BACKGROUND Myocarditis is an important cause of morbidity and mortality in children, leading to long-term sequelae including chronic congestive heart failure, dilated cardiomyopathy, heart transplantation, and death. ...BACKGROUND Myocarditis is an important cause of morbidity and mortality in children, leading to long-term sequelae including chronic congestive heart failure, dilated cardiomyopathy, heart transplantation, and death. The initial diagnosis of myocarditis is usually based on clinical presentation, but this widely ranges from the severe sudden onset of a cardiogenic shock to asymptomatic patients. Early recognition is essential in order to monitor and start supportive treatment prior to the development of severe adverse events. Of note, many cases of fulminant myocarditis are usually misdiagnosed as otherwise minor conditions during the weeks before the unexpected deterioration.AIM To provide diagnostic clues to make an early recognition of pediatric myocarditis.To investigate early predictors for poor outcomes.METHODS We conducted a retrospective cross-sectional single-center study from January 2008 to November 2017 at the Pediatric Department of our institution, including children < 18-years-old diagnosed with myocarditis. Poor outcome was defined as the occurrence of any of the following facts: death, heart transplant, persistent left ventricular systolic dysfunction or dilation at hospital discharge(early poor outcome), or after 1 year of follow-up(late poor outcome). We analyzed different clinical features and diagnostic test findings in order to provide diagnostic clues for myocarditis in children. Multivariable stepwise logistic regression analysis was performed using all variables that had been selected by univariate analysis to determine independent factors that predicted a poor early or late outcome in our study population.RESULTS A total of 42 patients [69% male; median age of 8(1.5-12) years] met study inclusion criteria. Chest pain(40%) was the most common specific cardiac symptom. Respiratory tract symptoms(cough, apnea, rhinorrhea)(38%),shortness of breath(35%), gastrointestinal tract symptoms(vomiting, abdominal pain, diarrhea)(33%), and fever(31%) were the most common non-cardiac initial complaints.展开更多
Background: The differential diagnosis for intracavitary cardiac masses is limited, typically including vegetations, tumors, or thrombi. Cardiac thrombi can often mimic cardiac tumors on imaging, creating a diagnostic...Background: The differential diagnosis for intracavitary cardiac masses is limited, typically including vegetations, tumors, or thrombi. Cardiac thrombi can often mimic cardiac tumors on imaging, creating a diagnostic challenge. Primary cardiac tumors are rare and usually benign, whereas most cardiac tumors result from metastases, commonly originating from malignancies in the breast, lung, or melanoma. Aim: This report highlights the importance of distinguishing various cardiac masses based on clinical presentations, clinical courses, and radiological features. Case Presentation: We describe two cases of cardiac masses with unique and diverse clinical features. Each case posed significant diagnostic challenges due to their distinct presentations and clinical progressions. Conclusion: These cases underscore the importance of considering both benign and metastatic origins in the differential diagnosis of intracavitary cardiac masses. Accurate differentiation between thrombi and tumors is crucial for appropriate management and treatment.展开更多
Fabry disease (FD) is a rare X-linked lysosomal accumulation disorder caused by a deficiency in the enzyme alpha-galactosidase A (Gal A), resulting in excessive storage of glycosphingolipids, particularly globotriaosy...Fabry disease (FD) is a rare X-linked lysosomal accumulation disorder caused by a deficiency in the enzyme alpha-galactosidase A (Gal A), resulting in excessive storage of glycosphingolipids, particularly globotriaosylceramide (Gb3). This leads to cellular dysfunction in various organs, with cardiovascular compromise being the major cause of morbidity and mortality. This study aimed to provide a comprehensive overview of FD focusing on its genetic, epidemiological, clinical, diagnostic, and therapeutic aspects. This study explored the genetic mutations associated with FD, its epidemiology, clinical phenotypes, cardiac manifestations, diagnostic approaches, and current treatment options. Background: FD is caused by mutations in GLA on the X chromosome, with over 1000 identified variants. Neonatal screening and specific studies have shown an increased incidence of FD. The clinical presentation varies between classic and late phenotypes, with cardiac involvement being a major concern, particularly in late-onset FD. Purpose: This study aimed to summarize the current knowledge on FD, emphasizing cardiac involvement, diagnostic modalities, and treatment options. Methods: A literature review of relevant studies on FD, including genetics, epidemiology, clinical presentation, diagnostic methods, and treatment options, was conducted. Results: Cardiac manifestations of FD included left ventricular hypertrophy (LVH), heart failure, arrhythmias, and sudden death. Diagnostic approaches such as electrocardiography, echocardiography, and cardiac magnetic resonance imaging play crucial roles in the early detection and monitoring of cardiac involvement. Enzyme replacement therapy (ERT) and emerging treatments have shown promise in managing FD, although challenges remain. Conclusions: FD remains a challenging condition in cardiology, with under-diagnosis being a concern. Early detection and specific therapy are essential to improve patient outcomes. Echocardiography and cardiac MRI are valuable tools for diagnosis and follow-up. De展开更多
文摘BACKGROUND Myocarditis is an important cause of morbidity and mortality in children, leading to long-term sequelae including chronic congestive heart failure, dilated cardiomyopathy, heart transplantation, and death. The initial diagnosis of myocarditis is usually based on clinical presentation, but this widely ranges from the severe sudden onset of a cardiogenic shock to asymptomatic patients. Early recognition is essential in order to monitor and start supportive treatment prior to the development of severe adverse events. Of note, many cases of fulminant myocarditis are usually misdiagnosed as otherwise minor conditions during the weeks before the unexpected deterioration.AIM To provide diagnostic clues to make an early recognition of pediatric myocarditis.To investigate early predictors for poor outcomes.METHODS We conducted a retrospective cross-sectional single-center study from January 2008 to November 2017 at the Pediatric Department of our institution, including children < 18-years-old diagnosed with myocarditis. Poor outcome was defined as the occurrence of any of the following facts: death, heart transplant, persistent left ventricular systolic dysfunction or dilation at hospital discharge(early poor outcome), or after 1 year of follow-up(late poor outcome). We analyzed different clinical features and diagnostic test findings in order to provide diagnostic clues for myocarditis in children. Multivariable stepwise logistic regression analysis was performed using all variables that had been selected by univariate analysis to determine independent factors that predicted a poor early or late outcome in our study population.RESULTS A total of 42 patients [69% male; median age of 8(1.5-12) years] met study inclusion criteria. Chest pain(40%) was the most common specific cardiac symptom. Respiratory tract symptoms(cough, apnea, rhinorrhea)(38%),shortness of breath(35%), gastrointestinal tract symptoms(vomiting, abdominal pain, diarrhea)(33%), and fever(31%) were the most common non-cardiac initial complaints.
文摘Background: The differential diagnosis for intracavitary cardiac masses is limited, typically including vegetations, tumors, or thrombi. Cardiac thrombi can often mimic cardiac tumors on imaging, creating a diagnostic challenge. Primary cardiac tumors are rare and usually benign, whereas most cardiac tumors result from metastases, commonly originating from malignancies in the breast, lung, or melanoma. Aim: This report highlights the importance of distinguishing various cardiac masses based on clinical presentations, clinical courses, and radiological features. Case Presentation: We describe two cases of cardiac masses with unique and diverse clinical features. Each case posed significant diagnostic challenges due to their distinct presentations and clinical progressions. Conclusion: These cases underscore the importance of considering both benign and metastatic origins in the differential diagnosis of intracavitary cardiac masses. Accurate differentiation between thrombi and tumors is crucial for appropriate management and treatment.
文摘目的：探讨急性心肌梗死患者早期血清可溶性ST2（sST2）水平及其与心肌活性的关系。方法采用ELISA法检测30例发病12 h以内的非ST段抬高型心肌梗死（NSTEMI）患者血清sST2水平，于发病后第7天行心脏磁共振检查并根据磁共振结果将患者分为透壁增强组、非透壁增强组和混合组。于7-14 d行PCI术，并于术后6个月再次行心脏磁共振检查评估心肌活性，观察指标包括梗死心肌质量、左心室射血分数及室壁运动异常评分在术前及术后的变化及其与心肌梗死早期血清sST2水平的关系。结果透壁增强组血清sST2的水平较之非透壁增强组及混合组明显升高（P＜0.05），混合组较非透壁增强组高（P＜0.05）；梗死心肌质量及室壁运动异常评分在3组患者PCI术后均减少，梗死心肌质量在非透壁增强组及混合组中减少有统计学差异（P＜0.05），室壁运动异常评分在非透壁增强组降低显著（P＜0.05）；左心室射血分数在3组患者PCI术后均增加，在非透壁增强组及混合组中增加有统计学差异（P＜0.05）。心肌梗死早期血清sST2水平与PCI术前及PCI术后6个月的心肌活性相关。结论急性心肌梗死早期血清中sST2的水平可反映心肌受损情况并可预测心肌梗死7 d PCI术前及PCI术后6个月的心肌活性。
文摘Fabry disease (FD) is a rare X-linked lysosomal accumulation disorder caused by a deficiency in the enzyme alpha-galactosidase A (Gal A), resulting in excessive storage of glycosphingolipids, particularly globotriaosylceramide (Gb3). This leads to cellular dysfunction in various organs, with cardiovascular compromise being the major cause of morbidity and mortality. This study aimed to provide a comprehensive overview of FD focusing on its genetic, epidemiological, clinical, diagnostic, and therapeutic aspects. This study explored the genetic mutations associated with FD, its epidemiology, clinical phenotypes, cardiac manifestations, diagnostic approaches, and current treatment options. Background: FD is caused by mutations in GLA on the X chromosome, with over 1000 identified variants. Neonatal screening and specific studies have shown an increased incidence of FD. The clinical presentation varies between classic and late phenotypes, with cardiac involvement being a major concern, particularly in late-onset FD. Purpose: This study aimed to summarize the current knowledge on FD, emphasizing cardiac involvement, diagnostic modalities, and treatment options. Methods: A literature review of relevant studies on FD, including genetics, epidemiology, clinical presentation, diagnostic methods, and treatment options, was conducted. Results: Cardiac manifestations of FD included left ventricular hypertrophy (LVH), heart failure, arrhythmias, and sudden death. Diagnostic approaches such as electrocardiography, echocardiography, and cardiac magnetic resonance imaging play crucial roles in the early detection and monitoring of cardiac involvement. Enzyme replacement therapy (ERT) and emerging treatments have shown promise in managing FD, although challenges remain. Conclusions: FD remains a challenging condition in cardiology, with under-diagnosis being a concern. Early detection and specific therapy are essential to improve patient outcomes. Echocardiography and cardiac MRI are valuable tools for diagnosis and follow-up. De
