Publications by authors named "Jyh-Ming Jimmy Juang"

Article Synopsis
  • Stereotactic arrhythmia radioablation (STAR) has shown effectiveness for treating refractory ventricular tachyarrhythmia (VT), with a focus on a Taiwanese patient group.
  • The study involved 11 patients, using advanced imaging techniques to treat VT with a single radiation dose, resulting in a significant 88% reduction in VT episodes within the first six months post-treatment.
  • Despite initial success, many patients experienced late VT recurrence, with a one-year survival rate of 83%, leading to some requiring additional treatments such as heart transplants or HeartMate III implantation.
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YKL-40 is significantly associated with the prevalence and severity of coronary artery disease (CAD). YKL-40 levels are significantly associated with variations in the CHI3L1 and TRIB1 genes. We investigated candidate genes for YKL-40 levels and evaluated the prognostic value of this biomarker and corresponding variants for long-term outcomes in patients with CAD.

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  • A study investigated the impact of drugs that prolong the corrected QT interval (QTc) on hospitalized patients over 45, focusing on drug-drug interactions (DDIs) and their real-world implications.* -
  • Among 2,276 patients, the use of QT-prolonging drugs significantly heightened the risk of QTc prolongation, particularly with "known risk" drugs and when multiple risky medications were involved.* -
  • The findings suggest careful prescribing and monitoring for patients with risk factors is essential to prevent QTc prolongation, weighing the risks against treatment benefits for overall patient care.*
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Background: To investigate the outcomes, clinical prognosticators, and genetic profiles of pediatric left ventricular non-compaction (LVNC).

Methods: All subjects were <18 years old, diagnosed with LVNC between January 2008 and December 2020. Whole-exome sequencing was undertaken.

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  • Fabry disease (FD) is an X-linked lysosomal storage disorder caused by mutations in the α-galactosidase A gene, leading to harmful buildup of glycosphingolipids in various tissues and classified into classic and late-onset phenotypes.
  • Classic phenotype shows severely reduced enzyme activity, resulting in a progressive disease with multi-organ issues, while late-onset often presents with milder symptoms and mainly affects the heart due to some remaining enzyme activity.
  • Early diagnosis through enzyme testing, imaging, and genotyping is crucial for effective treatment, which includes enzyme replacement therapy and new pharmacological options to prevent irreversible damage and optimize patient care based on genetic and gender considerations.
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  • * The guidelines address various aspects of ASCVD, including chronic coronary syndrome and cerebrovascular diseases, while promoting health education and the management of clinical risk factors like diet, exercise, and smoking.
  • * A catchy slogan and a memorable acronym were proposed to reinforce lifestyle changes related to modifiable risk factors, emphasizing the importance of healthy eating, physical activity, and overall well-being for ASCVD prevention.
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  • Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a serious heart condition primarily caused by mutations in the RYR2 gene, which accounts for about 60% of cases and is inherited in an autosomal dominant manner.!* -
  • This study, which used data from the Sudden Arrhythmia Death Syndrome Registry in Taiwan, analyzed 40 Taiwanese CPVT patients and found 55% had RYR2 mutations, similar to global rates, with some novel variants identified.!* -
  • Exercise-induced symptoms like fainting and cardiac arrest were reported more frequently in East Asian cohorts compared to Caucasians, highlighting the need for genetic testing tailored to different ethnic groups.!*
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Background: Lacosamide is frequently used as a mono- or adjunctive therapy for the treatment of adults with epilepsy. Although lacosamide is known to act on both neuronal and cardiac sodium channels, potentially leading to cardiac arrhythmias, including Brugada syndrome (BrS), its adverse effects in individuals with genetic susceptibility are less understood.

Case: We report a 33-year-old female with underlying epilepsy who presented to the emergency department with a four-day history of seizure clusters, and was initially treated with lacosamide therapy.

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Background: Diastolic dysfunction and alterations in cardiac geometry are early indicators of diabetic cardiomyopathy. However, the association between cardiac changes across the glucose continuum and the contribution of epicardial adipose tissue (EAT) to these changes has not yet been investigated.

Purpose: In this study, we aim to investigated the EAT on cardiac diastolic function and structural alterations along the diabetic continuum using cardiac magnetic resonance imaging (CMRI).

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Background And Aims: Brugada syndrome (BrS) is an inherited arrhythmia with a higher disease prevalence and more lethal arrhythmic events in Asians than in Europeans. Genome-wide association studies (GWAS) have revealed its polygenic architecture mainly in European populations. The aim of this study was to identify novel BrS-associated loci and to compare allelic effects across ancestries.

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Article Synopsis
  • Heart failure with preserved ejection fraction (HFpEF) is a complex condition affecting the heart and other organs, leading to significant health issues, especially in younger patients in Asia with high rates of diabetes and kidney disease.
  • The diagnosis involves a 2-step algorithm focusing on symptoms, ejection fraction, natriuretic peptide levels, and tests for heart abnormalities; if uncertain, additional stress testing may be required.
  • Management includes controlling comorbidities, lifestyle changes like weight loss and exercise, and pharmacological treatments such as diuretics and SGLT2 inhibitors to improve patient outcomes.
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Objectives: To identify the predictors of left ventricular ejection fraction (LVEF) recovery in patients with heart failure with reduced ejection fraction (HFrEF) and compare the mortality rate between patients with HFrEF and heart failure with improved ejection fraction (HFimpEF).

Methods: Patients in a post-acute care program from 2018 to 2021 were enrolled. A series of echocardiograms were arranged during follow-up.

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Objectives: Circulating serum amyloid A (SAA) levels are strongly associated with atherosclerotic cardiovascular disease risk and severity. The association between genetic variants, SAA levels, inflammatory marker levels, and coronary artery disease (CAD) prognosis has not been fully understood.

Materials And Methods: In total, 2199 Taiwan Biobank (TWB) participants were enrolled for a genome-wide association study (GWAS), and the long-term outcomes in 481 patients with CAD were analyzed.

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Article Synopsis
  • Next generation sequencing data allows for the analysis of low-frequency and rare genetic variants, which are crucial for understanding complex diseases that can't be fully explained by common variants. CLIN_SKAT is an R package developed to address the challenges of analyzing this data efficiently.
  • The package provides four user-friendly functions for obtaining clinically relevant variants, conducting gene-based association analyses, and visualizing results, making it easier to interpret findings.
  • CLIN_SKAT includes pre-analysis steps and customizable features to enhance the clinical relevance of its results, and it's available for free on multiple operating systems for users to download and use in R.
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  • A study was conducted to investigate the epidemiology of cardiomyopathy (CMP) in Taiwan, focusing on patients under 65 years old from 2001 to 2014.
  • Out of 38,868 identified CMP patients, about half had known causes, with the highest incidence rates in adults aged 60-64, while pediatric incidence peaked during infancy.
  • Although mortality was higher in pediatric patients (11.4% vs. 1.5% in adults), the incidence of sudden death was similar across both groups, highlighting concerns for sudden death risks in both age categories.
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Objective: Late-onset hereditary transthyretin amyloidosis with polyneuropathy (ATTRv-PN) is often associated with heart involvement. Recent advances in cardiac imaging allow the detection of cardiac amyloidosis. This study aimed to explore cardiomyopathy by cardiac imaging and its clinical correlates with polyneuropathy in late-onset ATTRv-PN.

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Resistin and soluble suppression of tumorigenicity 2 (sST2) are useful predictors in patients with coronary artery disease (CAD). Their serum levels are significantly attributed to variations in and loci. We investigated candidate variants in the for resistin levels and those in the locus for sST2 levels and evaluated the prognostication of these two biomarkers and the corresponding variants for long-term outcomes in the patients with CAD.

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Article Synopsis
  • - The study focuses on assessing cardiovascular mortality risk in an aging population through a combined score based on electrocardiographic (ECG) parameters, aiming for primary prevention strategies.
  • - Conducted on over 5,300 elderly subjects in Taiwan, results showed that specific ECG abnormalities like left ventricular hypertrophy (LVH) and prolonged QTc and PR intervals were significantly linked to all-cause and cardiovascular death.
  • - The research developed predictive models using an ECG abnormality score, which effectively identified high-risk groups, demonstrating robust statistical improvements in predicting outcomes related to mortality.
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  • Human copy number variations (CNVs) and copy number alterations (CNAs) are significant DNA changes that can lead to diseases like cancer, while also driving genetic diversity and evolution in populations.
  • The research community is working on large-scale databases such as ExAC, COSMIC, and CCLE to catalog these variations in both healthy individuals and cancer patients, although a fully comprehensive resource has been lacking.
  • CNVIntegrate is a new web-based platform that integrates CNV and CNA data from healthy individuals and cancer patients, including unique data from Taiwan, and facilitates statistical comparisons across different ethnic populations, aiding researchers and clinicians in drug discovery and precision treatments.
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This international multidisciplinary document intends to provide clinicians with evidence-based practical patient-centered recommendations for evaluating patients and decedents with (aborted) sudden cardiac arrest and their families. The document includes a framework for the investigation of the family allowing steps to be taken, should an inherited condition be found, to minimize further events in affected relatives. Integral to the process is counseling of the patients and families, not only because of the emotionally charged subject, but because finding (or not finding) the cause of the arrest may influence management of family members.

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Objective: To retrospectively investigate the clinical spectrum, genetic profiles and outcomes of survivors of paediatric sudden cardiac arrest (SCA).

Design And Patients: All 66 patients (aged 1-20 years), with unexpected SCA or syncope related to ventricular tachycardia (VT)/fibrillation and who survived to discharge from a tertiary centre, were enrolled from 1995 to 2018. Of these, 30 with underlying diseases prior to the events were excluded.

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Irritable bowel syndrome (IBS) is one of the most widely recognized functional bowel disorders (FBDs) with a genetic component. SCN5A gene and SCN1B loci have been identified in population-based IBS cohorts and proposed to have a mechanistic role in the pathophysiology of IBS. These same genes have been associated with Brugada syndrome (BrS).

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