Activated fibroblasts drive cellular interactions in end-stage pediatric hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is a relatively rare but debilitating diagnosis in the pediatric population and patients with end-stage HCM require heart transplantation. In this study, we performed single-nucleus RNA sequencing on pediatric HCM and control myocardium. We identified distinct underling cellular processes in pediatric, end-stage HCM in cardiomyocytes, fibroblasts, endothelial cells, and myeloid cells, compared to controls.

Junctional Ectopic Tachycardia Caused by Junctophilin-2 Expression Silencing Is Selectively Sensitive to Ryanodine Receptor Blockade.

Junctional ectopic tachycardia (JET) is a potentially fatal cardiac arrhythmia. Hcn4:shJph2 mice serve as a model of nodal arrhythmias driven by ryanodine type 2 receptor (RyR2)-mediated Ca leak. EL20 is a small molecule that blocks RyR2 Ca leak.

: A Web-Based Precision Medicine Tool for Predicting Variant Pathogenicity in Cardiomyopathy- and Channelopathy-Associated Genes.

Background: With genetic testing advancements, the burden of incidentally identified cardiac disease-associated gene variants is rising. These variants may carry a risk of sudden cardiac death, highlighting the need for accurate diagnostic interpretation. We sought to identify pathogenic hotspots in sudden cardiac death-associated genes using amino acid-level signal-to-noise (S:N) analysis and develop a web-based precision medicine tool, , to improve variant evaluation.

Green Synthesis of TiO Nanoparticles Using Natural Marine Extracts for Antifouling Activity.

Titanium dioxide (TiO) nanoparticles were synthesized via a novel eco-friendly green chemistry approach using marine natural extracts of two red algae ( and ) a green alga (), and a brown alga () along with a marine sponge sample identified as . X-ray diffraction (XRD), scanning electron microscope (SEM), UV-Vis, X-ray spectroscopy (EDS), X-ray photoelectron spectroscopy (XPS), and Fourier-transform infrared spectroscopy (FTIR) were employed to characterize the crystal structure, surface morphology, and optical properties of the synthesized nanoparticles. Each of the as-synthesized marine extract based TiO nanoparticles was individually incorporated as an antifouling agent to form a newly fabricated marine paint formulation.

Assessment of parental decision making in congenital heart disease, cardiomyopathy and heart transplantation: an observational study analysing decisional characteristics and preferences.

Objective: We explore shared decision making (DM) in guardians of children with heart disease by assessing the desired weight of influence on DM and factors that may alter the relative weight of parent or medical team influence.

Methods: Guardians of patients <21 years and admitted >1 week in the paediatric cardiac intensive care unit (PCICU) were recruited. Twelve vignettes were designed including technical (antibiotic selection, intubation, peripherally inserted central catheter placement, ventricular assist device placement, heart transplant, organ rejection, heart rhythm abnormalities and resuscitation effort) and non-technical vignettes (cessation of life-sustaining therapies, depression treatment, obesity and palliative care referral).

Arrhythmogenic cardiomyopathy is under-recognized in end-stage pediatric heart failure: A 36-year single-center experience.

Background: Although ventricular failure is a late finding in adults with AC, we hypothesize that this is a presenting symptom in pediatric heart failure patients who undergo HT and that their ventricular arrhythmia burden could differentiate AC from other cardiomyopathies.

Methods: We performed a single-center retrospective cohort study reviewing 457 consecutive pediatric (≤18 years) HT recipients at our institution. Explanted hearts were examined to establish the primary diagnosis, based on pathologic findings.

Left ventricular non-compaction in paediatrics: a novel semi-automated imaging technique bridging imaging findings and clinical outcomes.

Aims: We set out to design a reliable, semi-automated, and quantitative imaging tool using cardiac magnetic resonance (CMR) imaging that captures LV trabeculations in relation to the morphologic endocardial and epicardial surface, or perimeter-derived ratios, and assess its diagnostic and prognostic utility.

Methods And Results: We queried our institutional database between January 2008 and December 2018. Non-compacted (NC)-to-compacted (C) (NC/C) myocardium ratios were calculated and our tool was used to calculate fractal dimension (FD), total mass ratio (TMR), and composite surface ratios (SRcomp).

Signal-to-Noise Analysis Can Inform the Likelihood That Incidentally Identified Variants in Sarcomeric Genes Are Associated with Pediatric Cardiomyopathy.

Background: Hypertrophic cardiomyopathy (HCM) is the most common heritable cardiomyopathy and can predispose individuals to sudden death. Most pediatric HCM patients host a known pathogenic variant in a sarcomeric gene. With the increase in exome sequencing (ES) in clinical settings, incidental variants in HCM-associated genes are being identified more frequently.

Balloon-Expandable Valve for Treatment of Evolut Valve Failure: Implications on Neoskirt Height and Leaflet Overhang.

Objectives: This study sought to determine the degree of Evolut (Medtronic) leaflet pinning, diameter expansion, leaflet overhang, and performance at different implant depths of the balloon-expandable Sapien 3 (S3, Edwards Lifesciences LLC) transcatheter heart valve (THV) within the Evolut THV.

Background: Preservation of coronary access and flow is a major factor when considering the treatment of failed Evolut THVs.

Methods: An in vitro study was performed with 20-, 23-, 26-, and 29-mm S3 THVs deployed within 23-, 26-, 29-, and 34-mm Evolut R THVs, respectively.

Risk factors associated with post-transplant superior caval vein stenosis in paediatric heart transplantation.

Superior caval vein stenosis is a known complication following paediatric heart transplantation. Herein, we sought to assess the incidence of superior caval vein stenosis and need for intervention in a single centre paediatric heart transplantation programme. A retrospective review was performed to identify variables associated with superior caval vein stenosis and need for intervention.

Family functioning in the families of paediatric heart transplant recipients prior to outpatient visits and procedures.

In a cross-sectional study, we assessed family functioning prior to outpatient visits and routine heart catheterisations in the families of paediatric heart transplant recipients. Caregivers rated higher short-term family functioning prior to outpatient visits, but not prior to catheterisations. This finding may indicate that family functioning benefits from the support provided during outpatient visits.

A comprehensive guide to genetic variants and post-translational modifications of cardiac troponin C.

Familial cardiomyopathy is an inherited disease that affects the structure and function of heart muscle and has an extreme range of phenotypes. Among the millions of affected individuals, patients with hypertrophic (HCM), dilated (DCM), or left ventricular non-compaction (LVNC) cardiomyopathy can experience morphologic changes of the heart which lead to sudden death in the most detrimental cases. TNNC1, the gene that codes for cardiac troponin C (cTnC), is a sarcomere gene associated with cardiomyopathies in which probands exhibit young age of presentation and high death, transplant or ventricular fibrillation events relative to TNNT2 and TNNI3 probands.

Family functioning in pediatric heart transplantation: Variables associated with poor outcomes.

Family functioning is integral in a child's life and is linked to quality of life in health as well as disease. This has been scarcely studied in pediatric orthotopic heart transplantation (pOHT). In this study, we evaluate demographic and clinical factors associated with family functioning in this patient population.

Postoperative vasoactive inotropic score is predictive of outcomes in pediatric heart transplantation.

Vasoactive inotrope score (VIS) is scarcely studied in pediatric orthotopic heart transplantation (pOHT). We conducted a retrospective review of pOHT (<21 years) recipients. Max VIS and mean VIS were calculated at 0-24 and 24-48 hours post-pOHT.

Meta-analysis of cardiomyopathy-associated variants in troponin genes identifies loci and intragenic hot spots that are associated with worse clinical outcomes.

Introduction: Troponin (TNN)-encoded cardiac troponins (Tn) are critical for sensing calcium and triggering myofilament contraction. TNN variants are associated with development of cardiomyopathy; however, recent advances in genetic analysis have identified rare population variants. It is unclear how certain variants are associated with disease while others are tolerated.

Sub-acute neonatal hemochromatosis in an infant with hypoplastic left heart syndrome on ventricular assist device awaiting transplantation.

Single-ventricle pediatric patients, amongst other children waiting for OHT, are a vulnerable population, especially if candidacy is established before any palliation. NH is a rare disease with poor prognosis in the post-natal period. We present a case of sub-acute NH diagnosed in an infant with HLHS who was listed for OHT while bridged with a pulsatile paracorporeal VAD, with an emphasis on the evolution of the condition throughout the patient's clinical course and the ultimate decision for compassionate deactivation of VAD.

Copy Number Variants of Undetermined Significance Are Not Associated with Worse Clinical Outcomes in Hypoplastic Left Heart Syndrome.

Objective: To determine the prevalence, spectrum, and prognostic significance of copy number variants of undetermined significance (cnVUS) seen on chromosomal microarray (CMA) in neonates with hypoplastic left heart syndrome (HLHS).

Study Design: Neonates with HLHS who presented to Texas Children's Hospital between June 2008 and December 2016 were identified. CMA results were abstracted and compared against copy number variations (CNVs) in ostensibly healthy individuals gathered from the literature.

Mining marine shell wastes for polyelectrolyte chitosan anti-biofoulants: Fabrication of high-performance economic and ecofriendly anti-biofouling coatings.

Banning organotins as antifouling biocides in 2003 was the starting point for many researchers to search for novel economic and environmentally-friendly anti-fouling biocides. In our present contribution, we have successfully functionalized a natural biopolymer, chitosan (CS), isolated from marine wastes with polyelectrolyte brushes akin to ionic liquids. These antifouling biopolymers anchoring polyelectrolyte brushes were in vitro assessed for their ability to eradicate or inhibit the Staphylococcal/Escherichia biofilms.

A Reminder of Methylene Blue's Effectiveness in Treating Vasoplegic Syndrome after On-Pump Cardiac Surgery.

The inflammatory response induced by cardiopulmonary bypass decreases vascular tone, which in turn can lead to vasoplegic syndrome. Indeed the hypotension consequent to on-pump cardiac surgery often necessitates vasopressor and intravenous fluid support. Methylene blue counteracts vasoplegic syndrome by inhibiting the formation of nitric oxide.

Crestal remodelling and osseointegration at surface-modified commercially pure titanium and titanium alloy implants in a canine model.

Background: Ti-6Al-7Nb alloys exhibit enhanced mechanical properties and corrosion resistance and may represent an improvement to present commercially pure (CP) titanium oral implant technology.

Objectives: To evaluate crestal remodelling and osseointegration at CP titanium compared with Ti-6Al-7Nb alloy oral implants using a canine model.

Methods: Two threaded anodized CP titanium and two Ti-6Al-7Nb alloy anodized oral implants (ø4.