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1.
Medicine (Baltimore) ; 99(40): e22640, 2020 Oct 02.
Artigo em Inglês | MEDLINE | ID: mdl-33019488

RESUMO

INTRODUCTION: Danon disease is a rare X-linked dominant genetic disorder caused by defects in the lysosome-associated membrane protein 2 (LAMP2) gene. Unless treated, cardiogenic death is the main cause of mortality. This case report describes a 19-year-old man who was diagnosed with Danon disease and survived for 3 years from symptom onset to death. The mutation in his LAMP2 gene (p.Gly221Ilefs*19) had not been previously reported. PATIENT CONCERNS: A 19-year-old man patient was hospitalized for intermittent palpitations. He had no family history of cardiomyopathy, arrhythmia, or sudden cardiac death, but his sister had died of cirrhosis at age 12 years, but the exact cause of cirrhosis was unknown. DIAGNOSIS: Exome sequencing and Sanger sequencing identified a novel missense mutation (p.Gly221Ilefs*19) in the LAMP2 gene of the proband. This mutation was also detected in his mother, confirming the diagnosis of Danon disease. INTERVENTIONS: The patient experienced various types of arrhythmia throughout the clinical process, including Wolff-Parkinson-White syndrome, non-sustained atrial tachycardia, atrial flutter, and third-degree atrioventricular block. He was therefore treated with cardiac ablation procedures and cardiac resynchronization therapy. OUTCOMES: The period from the onset of symptoms to the onset of heart failure was 2 years. The patient died of cardiogenic death during the third year, at age 22 years. LESSONS: Danon disease is a rare disease that is difficult to recognize because of its hidden early manifestations. Early identification of its clinical symptoms can lead to early diagnosis and treatment.


Assuntos
Doença de Depósito de Glicogênio Tipo IIb/genética , Doença de Depósito de Glicogênio Tipo IIb/terapia , Proteína 2 de Membrana Associada ao Lisossomo/genética , Arritmias Cardíacas/etiologia , Terapia de Ressincronização Cardíaca/métodos , Ablação por Cateter/métodos , Evolução Fatal , Doença de Depósito de Glicogênio Tipo IIb/complicações , Doença de Depósito de Glicogênio Tipo IIb/diagnóstico , Insuficiência Cardíaca/etiologia , Humanos , Masculino , Mutação de Sentido Incorreto/genética , Sequenciamento Completo do Exoma/métodos , Síndrome de Wolff-Parkinson-White/genética , Síndrome de Wolff-Parkinson-White/terapia , Adulto Jovem
2.
Int Heart J ; 61(5): 1005-1013, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32999188

RESUMO

This study sought to evaluate clinical features, treatment patterns, and outcomes of patients with idiopathic inflammatory myopathy (IIM) complicated by heart failure (HF). Thirty-two patients with IIM-HF admitted to the Peking Union Medical College Hospital between January 1999 and January 2018 were retrospectively reviewed, including 14 patients with polymyositis, 11 with dermatomyositis, and 7 with overlap syndrome. Survivors and no-survivors were compared on clinical characteristics and treatment. Although systemic symptoms were variable, all patients presented with elevated troponin I. Rapid atrial arrhythmia was the most frequent arrhythmia. Systolic dysfunction and restrictive diastolic dysfunction were typical presentations in echocardiography. Twenty-nine patients were followed up for a median of 2.8 years (0.1 month to 11 years). We recorded 13 deaths of cardiogenic cause, 1 of serious IIM, and 3 of infective complications. The median survival time from diagnosis of IIM-HF to all-cause mortality was 8.4 months (range from 1 month to 5 years). Both all-cause deaths and cardiogenic deaths were more reported in the methotrexate-alone group than in the combination therapy group (6/7 versus 3/10, P = 0.050; 5/6 versus 2/9, P = 0.041). Combination therapy including methotrexate (HR = 0.188, 95%CI 0.040-0.871, P = 0.033) and taking ß-receptor blockers (HR = 0.249, 95%CI 0.086-0.719, P = 0.010) was associated with reduced risk of all-cause deaths. In conclusion, elevated troponin I, atrial arrhythmia, and systolic and restrictive diastolic dysfunction are typical characteristics of IIM-HF. Combined immunosuppression that includes methotrexate and ß-receptor blockers seems to be important to improve survival.


Assuntos
Imunossupressores/uso terapêutico , Metotrexato/uso terapêutico , Miosite/tratamento farmacológico , Miosite/mortalidade , Adolescente , Antagonistas Adrenérgicos beta/uso terapêutico , Adulto , Idoso , China/epidemiologia , Quimioterapia Combinada , Feminino , Glucocorticoides/uso terapêutico , Insuficiência Cardíaca/etiologia , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Masculino , Metilprednisolona/uso terapêutico , Pessoa de Meia-Idade , Miosite/complicações , Estudos Retrospectivos , Adulto Jovem
3.
Int Heart J ; 61(5): 1079-1083, 2020 Sep 29.
Artigo em Inglês | MEDLINE | ID: mdl-32879264

RESUMO

A Japanese girl with polycystic kidney disease (PKD) developed normally, but at 8 months of age, she was hospitalized for acute onset dyspnea. On the day after admission to hospital, her general condition suddenly became worse. An echocardiogram showed left ventricular dilatation with thin walls, severe mitral valve regurgitation, and a reduced ejection fraction. She died of acute cardiac failure 3 hours after the sudden change. Postmortem analysis with light microscopy showed disarray of cardiomyocytes without obvious infiltration of lymphocytes, and we diagnosed her heart failure as idiopathic dilated cardiomyopathy (DCM). Clinical exome sequencing showed compound heterozygous variants in JPH2 (p.T237A/p.I414L) and a heterozygous nonsense mutation in PKD1 (p.Q4193*). To date, several variants in the JPH2 gene have been reported to be pathogenic for adult-onset hypertrophic cardiomyopathy or DCM in an autosomal dominant manner and infantile-onset DCM in an autosomal recessive manner. Additionally, autosomal dominant polycystic kidney disease is a systemic disease associated with several extrarenal manifestations, such as cardiomyopathy. Here we report a sudden infant death case of DCM and discuss the genetic variants of DCM and PKD.


Assuntos
Cardiomiopatia Dilatada/genética , Proteínas de Membrana/genética , Proteínas Musculares/genética , Rim Policístico Autossômico Dominante/genética , Canais de Cátion TRPP/genética , Cardiomiopatia Dilatada/complicações , Cardiomiopatia Dilatada/patologia , Morte Súbita Cardíaca/etiologia , Evolução Fatal , Feminino , Insuficiência Cardíaca/sangue , Insuficiência Cardíaca/etiologia , Heterozigoto , Humanos , Lactente , Insuficiência da Valva Mitral/etiologia , Miocárdio/patologia , Peptídeo Natriurético Encefálico/sangue , Fragmentos de Peptídeos/sangue
4.
Medwave ; 20(7): e8008, 2020 Aug 28.
Artigo em Espanhol | MEDLINE | ID: mdl-32877391

RESUMO

In December 2019, a new strain of the SARS-CoV-2 coronavirus was reported in Wuhan, China, which produced severe lung involvement and progressed to respiratory distress. To date, more than seventeen million confirmed cases and more than half a million died worldwide from COVID-19. Patients with cardiovascular disease are more susceptible to contracting this disease and presenting more complications. We did a literature search on the association of cardiovascular disease and COVID-19 in databases such as Scopus, PubMed/MEDLINE, and the Cochrane Library. The purpose of this review is to provide updated information for health professionals who care for patients with COVID-19 and cardiovascular disease, given that they have a high risk of complications and mortality. Treatment with angiotensin-converting enzyme inhibitors and receptor blockers is controversial, and there is no evidence not to use these medications in patients with COVID-19. Regarding treatment with hydroxychloroquine associated or not with azithromycin, there is evidence of a higher risk with its use than clinical benefit and decreased mortality. Likewise, patients with heart failure are an important risk group due to their condition per se. Patients with heart failure and COVID-19 are a diagnostic dilemma because the signs of acute heart failure could be masked. On the other hand, in patients with acute coronary syndrome, the initial therapeutic approach could change in the context of the pandemic, although only based on expert opinions. Nonetheless, many controversial issues will be the subject of future research.


Assuntos
Betacoronavirus , Doenças Cardiovasculares/complicações , Infecções por Coronavirus/complicações , Pneumonia Viral/complicações , Síndrome Coronariana Aguda/etiologia , Síndrome Coronariana Aguda/terapia , Algoritmos , Inibidores da Enzima Conversora de Angiotensina/uso terapêutico , Antivirais/efeitos adversos , Azitromicina/efeitos adversos , Infecções por Coronavirus/tratamento farmacológico , Quimioterapia Combinada , Eletrocardiografia/efeitos dos fármacos , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/terapia , Humanos , Hidroxicloroquina/efeitos adversos , Hipertensão/complicações , Hipertensão/tratamento farmacológico , Pandemias , Peptidil Dipeptidase A/metabolismo , Pneumonia Viral/tratamento farmacológico , Prognóstico , Sistema Renina-Angiotensina/fisiologia
5.
Am J Chin Med ; 48(6): 1369-1383, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32933311

RESUMO

Age-related myocardial dysfunction is a very large healthcare burden. Here, we aimed to investigate whether ginsenoside Rb1 (Rb1) improves age-related myocardial dysfunction and to identify the relevant molecular mechanism. Young mice and aged mice were injected with Rb1 or vehicle for 3 months. Then, their cardiac function was inspected by transthoracic echocardiography. Serum and myocardium tissue were collected from all mice for histological or molecular expression analyses, including aging-related proteins, markers relevant to fibrosis and inflammation, and markers indicating the activation of the nuclear factor-kappa B (NF-[Formula: see text]B) pathway. Compared with the control condition, Rb1 treatment significantly increased the ejection fraction percentage and significantly decreased the internal diameter and volume of the left ventricle at the end-systolic and end-diastolic phases in aged mice. Rb1 treatment reduced collagen deposition and collagen I, collagen III, and transforming growth factor-[Formula: see text]1 protein expression levels in aged hearts. Rb1 also decreased the aging-induced myocardial inflammatory response, as measured by serum or myocardial interleukin-6 and tumor necrosis factor-[Formula: see text] levels. Furthermore, Rb1 treatment in aged mice increased cytoplasmic NF-[Formula: see text]B but decreased nuclear NF-[Formula: see text]B, which indicated the suppression of the NF-[Formula: see text]B signaling pathway by regulating the translocation of NF-[Formula: see text]B. Rb1 could alleviate aging-related myocardial dysfunction by suppressing fibrosis and inflammation, which is potentially associated with regulation of the NF-[Formula: see text]B signaling pathway.


Assuntos
Envelhecimento , Ginsenosídeos/farmacologia , Ginsenosídeos/uso terapêutico , Insuficiência Cardíaca/tratamento farmacológico , NF-kappa B/genética , NF-kappa B/metabolismo , Fitoterapia , Animais , Anti-Inflamatórios , Colágeno/metabolismo , Feminino , Expressão Gênica/efeitos dos fármacos , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/genética , Insuficiência Cardíaca/metabolismo , Camundongos Endogâmicos C57BL , Transdução de Sinais , Volume Sistólico/efeitos dos fármacos
6.
Nat Commun ; 11(1): 4664, 2020 09 16.
Artigo em Inglês | MEDLINE | ID: mdl-32938919

RESUMO

Cardiorenal syndrome type 4 (CRS4) is a common complication of chronic kidney disease (CKD), but the pathogenic mechanisms remain elusive. Here we report that morphological and functional changes in myocardial mitochondria are observed in CKD mice, especially decreases in oxidative phosphorylation and fatty acid metabolism. High phosphate (HP), a hallmark of CKD, contributes to myocardial energy metabolism dysfunction by downregulating peroxisome proliferator-activated receptor gamma coactivator 1 alpha (PGC1α). Furthermore, the transcriptional factor interferon regulatory factor 1 (IRF1) is revealed as the key molecule upregulated by HP through histone H3K9 acetylation, and responsible for the HP-mediated transcriptional inhibition of PGC1α by directly binding to its promoter region. Conversely, restoration of PGC1α expression or genetic knockdown of IRF1 significantly attenuates HP-induced alterations in vitro and in vivo. These findings demonstrate that IRF1-PGC1α axis-mediated myocardial energy metabolism remodeling plays a crucial role in the pathogenesis of CRS4.


Assuntos
Síndrome Cardiorrenal/metabolismo , Fator Regulador 1 de Interferon/metabolismo , Coativador 1-alfa do Receptor gama Ativado por Proliferador de Peroxissomo/metabolismo , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Animais , Síndrome Cardiorrenal/patologia , Modelos Animais de Doenças , Regulação para Baixo , Metabolismo Energético , Técnicas de Silenciamento de Genes , Taxa de Filtração Glomerular , Glucuronidase/genética , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/metabolismo , Humanos , Fator Regulador 1 de Interferon/genética , Masculino , Camundongos Endogâmicos BALB C , Camundongos Endogâmicos C57BL , Camundongos Knockout , Pessoa de Meia-Idade , Mitocôndrias Cardíacas/metabolismo , Mitocôndrias Cardíacas/patologia , Miócitos Cardíacos/efeitos dos fármacos , Miócitos Cardíacos/metabolismo , Coativador 1-alfa do Receptor gama Ativado por Proliferador de Peroxissomo/genética , Fosfatos/metabolismo , Regiões Promotoras Genéticas , Ratos , Insuficiência Renal Crônica/complicações , Insuficiência Renal Crônica/metabolismo , Adulto Jovem
7.
Anticancer Res ; 40(9): 5301-5307, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32878821

RESUMO

BACKGROUND/AIM: The aim of this single center, non-randomized, open-label, uncontrolled, interventional trial was to determine the feasibility of continuous administration of low-dose human atrial natriuretic peptide (hANP) perioperatively during curative operation for colorectal cancer patients without history of acute heart failure. PATIENTS AND METHODS: The study included three males and two females ranging from 27 to 70 years old. Continuous intravenous injection of hANP solution was started before surgery. The primary endpoint was safety of hANP administration, and the secondary endpoints were perioperative changes in ANP, b-type natriuretic peptide, electrocardiogram (ECG), and lung function. RESULTS: The American Society of Anaesthesiologists physical status was 1, 2, and 3 in three, one, and one patient, respectively. Grade 2 hypotension was observed in one case. No marked changes were observed between pre- and post-operation in all cases. CONCLUSION: Perioperative low-dose hANP administration is feasible and safe in patients with curative colorectal cancer.


Assuntos
Fator Natriurético Atrial/administração & dosagem , Neoplasias Colorretais/complicações , Neoplasias Colorretais/patologia , Insuficiência Cardíaca/prevenção & controle , Assistência Perioperatória , Complicações Pós-Operatórias/prevenção & controle , Adulto , Idoso , Biomarcadores , Neoplasias Colorretais/cirurgia , Eletrocardiografia , Feminino , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/etiologia , Humanos , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/etiologia , Avaliação de Sintomas , Resultado do Tratamento
8.
Cardiol Rev ; 28(6): 308-311, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32941261

RESUMO

Cardiac involvement as a complication of severe acute respiratory syndrome coronavirus 2 infection in children is a relatively new entity. We present our initial experience managing children with coronavirus disease 2019-related acute myocardial injury. The 3 patients presented here represent a spectrum of the cardiac involvement noted in children with coronavirus disease 2019-related multisystem inflammatory syndrome, including myocarditis presenting as cardiogenic shock or heart failure with biventricular dysfunction, valvulitis, coronary artery changes, and pericardial effusion.


Assuntos
Betacoronavirus , Infecções por Coronavirus , Insuficiência Cardíaca , Doenças das Valvas Cardíacas , Miocardite , Pandemias , Administração dos Cuidados ao Paciente/métodos , Derrame Pericárdico , Pneumonia Viral , Síndrome de Resposta Inflamatória Sistêmica , Adolescente , Betacoronavirus/isolamento & purificação , Betacoronavirus/patogenicidade , Técnicas de Imagem Cardíaca/métodos , Criança , Pré-Escolar , Vasos Coronários/diagnóstico por imagem , Vasos Coronários/patologia , Infecções por Coronavirus/diagnóstico , Infecções por Coronavirus/fisiopatologia , Infecções por Coronavirus/terapia , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/terapia , Doenças das Valvas Cardíacas/diagnóstico , Doenças das Valvas Cardíacas/virologia , Humanos , Miocardite/terapia , Miocardite/virologia , Derrame Pericárdico/terapia , Derrame Pericárdico/virologia , Pneumonia Viral/diagnóstico , Pneumonia Viral/fisiopatologia , Pneumonia Viral/terapia , Choque Cardiogênico/etiologia , Choque Cardiogênico/terapia , Síndrome de Resposta Inflamatória Sistêmica/diagnóstico , Síndrome de Resposta Inflamatória Sistêmica/fisiopatologia , Síndrome de Resposta Inflamatória Sistêmica/terapia , Resultado do Tratamento
9.
G Ital Cardiol (Rome) ; 21(10): 739-749, 2020 Oct.
Artigo em Italiano | MEDLINE | ID: mdl-32968306

RESUMO

Coronavirus 2019 disease (COVID-19), caused by SARS-CoV-2, can lead to cardiac impairment with various types of clinical manifestations, including heart failure and cardiogenic shock. A possible expression of cardiac impairment is non-ischemic ventricular dysfunction, which can be related to different pathological conditions, such as myocarditis, stress and cytokine-related ventricular dysfunction. The diagnosis of these pathological conditions can be challenging during COVID-19; furthermore, their prevalence and prognostic significance have not been elucidated yet. The purpose of this review is to take stock of the various aspects of non-ischemic ventricular dysfunction that may occur during COVID-19 and of the diagnostic implications related to the use of cardiac imaging techniques.


Assuntos
Betacoronavirus , Infecções por Coronavirus/complicações , Miocardite/diagnóstico por imagem , Pneumonia Viral/complicações , Disfunção Ventricular Esquerda/diagnóstico por imagem , Disfunção Ventricular Direita/diagnóstico por imagem , Doença Aguda , Infecções Assintomáticas , Infecções por Coronavirus/sangue , Infecções por Coronavirus/diagnóstico por imagem , Síndrome da Liberação de Citocina/complicações , Ecocardiografia , Eletrocardiografia , Insuficiência Cardíaca/etiologia , Humanos , Miocardite/sangue , Miocardite/etiologia , Pandemias , Pneumonia Viral/sangue , Pneumonia Viral/diagnóstico por imagem , Volume Sistólico/fisiologia , Cardiomiopatia de Takotsubo/diagnóstico por imagem , Cardiomiopatia de Takotsubo/etiologia , Troponina/sangue , Disfunção Ventricular Esquerda/sangue , Disfunção Ventricular Esquerda/etiologia , Disfunção Ventricular Direita/sangue , Disfunção Ventricular Direita/etiologia
10.
Medwave ; 20(7)31-08-2020.
Artigo em Inglês, Espanhol | LILACS | ID: biblio-1122676

RESUMO

En diciembre de 2019 se reportó en Wuhan, China, la aparición de una nueva cepa de coronavirus SARS-CoV-2 que producía un compromiso pulmonar severo y progresaba a estrés respiratorio agudo. A la fecha, son más de diecisiete millones los casos confirmados y más de medio millón los fallecidos en todo el mundo a causa de COVID-19. Los estudios reportan que los pacientes con enfermedad cardiovascular son más susceptibles a contraer esta enfermedad y a presentar más complicaciones. El propósito de esta revisión es proporcionar información actualizada para los profesionales de la salud que atienden a pacientes con COVID-19 y que tienen además enfermedad cardiovascular y por ende un riesgo elevado de complicaciones y mortalidad. Realizamos una búsqueda de bibliografía científica acerca de la asociación de enfermedad cardiovascular y COVID-19 en diferentes bases de datos como Scopus, MEDLINE vía PubMed y Cochrane Library. El tratamiento con inhibidores de la enzima convertidora de angiotensina y bloqueadores del receptor de angiotensina ha sido motivo de discusión y no hay evidencia sólida para contraindicarlo en pacientes con COVID-19. Respecto al tratamiento con hidroxicloroquina asociado o no con azitromicina, hay evidencia que demuestra un mayor riesgo con su utilización, que beneficio clínico y/o disminución de mortalidad. En este contexto, los pacientes con insuficiencia cardíaca representan un grupo importante de riesgo por su condición per se y por el dilema diagnóstico generado al evaluar un paciente con COVID-19, en el que los signos de insuficiencia cardíaca aguda podrían enmascararse. Por otro lado, en los pacientes con síndrome coronario agudo, el enfoque terapéutico inicial podría cambiar en el contexto de la pandemia, aunque sólo sobre la base de opiniones de expertos. Quedan, sin embargo, muchos temas en controversia que serán motivo de investigaciones futuras.


In December 2019, a new strain of the SARS-CoV-2 coronavirus was reported in Wuhan, China, which produced severe lung involvement and progressed to respiratory distress. To date, more than seventeen million confirmed cases and more than half a million died worldwide from COVID-19. Patients with cardiovascular disease are more susceptible to contracting this disease and presenting more complications. We did a literature search on the association of cardiovascular disease and COVID-19 in databases such as Scopus, PubMed/MEDLINE, and the Cochrane Library. The purpose of this review is to provide updated information for health professionals who care for patients with COVID-19 and cardiovascular disease, given that they have a high risk of complications and mortality. Treatment with angiotensin-converting enzyme inhibitors and receptor blockers is controversial, and there is no evidence not to use these medications in patients with COVID-19. Regarding treatment with hydroxychloroquine associated or not with azithromycin, there is evidence of a higher risk with its use than clinical benefit and decreased mortality. Likewise, patients with heart failure are an important risk group due to their condition per se. Patients with heart failure and COVID-19 are a diagnostic dilemma because the signs of acute heart failure could be masked. On the other hand, in patients with acute coronary syndrome, the initial therapeutic approach could change in the context of the pandemic, although only based on expert opinions. Nonetheless, many controversial issues will be the subject of future research.


Assuntos
Humanos , Pneumonia Viral/complicações , Doenças Cardiovasculares/complicações , Infecções por Coronavirus/complicações , Betacoronavirus , Antivirais/efeitos adversos , Pneumonia Viral/tratamento farmacológico , Prognóstico , Sistema Renina-Angiotensina/fisiologia , Algoritmos , Inibidores da Enzima Conversora de Angiotensina/uso terapêutico , Infecções por Coronavirus/tratamento farmacológico , Azitromicina/efeitos adversos , Peptidil Dipeptidase A/metabolismo , Quimioterapia Combinada , Eletrocardiografia/efeitos dos fármacos , Síndrome Coronariana Aguda/etiologia , Síndrome Coronariana Aguda/terapia , Pandemias , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/terapia , Hidroxicloroquina/efeitos adversos , Hipertensão/complicações , Hipertensão/tratamento farmacológico
11.
Ann Hematol ; 99(10): 2289-2294, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32737633

RESUMO

Iron overload-induced cardiomyopathy is the leading cause of death in patients with transfusion-dependent thalassemia (TDT). The mortality is extremely high in these patients with severe cardiac complications, and how to rescue them remains a challenge. It is reasonable to use combined chelation with deferiprone (L1) and deferoxamine (DFO) because of their shuttle and synergistic effects on iron chelation. Here, seven consecutive patients with TDT who had severe cardiac complications between 2002 and 2019 and received combined chelation therapy with oral high-dose L1 (100 mg/kg/day) and continuous 24-h DFO infusion (50 mg/kg/day) in our hospital were reported. Survival for eight consecutive patients receiving DFO monotherapy for their severe cardiac complications between 1984 and 2001 was compared. We found that combined chelation therapy with high-dose L1 and DFO was efficient to improve survival and cardiac function in patients with TDT presenting severe cardiac complications. Reversal of arrhythmia to sinus rhythm was noted in all patients. Their 1-month follow-up left ventricular ejection fraction increased significantly (P < 0.001). There were no deaths, and all patients were discharged from hospital with good quality of life. In contrast, all the eight patients receiving DFO monotherapy died (P < 0.001). Accordingly, combined chelation therapy with high-dose L1 and DFO should be considered in patients with TDT presenting cardiac complications.


Assuntos
Arritmias Cardíacas/tratamento farmacológico , Terapia por Quelação/métodos , Deferiprona/uso terapêutico , Desferroxamina/uso terapêutico , Insuficiência Cardíaca/tratamento farmacológico , Quelantes de Ferro/uso terapêutico , Sobrecarga de Ferro/tratamento farmacológico , Talassemia/terapia , Arritmias Cardíacas/etiologia , Arritmias Cardíacas/fisiopatologia , Transfusão de Sangue , Deferiprona/administração & dosagem , Desferroxamina/administração & dosagem , Avaliação de Medicamentos , Quimioterapia Combinada , Feminino , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/fisiopatologia , Humanos , Quelantes de Ferro/administração & dosagem , Sobrecarga de Ferro/etiologia , Masculino , Qualidade de Vida , Estudos Retrospectivos , Talassemia/complicações , Reação Transfusional , Resultado do Tratamento , Função Ventricular Esquerda
14.
Am Heart J ; 228: 57-64, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32828047

RESUMO

AIMS: Data regarding outcomes for patients with severe aortic stenosis (AS) with concomitant aortic insufficiency (AI), undergoing transcatheter aortic valve replacement (TAVR) are limited. This study aimed to analyze the prevalence of severe AS with concomitant AI among patients undergoing TAVR and outcomes of TAVR in this patient group. METHODS AND RESULTS: Using data from the STS/ACC-TVT Registry, we identified patients with severe AS with or without concomitant AI who underwent TAVR between 2011 and 2016. Patients were categorized based on the severity of pre-procedural AI. Multivariable proportional hazards regression models were used to examine all-cause mortality and heart failure (HF) hospitalization at 1-year. Among 54,535 patients undergoing TAVR, 42,568 (78.1%) had severe AS with concomitant AI. Device success was lower in patients with severe AS with concomitant AI as compared with isolated AS. The presence of baseline AI was associated with lower 1 year mortality (HR 0.94 per 1 grade increase in AI severity; 95% CI, 0.91-0.98, P < .001) and HF hospitalization (HR 0.87 per 1 grade increase in AI severity; 95% CI, 0.84-0.91, P < .001). CONCLUSIONS: Severe AS with concomitant AI is common among patients undergoing TAVR, and is associated with lower 1 year mortality and HF hospitalization. Future studies are warranted to better understand the mechanisms underlying this benefit. SHORT ABSTRACT: In this nationally representative analysis from the United States, 78.1% of patients undergoing TAVR had severe AS with concomitant AI. Device success was lower in patients with severe AS with concomitant AI as compared with isolated AS. The presence of baseline AI was associated with lower 1 year mortality (HR 0.94 per 1 grade increase in AI severity; 95% CI, 0.91-0.98, P < .001) and HF hospitalization (HR 0.87 per 1 grade increase in AI severity; 95% CI, 0.84-0.91, P < .001).


Assuntos
Insuficiência da Valva Aórtica , Estenose da Valva Aórtica , Valva Aórtica , Insuficiência Cardíaca , Complicações Pós-Operatórias , Substituição da Valva Aórtica Transcateter , Idoso , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/fisiopatologia , Valva Aórtica/cirurgia , Insuficiência da Valva Aórtica/complicações , Insuficiência da Valva Aórtica/diagnóstico , Insuficiência da Valva Aórtica/cirurgia , Estenose da Valva Aórtica/complicações , Estenose da Valva Aórtica/diagnóstico , Estenose da Valva Aórtica/epidemiologia , Estenose da Valva Aórtica/cirurgia , Feminino , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/mortalidade , Insuficiência Cardíaca/terapia , Próteses Valvulares Cardíacas , Hospitalização/estatística & dados numéricos , Humanos , Masculino , Avaliação de Resultados em Cuidados de Saúde , Complicações Pós-Operatórias/mortalidade , Complicações Pós-Operatórias/terapia , Sistema de Registros/estatística & dados numéricos , Índice de Gravidade de Doença , Substituição da Valva Aórtica Transcateter/efeitos adversos , Substituição da Valva Aórtica Transcateter/métodos , Estados Unidos/epidemiologia
15.
J Am Coll Cardiol ; 76(11): 1318-1324, 2020 09 15.
Artigo em Inglês | MEDLINE | ID: mdl-32828614

RESUMO

BACKGROUND: ST-segment elevation myocardial infarction (STEMI) is a fatal cardiovascular emergency requiring rapid reperfusion treatment. During the coronavirus disease-2019 (COVID-19) pandemic, medical professionals need to strike a balance between providing timely treatment for STEMI patients and implementing infection control procedures to prevent nosocomial spread of COVID-19 among health care workers and other vulnerable cardiovascular patients. OBJECTIVES: This study evaluates the impact of the COVID-19 outbreak and China Chest Pain Center's modified STEMI protocol on the treatment and prognosis of STEMI patients in China. METHODS: Based on the data of 28,189 STEMI patients admitted to 1,372 Chest Pain Centers in China between December 27, 2019 and February 20, 2020, the study analyzed how the COVID-19 outbreak and China Chest Pain Center's modified STEMI protocol influenced the number of admitted STEMI cases, reperfusion strategy, key treatment time points, and in-hospital mortality and heart failure for STEMI patients. RESULTS: The COVID-19 outbreak reduced the number of STEMI cases reported to China Chest Pain Centers. Consistent with China Chest Pain Center's modified STEMI protocol, the percentage of patients undergoing primary percutaneous coronary intervention declined while the percentage of patients undergoing thrombolysis increased. With an average delay of approximately 20 min for reperfusion therapy, the rate of in-hospital mortality and in-hospital heart failure increased during the outbreak, but the rate of in-hospital hemorrhage remained stable. CONCLUSIONS: There were reductions in STEMI patients' access to care, delays in treatment timelines, changes in reperfusion strategies, and an increase of in-hospital mortality and heart failure during the COVID-19 pandemic in China.


Assuntos
Infecções por Coronavirus , Controle de Infecções , Pandemias , Intervenção Coronária Percutânea , Pneumonia Viral , Infarto do Miocárdio com Supradesnível do Segmento ST , Terapia Trombolítica , Betacoronavirus , China/epidemiologia , Angiografia Coronária/métodos , Angiografia Coronária/estatística & dados numéricos , Infecções por Coronavirus/epidemiologia , Infecções por Coronavirus/prevenção & controle , Feminino , Insuficiência Cardíaca/epidemiologia , Insuficiência Cardíaca/etiologia , Mortalidade Hospitalar , Hospitalização , Humanos , Controle de Infecções/métodos , Controle de Infecções/organização & administração , Masculino , Pessoa de Meia-Idade , Inovação Organizacional , Avaliação de Processos e Resultados em Cuidados de Saúde , Pandemias/prevenção & controle , Assistência ao Paciente/métodos , Assistência ao Paciente/tendências , Intervenção Coronária Percutânea/métodos , Intervenção Coronária Percutânea/estatística & dados numéricos , Pneumonia Viral/epidemiologia , Pneumonia Viral/prevenção & controle , Infarto do Miocárdio com Supradesnível do Segmento ST/complicações , Infarto do Miocárdio com Supradesnível do Segmento ST/mortalidade , Infarto do Miocárdio com Supradesnível do Segmento ST/terapia , Terapia Trombolítica/métodos , Terapia Trombolítica/estatística & dados numéricos
17.
Cardiol Young ; 30(9): 1346-1349, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32600496

RESUMO

We present our recent experience with a 6-month-old infant with a personal history of short bowel syndrome that presented with fever, cyanosis, and cardiogenic shock secondary to severe pulmonary hypertension and right ventricular failure without pulmonary thromboembolism. He did not present signs of toxin-mediated disease or Kawasaki disease. He was finally diagnosed with SARS-CoV-2 infection. If this presentation is confirmed in future research, the severe cardiovascular impairment in children with COVID-19 could be also attributable to the primary pulmonary infection, not only to a multisystem inflammatory syndrome but also in children without heart disease.


Assuntos
Angiografia por Tomografia Computadorizada , Infecções por Coronavirus , Insuficiência Cardíaca , Hipertensão Pulmonar , Pandemias , Pneumonia Viral , Choque Cardiogênico , Síndrome de Resposta Inflamatória Sistêmica , Betacoronavirus/isolamento & purificação , Angiografia por Tomografia Computadorizada/métodos , Infecções por Coronavirus/complicações , Infecções por Coronavirus/diagnóstico , Infecções por Coronavirus/tratamento farmacológico , Infecções por Coronavirus/fisiopatologia , Infecções por Coronavirus/terapia , Cuidados Críticos/métodos , Ecocardiografia/métodos , Eletrocardiografia/métodos , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/fisiopatologia , Insuficiência Cardíaca/terapia , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/etiologia , Lactente , Masculino , Pneumonia Viral/complicações , Pneumonia Viral/diagnóstico , Pneumonia Viral/fisiopatologia , Pneumonia Viral/terapia , Radiografia Torácica , Respiração Artificial/métodos , Índice de Gravidade de Doença , Choque Cardiogênico/diagnóstico , Choque Cardiogênico/etiologia , Síndrome de Resposta Inflamatória Sistêmica/diagnóstico , Síndrome de Resposta Inflamatória Sistêmica/fisiopatologia , Resultado do Tratamento
18.
Rev Cardiovasc Med ; 21(2): 181-190, 2020 Jun 30.
Artigo em Inglês | MEDLINE | ID: mdl-32706207

RESUMO

Heart failure with preserved ejection fraction is a very common clinical problem. Its prevalence is increasing with aging of the population. A diverse group of risk factors and etiologies comprise the HFpEF syndrome. No specific therapies have been shown to improve survival for the vast majority of HFpEF cases. Restrictive cardiomyopathies account for a significant portion of HFpEF patients and are characterized by diastolic dysfunction due to infiltration of the myocardium or ventricular hypertrophy. Many of these restrictive diseases occur in the context of myocardial infiltration by other substances such as amyloid, iron or glycogen or endomyocardial fibrosis. These infiltrative diseases usually have important clues in the clinical picture and on cardiac imaging that may allow differentiation from the usual HFpEF phenotype (that is commonly seen in the older, hypertensive patient). Noninvasive diagnosis has replaced endomyocardial biopsy for most instances in the workup of these conditions. Early recognition is important to institute specific therapies and to improve prognosis. In this review, we describe 4 major infiltrative cardiomyopathies (Cardiac Amyloidosis, Sarcoidosis, Hemochromatosis and Fabry disease), and their key imaging features.


Assuntos
Amiloidose/diagnóstico por imagem , Cardiomiopatias/diagnóstico por imagem , Doença de Fabry/diagnóstico por imagem , Insuficiência Cardíaca/etiologia , Hemocromatose/diagnóstico por imagem , Volume Sistólico , Função Ventricular Esquerda , Amiloidose/complicações , Amiloidose/fisiopatologia , Amiloidose/terapia , Cardiomiopatias/complicações , Cardiomiopatias/fisiopatologia , Cardiomiopatias/terapia , Diagnóstico Diferencial , Diagnóstico Precoce , Doença de Fabry/complicações , Doença de Fabry/fisiopatologia , Doença de Fabry/terapia , Insuficiência Cardíaca/diagnóstico por imagem , Insuficiência Cardíaca/fisiopatologia , Insuficiência Cardíaca/terapia , Hemocromatose/complicações , Hemocromatose/fisiopatologia , Hemocromatose/terapia , Humanos , Valor Preditivo dos Testes , Prognóstico
19.
JAMA ; 324(1): 79-89, 2020 07 07.
Artigo em Inglês | MEDLINE | ID: mdl-32633805

RESUMO

Importance: Many patients with systemic amyloidosis are underdiagnosed. Overall, 25% of patients with immunoglobulin light chain (AL) amyloidosis die within 6 months of diagnosis and 25% of patients with amyloid transthyretin (ATTR) amyloidosis die within 24 months of diagnosis. Effective therapy exists but is ineffective if end-organ damage is severe. Objective: To provide evidence-based recommendations that could allow clinicians to diagnose this rare set of diseases earlier and enable accurate staging and counseling about prognosis. Evidence Review: A comprehensive literature search was conducted by a reference librarian with publication dates from January 1, 2000, to December 31, 2019. Key search terms included amyloid, amyloidosis, nephrotic syndrome, heart failure preserved ejection fraction, and peripheral neuropathy. Exclusion criteria included case reports, non-English-language text, and case series of fewer than 10 patients. The authors independently selected and appraised relevant literature. Findings: There was a total of 1769 studies in the final data set. Eighty-one articles were included in this review, of which 12 were randomized clinical trials of therapy that included 3074 patients, 9 were case series, and 3 were cohort studies. The incidence of AL amyloidosis is approximately 12 cases per million persons per year and there is an estimated prevalence of 30 000 to 45 000 cases in the US and European Union. The incidence of variant ATTR amyloidosis is estimated to be 0.3 cases per year per million persons with a prevalence estimate of 5.2 cases per million persons. Wild-type ATTR is estimated to have a prevalence of 155 to 191 cases per million persons. Amyloidosis should be considered in the differential diagnosis of adult nondiabetic nephrotic syndrome; heart failure with preserved ejection fraction, particularly if restrictive features are present; unexplained hepatomegaly without imaging abnormalities; peripheral neuropathy with distal sensory symptoms, such as numbness, paresthesia, and dysesthesias (although the autonomic manifestations occasionally may be the presenting feature); and monoclonal gammopathy of undetermined significance with atypical clinical features. Staging can be performed using blood testing only. Therapeutic decision-making for AL amyloidosis involves choosing between high-dose chemotherapy and stem cell transplant or bortezomib-based chemotherapy. There are 3 therapies approved by the US Food and Drug Administration for managing ATTR amyloidosis, depending on clinical phenotype. Conclusions and Relevance: All forms of amyloidosis are underdiagnosed. All forms now have approved therapies that have been demonstrated to improve either survival or disability and quality of life. The diagnosis should be considered in patients that have a multisystem disorder involving the heart, kidney, liver, or nervous system.


Assuntos
Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico , Algoritmos , Benzoxazóis/uso terapêutico , Dexametasona/uso terapêutico , Diagnóstico Diferencial , Inativação Gênica , Insuficiência Cardíaca/etiologia , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina/mortalidade , Amiloidose de Cadeia Leve de Imunoglobulina/fisiopatologia , Amiloidose de Cadeia Leve de Imunoglobulina/terapia , Transplante de Fígado , Melfalan/uso terapêutico , Prognóstico , Proteinúria/etiologia , Transplante de Células-Tronco
20.
Medicine (Baltimore) ; 99(29): e21133, 2020 Jul 17.
Artigo em Inglês | MEDLINE | ID: mdl-32702866

RESUMO

The aim of the study is to discuss the risk factor of right heart failure (RHF) especially the association of iron deficiency with RHF in Tibetan children who live in high altitude area. In this retrospective study, we collected the data of Tibetan children from January 2011 to December 2018 in our hospital. The patients included in the study had the following data: age, gender, ferritin, echocardiography, hemoglobin, C-reaction protein, and altitude of residence. According to whether RHF was diagnosed, the patients were divided into RHF group and non-RHF group. Totally 133 patients were included with 59 in RHF group and 74 in non-RHF group. In single factor analysis, age (P = .008), altitude of residence (P < .001), ferritin (P < .001), and pulmonary arterial systolic pressure (P < .001) showed significant difference between the 2 groups. Binary logistic regression was performed to further identify the association of the clinical factors with RHF. Higher pulmonary arterial systolic pressure (odds ratio: 29.303, 95% confidence interval: 5.249-163.589, P < .001) and lower ferritin level (odds ratio: 5.849, 95% confidence interval: 1.585-21.593, P = .008) were independent risk factors associated with RHF. In receiver-operating characteristic curve, the optimal cutoff value of ferritin level was 14.6 µg/L with the sensitivity of 81.4% and specificity of 89.2%. As continuous variable, the correlation between ferritin and RHF was not certain (P = .281). Due to the possibility that iron deficiency be a risk factor of RHF in Tibetan children, prevention and treatment of iron deficiency might be a potential way in reducing the incidence of RHF in this high altitude area.


Assuntos
Altitude , Anemia Ferropriva/complicações , Insuficiência Cardíaca/etiologia , Anemia Ferropriva/epidemiologia , Anemia Ferropriva/fisiopatologia , Proteína C-Reativa/análise , Distribuição de Qui-Quadrado , Ecocardiografia/métodos , Ecocardiografia/estatística & dados numéricos , Feminino , Ferritinas/análise , Ferritinas/sangue , Insuficiência Cardíaca/epidemiologia , Insuficiência Cardíaca/fisiopatologia , Hemoglobinas/análise , Humanos , Lactente , Modelos Logísticos , Masculino , Estudos Retrospectivos , Tibet/epidemiologia , Disfunção Ventricular Direita/sangue , Disfunção Ventricular Direita/epidemiologia , Disfunção Ventricular Direita/etiologia
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