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.

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.

Anatomic and non-anatomic substrates in infants with two ventricles undergoing aortic arch repair.

Objectives: We sought to examine the relative importance of surgical lesion complexity versus the presence of genetic/syndromic/extracardiac anomalies (GSAs) in determining survival, morbidity or need for reinterventions following repair for aortic arch hypoplasia.

Methods: A single-centre, retrospective cohort study of infants undergoing biventricular aortic arch repair sternotomy from 2010 to 2021 was conducted. Survival analysis was performed using Kaplan-Meier methods, with additional Bayesian survival modelling for subgroups.

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.

Long-term Outcome After Repair of Transposition of the Great Arteries With Aortic Arch Obstruction.

Background: This study compares the long-term outcomes of patients after repair of transposition of the great arteries (TGA) with and without aortic arch obstruction (AAO).

Methods: This is a single-institution, retrospective study between October 2004 and February 2023. Patients who underwent arterial switch operation and aortic arch repair (ASO-AAR group) with patch augmentation were compared with those without AAO (ASO group).

Using DaSilva Cone Operation to Establish 1.5 or 2 Ventricle Circulation After Initial Single Ventricle Palliation with Starnes Procedure.

Ebstein anomaly (EA) is a congenital dysplasia of the tricuspid valve resulting in reduced right ventricular (RV) volume and tricuspid regurgitation. Severe EA in the neonatal period is associated with high mortality. The Starnes procedure (fenestrated RV exclusion) is reserved for EA patients with cardiogenic shock and has previously committed patients to single ventricle (SV) palliation.

Optimizing Surgical Selection for Transposition With Left Ventricular Outflow Tract Obstruction.

Background: Studies that have assessed the Rastelli and Nikaidoh operations for transposition of the great arteries (TGA) with obstructed left ventricular outflow tract obstruction (LVOTO) have not fully evaluated the anatomic drivers that may contribute to surgical selection. We present our procedural selection process for optimizing outcomes of complex TGA in the modern era.

Methods: This is a single-center, retrospective study that included pediatric patients who underwent either a Nikaidoh or Rastelli operation for the treatment of TGA-LVOTO, congenitally corrected TGA-LVOTO, or double-outlet right ventricle TGA type-LVOTO from June 2004 to June 2021.

Pulmonary Homograft vs Handmade Polytetrafluoroethylene-Valved Conduits After the Ross Procedure.

Background: This study aimed to longitudinally compare expanded polytetrafluoroethylene (ePTFE)-valved conduits vs pulmonary homograft (PH) conduits after right ventricular outflow tract reconstruction in the Ross procedure.

Methods: Patients undergoing a Ross procedure from June 2004 to December 2021 were identified. Echocardiographic data, catheter-based interventions, or conduit replacements, as well as time to first reintervention or replacement, were comparatively assessed between handmade ePTFE-valved conduits and PH conduits.

Anatomic Position and Durability of Polytetrafluoroethylene Conduit ≥18 mm: Single-Center Experience.

Background: Conduit longevity after right ventricular outflow tract (RVOT) reconstruction is determined by the interaction of different factors. We evaluated the relationship between conduit anatomic position and long-term durability among ≥18 mm polytetrafluoroethylene (PTFE) conduits.

Methods: A single-institution RVOT reconstructions using a PTFE conduit ≥18 mm were identified.

Congenital Heart Surgery Training Experiences and Perceptions Among Cardiothoracic Surgery Residents.

Interest and core training in congenital heart surgery (CHS) has not been characterized among current cardiothoracic surgical trainees. This study aimed to evaluate perceptions, interest, exposure, and experience among current trainees. A 22 question survey was distributed to all cardiothoracic surgical trainees in ACGME-accredited thoracic surgery residencies.

A High-Fidelity Percutaneous Model Used to Demonstrate ECMO Cannulation.

Medical simulation provides a realistic environment for practitioners to experience a planned clinical event in a controlled educational setting. We established a simulation model composed of synthetic ballistic gelatin that provided an inexpensive high-fidelity model for our extracorporeal membrane oxygenation (ECMO) team members to develop, master, and maintain clinical skills necessary for percutaneous cervical or femoral cannulation. The simulation component includes a cervical torso or femoral percutaneous synthetic gelatin model that is attached to either a static fluid model or to the high-fidelity perfusion simulator.

High Risk Status for Stage I Palliation Increases Mortality After Stage II But Not Stage III.

Background: High risk (HR) factors have been shown to have increased rates of mortality after stage 1 palliation (S1P) for single ventricle physiology. It remains unclear how initial HR status affects longitudinal outcomes after subsequent stage 2 palliation (S2P) and stage 3 palliation (S3P).

Methods: Single ventricle patients undergoing S1P between July 2004 and October 2018 at a single institution were included.

Design and rationale of a clinical trial to increase cardiomyocyte division in infants with tetralogy of Fallot.

Background: Patients with Tetralogy of Fallot with pulmonary stenosis (ToF/PS), the most common form of cyanotic congenital heart disease (CHD), develop adverse right ventricular (RV) remodeling, leading to late heart failure and arrhythmia. We recently demonstrated that overactive β-adrenergic receptor signaling inhibits cardiomyocyte division in ToF/PS infants, providing a conceptual basis for the hypothesis that treatment with the β-adrenergic receptor blocker, propranolol, early in life would increase cardiomyocyte division. No data are available in ToF/PS infants on the efficacy of propranolol as a possible novel therapeutic option to increase cardiomyocyte division and potentially reduce adverse RV remodeling.

Utilization and Outcomes of the Nikaidoh, Rastelli, and REV Procedures: An Analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database.

Background: Aortic root translocation (Nikaidoh), Rastelli, and réparation à l'etage ventriculaire (REV) are repair options for transposition of the great arteries (TGA) with ventricular septal defects and left ventricular outflow tract obstruction (VSD-LVOTO) or double outlet right ventricle TGA type (DORV-TGA).

Methods: This retrospective study using The Society of Thoracic Surgeons Congenital Heart Surgery Database evaluates surgical procedure utilization and outcomes of patients undergoing repair of TGA-VSD-LVOTO and DORV-TGA with a Nikaidoh, Rastelli, or REV procedure.

Results: A total of 293 patients underwent repair at 82 centers (January 2010 to June 2019).

Polytetrafluoroethylene Tricuspid Valved Conduit for Right Ventricular Outflow Tract Reconstruction.

The ideal conduit for reconstruction of the right ventricular outflow tract in pediatric patients remains a topic of discussion. We present a technique for construction of a handmade tricuspid valved polytetrafluoroethylene conduit for use in patients of all ages requiring right ventricular outflow tract reconstruction at the time of congenital cardiac surgery. This conduit provides an economically advantageous and readily available option globally when compared with homograft, without sacrificing surgical results.

A Risk Score for Adults With Congenital Heart Disease Undergoing Heart Transplantation.

Background: This study derived and validated a risk score for 1-year mortality in patients with adult congenital heart disease (ACHD) undergoing orthotopic heart transplantation (OHT).

Methods: The United Network for Organ Sharing registry identified patients with ACHD (≥18 years of age) who underwent OHT between 1987 and 2018. The primary outcome was 1-year mortality.

Bidirectional Glenn Procedure in Patients Less Than 3 Months of Age: A 14-Year Experience.

Background: Contradictory data exist regarding timing of stage 2 palliation (S2P). Prolonged interstage hospitalizations and home surveillance programs have contributed to a more rapid progression to S2P. Our goal is to describe the S2P population and explore the relationships of clinical outcomes and S2P timing at our institution over the last 14 years.

Early experience with the HeartMate 3 continuous-flow ventricular assist device in pediatric patients and patients with congenital heart disease: A multicenter registry analysis.

Background: The HeartMate 3 ventricular assist device (VAD) is a newer centrifugal continuous-flow VAD used for bridge-to-transplant and destination therapy in adults. However, there is limited experience regarding its use in children and adults with complex congenital heart disease (CHD).

Methods: The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) is a multicenter learning network comprised of pediatric hospitals implanting VADs in children and adults with complex CHD.