Increased Body Mass Index and Ventricular Cardiac Magnetic Resonance Characteristics in Adults With Fontan Circulation.

Background: In structurally normal hearts, increased body mass index (BMI) is associated with larger ventricular mass and volume, however, this association has yet to be described in adult Fontan patients.

Objectives: This study evaluates the relationship of increased BMI and ventricular characteristics by cardiac magnetic resonance imaging (CMR) in a multi-institutional adult Fontan population.

Methods: We conducted a multicenter, cross-sectional study using the Fontan Outcomes Registry using CMR Examinations.

Mid-Term Recovery of Right Ventricular Function and Improvement of Left Ventricular Function After Da Silva Cone Procedure for Ebstein Anomaly.

Background: The Da Silva Cone procedure for Ebstein anomaly has dramatically improved tricuspid valve competence and clinical outcomes. However, preoperative left ventricular (LV) dysfunction and immediate postoperative right ventricular (RV) systolic dysfunction are frequently observed. While excellent valve outcomes are well established, recovery of biventricular function following the Cone remains less defined.

Defining diastolic dysfunction post-Fontan: Threshold, risk factors, and associations with outcomes.

Background: Following the Fontan procedure, patients with single ventricle physiology are at high risk of diastolic dysfunction (DD) and elevated end-diastolic pressure (EDP).

Objective: This study aims to determine (1) the optimal EDP threshold correlated with adverse outcomes post-Fontan and (2) the clinical and imaging predictors of DD.

Methods: The study included patients from the Fontan Outcome Registry using CMR Examinations (FORCE) who underwent cardiac catheterization and cardiac magnetic resonance (CMR) within a 2-year window.

The association of the child opportunity index on outcomes in paediatric patients with Wolff-Parkinson-White.

Background: The Child Opportunity Index is an index of 29 indicators of social determinants of health linked to the United States of America Census. Disparities in the treatment of Wolff-Parkinson-White have not be reported. We hypothesise that lower Child Opportunity Index levels are associated with greater disease burden (antiarrhythmic use, ablation success, and Wolff-Parkinson-White recurrence) and ablation utilisation.

Differences in Exercise Performance in Fontan Patients with Extracardiac Conduit and Lateral Tunnel: A FORCE Fontan Registry Study.

To explore the differences in exercise capacity between the extracardiac conduit (ECC) and lateral tunnel (LT) Fontan. 2169 patients (36% LT ( = 774); 64% ECC ( = 1395)) underwent a Fontan operation between 2000 to 2023 in a multi-institutional Fontan registry. LT patients were age-matched to ECC patients, and cardiopulmonary exercise test (CPET) results were compared.

The long-term effect of the Fontan fenestration on clinical outcomes: A FORCE registry study.

Background: The long-term effect of open fenestration in Fontan patients is unclear, leading to wide practice variation of fenestration creation and closure. We evaluated the long-term outcomes of the fenestration using data from the Fontan Outcome Registry using Cardiac Magnetic Resonance Examinations (FORCE) Study.

Methods: Patients were categorized by fenestration status determined by post-Fontan cardiac magnetic resonance imaging (CMR) as open fenestration, nonfenestrated Fontan, spontaneous closure, and device closure.

Lower Child Opportunity Index is Associated with Lower Exercise Capacity Post-Fontan Palliation.

The Child Opportunity Index (COI) is a validated measurement that uses a composite index of 29 indicators of social determinants of health linked to the US Census. Patients post-Fontan palliation for single ventricle physiology often have reduced exercise capacity compared to the general population. Our hypothesis is that COI levels are directly associated with exercise capacity and inversely with late outcomes.

The cone repair allows right ventricle rehabilitation with excellent tricuspid valve function following the Starnes procedure.

Background: We present a case series of right ventricle (RV) rehabilitation after the Starnes procedure in patients with Ebstein anomaly (EA), applying the Cone repair of the tricuspid valve (TV) to achieve 2-ventricle or 1.5-ventricle physiology.

Methods: This is a retrospective database analysis from 2 institutions in North America.

Impact of guideline directed medical therapy on myocardial function in adults with congenital heart disease.

Background: Guideline-directed heart failure therapy with angiotensin receptor blocker/neprilysin inhibitor (ARNi) and sodium-glucose transporter inhibitors (SGLT2i) has been incrementally beneficial in improving outcomes in heart failure patients.

Objective: Evaluate the feasibility and efficacy of guideline-directed medical therapy (GDMT) in adults congenital heart disease (ACHD) patients.

Methods: In a retrospective cohort study, ACHD patients with either New York Heart Association (NYHA) Class II symptoms or systemic ejection fraction (EF) <45%, optimized on a combination of beta-blocker (BB), ARNi, mineralocorticoid receptor antagonist (MRA) and SGLT2i were evaluated.

From Other Journals: A Review of Recent Articles by Our Editorial Team.

In this review we provide a brief description of recently published articles addressing topics relevant to pediatric cardiologists. Our hope is to provide a summary of the latest articles published recently in other journals in our field. The articles address: 1- The use of AI in fetal echocardiography, 2- The role of Apixaban in thromboembolism prevention in pediatric congenital heart disease, 3- Cardiovascular events in childhood cancer survivors, and lastly 4- the new consensus statement on cardiac catheterization for pediatrics and adults with congenital heart disease.

Large Right Atrial Size on Cardiac MRI is Associated with Post-operative Right Ventricular Dysfunction After the Cone Operation for Ebstein Anomaly.

The cone operation has revolutionized care for patients with Ebstein anomaly; however, acute post-operative right ventricular dysfunction (RVD) is common in this patient population. A single-center, retrospective review of 28 patients with Ebstein anomaly who underwent cardiac MRI (CMR) prior to cone reconstruction of the tricuspid valve was conducted. Measurements of atrial and ventricular size/function were assessed.

Assessing Left Ventricular Pathology in Patients with Ebstein Anomaly Using Cardiovascular Magnetic Resonance: Looking Past the Right Heart.

Ebstein Anomaly (EA) is a malformation of the right heart, but there is data to suggest that the left ventricle (LV) can suffer from intrinsic structural and functional abnormalities which affect surgical outcomes. The LV in patients with EA is hypertrabeculated with abnormalities in LV function and strain. In this retrospective single-center study, patients with EA who underwent pre-operative cardiac MRI (CMR) between the periods of 2014-2024 were included along with a group of healthy-age-matched controls.

Augmented Biphasic Breathing Using Sniff and an Oral Positive Expiratory Pressure Device (Sniff-PEP) in Fontan Patients.

The use of an oral positive expiratory pressure device (oPEP) with sniff breathing (Sniff-PEP) mimics biphasic ventilation. Biphasic ventilation increases pulmonary blood flow and cardiac output in Fontan patients. The aim of this study was to assess the effect of Sniff-PEP on Fontan flow velocities.

Thrombocytopenia is Associated with Higher Fontan Pressure and Increased Morbidity in Patients with Fontan Circulation.

After the Fontan procedure, patients require lifelong follow-up due to significant late morbidity and mortality. Thrombocytopenia is seen frequently post-Fontan, likely due to secondary hypersplenism from elevated Fontan pressure. We investigated platelet counts in patients with a Fontan circulation and assessed associations with catheterization data and clinical outcomes.

T1 mapping: a non-invasive tool to assess the systemic right ventricle.

Ventricular remodeling leads to fibrotic changes in systemic right ventricles (RV). Native T1 mapping provides a quantitative measure in myocardial tissue characterization. The aim of our study was to correlate native T1 values of the systemic RV to function and volumetric data.

Prenatal Predictors for Pulmonary Balloon Valvuloplasty in the Newborn.

Pulmonary balloon valvuloplasty (PBV) is the treatment of choice for subjects with isolated pulmonary valve stenosis (IPS). The purpose of this study was to define fetal echocardiographic features associated with an inpatient PBV prior to newborn hospital discharge and characterize resource utilization of IPS fetuses among participating centers. Six center, retrospective case series of singleton fetuses identified between 2010 and 2020 with IPS.

Participation in Competitive Sports by Patients With Congenital Heart Disease: AHA/ACC and EAPC/ESC/AEPC Guidelines Comparison.

Sports participation in patients with congenital heart disease is an evolving subject. The American Heart Association/American College of Cardiology released a set of guidelines that advise the type and level of sports participation based primarily on anatomical defects with secondary consideration given to hemodynamic effects. Recently, the European Association of Preventive Cardiology/European Society of Cardiology/Association for European Paediatric and Congenital Cardiology offered a contrasting approach to sports participation that is based on hemodynamic and electrophysiological profiles of each patient, regardless of anatomical consideration.

From Other Journals: A Review of Recent Articles by Our Editorial Team.

In this review, we provide a brief description of recently published articles addressing topics relevant to pediatric cardiologists. Our hope is to provide a summary of the latest articles published recently in other journals in our field. The articles address (1) A new index for prenatal diagnosis of total anomalous pulmonary venous return, (2) Outcomes of patients with Tetralogy of Fallot after pulmonary valve replacement (PVR), and (3) Short-term outcomes of the self expanding Harmony valve for transcatheter PVR.

Persistent Patent Vertical Vein After Repair of Total Anomalous Pulmonary Venous Connection (TAPVR): A Rare Cause of Hypoxemia Post-Fontan Procedure.

Vertical vein (VV) ligation during total anomalous pulmonary venous return (TAPVR) repair is controversial. While some surgeons prefer ligation of the VV to prevent adverse sequelae of shunting across it and to promote flow through the newly created anastomosis, others leave it to serve as a "pop off valve" to the left heart structures, which are believed to be hypoplastic and noncompliant, presumably contributing to a more favorable post-operative outcome. We report two patients post-Fontan procedure, who underwent cardiac catheterization to explore the etiology of hypoxia and were found to have a persistent VV responsible for right to left shunting.

Right Atrial Enlargement on Electrocardiogram in Previously Healthy Young Patients.

Objectives: To assess whether right atrial enlargement (RAE) on electrocardiogram (ECG) correlates with true RAE on echocardiogram in previously healthy young patients and to understand which patients with RAE on ECG may warrant additional testing.

Study Design: A single-center, retrospective review of previously healthy young patients with (1) ECGs that were read as RAE by a pediatric cardiologist and (2) echocardiograms obtained within 90 days of the ECG. ECGs were reviewed to confirm RAE and determine which leads met criteria.