Exercise as therapy for neurodevelopmental and cognitive dysfunction in people with a Fontan circulation: A narrative review.

People with a Fontan circulation are at risk of neurodevelopmental delay and disability, and cognitive dysfunction, that has significant implications for academic and occupational attainment, psychosocial functioning, and overall quality of life. Interventions for improving these outcomes are lacking. This review article discusses current intervention practices and explores the evidence supporting exercise as a potential intervention for improving cognitive functioning in people living with a Fontan circulation.

Speckle Tracking Analysis in Fetuses with D-Transposition: Predicting the Need for Urgent Neonatal Balloon Atrial Septostomy.

Introduction: Speckle tracking analysis of the endocardium of the right (RV) and left (LV) ventricles was used to evaluate the size, shape, and contractility of these chambers in fetuses with D-Transposition of the great arteries (D-TGA) to identify fetuses that would require emergent balloon atrial septostomy (BAS) after birth.

Methods: This was a retrospective analysis of fetuses with D-TGA and intact ventricular septum that were divided into 2 groups. Group 1 underwent urgent BAS after birth because of a restrictive atrial septum and group 2 did not.

Defining Expectations for Infants With Hypoplastic Left Heart Syndrome Who Survive Initial Surgical Palliation.

Background: Overall one-year non-mortality outcomes for surgically palliated hypoplastic left heart syndrome (HLHS) patients remain understudied. Using the metric Days Alive and Outside of Hospital (DAOH), the present study sought to characterize expectations for surgically palliated patients' first year of life.

Methods: The Pediatric Health Information System database was used to identify by code all HLHS patients who underwent surgical palliation (Norwood/hybrid and/or heart transplantation [HTx]) during their index neonatal admission and were successfully discharged alive (n  =  2227) and for whom one-year DAOH could be calculated.

Comparison of Invasive Pancreatic Ductal Adenocarcinoma versus Intraductal Papillary Mucinous Neoplasm: A National Cancer Database Analysis.

Background: Current evidence on overall survival (OS) between invasive pancreatic ductal adenocarcinoma (PDAC) and intraductal papillary mucinous neoplasm (IPMN) is limited to single-center reports. We aimed to compare the characteristics, management, and OS of invasive PDAC vs. IPMN using a national United States (US) database.

Patient-Reported Outcomes to Describe Global Health and Family Relationships in Pediatric Weight Management.

Patient-reported outcomes (PROs) can assess chronic health. The study aims were to pilot a survey through the PEDSnet Healthy Weight Network (HWN), collecting PROs in tertiary care pediatric weight management programs (PWMP) in the United States, and demonstrate that a 50% enrollment rate was feasible; describe PROs in this population; and explore the relationship between child/family characteristics and PROs. Participants included 12- to 18-year-old patients and parents of 5- to 18-year-olds receiving care at PWMP in eight HWN sites.

Contemporary Outcomes of Heart Transplantation in Children with Heterotaxy Syndrome: Sub-Optimal Pre-Transplant Optimization Translates into Early Post-Transplant Mortality.

Patients with heterotaxy syndrome and congenital heart disease (CHD) experience inferior cardiac surgical outcomes. Heart transplantation outcomes are understudied, however, particularly compared to non-CHD patients. Data from UNOS and PHIS were used to identify 4803 children (< 18 years) undergoing first-time heart transplant between 2003 and 2022 with diagnoses of heterotaxy (n = 278), other-CHD (n = 2236), and non-CHD cardiomyopathy (n = 2289).

Seasonal variability in body mass index change among children enrolled in the Pediatric Obesity Weight Evaluation Registry: A step in the right direction.

Background: Research has shown children disproportionately gain excess weight over the summer months (vs. school months), with stronger effects for children with obesity. However, the question has not been investigated among children receiving care in paediatric weight management (PWM) programs.

Aortic root rotational position associates with aortic valvar incompetence and aortic dilation after arterial switch operation for transposition of the great arteries.

Purpose: Aortic dilation and valvar regurgitation can develop in transposition of the great arteries (TGA) after the arterial switch operation (ASO). Variation in aortic root rotational position affects flow dynamics in patients without congenital heart disease. The aim of this study was to assess neo-aortic root (neo-AoR) rotational position and its association with neo-AoR dilation, ascending aorta (AAo) dilation, and neo-aortic valvar regurgitation in TGA following ASO.

Novel Application of Large Bore Aspiration in the Treatment of COVID-19 Associated Acute Limb Ischemia Using the Penumbra CAT 12 Lightning Aspiration System.

COVID-19 associated cardiovascular complications have been well documented throughout the pandemic and are associated with higher rates of morbidity and mortality. Hypercoagulable states resulting from systemic inflammation have been associated with increasing incidences of acute myocardial infarctions (AMI) and acute limb ischemia (ALI). Herein, we present a case of AMI complicated by ALI in a patient with COVID-19 in which aspiration thrombectomy using the Penumbra CAT 12 thrombectomy catheter was used in the management of acute popliteal artery occlusion.

Improvement in Multidisciplinary Provider Rounding (Surgical Rounds) in the Pediatric Cardiac ICU: An Application of Lean Methodology.

Objectives: Provider-only, combined surgical, and medical multidisciplinary rounds ("surgical rounds") are essential to achieve optimal outcomes in large pediatric cardiac ICUs. Lean methodology was applied with the aims of identifying areas of waste and nonvalue-added work within the surgical rounds process. Thereby, the goals were to improve rounding efficiency and reduce rounding duration while not sacrificing critical patient care discussion nor delaying bedside rounds or surgical start times.

Evidence-Based Assessment of Congenital Heart Disease Genes to Enable Returning Results in a Genomic Study.

Background: Congenital heart disease (CHD) is the most common major congenital anomaly and causes significant morbidity and mortality. Epidemiologic evidence supports a role of genetics in the development of CHD. Genetic diagnoses can inform prognosis and clinical management.

Pulmonary artery compliance in different forms of pulmonary hypertension.

Objective: Pulmonary artery compliance (PAC), estimated as stroke volume (SV) divided by pulmonary artery pulse pressure (PP), may be a predictor of survival in pulmonary arterial hypertension (PAH). Resistance-compliance (RC) time, the product of PAC and pulmonary vascular resistance, is reported to be a physiological constant. We investigated if differences in PAC and RC time exist between pulmonary hypertension (PH) subgroups and examined whether PAC is an independent predictor of transplant-free survival in PAH.

1-year outcomes of patients implanted with the Perceval sutureless valve: the Japanese post-marketing surveillance study.

Sutureless offers an alternative to standard valves in surgical aortic valve replacement (SAVR). We sought to confirm the efficacy and safety of the Perceval sutureless valve in Japanese patients. Prospective observational study of 204 patients who underwent SAVR with Perceval at 19 sites in Japan between March and December 2019.

Long-term exercise and pulmonary function outcomes in a contemporary cohort of children with congenital diaphragmatic hernia.

Objective: Congenital diaphragmatic hernia (CDH) survivors are at risk for long-term exercise impairment and pulmonary morbidity, but the generalizability of prior reported cohorts are limited by reduced disease severity and older surgical eras. We assessed the mid-childhood exercise and pulmonary function outcomes in a contemporary cohort of CDH survivors.

Study Design: In this retrospective cohort study, we identified 36 consecutive pediatric CDH survivors who underwent cardiopulmonary exercise testing (CPET) and spirometry from 2014 to 2021.

Staged vs Complete Repair in Tetralogy of Fallot With Pulmonary Atresia.

Background: We sought to compare outcomes for infants with tetralogy of Fallot with pulmonary atresia (TOF/PA) and confluent pulmonary arteries who underwent staged or primary complete surgical repair.

Methods: This retrospective study included infants undergoing initial surgical intervention between 0 and 60 days of age with TOF/PA without aortopulmonary collaterals from 2009 to 2018 at 20 centers. The primary outcome was days alive and out of the hospital in the first year of life (DAOH365).

Absolute Versus Relative Near-Infrared Spectroscopy in Pediatric Cardiac Patients.

Objectives: Near-infrared spectroscopy (NIRS) has been increasingly accepted as a noninvasive marker of regional tissue oxygenation despite concerns of imprecision and wide limits of agreement (LOA) with invasive oximetry. New generation absolute monitors may have improved accuracy compared with trend monitors. We sought to compare the concordance with invasive venous oximetry of a new generation absolute NIRS-oximeter (FORESIGHT ELITE; CASMED, Branford, CT) with a modern widely used trend monitor (INVOS 5100C; Medtronic, Minneapolis, MN).

Cardiovascular Complications of Down Syndrome: Scoping Review and Expert Consensus.

Cardiovascular disease is a leading cause of morbidity and mortality in individuals with Down syndrome. Congenital heart disease is the most common cardiovascular condition in this group, present in up to 50% of people with Down syndrome and contributing to poor outcomes. Additional factors contributing to cardiovascular outcomes include pulmonary hypertension; coexistent pulmonary, endocrine, and metabolic diseases; and risk factors for atherosclerotic disease.

Racial disparity exists in the utilization and post-transplant survival benefit of ventricular assist device support in children.

Purpose: Children of minority race and ethnicity experience inferior outcomes postheart transplantation (HTx). Studies have associated ventricular assist device (VAD) bridge-to-transplant (BTT) with similar-to-superior post-transplant-survival (PTS) compared to no mechanical circulatory support. It is unclear whether racial and ethnic discrepancies exist in VAD utilization and outcomes.

Dwarf Open Reading Frame (DWORF) Gene Therapy Ameliorated Duchenne Muscular Dystrophy Cardiomyopathy in Aged mdx Mice.

Background Cardiomyopathy is a leading health threat in Duchenne muscular dystrophy (DMD). Cytosolic calcium upregulation is implicated in DMD cardiomyopathy. Calcium is primarily removed from the cytosol by the sarcoendoplasmic reticulum calcium ATPase (SERCA).

Therapeutic CCR2 Blockade Prevents Inflammation and Alleviates Myxomatous Valve Disease in Marfan Syndrome.

Myxomatous valve disease (MVD) can lead to cardiac dysfunction and heart failure, yet medical therapies are lacking. C-C chemokine receptor type 2 (CCR2)+ immune cell infiltration promotes mitral valve inflammation in a Marfan syndrome (MFS) mouse model. The CCR2 genetic knockout reduces inflammation with downregulated proteases and improved extracellular matrix integrity.

Reasons for disagreement between screening and standard echocardiography in primary care: data from the PROVAR + study : Disagreement between screening and standard echo.

We aimed to evaluate the reasons for disagreement between screening echocardiography (echo), acquired by nonexperts, and standard echo in the Brazilian primary care (PC). Over 20 months, 22 PC workers were trained on simplified handheld (GE VSCAN) echo protocols. Screening groups, consisting of patients aged 17-20, 35-40 and 60-65 years, and patients referred for clinical indications underwent focused echo.