Pediatric flecainide pharmacogenomics: a roadmap to delivering precision-based care to pediatrics arrhythmias.

Flecainide acetate is a Class 1c anti-arrhythmic with a potent sodium voltage gated channel blockade which is utilized for the second-line treatment of tachyarrhythmias in children and adults. Given its narrow therapeutic index, the individualization of drug therapy is of utmost importance for clinicians. Despite efforts to improve anti-arrhythmic drug therapy, there remain knowledge gaps regarding the impact of variation in the genes relevant to flecainide's disposition and response.

Association of frailty and chronic limb-threatening ischemia in patients on maintenance hemodialysis: a prospective cohort study.

Chronic limb-threatening ischemia (CLTI) is a prevalent yet unpredictable complication among patients undergoing hemodialysis, and frailty is linked to adverse outcomes in this population. This study examined the influence of clinical factors on vascular events in patients undergoing hemodialysis. This multicenter prospective cohort study included patients undergoing maintenance hemodialysis since January 2008.

Over 100 Fontan Patients on Systemic Ventricular Assist Device Support: An ACTION Update.

As the Fontan population grows, understanding successful strategies for ventricular assist device (VAD) support of the failing Fontan circulation is needed. We performed a retrospective analysis of patients with Fontan circulation and systemic VAD support in the Advanced Cardiac Therapies Improving Outcomes Network (ACTION) registry. Competing outcomes and Kaplan-Meier estimated survival methods were used.

Stem Cell Markers LGR5, LGR4 and Their Immediate Signalling Partners are Dysregulated in Preeclampsia.

Leucine-rich repeat-containing G protein-coupled receptors 5/4 (LGR5/LGR4) are critical stem cell markers in epithelial tissues including intestine. They agonise wingless-related integration site (WNT) signalling. Until now, LGR5/LGR4 were uncharacterised in placenta, where analogous functions may exist.

Multielectrode Radiofrequency Balloon Catheter for Paroxysmal Atrial Fibrillation: Results From the Global, Multicenter, STELLAR Study.

Introduction: The safety and efficacy of paroxysmal atrial fibrillation (PAF) ablation with the HELIOSTAR multielectrode radiofrequency (RF) balloon catheter have been demonstrated in European studies; data from elsewhere are lacking. This prospective, multicenter study conducted in the United States, Italy, and China investigated the safety and efficacy of pulmonary vein isolation (PVI) using HELIOSTAR in drug-refractory symptomatic PAF.

Methods: The primary effectiveness endpoint (PEE) was 12-month freedom from documented atrial fibrillation/atrial flutter/atrial tachycardia plus freedom from acute procedural failure, nonstudy catheter failure, repeat ablation failure, direct current cardioversion (DCCV), and Class I/III antiarrhythmic drug (AAD) failure.

Factors Associated With Shock at Presentation in Kawasaki Disease Versus Multisystem Inflammatory Syndrome in Children Associated With Covid-19.

Background: Although clinical overlap between Kawasaki disease (KD) and multisystem inflammatory syndrome in children (MIS-C) has been evident, information regarding those presenting with shock has been limited. We sought to determine associations with shock within and between diagnosis groups.

Methods: The International KD Registry enrolled contemporaneous patients with either KD or MIS-C from 39 sites in 7 countries from January 1, 2020, to January 1, 2023.

Midterm survival, clinical, and hemodynamic outcomes of a novel mechanical mitral valve prosthesis.

Objective: To evaluate the midterm survival, clinical, and hemodynamic outcomes of the On-X mechanical mitral valve, based on the 5-year results of the Prospective Randomized On-X Anticoagulation Clinical Trial (PROACT).

Method: PROACT Mitral was a multicenter study evaluating 401 patients who underwent mitral valve replacement (MVR) with either Standard or Conform-X On-X mitral valves, comparing low-dose and standard-dose warfarin. Here we report prespecified secondary outcomes of survival, New York Heart Association (NYHA) functional classification, and valve hemodynamics as assessed by core lab-adjudicated echocardiography at 1, 3, and 5 years in the pooled population.

Variants Are Associated with Atrial Dysfunction in Neonates with Hypoplastic Left Heart Syndrome.

: variants are the most well-known genetic risk factor (10%) for hypoplastic left heart syndrome (HLHS) and are associated with decreased cardiac transplant-free survival. encodes for α-myosin heavy chain (α-MHC), a contractile protein expressed in the neonatal atria. We therefore assessed atrial function in HLHS patients with variants.

Cardiac Papillary Fibroelastoma and Cerebrovascular Events: A Systematic Review.

Background: Cardiac papillary fibroelastomas (CPFs) are benign endocardial tumours with embolic potential. This article is a systematic review to identify the clinical profile, diagnosis, tumour characteristics, and treatment modalities in patients with CPF presenting with stroke or transient ischemic attack (TIA).

Methods: This systematic review was conducted using the PubMed and Embase databases, including case reports and/or series and observational studies (for a search period of up until April 2022).

Decreasing Endomyocardial Biopsy Frequency in Pediatric Heart Transplantation Using A Rejection Risk Prediction Score-A Single Center Study.

Background: Rejection remains an important cause of morbidity and mortality after pediatric heart transplantation (HT). Endomyocardial biopsy (EMB) is the gold standard for rejection diagnosis, but it comes with procedural risk. The frequency of EMB varies significantly across centers.

Messenger interference RNA therapies targeting apolipoprotein C-III and angiopoietin-like protein 3 for mixed hyperlipidemia: the future of plozasiran and zodasiran.

Introduction: Mixed hyperlipidemia represents a substantial public health issue and a considerable burden on healthcare systems. Although the introduction of statins and LDL-cholesterol lowering agents have significantly reduced the incidence of atherosclerotic cardiovascular diseases (ASCVD), a significant portion of the population continues to exhibit ASCVD progression due to elevated triglyceride-rich lipoprotein (TRL) levels. This persistent risk has catalyzed the development of novel pharmacological interventions targeting these lipoproteins.

Senescent Syncytiotrophoblast Secretion During Early Onset Preeclampsia.

Background: Preeclampsia is a severe hypertensive disorder in pregnancy that causes preterm delivery, maternal and fetal morbidity, mortality, and life-long sequelae. Understanding the pathogenesis of preeclampsia is a critical first step toward protecting mother and child from this syndrome and increased risk of cardiovascular disease later in life. However, effective early predictive tests and therapies for preeclampsia are scarce.

Poor correlation of venous lactate with systemic oxygen saturation in the paediatric cardiac ICU: a pilot study.

Introduction: Cardiac intensive care providers require a comprehensive understanding of cardiac output and oxygen delivery. The estimation of cardiac output in clinical practice often relies on thermodilution and the Fick principle. Central venous saturation and lactate levels are commonly used indicators for cardiac output assessment.

ACTION-ARC Pediatric and Adult Congenital Heart Disease Ventricular Assist Device Adverse Event Definitions-2023.

Adverse events (AEs) experienced by children and adults with congenital heart disease (CHD) on ventricular assist devices (VADs) are sometimes unique to these populations. The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) and the Academic Research Consortium (ARC) aimed to harmonize definitions of pediatric and CHD AEs for use in clinical trials, registries, and regulatory evaluation. Data from the ACTION registry and adjudication committee were used to adapt general mechanical circulatory support ARC definitions.

Rationale and design of CHD PULSE: Congenital Heart Disease Project to Understand Lifelong Survivor Experience.

Background: With improved survival of adults with congenital heart disease (CHD) comes a need to understand the lifelong outcomes of this population. The aim of this paper is to describe the rationale and design of Congenital Heart Disease Project to Understand Lifelong Survivor Experience (CHD PULSE), a study to determine long-term medical, neurocognitive, and psychosocial outcomes among adults with a history of intervention for CHD and to identify factors associated with those outcomes.

Methods: CHD PULSE is a cross-sectional survey conducted from September 2021 to April 2023 among adults aged 18 and older with a history of at least 1 intervention for CHD at 1 of 11 participating U.

Characteristics and Outcomes of Children and Young Adults With Sepsis Requiring Continuous Renal Replacement Therapy: A Comparative Analysis From the Worldwide Exploration of Renal Replacement Outcomes Collaborative in Kidney Disease (WE-ROCK).

Objectives: Pediatric sepsis-associated acute kidney injury (AKI) often requires continuous renal replacement therapy (CRRT), but limited data exist regarding patient characteristics and outcomes. We aimed to describe these features, including the impact of possible dialytrauma (i.e.

Recommendations for Cardiac Point-of-Care Ultrasound Nomenclature.

Point-of-care ultrasound (POCUS) involves the acquisition, interpretation, and immediate clinical integration of ultrasonographic imaging performed by a treating clinician. The current state of cardiac POCUS terminology is heterogeneous and ambiguous, in part because it evolved through siloed specialty practices. In particular, the medical literature and colloquial medical conversation contain a wide variety of terms that equate to cardiac POCUS.

Representation of obesity in contemporary atrial fibrillation ablation randomized controlled trials.

Background: The prevalence and impact of obesity on outcomes of atrial fibrillation (AF) ablation randomized controlled trials (RCTs) have not been well studied.

Objective: To examine the proportion of participants with obesity enrolled in RCTs of AF ablation and outcomes of ablation when subgroup analysis of participants with obesity were available.

Methods: We systematically searched PubMed and EMBASE for AF ablation RCTs published between January 1, 2015 to May 31, 2022.