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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
4.
Int Heart J ; 60(5): 1219-1221, 2019 Sep 27.
Artigo em Inglês | MEDLINE | ID: mdl-31484873

RESUMO

We recently experienced a 43-year-old man with dilated cardiomyopathy transported under the Impella support to a high-volume left ventricular assist device (LVAD) center. Stabilized hemodynamics with the Impella and firm fixation of the device were important for safe transportation of the patient.


Assuntos
Cardiomiopatia Dilatada/cirurgia , Coração Auxiliar , Segurança do Paciente , Adulto , Cardiomiopatia Dilatada/diagnóstico , Humanos , Japão , Masculino , Monitorização Fisiológica/métodos , Medição de Risco , Transporte de Pacientes/métodos
7.
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
8.
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
9.
Cardiology ; 142(3): 167-174, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31189164

RESUMO

PURPOSE: The aim of this study was to quantify the value of various clinical, laboratory, and instrumental signs in the diagnosis of myocarditis in comparison with morphological studies of the myocardium. METHODS: In 100 patients (65 men, 44.7 ± 12.5 years old) with "idiopathic" arrhythmias (n = 20) and dilated cardiomyopathy (DCM; n = 80), we performed the following: 71 endomyocardial biopsies (EMB), 13 intraoperative biopsies, 5 studies of explanted hearts, and 11 autopsies with virus investigation (real-time PCR) of the blood and myocardium. Antiheart antibodies (AHA) were also measured as well as cardiac CT (n = 45), MRI (n = 25), and coronary angiography (n = 47). The comparison group included 50 patients (25 men, 53.7 ± 11.7 years old) with noninflammatory heart diseases who underwent open heart surgery. RESULTS: Active/borderline myocarditis was diagnosed in 76.0% of the study group and in 21.6% of patients in the comparison group (p < 0.001). The myocardial viral genome was observed more frequently in patients in the comparison group than in the study group (65.0 and 40.2%; p < 0.01). We evaluated the diagnostic value of noninvasive markers of myocarditis. The panel of AHA had the greatest importance in the identification of myocarditis: sensitivity was 81.5%, and the positive and negative predictive values were 75.0 and 60.5%. This defined the diagnostic value of noninvasive markers of myocarditis and established a diagnostic algorithm providing an individual assessment of the likelihood of myocarditis development. CONCLUSION: AHA have the greatest significance in the diagnosis of latent myocarditis in patients with "idiopathic" arrhythmias and DCM. The use of a complex of noninvasive criteria allows the probability of myocarditis to be estimated and the indications for EMB to be determined.


Assuntos
Anticorpos/análise , Arritmias Cardíacas/diagnóstico , Cardiomiopatia Dilatada/diagnóstico , Miocardite/diagnóstico , Miocárdio/patologia , Adulto , Antiestreptolisina/sangue , Arritmias Cardíacas/sangue , Biópsia , Técnicas de Imagem Cardíaca , Cardiomiopatia Dilatada/sangue , Diagnóstico Diferencial , Feminino , Genoma Viral , Humanos , Masculino , Pessoa de Meia-Idade , Miocardite/sangue , Miocárdio/imunologia , Valor Preditivo dos Testes , Medição de Risco , Fatores de Risco , Federação Russa
10.
Pediatr. aten. prim ; 21(82): e77-e79, abr.-jun. 2019. ilus
Artigo em Espanhol | IBECS | ID: ibc-184592

RESUMO

La insuficiencia cardiaca es una patología poco frecuente en las consultas médicas pediátricas. Las formas en las que se expresa clínicamente esta incapacidad son muy diversas, sin embargo, en el paciente adulto suelen detectarse a través de síntomas clásicos como la disnea. En el caso del paciente pediátrico este espectro de presentación es mucho más amplio e inespecífico. El porcentaje de pacientes que no son correctamente diagnosticados en la primera consulta médica asciende hasta el 50%, y muchos de ellos reciben terapia antibiótica al haber sido etiquetado de infección bacteriana. Por ello presentamos el caso de un niño de tres años diagnosticado de insuficiencia cardiaca en nuestro servicio de urgencias pediátricas


Heart failure is an uncommon pathology in medical pediatric consultations. The forms in which this disability is clinically expressed are diverse, however, in the adult patient it is often detected through classic symptoms such as dyspnea. In the case of the pediatric patient this spectrum of presentation is much wider and non-specific. The percentage of patients who are not correctly diagnosed at the first medical consultation amounts to 50%, receiving many of them antibiotic therapy after having been labelled as bacterial infection. Therefore, we present the case of a three-year-old child diagnosed with heart failure in our pediatric emergency department


Assuntos
Humanos , Masculino , Pré-Escolar , Insuficiência Cardíaca/diagnóstico , Insuficiência da Valva Mitral/diagnóstico , Cardiomiopatia Dilatada/diagnóstico , Tratamento de Emergência/métodos , Dor Abdominal/etiologia , Diagnóstico Tardio , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos/prevenção & controle
11.
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
13.
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
14.
Pan Afr Med J ; 32: 34, 2019.
Artigo em Francês | MEDLINE | ID: mdl-31143339

RESUMO

We here report the case of a 38-year old woman with dilated cardiomyopathy induced by hypocalcaemia secondary to hypoparathyroidism. The patient had low calcium level (30 mg/L) and echocardiography showed dilated-hypokinetic cardiomyopathy with reduced left ventricular ejection fraction (31.4%). She received calcitherapy associated with vitamin D3 and her evolution was marked by the normalization of the size of the cardiac cavities and of the left ventricular ejection fraction after normocalcemia.


Assuntos
Cardiomiopatia Dilatada/etiologia , Hipocalcemia/complicações , Hipoparatireoidismo/complicações , Tireoidectomia/métodos , Adulto , Cálcio/administração & dosagem , Cálcio/sangue , Cardiomiopatia Dilatada/diagnóstico , Cardiomiopatia Dilatada/fisiopatologia , Colecalciferol/administração & dosagem , Feminino , Humanos , Hipocalcemia/etiologia , Hipoparatireoidismo/etiologia , Volume Sistólico , Resultado do Tratamento
15.
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
16.
Pediatr Cardiol ; 40(5): 950-957, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30993396

RESUMO

Dilated cardiomyopathy (DCM) is characterized by left or bilateral ventricular dilation and systolic dysfunction without rational conditions, which can lead to progressive heart failure and sudden cardiac death. Most of the pathogenic genes have been reported in adult population by locus mapping in familial cases and animal model studies. However, it still remains challenging to decipher the role of genetics in the etiology of pediatric DCM. We applied whole-exome sequencing (WES) for 30 sporadic pediatric DCM subjects and 100 non-DCM local controls. We identified the pathogenic mutations using bioinformatics tools based on genomic strategies synergistically and confirmed mutations by Sanger sequencing. We identified compound heterozygous nonsense mutations in DSP (c.3799C > T, p.R1267X; c.4444G > T, p.E1482X). In sporadic cases, the two heterozygous mutations in XIRP2 were identified. Then we performed an exome-wide association study with 30 case and 100 control subjects. Interestingly, we could not identify TTN truncating variants in all cases. Collectively, we observed a significant risk signal between carriers of TTN deleterious missense variants and DCM risk (odds ratio 4.0, 95% confidence interval 1.1-22.2, p = 3.12 × 10-2). Our observations expanded the spectrum of mutations and were valuable in the pre- and postnatal screening and genetic diagnosis for DCM.


Assuntos
Cardiomiopatia Dilatada/genética , Heterogeneidade Genética , Sequenciamento Completo do Exoma/métodos , Cardiomiopatia Dilatada/diagnóstico , Estudos de Casos e Controles , Criança , Pré-Escolar , China , Códon sem Sentido , Feminino , Humanos , Masculino , Linhagem , Mutação Puntual
17.
Cell Physiol Biochem ; 52(3): 435-438, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30873819

RESUMO

BACKGROUND/AIMS: Tachycardiomyopathy (TCM) is a largely reversible form of non-ischemic heart failure. The underlying mechanism are, however, still today poorly understood. Recent data indicate distinct changes in mitochondrial distribution in these patients, compared to other non-ischemic cardiomyopathies.This study investigated underlying mechanisms in mitochondrial dynamics in endomyocardial biopsy samples (EMB) from patients with TCM and compared them to patients with dilated cardiomyopathy (DCM), which show similar clinical features. METHODS: Focused mRNA analyses were performed on routinely obtained paraffinfixed EMB specimen from patients fulfilling TCM diagnosis criteria, as well as patients with DCM to elucidate regulatory changes in mitochondrial fusion, fission and mitophagy. RESULTS: In patients with TCM we were able to identify mRNA of Mitofusin 1 and 2, two effector proteins regulating mitochondrial fusion, to be strongly upregulated compared to patients with DCM. Conclusively, we did not find differences in the mRNA expression of mitochondrial fission regulators including DRP1, Fis1, MFF, MiD49, and MiD51. Furthermore, we did not find significant changes in PINK1 expression, an important mediator for mitochondrial autophagy. CONCLUSION: The mRNA upregulation of Mitofusin 1 and 2 provides first insight into the complex changes of mitochondrial dynamics in cardiomyocytes of patients with reversible heart failure due to TCM.


Assuntos
Cardiomiopatia Dilatada/genética , GTP Fosfo-Hidrolases/genética , Mitocôndrias/genética , Dinâmica Mitocondrial/genética , Proteínas de Transporte da Membrana Mitocondrial/genética , Proteínas Mitocondriais/genética , RNA Mensageiro/genética , Biópsia , Cardiomiopatia Dilatada/classificação , Cardiomiopatia Dilatada/diagnóstico , Cardiomiopatia Dilatada/fisiopatologia , GTP Fosfo-Hidrolases/metabolismo , Regulação da Expressão Gênica , Frequência Cardíaca/fisiologia , Humanos , Proteínas de Membrana/genética , Proteínas de Membrana/metabolismo , Proteínas Associadas aos Microtúbulos/genética , Proteínas Associadas aos Microtúbulos/metabolismo , Mitocôndrias/metabolismo , Mitocôndrias/patologia , Proteínas de Transporte da Membrana Mitocondrial/metabolismo , Proteínas Mitocondriais/metabolismo , Miócitos Cardíacos/metabolismo , Miócitos Cardíacos/patologia , Fatores de Alongamento de Peptídeos/genética , Fatores de Alongamento de Peptídeos/metabolismo , Proteínas Quinases/genética , Proteínas Quinases/metabolismo , RNA Mensageiro/metabolismo
18.
Zhonghua Xin Xue Guan Bing Za Zhi ; 47(3): 197-203, 2019 Mar 24.
Artigo em Chinês | MEDLINE | ID: mdl-30897878

RESUMO

Objective: Screen the pathogenic genes of a pedigree with clinical manifestation of familial dilated cardiomyopathy in Inner Mongolia. Methods: A total of 3 patients with dilated cardiomyopathy and 20 family members from the same family were examined in Ordos Central Hospital in Inner Mongolia from October, 2003 to August, 2017. Data on medical history, physical examinations, electrocardiograms, and echocardiography were obtained. 5 ml peripheral blood was sampled for per person. Chip Capture Sequencing technology was used to capture all the exons and splice sites of the genes that associated with hereditary cardiomyopathy and hereditary arrhythmia. The mutations in these genes were detected by high-throughput sequencing. All suspected pathogenic loci identified by high-throughput sequencing were verified by Sanger sequencing used for mutation detection. One hundred and fifty gender, age and race matched healthy people were included as the control group. Results: Pathogenic gene variations were detected in 3 symptomatic family members and 1 carrier from the pedigree. Five pathogenic gene variations were identified in the proband (Ⅱ1), a pSer236Gly and a pArg215Cys variation in the MYBPC3 gene, a pGln90Arg variation in the DSP gene, and pAsn2912Asp and pGlu2910Val variation in the DMD gene. One pathogenic variation was detected in Ⅲ3, which was a pArg215Cys variation in the MYBPC3 gene. Two pathogenic variations were detected in Ⅲ7, a pSer236Gly variation in the MYBPC3 gene and a pGln90Arg variation in the DSP gene. Two pathogenic variations were detected in the Ⅳ7, a pSer236Gly variation in the MYBPC3 gene and a pGln90Arg variation in the DSP gene. No gene variation loci were detected in the other family members and the control group. Conclusion: MYBPC3 gene, DSP gene and DMD gene variations are present in the familial dilated cardiomyopathy pedigree from Inner Mongolia, and these variations may be related with familial dilated cardiomyopathy.


Assuntos
Cardiomiopatia Dilatada , Cardiomiopatia Dilatada/diagnóstico , Cardiomiopatia Dilatada/genética , Proteínas de Transporte , China , Humanos , Mutação , Linhagem
19.
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
20.
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
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