Mitochondrial transplantation normalizes transcriptomic and proteomic shift associated with ischemia reperfusion injury in neonatal hearts donated after circulatory death.

Heart transplantation remains the ultimate treatment strategy for neonates and children with medically refractory end-stage heart failure and utilization of donors after circulatory death (DCD) can expand th donor pool. We have previously shown that mitochondrial transplantation preserves myocardial function and viability in neonatal swine DCD hearts to levels similar to that observed in donation after brain death (DBD). Herein, we sought to investigate the transcriptomic and proteomic pathways implicated in these phenotypic changes using ex situ perfused swine hearts.

Cross-species analyses of thymic mimetic cells reveal evolutionarily ancient origins and both conserved and species-specific elements.

Thymic mimetic cells are molecular hybrids between medullary-thymic-epithelial cells (mTECs) and diverse peripheral cell types. They are involved in eliminating autoreactive T cells and can perform supplementary functions reflective of their peripheral-cell counterparts. Current knowledge about mimetic cells derives largely from mouse models.

Challenges in the Development and Evaluation of Pediatric Heart Valve Technologies.

Background: In October 2022, the Heart Valve Collaboratory and Food and Drug Administration convened a global multidisciplinary workshop to address the unmet clinical need to promote and accelerate the development of pediatric-specific heart valve technologies.

Methods: The Pediatric Heart Valve Global Multidisciplinary Workshop was convened in October 2022. Key stakeholders, including expert clinicians in pediatric cardiology and cardiac surgery, valve manufacturers, engineers and scientists were assembled to review the current state-of-the-art, discuss unique challenges in the pre-and post-market evaluation of pediatric valve therapies, and highlight emerging technologies that show potential to address some of the key unmet needs of children with valve disease.

Ventricular septation for double inlet ventricle: Avoiding conduction injury.

Objectives: Although conduction location can be reliably predicted in double inlet ventricle, ventricular septation continues to carry a significant risk of complete heart block. This study describes our experience using intraoperative conduction mapping during ventricular septation.

Methods: Patients undergoing ventricular septation from 2017 to 2023 were identified.

Impact of permanent pacemaker for iatrogenic atrioventricular block on outcomes after congenital heart surgery.

Objective: The need for permanent pacemaker (PPM) for iatrogenic atrioventricular block (AVB) after congenital heart surgery is approximately 1%. We aimed to evaluate the long-term outcomes of patients with PPM for iatrogenic AVB and compare them with patients with an optimal repair (trivial/no residual) Residual Lesion Score (RLS) Class 1 repair without PPM need.

Methods: We reviewed 183 patients discharged with PPM for iatrogenic AVB from 2011 to 2022.

Multiphysiologic State Computational Fluid Dynamics Modeling for Planning Fontan With Interrupted Inferior Vena Cava.

Background: Single ventricle (SV) patients with interrupted inferior vena cava (iIVC) and azygos continuation are at high risk for unbalanced hepatic venous flow (HVF) distribution to the lungs after Fontan completion and subsequent pulmonary arteriovenous malformations (AVMs) formation.

Objectives: The aim of the study was to utilize computational fluid dynamics (CFD) analysis to avoid maldistribution of HVF to the lungs after Fontan surgery.

Methods: Four SV subjects with iIVC were prospectively studied with a 3-dimensional (3D) modeling workflow with digital 3D models created from segmented magnetic resonance images or computer tomography scans, virtual surgery, and CFD analysis over multiple physiologic states for the evaluation of operative plans to achieve balanced HVF to both lungs.

Clinical and 2D/3D-Echo Cardiography Determinants of Mitral Valve Reoperation in Children With Congenital Mitral Valve Disease.

Background: Congenital mitral valve disease (CMVD) presents major challenges in its medical and surgical management.

Objectives: The purpose of this study was to investigate the value of 3-dimensional echocardiography (3DE) and identify associations with MV reoperation in this setting.

Methods: All children <18 years of age who underwent MV reconstruction for CMVD in 2002 to 2018 were included.

Surgical debulking of large ventricular fibromas in children.

Objective: This study aims to provide an update on the clinical presentation, diagnostic workup, operative strategies, and midterm outcomes in children undergoing ventricular fibroma resection.

Methods: Single-center, retrospective cohort study of patients undergoing ventricular fibroma resection between 2000 and 2023.

Results: Among 52 patients, median age at surgery was 2.

Biomechanical Analysis of Age-Dependent Changes in Fontan Power Loss.

The hemodynamics in Fontan patients with single ventricles rely on favorable flow and energetics, especially in the absence of a subpulmonary ventricle. Age-related changes in energetics for extracardiac and lateral tunnel Fontan procedures are not well understood. Vorticity (VOR) and viscous dissipation rate (VDR) are two descriptors that can provide insights into flow dynamics and dissipative areas in Fontan pathways, potentially contributing to power loss.

Intervention for critical aortic stenosis in Hutchinson-Gilford progeria syndrome.

Hutchinson-Gilford Progeria Syndrome (HGPS) is an ultra-rare genetic premature aging disease that is historically fatal in teenage years, secondary to severe accelerated atherosclerosis. The only approved treatment is the farnesyltransferase inhibitor lonafarnib, which improves vascular structure and function, extending average untreated lifespan of 14.5 years by 4.

Early and long-term outcomes following cardiac surgery for patients with heterotaxy syndrome.

Objective: Heterotaxy syndrome is a complex multisystem abnormality historically associated with high morbidity and mortality. We sought to evaluate the early and long-term outcomes after cardiac surgery in heterotaxy syndrome.

Methods: This is a single-center retrospective review of patients with heterotaxy syndrome undergoing single-ventricle palliation or primary or staged biventricular repair from 1998 to 2018.

Impact of Age-Related Change in Caval Flow Ratio on Hepatic Flow Distribution in the Fontan Circulation.

Background: The Fontan operation is a palliative technique for patients born with single ventricle heart disease. The superior vena cava (SVC), inferior vena cava (IVC), and hepatic veins are connected to the pulmonary arteries in a total cavopulmonary connection by an extracardiac conduit or a lateral tunnel connection. A balanced hepatic flow distribution (HFD) to both lungs is essential to prevent pulmonary arteriovenous malformations and cyanosis.

Erector spinae plane blocks for opioid-sparing multimodal pain management after pediatric cardiac surgery.

Objective: Peripheral regional anesthesia is proposed to enhance recovery. We sought to evaluate the efficacy of bilateral continuous erector spinae plane blocks (B-ESpB) for postoperative analgesia and the impact on recovery in children undergoing cardiac surgery.

Methods: Patients aged 2 through 17 years undergoing cardiac surgery in the enhanced recovery after cardiac surgery program were prospectively enrolled to receive B-ESpB at the end of the procedure, with continuous infusions via catheters postoperatively.

Association of tetralogy of Fallot and complete atrioventricular canal: a single-centre 40-year experience.

Objectives: Outcome data in tetralogy of Fallot (ToF) and complete atrioventricular canal (CAVC) are limited. We report our experience for over 40 years in this patient population.

Methods: Single-centre, retrospective analysis of patients who underwent surgical repair with the diagnosis of ToF-CAVC from 1979 to 2022, divided into 2 different periods and compared.