Fatores de Risco , Índice de Gravidade de Doença
19.
Circulation ; 137(23): 2514-2527, 2018 06 05.
Artigo em Inglês | MEDLINE | ID: mdl-29866775

RESUMO

The etiology of dilated cardiomyopathy (DCM) can be grouped as either genetic or nongenetic. More than 50 pathogenic genes have been described, with sarcomeric and lamin A/C mutations being the most common. Mutation carriers for genetic DCM are often asymptomatic until cardiac disease manifests with heart failure, arrhythmias, or sudden cardiac death. Preventive strategies are promising but can only be applied and tested adequately if genetic DCM can be diagnosed at an early stage. Early diagnosis of mutation carriers that may develop overt DCM requires advanced imaging techniques that can detect subtle structural and functional abnormalities. Advanced echocardiographic techniques such as tissue Doppler imaging and speckle tracking strain analysis permit early detection of functional abnormalities, whereas cardiovascular magnetic resonance techniques provide information on tissue characterization and myocardial energetics that may be altered at an early stage. Furthermore, nuclear imaging techniques provide information on cellular function (metabolism, perfusion). Once the diagnosis of overt DCM has been established, various imaging parameters such as echocardiography-based myocardial mechanics and cardiovascular magnetic resonance-based tissue characterization have shown incremental benefit to left ventricular ejection fraction in risk stratification. Further research is required to understand how imaging techniques may help to choose management strategies that could delay progression when instituted early in the course of the disease. The present article reviews the role of imaging in the risk stratification of genetic DCM in general, with specific emphasis on DCM associated with neuromuscular disorders.


Assuntos
Cardiomiopatia Dilatada , Morte Súbita Cardíaca , Ecocardiografia Doppler , Doenças Genéticas Inatas , Lamina Tipo A/genética , Mutação , Doenças Neuromusculares , Cardiomiopatia Dilatada/diagnóstico por imagem , Cardiomiopatia Dilatada/genética , Cardiomiopatia Dilatada/mortalidade , Cardiomiopatia Dilatada/fisiopatologia , Feminino , Doenças Genéticas Inatas/diagnóstico por imagem , Doenças Genéticas Inatas/genética , Doenças Genéticas Inatas/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Doenças Neuromusculares/diagnóstico por imagem , Doenças Neuromusculares/genética , Doenças Neuromusculares/fisiopatologia , Medição de Risco , Volume Sistólico , Função Ventricular Esquerda
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