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2.
J Interv Cardiol ; 2020: 4386841, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32549801

RESUMO

Background: Voltage mapping allows identifying the arrhythmogenic substrate during scar-related ventricular arrhythmia (VA) ablation procedures. Slow conducting channels (SCCs), defined by the presence of electrogram (EGM) signals with delayed components (EGM-DC), are responsible for sustaining VAs and constitute potential ablation targets. However, voltage mapping, as it is currently performed, is time-consuming, requiring a manual analysis of all EGMs to detect SCCs, and its accuracy is limited by electric far-field. We sought to evaluate an algorithm that automatically identifies EGM-DC, classifies mapping points, and creates new voltage maps, named "Slow Conducting Channel Maps" (SCC-Maps). Methods: Retrospective analysis of electroanatomic maps (EAM) from 20 patients (10 ischemic, 10 with arrhythmogenic right ventricular dysplasia/cardiomyopathy) was performed. EAM voltage maps were acquired during sinus rhythm and used for ablation. Preprocedural contrast-enhanced cardiac magnetic resonance (Ce-CMR) imaging was available for the ischemic population. Three mapping modalities were analysed: (i) EAM voltage maps using standard (EAM standard) or manual (EAM screening) thresholds for defining core and border zones; (ii) SCC-Maps derived from the use of the novel SCC-Mapping algorithm that automatically identify EGM-DCs measuring the voltage of the local component; and (iii) Ce-CMR maps (when available). The ability of each mapping modality in identifying SCCs and their agreement was evaluated. Results: SCC-Maps and EAM screening identified a greater number of SCC entrances than EAM standard (3.45 ± 1.61 and 2.95 ± 2.31, resp., vs. 1.05 ± 1.10; p < 0.01). SCC-Maps and EAM screening highly correlate with Ce-CMR maps in the ischemic population when compared to EAM standard (Lin's correlation = 0.628 and 0.679, resp., vs. 0.212, p < 0.01). Conclusion: The SCC-Mapping algorithm allows an operator-independent analysis of EGM signals showing better identification of the arrhythmogenic substrate characteristics when compared to standard voltage EAM.


Assuntos
Displasia Arritmogênica Ventricular Direita/diagnóstico , Displasia Arritmogênica Ventricular Direita/fisiopatologia , Ablação por Cateter , Taquicardia Ventricular/etiologia , Adulto , Idoso , Arritmias Cardíacas/cirurgia , Displasia Arritmogênica Ventricular Direita/cirurgia , Cicatriz/patologia , Cicatriz/cirurgia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Taquicardia Ventricular/diagnóstico
5.
Herz ; 45(3): 243-251, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32170341

RESUMO

Arrhythmogenic right ventricular cardiomyopathy (ARVC), an inherited heart muscle disease, is characterized by a progressive replacement of viable, in its classic form predominantly right ventricular myocardium by fibro-fatty tissue. These pathological alterations may provide the substrate for the occurrence of life-threatening ventricular tachyarrhythmias, heart failure, and sudden cardiac death. The clinical course in this young patient population is highly variable, diagnostic algorithms complex, and individualized treatment strategies yet to be refined. Molecular genetic analyses have revealed both heterozygous and compound mutations in genes encoding for desmosomal proteins that are an integral part of the intercellular architecture. However, its diagnostic and prognostic impact remains to be elucidated. Over time, other genetic (i.e., non-desmosomal) and non-genetic causes (phenocopies) have been identified, and biventricular and left dominant manifestations (ALVC) are known. Based on a qualitative scoring system, initially published in 1994, diagnostic criteria were revised and substantiated by quantitative criteria in 2010 followed by a critical appraisal 9 years later. In 1995, ARVC was included in the classification of cardiomyopathies of the World Health Organization but was recently proposed to be subsumed in a broader concept termed "arrhythmogenic cardiomyopathy" (AC). This review provides an update on the clinical diagnosis and differential diagnoses of ARVC as well as our current understanding of the underlying pathogenesis, and it sheds light on new efforts in risk stratification.


Assuntos
Displasia Arritmogênica Ventricular Direita , Cardiomiopatias , Arritmias Cardíacas , Displasia Arritmogênica Ventricular Direita/diagnóstico , Displasia Arritmogênica Ventricular Direita/terapia , Ventrículos do Coração , Humanos , Miocárdio
6.
G Ital Cardiol (Rome) ; 21(3): 195-208, 2020 Mar.
Artigo em Italiano | MEDLINE | ID: mdl-32100732

RESUMO

Cardiomyopathies are a heterogeneous group of cardiac diseases for which diagnosis and treatment are not always simple. The diagnosis of cardiomyopathy, in particular the etiology, comes from an integration between symptoms and results collected by several instrumental exams. The brain storming for the diagnosis includes also the identification of the "red flags", i.e. the pathognomonic features for each etiology that can drive the choice of appropriate diagnostic tests and therapy. In this review, we provide a step by step approach in order to help cardiologists, not specifically dedicated to cardiomyopathies, to draw the diagnosis, therapy and follow-up. This approach will be accompanied by the consultation of other specialists to discuss together the results of the exams performed and to deepen extracardiac signs and symptoms.


Assuntos
Cardiomiopatias/diagnóstico , Cardiomiopatias/genética , Fenótipo , Avaliação de Sintomas , Displasia Arritmogênica Ventricular Direita/diagnóstico , Displasia Arritmogênica Ventricular Direita/genética , Displasia Arritmogênica Ventricular Direita/terapia , Cardiomiopatias/terapia , Cardiomiopatia Dilatada/diagnóstico , Cardiomiopatia Dilatada/genética , Cardiomiopatia Dilatada/terapia , Cardiomiopatia Hipertrófica/diagnóstico , Cardiomiopatia Hipertrófica/genética , Cardiomiopatia Hipertrófica/terapia , Cardiomiopatia Restritiva/diagnóstico , Cardiomiopatia Restritiva/etiologia , Cardiomiopatia Restritiva/terapia , Diagnóstico Diferencial , Ecocardiografia , Eletrocardiografia , Humanos , Imagem Cinética por Ressonância Magnética , Tomografia por Emissão de Pósitrons , Encaminhamento e Consulta , Sarcoidose/diagnóstico
7.
Int J Mol Sci ; 21(4)2020 Feb 24.
Artigo em Inglês | MEDLINE | ID: mdl-32102357

RESUMO

Arrhythmogenic Cardiomyopathy (AC) is a clinically and genetically heterogeneous myocardial disease. Half of AC patients harbour private desmosomal gene variants. Although microRNAs (miRNAs) have emerged as key regulator molecules in cardiovascular diseases and their involvement, correlated to phenotypic variability or to non-invasive biomarkers, has been advanced also in AC, no data are available in larger disease cohorts. Here, we propose the largest AC cohort unbiased by technical and biological factors. MiRNA profiling on nine right ventricular tissue, nine blood samples of AC patients, and four controls highlighted 10 differentially expressed miRNAs in common. Six of these were validated in a 90-AC patient cohort independent from genetic status: miR-122-5p, miR-133a-3p, miR-133b, miR-142-3p, miR-182-5p, and miR-183-5p. This six-miRNA set showed high discriminatory diagnostic power in AC patients when compared to controls (AUC-0.995), non-affected family members of AC probands carrying a desmosomal pathogenic variant (AUC-0.825), and other cardiomyopathy groups (Hypertrophic Cardiomyopathy: AUC-0.804, Dilated Cardiomyopathy: AUC-0.917, Brugada Syndrome: AUC-0.981, myocarditis: AUC-0.978). AC-related signalling pathways were targeted by this set of miRNAs. A unique set of six-miRNAs was found both in heart-tissue and blood samples of AC probands, supporting its involvement in disease pathogenesis and its possible role as a non-invasive AC diagnostic biomarker.


Assuntos
Displasia Arritmogênica Ventricular Direita/genética , Biomarcadores/metabolismo , Perfilação da Expressão Gênica/métodos , MicroRNAs/genética , Miocárdio/metabolismo , Adolescente , Adulto , Idoso , Displasia Arritmogênica Ventricular Direita/sangue , Displasia Arritmogênica Ventricular Direita/diagnóstico , Biomarcadores/sangue , Criança , Estudos de Coortes , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Miocárdio/patologia , Curva ROC , Transdução de Sinais/genética , Adulto Jovem
8.
J Am Heart Assoc ; 9(3): e013695, 2020 02 04.
Artigo em Inglês | MEDLINE | ID: mdl-32009524

RESUMO

Background Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is characterized by high arrhythmic burden and progressive heart failure, which can prompt referral for heart transplantation. Cardiopulmonary exercise testing (CPET) has an established role in risk stratification for advanced heart failure therapies, but has not been described in ARVC/D. This study sought to determine the safety and prognostic utility of CPET in patients with ARVC/D. Methods and Results Using the Johns Hopkins ARVC/D Registry, we examined patients with ARVC/D undergoing CPET. Baseline characteristics and transplant-free survival were compared on the basis of peak oxygen consumption (pVO2) (≤14 or >14 mL/kg per minute) and ventilatory efficiency (Ve/VCO2 slope ≤34 or >34). Thirty-eight patients underwent 50 CPETs. There were no sustained arrhythmic events. Twenty-nine patients achieved a maximal test. Patients with pVO2 ≤14 mL/kg per minute were more often men (P=0.042) compared with patients with pVO2 >14 mL/kg per minute. Patients with Ve/VCO2 slope >34 tended to have more moderate/severe right ventricular dilation (7/9 [78%] versus 10/26 [38%]; P=0.060) and clinical heart failure (8/9 [89%] versus 13/26 [50%]; P=0.056) compared with patients with Ve/VCO2 slope ≤34. Patients who underwent heart transplantation were more likely to have clinical heart failure (10/10 [100%] versus 13/28 [46%]; P=0.003). Patients with Ve/VCO2 slope >34 had worse transplant-free survival compared with patients with Ve/VCO2 slope ≤34 (n=35; hazard ratio, 6.57 [95% CI, 1.28-33.72]; log-rank P=0.010), whereas transplant-free survival was similar on the basis of pVO2 groups (n=29; hazard ratio, 3.38 [95% CI, 0.75-15.19]; log-rank P=0.092). Conclusions CPET is safe to perform in patients with ARVC/D. Ve/VCO2 slope may be used for risk stratification and guide referral for heart transplantation in ARVC/D.


Assuntos
Displasia Arritmogênica Ventricular Direita/diagnóstico , Teste de Esforço , Tolerância ao Exercício , Insuficiência Cardíaca/diagnóstico , Consumo de Oxigênio , Adolescente , Adulto , Displasia Arritmogênica Ventricular Direita/fisiopatologia , Displasia Arritmogênica Ventricular Direita/cirurgia , Teste de Esforço/efeitos adversos , Feminino , Insuficiência Cardíaca/fisiopatologia , Insuficiência Cardíaca/cirurgia , Transplante de Coração , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Intervalo Livre de Progressão , Sistema de Registros , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Adulto Jovem
10.
Heart Lung Circ ; 29(4): 547-555, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31964580

RESUMO

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a group of cardiomyopathies associated with ventricular arrhythmias predominantly arising from the right ventricle, sudden cardiac death and right ventricular failure, caused largely due to inherited mutations in proteins of the desmosomal complex. Whilst long recognised as a cause of sudden cardiac death (SCD) during exercise, it has recently been recognised that intense and prolonged exercise can worsen the disease resulting in earlier and more severe phenotypic expression. Changes in cardiac structure and function as a result of exercise training also pose challenges with diagnosis as enlargement of the right ventricle is commonly seen in endurance athletes. Advice regarding restriction of exercise is an important part of patient management, not only of those with established disease, but also in individuals known to carry gene mutations associated with development of ARVC.


Assuntos
Displasia Arritmogênica Ventricular Direita , Atletas , Morte Súbita Cardíaca , Exercício Físico , Mutação , Displasia Arritmogênica Ventricular Direita/diagnóstico , Displasia Arritmogênica Ventricular Direita/genética , Displasia Arritmogênica Ventricular Direita/mortalidade , Displasia Arritmogênica Ventricular Direita/fisiopatologia , Humanos
11.
Zhonghua Er Ke Za Zhi ; 57(9): 700-704, 2019 Sep 02.
Artigo em Chinês | MEDLINE | ID: mdl-31530356

RESUMO

Objective: To analyze and summarize the diagnosis and treatment experience of common inherited cardiac arrhythmia syndrome in pediatric patients, and explore the most appropriate therapy. Methods: A retrospective review identified 30 pediatric cases (19 males, 11 females) diagnosed with long QT syndrome (LQTS), Brugada syndrome (BrS), catecholaminergic polymorphic ventricular tachycardia (CPVT), hypertrophic cardiomyopathy (HCM), arrhythmogenc right ventricular cardiomyopathy (ARVC) from January 2008 to December 2018 in the Pediatric Cardiology Department, Guangdong Provincial People's Hospital. Data obtained included the diagnosis, treatment and follow-up outcome. Results: The most common inherited cardiac arrhythmia syndromes were LQTS (n=14) including 1 case with epilepsy, CPVT (n=5), HCM (n=7), ARVC (n=1), and BrS (n=3). Twenty-seven cases were admitted to hospital due to syncope, whereas the remaining 3 cases of BrS had not presented with syncope before admission. The average onset age of inherited arrhythmia was (10.0±3.3) years. Genetic testing was performed on 20 patients. The median follow-up time was 40 months. Among 15 patients who underwent implantable cardioverter defibrillator (ICD) and survived, 2 patients had frequent ICD discharge. One patient underwent radiofrequency ablation, and the other one received left cardiac sympathetic denervation and an increased ICD defibrillation threshold, and the number of ICD discharge was significantly reduced. Among 10 patients who received drug therapy, 4 patients including two patients who discontinued treatment without advices died. Two patients whose parents refused treatment died, 1 case diagnosed with unexplained sudden cerebral death, and the remaining 2 cases without indication for drug therapy survived without any treatment. Conclusions: Mortality rate is high in pediatric patients with inherited cardiac arrhythmia and syncope. The therapeutic effect of drugs are not satisfactory, ICD implantation is the most effective treatment to prevent sudden cardiac death currently, but the postoperative frequent discharge should be brought to the forefront and handled in time.


Assuntos
Displasia Arritmogênica Ventricular Direita/genética , Síndrome de Brugada/genética , Cardiomiopatia Hipertrófica/genética , Síndrome do QT Longo/genética , Taquicardia Ventricular/genética , Arritmias Cardíacas , Displasia Arritmogênica Ventricular Direita/diagnóstico , Displasia Arritmogênica Ventricular Direita/mortalidade , Displasia Arritmogênica Ventricular Direita/terapia , Síndrome de Brugada/diagnóstico , Síndrome de Brugada/mortalidade , Síndrome de Brugada/terapia , Cardiomiopatia Hipertrófica/diagnóstico , Cardiomiopatia Hipertrófica/mortalidade , Cardiomiopatia Hipertrófica/terapia , Criança , Morte Súbita Cardíaca , Desfibriladores Implantáveis , Feminino , Seguimentos , Testes Genéticos , Humanos , Síndrome do QT Longo/diagnóstico , Síndrome do QT Longo/mortalidade , Síndrome do QT Longo/terapia , Masculino , Estudos Retrospectivos , Síncope , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/mortalidade , Taquicardia Ventricular/terapia , Resultado do Tratamento
12.
Am J Cardiol ; 124(5): 715-722, 2019 09 01.
Artigo em Inglês | MEDLINE | ID: mdl-31284935

RESUMO

Some Brugada syndrome (BrS) patients have been suspected of being in the initial state of arrhythmogenic right ventricular cardiomyopathy (ARVC). This study aimed to clarify the electrocardiographic (ECG) and clinical differences between BrS and ARVC in long-term follow-up (mean 11.9 ± 6.3 years). A total of 50 BrS and 65 ARVC patients with fatal ventricular tachyarrhythmia (VTA) were evaluated according to the revised Task Force Criteria for ARVC. Based on the current diagnostic criteria concerning electrocardiographic, repolarization abnormality was positive in 2.0% and 2.6% of BrS patients at baseline and follow-up, and depolarization abnormality was positive in 6.0% and 12.8% of BrS patients at baseline and follow-up, respectively. At baseline, none of the BrS patients were definitively diagnosed with ARVC. Considering patients' lives since birth, Kaplan-Meier analysis revealed that age at first VTA attack showed the same tendency between the groups (BrS: mean 42.2 ± 12.5 years old vs ARVC: mean 44.8 ± 13.7 years old, log-rank p = 0.123). Moreover, the incidence of VTA recurrence was similar between the groups during follow-up (log-rank p = 0.906). Incidence of sustained monomorphic ventricular tachycardia was significantly higher in ARVC than in BrS whereas the opposite was true for ventricular fibrillation (log-rank p <0.001 and p <0.001, respectively). None of the diagnoses of BrS patients were changed to ARVC during follow-up. During long-term follow-up, although age at first VTA attack and VTA recurrence were similar, BrS consistently exhibited features that differed from those of ARVC.


Assuntos
Displasia Arritmogênica Ventricular Direita/diagnóstico , Síndrome de Brugada/diagnóstico , Eletrocardiografia , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/mortalidade , Adulto , Fatores Etários , Displasia Arritmogênica Ventricular Direita/mortalidade , Síndrome de Brugada/mortalidade , Estudos de Coortes , Seguimentos , Humanos , Japão , Estimativa de Kaplan-Meier , Pessoa de Meia-Idade , Estudos Retrospectivos , Medição de Risco , Índice de Gravidade de Doença , Fatores Sexuais , Análise de Sobrevida , Fatores de Tempo
13.
Chin Med J (Engl) ; 132(12): 1406-1413, 2019 Jun 20.
Artigo em Inglês | MEDLINE | ID: mdl-31205097

RESUMO

BACKGROUND: The long-term predicted value of microvolt T-wave alternans (MTWA) for ventricular tachyarrhythmia in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) remains unclear. Our study explored the characteristics of MTWA and its prognostic value when combined with an electrophysiologic study (EPS) in patients with ARVC. METHODS: All patients underwent non-invasive MTWA examination with modified moving average (MMA) analysis and an EPS. A positive event was defined as the first occurrence of sudden cardiac death, documented sustained ventricular tachycardia (VT), ventricular fibrillation, or the administration of appropriate implantable cardioverter defibrillator therapy including shock or anti-tachycardia pacing. RESULTS: Thirty-five patients with ARVC (age 38.6 ±â€Š11.0 years; 28 males) with preserved left ventricular (LV) function were recruited. The maximal TWA value (MaxValt) was 17.0 (11.0-27.0) µV. Sustained VT was induced in 22 patients by the EPS. During a median follow-up of 99.9 ±â€Š7.7 months, 15 patients had positive clinical events. When inducible VT was combined with the MaxValt, the area under the curve improved from 0.739 to 0.797. The receiver operating characteristic curve showed that a MaxValt of 23.5 µV was the optimal cutoff value to identify positive events. The multivariate Cox regression model for survival showed that MTWA (MaxValt, hazard ratio [HR], 1.06; 95% confidence interval [CI], 1.01-1.11; P = 0.01) and inducible VT (HR, 5.98; 95% CI, 1.33-26.8; P = 0.01) independently predicted positive events in patients with ARVC. CONCLUSIONS: MTWA assessment with MMA analysis complemented by an EPS might provide improved prognostic ability in patients with ARVC with preserved LV function during long-term follow-up.


Assuntos
Arritmias Cardíacas/diagnóstico , Displasia Arritmogênica Ventricular Direita/diagnóstico , Eletrocardiografia/métodos , Eletrofisiologia/métodos , Taquicardia Ventricular/diagnóstico , Adulto , Teste de Esforço , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Função Ventricular Esquerda/fisiologia
14.
Biochemistry (Mosc) ; 84(3): 272-282, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31221065

RESUMO

Differential diagnosis of arrhythmogenic cardiomyopathy (ACM) during the disease latent phase is a challenging clinical problem that requires identification of early ACM biomarkers. Because extracellular nucleic acids are stable, specific, and can be easily detected, they can be used as reliable biomarkers of various diseases. In this study, we analyzed the levels of extracellular microRNAs and mitochondrial DNA in the conditioned medium collected from cardiomyocytes differentiated from induced pluripotent stem cells of ACM patients and healthy donor. Several microRNAs were expressed differently by the affected and healthy cardiomyocytes; therefore, they could be considered as potential ACM biomarkers.


Assuntos
Displasia Arritmogênica Ventricular Direita/diagnóstico , DNA Mitocondrial/análise , MicroRNAs/análise , Biomarcadores/análise , Células Cultivadas , DNA Mitocondrial/genética , Humanos , MicroRNAs/genética
15.
Prog Cardiovasc Dis ; 62(3): 217-226, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31004608

RESUMO

Sudden cardiac death (SCD) is a devastating possible outcome of all cardiomyopathies. The risk of SCD is increased in patients with structural heart disease and continues to increase as ventricular dysfunction worsens. There is, however, a subset of cardiomyopathy, so-called "arrhythmogenic cardiomyopathy" (ACM), that carries an inherent propensity for arrhythmia in all stages of the disease, even preceding ventricular dysfunction. The aim of this review is to identify cardiomyopathies, other than ischemic and dilated cardiomyopathies, that are associated with ventricular arrhythmias (VAs) and SCD. We discuss prevalence, diagnosis, natural history and management of arrhythmogenic right ventricular dysplasia/cardiomyopathy, ACM, and exercise-induced cardiomyopathy, with emphasis on the morbidity and mortality of VAs associated with these cardiomyopathies and how they can be mitigated through lifestyle modification, medical management, and implantation of cardioverter defibrillators.


Assuntos
Arritmias Cardíacas/etiologia , Displasia Arritmogênica Ventricular Direita/complicações , Cardiomiopatias/complicações , Morte Súbita Cardíaca/etiologia , Desfibriladores Implantáveis , Arritmias Cardíacas/mortalidade , Displasia Arritmogênica Ventricular Direita/diagnóstico , Cardiomiopatias/diagnóstico , Morte Súbita Cardíaca/prevenção & controle , Exercício Físico , Humanos , Prevalência
17.
Monaldi Arch Chest Dis ; 89(1)2019 Mar 27.
Artigo em Inglês | MEDLINE | ID: mdl-30968669

RESUMO

We reported a case of a young adult male aged 18 years admitted in our institution for syncope during a basketball match. No previous symptoms were reported. Electrocardiogram (ECG) showed T-wave inversion in the anterior leads and an incomplete right bundle branch block. Surprisingly, a complete echocardiographic evaluation demonstrated the presence of severe right ventricular enlargement with significant wall motion abnormalities, apical aneurysm and reduced systolic function. Cardiac Magnetic Resonance was pathognomonic for a fibro-fatty replacement of both ventricles. We decided for a subcutaneous defibrillator implantation and, after inducing a ventricular fibrillation to test the device status, epsilon wave appeared on the ECG. This clinical scenario depicted an advanced arrhythmogenic right ventricular cardiomyopathy at its first clinical manifestation.


Assuntos
Displasia Arritmogênica Ventricular Direita/diagnóstico , Bloqueio de Ramo/diagnóstico , Desfibriladores Implantáveis , Ventrículos do Coração/fisiopatologia , Adolescente , Displasia Arritmogênica Ventricular Direita/fisiopatologia , Displasia Arritmogênica Ventricular Direita/terapia , Ecocardiografia/métodos , Eletrocardiografia/métodos , Humanos , Imagem por Ressonância Magnética/métodos , Masculino , Síncope/etiologia
18.
Clin Res Cardiol ; 108(10): 1147-1162, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30868222

RESUMO

OBJECTIVES: As underlying heart diseases of right ventricular tachyarrhythmias, ARVC causes wall-motion abnormalities based on fibrofatty myocardial degeneration, while RVOT-VT and BrS are thought to lack phenotypic MR characteristics. To examine whether cardiac magnetic resonance (CMR) feature tracking (FT) in addition to ARVC objectively facilitates detection of myocardial functional impairments in RVOT-VT and BrS. METHODS: Cine MR datasets of four retrospectively enrolled, age-matched study groups [n = 65; 16 ARVC, 26 RVOT-VT, 9 BrS, 14 healthy volunteers (HV)] were independently assessed by two distinctly experienced investigators regarding myocardial function using CMR-FT. Global strain (%) and strainrate (s-1) in radial and longitudinal orientation were assessed at RVOT as well as for left (LV) and right (RV) ventricle at a basal, medial and apical section with the addition of a biventricular circumferential orientation. RESULTS: RV longitudinal and radial basal strain (%) in ARVC (- 12.9 ± 4.2; 11.4 ± 5.1) were significantly impaired compared to RVOT-VT (- 18.0 ± 2.5, p ≤ 0.005; 16.4 ± 5.2, p ≤ 0.05). Synergistically, RVOT endocardial radial strain (%) in ARVC (33.8 ± 22.7) was significantly lower (p ≤ 0.05) than in RVOT-VT (54.3 ± 14.5). For differentiation against BrS, RV basal and medial radial strain values (%) (13.3 ± 6.1; 11.8 ± 2.9) were significantly reduced when compared to HV (21.0 ± 6.9, p ≤ 0.05; 20.1 ± 6.6, p ≤ 0.005), even in case of a normal RV ejection fraction (EF) (> 45%; n = 6) (12.0 ± 2.7 vs. 20.1 ± 6.6, p ≤ 0.05). CONCLUSIONS: CMR-FT facilitates relevant differentiation in patients with right ventricular tachyarrhythmias: between ARVC against RVOT-VT and HV as well as between BrS with even a preserved EF against HV.


Assuntos
Displasia Arritmogênica Ventricular Direita/diagnóstico , Síndrome de Brugada/diagnóstico , Eletrocardiografia/métodos , Ventrículos do Coração/diagnóstico por imagem , Imagem Cinética por Ressonância Magnética/métodos , Contração Miocárdica/fisiologia , Disfunção Ventricular Direita/diagnóstico , Displasia Arritmogênica Ventricular Direita/fisiopatologia , Síndrome de Brugada/fisiopatologia , Diagnóstico Diferencial , Feminino , Seguimentos , Ventrículos do Coração/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Miocárdio/patologia , Reprodutibilidade dos Testes , Estudos Retrospectivos , Disfunção Ventricular Direita/fisiopatologia
19.
Int J Sports Med ; 40(5): 295-304, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30865997

RESUMO

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterized by ventricular arrhythmias and sudden death in the young and in competitive athletes. The deleterious role of exercise in the natural history of ARVC is clear. Even in the absence of a demonstrated arrhythmogenic substrate, family history or mutations of ARVC, intense physical exercise may in some individuals lead to the development of right ventricular dysfunction and arrhythmogenicity. This led to question the benignity of some adaptive features of the athlete's heart. In fact, there is an overlap between typical aspects of the athlete's heart and pathological changes described in ARVC, being challenging to distinguish the two conditions. The aim of this review is to highlight the aspects that help to distinguish between athlete's heart and ARVC, to review the major findings on exams helping in the differential diagnosis and to determine the implications on eligibility for leisure and competitive sports.


Assuntos
Displasia Arritmogênica Ventricular Direita/diagnóstico , Displasia Arritmogênica Ventricular Direita/patologia , Exercício Físico , Remodelação Ventricular , Atletas , Diagnóstico Diferencial , Humanos , Esportes
20.
Am J Cardiol ; 123(10): 1690-1695, 2019 05 15.
Artigo em Inglês | MEDLINE | ID: mdl-30885416

RESUMO

Less is known about bradyarrhythmias in arrhythmogenic right ventricular cardiomyopathy (ARVC). This cross-sectional study aimed to assess the prevalence and clinical significance of bradyarrhythmias in ARVC. From May 1995 to December 2017, bradyarrhythmias including sick sinus syndrome, atrioventricular block, and intraventricular conductional block (ICB) were investigated in 522 ARVC patients. A total of 169 patients (32.4%) presented with bradyarrhythmias including sick sinus syndrome in18 (3.5%), atrioventricular block in 56 (10.7%), and ICB in 118 patients (22.6%). Multivariate analysis showed right atrial dilation increased the risk of bradyarrhythmias (odds ratio [OR] 1.641, 95% confidence interval [CI] 1.081 to 2.492, p= 0.020). Bradyarrhythmias were not associated with death and heart transplantation. In patients with bradyarrhythmias, female gender, left atrial diameter >40 mm, and New York Heart Association Ⅲ/Ⅳ increased the risk of death and heart transplantation (hazards ratio [HR] = 2.790, 95% CI 1.220 to 6.377, p = 0.015; HR = 4.913, 95% CI 2.058 to 11.730, p <0.001; HR = 3.223, 95% CI 1.246 to 8.340, p = 0.016). Among the 23 patients who underwent device implantation, left atrial diameter >40mm was associated with death and heart transplantation (HR = 9.523, 95% CI 1.587 to 57.126, p = 0.014). In conclusion, bradyarrhythmias were commonly seen in ARVC, and ICB was the most common type. Female, left atrial diameter >40 mm, and NYHA class were associated with death and heart transplantation.


Assuntos
Displasia Arritmogênica Ventricular Direita/complicações , Bradicardia/etiologia , Átrios do Coração/diagnóstico por imagem , Ventrículos do Coração/diagnóstico por imagem , Função Ventricular Direita/fisiologia , Adulto , Displasia Arritmogênica Ventricular Direita/diagnóstico , Bradicardia/diagnóstico , Bradicardia/epidemiologia , China/epidemiologia , Estudos Transversais , Ecocardiografia , Eletrocardiografia , Feminino , Seguimentos , Átrios do Coração/fisiopatologia , Ventrículos do Coração/fisiopatologia , Humanos , Incidência , Masculino , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida/tendências
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