Publications by authors named "Taylor Howard"

Background: cardiomyopathy is a distinct subset of arrhythmogenic cardiomyopathy, reported primarily in adults, that has predominantly left ventricular involvement and features of myocarditis. Clinical characteristics, risk stratification, and management of pediatric patients with variants are not well known. We sought to identify phenotypic features and prognosis of pediatric patients with pathogenic or likely pathogenic variants.

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Article Synopsis
  • Event monitors are increasingly used to assess arrhythmias in patients with congenital heart disease, with only 8.8% showing critical events upon evaluation.
  • The study reviewed data from 2017 to 2020, finding that patients with complex congenital heart disease and cardiomyopathy were more likely to experience these critical events.
  • A significant number (65%) of patients with identified critical events received treatment, and usage of event monitors rose by 52% in the study period.
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Background: Infants with complete heart block (CHB) require epicardial pacemaker (PM) insertion. Prior studies described epicardial pacing outcomes in infants and children, although they were limited by small or heterogeneous populations.

Objective: This study aimed to explore patient- and procedure-level associations with device complications in infants with CHB who received a permanent PM.

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Asteroids with diameters less than about 5 km have complex histories because they are small enough for radiative torques (that is, YORP, short for the Yarkovsky-O'Keefe-Radzievskii-Paddack effect) to be a notable factor in their evolution. (152830) Dinkinesh is a small asteroid orbiting the Sun near the inner edge of the main asteroid belt with a heliocentric semimajor axis of 2.19 AU; its S-type spectrum is typical of bodies in this part of the main belt.

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This article reviews various opportunities to translate established and novel tools and techniques used in adult electrophysiology to pediatrics and the adult congenital heart disease population. There is a specific focus on preoperative management of special population, implantation techniques, and postoperative programming of devices.

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Heart rate variability (HRV) is a noninvasive indicator of the health of neurocardiac interactions of the autonomic nervous system. In adults, decreased HRV correlates with increased cardiovascular mortality. However, the relationship between HRV and outcomes in children with acute decompensated heart failure (ADHF) has not been described.

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Dexmedetomidine, an alpha 2 agonist, has emerged as a desirable sedative agent in the pediatric intensive care unit due to its minimal effect on respiratory status and reduction in delirium. Bradycardia and hypotension are common side effects, however there are emerging reports of more serious cardiovascular events, including sinus arrest and asystole. These case reports have been attributed to high vagal tone or underlying cardiac conduction dysfunction.

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Background: 1p36 deletion syndrome can predispose to pediatric-onset cardiomyopathy. Deletion breakpoints are variable and may delete the transcription factor . Early studies suggest that deletion of may underlie cardiomyopathy in patients with 1p36 deletion; however, the prognostic impact of loss is unknown.

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Background: Transcatheter Leadless Pacemakers (TLP) are a safe and effective option for adults with pacing indications. These devices may be an alternative in pediatric patients and patients with congenital heart disease for whom repeated sternotomies, thoracotomies, or transvenous systems are unfavorable. However, exemption of children from clinical trials has created uncertainty over the indications, efficacy, and safety of TLP in the pediatric population.

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Background: Genetic defects in the RAS/mitogen-activated protein kinase pathway are an important cause of hypertrophic cardiomyopathy (RAS-HCM). Unlike primary HCM (P-HCM), the risk of sudden cardiac death (SCD) and long-term survival in RAS-HCM are poorly understood.

Objectives: The study's objective was to compare transplant-free survival, incidence of SCD, and implantable cardioverter-defibrillator (ICD) use between RAS-HCM and P-HCM patients.

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  • - TANGO2 deficiency disorder (TDD) is a rare genetic condition that leads to developmental delays, seizures, and serious metabolic and cardiac issues, and this study aimed to outline its natural history.
  • - The research gathered data from 73 patients across 16 countries, revealing that most children show normal early development but experience a decline in milestones and various symptoms like ataxia and dystonia starting from ages 1 to 3.
  • - Findings suggest that B-complex or multivitamin supplements can significantly reduce the occurrence of metabolic crises in these patients, offering a potential preventive measure.
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  • - Atrial standstill (AS) is a rare condition marked by lack of electrical activity in the heart's atria, and this study aims to explore its clinical features, genetic causes, and patient outcomes.
  • - The research involved 20 patients diagnosed with AS at an average age of 6.6 years, revealing a high prevalence of arrhythmias (80%) and significant cardiac events, including cardiac arrests in 4 patients.
  • - Genetic testing showed SCN5A variants in 65% of patients, indicating that these variants may contribute to AS; patients unable to pace their atria face increased risks for blood clots, emphasizing the need for anticoagulant treatment.
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Background: Hypertrophic cardiomyopathy (HCM) can be associated with an abnormal exercise response. In adults with HCM, abnormal results on exercise stress testing are predictive of heart failure outcomes. Our goal was to determine whether an abnormal exercise response is associated with adverse outcomes in pediatric patients with HCM.

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Background: Atrial arrhythmia's (AA) following lung transplant in adults are a well-described clinical finding. In pediatrics, however, there are limited data with some reports suggesting that arrhythmias are rare.

Methods: We performed a single-center retrospective review of lung transplant recipients from January 2013 to June 2020.

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Background: Data regarding recurrence risk among infants with supraventricular tachycardia (SVT) are limited.

Objectives: The purpose of this study was to determine incidence and factors associated with SVT recurrence.

Methods: This was a retrospective single-center study (1984-2020) with prospective phone follow-up of infants with structurally normal hearts diagnosed at age ≤1 year with re-entrant SVT.

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The 2019 novel coronavirus infectious disease (COVID-19) pandemic has resulted in an unsustainable need for diagnostic tests. Currently, molecular tests are the accepted standard for the detection of SARS-CoV-2. Mass spectrometry (MS) enhanced by machine learning (ML) has recently been postulated to serve as a rapid, high-throughput, and low-cost alternative to molecular methods.

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Background: TANGO2 deficiency disorder (TDD) is an autosomal recessive disease associated with metabolic crisis, lethal cardiac arrhythmias, and cardiomyopathy. Data regarding treatment, management, and outcomes of cardiac manifestations of TDD are lacking.

Objective: The purpose of this study was to describe TDD-related cardiac crises.

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Introduction: Ablation for atrioventricular nodal reentrant tachycardia (AVNRT) classically utilizes evaluation of signal morphology within the anatomic region of the slow pathway (SP), which involves subjectivity. Ripple mapping (RM; CARTO-3 Biosense Webster Inc) displays each electrogram at its three-dimensional coordinate as a bar changing in length according to its voltage-time relationship. This allows prolonged, low-amplitude signals to be displayed in their entirety, helping identify propagation in low-voltage areas.

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Background: As pediatric implantable cardioverter-defibrillator (ICD) utilization increases, hospital admission rates will increase. Data regarding hospitalizations among pediatric patients with ICDs are lacking. In addition, hospital mortality rates are unknown.

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