Clinical manifestations, genetic profiles, and sudden cardiac arrest in pediatric hypertrophic cardiomyopathy: Challenges of risk prediction for initial sudden cardiac arrest presentations.

Background: Sudden cardiac arrest (SCA) is a leading cause of death in pediatric hypertrophic cardiomyopathy (HCM).

Objective: The study sought to analyze the clinical and genetic characteristics of pediatric HCM and assess the applicability of current SCA risk prediction models.

Methods: We enrolled individuals diagnosed as HCM before 20 years of age, between 2000 and 2020, excluding those secondary to hemodynamic causes and those associated with genetic syndromes other than RASopathies.

Impact of Tafamidis on [Tc]Tc-pyrophosphate Scintigraphy in Ala97Ser Hereditary Transthyretin amyloid cardiomyopathy: significant initial reduction with stable Long-Term effects.

Objective: Tafamidis has shown potential in slowing disease progression in patients with transthyretin amyloid cardiomyopathy (ATTR-CM). This study aimed to evaluate serial changes on [Tc]Tc-pyrophosphate (PYP) scintigraphy during tafamidis treatment for hereditary ATTR-CM.

Methods: We retrospectively analyzed a prospectively collected cohort of Ala97Ser (A97S) hereditary ATTR-CM patients treated with tafamidis (61 mg/day) and a control group comprising A97S hereditary ATTR-CM patients who had not received disease-modifying medications.

Circulating YKL-40 levels but not CHI3L1 or TRIB1 gene variants predict long-term outcomes in patients with angiographically confirmed multivessel coronary artery disease.

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.

2024 Guidelines of the Taiwan Society of Cardiology on the Primary Prevention of Atherosclerotic Cardiovascular Disease --- Part II.

For the primary prevention of atherosclerotic cardiovascular disease (ASCVD), the recommended treatment target for each modifiable risk factor is as follows: reducing body weight by 5-10%; blood pressure < 130/80 mmHg (systolic pressure < 120 mmHg in high-risk individuals); low-density lipoprotein cholesterol (LDL-C) < 100 mg/dL in high-risk individuals, LDL-C < 115 mg/dL in moderate-risk individuals, LDL-C < 130 mg/dL in low-risk individuals, and LDL-C < 160 mg/dL in those with a minimal; complete and persistent abstinence from cigarette smoking; hemoglobin A1C < 7.0%; fulfilling recommended amounts of the six food groups according to the Taiwan food guide; and moderate-intensity physical activity 150 min/wk or vigorous physical activity 75 min/wk. For the primary prevention of ASCVD by pharmacological treatment in individuals with modifiable risk factors/clinical conditions, statins are the first-line therapy for reducing LDL-C levels; some specific anti-diabetic drugs proven to be effective in randomized controlled trials for the primary prevention of ASCVD are recommended in patients with type 2 diabetes mellitus; pharmacological treatment is recommended to assist in weight management for obese patients with a body mass index ≥ 30 kg/m (or 27 kg/m who also have at least one ASCVD risk factor or obesity-related comorbidity); an angiotensin-converting enzyme inhibitor, a glucagon-like peptide-1 receptor agonist, a sodium-dependent glucose cotransporter-2 inhibitor, and finerenone can be used in diabetic patients with chronic kidney disease for the primary prevention of ASCVD.

Genophenotypic correlates and long-term outcome prognosticators of left ventricular non-compaction in children.

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.

Case Report: Lacosamide unmasking -associated Brugada syndrome in a young female with epilepsy.

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.

Epicardial Adipose Tissue Is Associated With Geometry Alteration and Diastolic Dysfunction in Prediabetic Cardiomyopathy.

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 aimed to investigate the EAT on cardiac diastolic function and structural alterations along the diabetic continuum using cardiac magnetic resonance imaging (CMRI).

Brugada syndrome in Japan and Europe: a genome-wide association study reveals shared genetic architecture and new risk loci.

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.

Predictors of Left Ventricular Ejection Fraction Improvement in Patients with Early-Stage Heart Failure with Reduced Ejection Fraction.

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.

Circulating serum amyloid A levels but not SAA1 variants predict long-term outcomes of angiographically confirmed coronary artery disease.

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.

Cardiomyopathy correlates to nerve damage in p.A117S late-onset transthyretin amyloid polyneuropathy.

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.

Synergistic Effects of Weighted Genetic Risk Scores and Resistin and sST2 Levels on the Prognostication of Long-Term Outcomes in Patients with Coronary Artery Disease.

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.

2020 APHRS/HRS expert consensus statement on the investigation of decedents with sudden unexplained death and patients with sudden cardiac arrest, and of their families.

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.