Durable left ventricular assist devices in pediatrics: impact of body size on outcomes and size limitations.

Despite the range of body sizes in children, few ventricular assist devices (VAD) exist to support pediatric patients with end-stage heart failure. Large registry data identified weight < 20 kg to be associated with higher rates of VAD-related stroke, compared to > 40 kg. Moreover, patients < 1 years of age experience the highest post-implant mortality, with 1-year survival improving in an age-dependent manner.

Impact of Hemoglobin Level in Ex Vivo Heart Perfusion on Donation After Circulatory Death Hearts: A Juvenile Porcine Experimental Model.

Background: Ex vivo heart perfusion (EVHP) of donation after circulatory death (DCD) hearts has become an effective strategy in adults; however, the small circulating volume in pediatrics poses the challenge of a low-hemoglobin (Hb) perfusate. We aimed to determine the impact of perfusate Hb levels during EVHP on DCD hearts using a juvenile porcine model.

Methods: Sixteen DCD piglet hearts (11-14 kg) were reperfused for 4 h in unloaded mode followed by working mode.

Outcomes of Children With Hypoplastic Left Heart Syndrome and Heart Failure on Medical Therapy.

Background: Systemic right ventricle (RV) dysfunction is associated with lower transplant-free survival (TFS) in hypoplastic left heart syndrome (HLHS), but the likelihood of functional improvement and utility of heart failure (HF) medications is not understood.

Objectives: The authors aimed to describe TFS, HF medication use, and surgical interventions in HLHS patients with RV dysfunction with and without subsequent improvement in function.

Methods: The SickKids HF Database is a retrospective cohort that includes all pediatric HLHS patients with RV dysfunction lasting >30 days.

Nutritional status and cannula infections in pediatric patients on ventricular assist device support.

Background: Ventricular assist devices (VADs) are used to bridge pediatric patients to heart transplantation. Paracorporeal VADs require the placement of cannulas, which can create an environment for infections. We examined cannula infections in pediatric VAD patients and the role of nutritional status.

Association of left ventricular outflow tract size with arch morphology in interrupted aortic arch.

Objectives: Left ventricular outflow tract obstruction (LVOTO) is a major cause of morbidity and mortality in infants with interrupted aortic arch (IAA). Left Ventricular Outflow Tract (LVOT) development may be flow-mediated, thus IAA morphology may influence LVOT diameter and subsequent reintervention. We investigated the association of IAA morphology [type and presence of aortic arch aberrancy (AAb)] with LVOT diameter and reintervention.

A modified intraventricular balloon method for functional assessment of hearts from donation after circulatory death.

Objective: Functional assessment of hearts during ex-vivo heart perfusion is not well-established. Conventional intraventricular balloon methods for large animals sacrifice the mitral valve. This study assessed the effectiveness of the modified intraventricular balloon method in comparison with other modalities used during working mode in juvenile pigs.

Longitudinal Evaluation of Congenital Cardiovascular Surgical Performance and Skills Retention Using Silicone-Molded Heart Models.

Hands-on surgical training (HOST) for congenital heart surgery (CHS), utilizing silicone-molded models created from 3D-printing of patients' imaging data, was shown to improve surgical skills. However, the impact of repetition and frequency of repetition in retaining skills has not been previously investigated. We aimed to longitudinally evaluate the outcome for HOST on two example procedures of different technical difficulties with repeated attempts over a 15-week period.

Early outcomes of moderate-to-high-risk pediatric congenital cardiac surgery and predictors of extracorporeal circulatory life support requirement.

Background: Cardiopulmonary failure refractory to medical management after moderate-to-high-risk congenital cardiac surgery may necessitate mechanical support with veno-arterial extracorporeal membrane oxygenation (ECMO). On the extreme, ECMO can also be initiated in the setting of cardiac arrest (extracorporeal cardiopulmonary resuscitation, ECPR) unresponsive to conventional resuscitative measures.

Methods: This was a single-center retrospective cohort study of patients ( = 510) aged <3 years old who underwent cardiac surgery with cardiopulmonary bypass with a RACHS-1 score ≥3 between 2011 and 2014.

A Pediatric Primary Cardiac Spindle Cell Neoplasm With a Rare PDGFRA::USP8 Gene Fusion: A Case Report.

We report a case of a primary cardiac spindle cell neoplasm with concerning histological features and a rare gene fusion in a 3 year old boy. The patient presented with a large cardiac mass predominantly in the right ventricle, originating from the ventricular septum. The mass was resected with grossly negative margins.

Coronary artery lesions are associated with adverse cardiac events in children undergoing supravalvular aortic stenosis repair.

Objectives: The aim of this study was to identify the prevalence and anatomic characteristics of coronary artery lesions and their associated postoperative risk in patients undergoing supravalvular aortic stenosis repair.

Methods: The association between structural risk factors, postoperative ST-segment changes, and major adverse cardiac events was explored using logistic regression and the Fisher's exact test.

Results: In 51 consecutive patients with supravalvular aortic stenosis treated between 2000 and 2017, a total of 48 coronary lesions were identified in 27 patients (53%).

Outcomes After Hybrid Palliation for Infants With Critical Left Heart Obstruction.

Background: Hybrid palliation (bilateral pulmonary artery banding with or without ductal stenting) is an initial management strategy for infants with critical left heart obstruction and serves as palliation until subsequent operations are pursued.

Objectives: This study sought to determine patient characteristics and factors associated with subsequent outcomes for infants who underwent hybrid palliation.

Methods: From 2005 to 2019, 214 of 1,236 prospectively enrolled infants within the Congenital Heart Surgeons' Society's critical left heart obstruction cohort underwent hybrid palliation across 24 institutions.

Mechanical atrioventricular valve replacement in patients with single ventricle palliation.

Objectives: Atrioventricular valve (AVV) replacements in patients with single-ventricle circulations pose significant surgical risks and are associated with high morbidity and mortality.

Methods: From 1997 to 2021, 16 consecutive patients with functionally single-ventricle physiology underwent mechanical AVV replacement. Primary outcome was transplant-free survival.

Left atrial appendage aneurysm in pediatrics: Case study and literature review.

Left atrial appendage aneurysm (LAAA) is a rare cardiac pathology that is often identified in adulthood. There are a myriad of presentations related to atrial appendage enlargement, but most are asymptomatic. Pediatric cases of LAAA are extremely rare.

Epinephrine Dosing Use During Extracorporeal Cardiopulmonary Resuscitation: Single-Center Retrospective Cohort.

Objectives: During pediatric cardiac arrest, contemporary guidelines recommend dosing epinephrine at regular intervals, including in patients requiring extracorporeal membrane oxygenation (ECMO). The impact of epinephrine-induced vasoconstriction on systemic afterload and venoarterial ECMO support is not well-defined.

Design: Nested retrospective observational study within a single center.

Heart transplantation in neonatal Marfan syndrome: Saving life in a rare and fatal condition.

Background: Neonatal Marfan syndrome is a rare disease with mortality in the first year of life reported as high as 95% predominantly due to progressive heart failure from valvar regurgitation and cardiomyopathy. Multisystem involvement and uncertain prognosis have historically precluded transplant candidacy, and current management options are of limited success.

Case Report: We present a baby girl with a postnatal diagnosis of neonatal Marfan syndrome who at 1 year of age underwent mitral valve and tricuspid valve repair with postoperative profound left ventricular and moderate right ventricular dysfunction necessitating biventricular assist device (BiVAD) support and subsequent heart transplant.

Hybrid palliation versus nonhybrid management for a multi-institutional cohort of infants with critical left heart obstruction.

Objective: To compare patient characteristics and overall survival for infants with critical left heart obstruction after hybrid palliation (bilateral pulmonary artery banding with or without ductal stenting) versus nonhybrid management (eg, Norwood, primary transplantation, biventricular repair, or transcatheter/surgical aortic valvotomy).

Methods: From 2005 to 2019, 1045 infants in the Congenital Heart Surgeons' Society critical left heart obstruction cohort underwent interventions across 28 institutions. Using a balancing score propensity analysis, 214 infants who underwent hybrid palliation and 831 infants who underwent nonhybrid management were proportionately matched regarding variables significantly associated with mortality and variables noted to significantly differ between groups.