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).

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.

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.

Double outlet right ventricle.

This review article addresses the history, morphology, anatomy, medical management, and different surgical options for patients with double outlet right ventricle.

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.

Educating Surgeons to Construct Valved Conduits: What Does It Take to Achieve Reproducible Results?

Background: Despite the demonstrated utility of surgeon-fashioned polytetrafluoroethylene (PTFE) valved conduits, methods for educating surgeons to reproducibly construct these conduits are lacking. We present a surgeon education process and early outcomes for children receiving surgeon-fashioned PTFE valved conduits during the initial learning curve.

Methods: The educational curriculum included 4 hours of proctored instruction/supervised valve construction, followed by 2 hours of individual practice.

Occlusion of the os of the left coronary artery by dysplastic aortic valve tissue presenting as progressive mitral insufficiency and cardiac arrest.

Mitral regurgitation in the neonatal period is relatively rare. It can be secondary to a congenital malformation of the valve apparatus or mitral valve dysfunction and deformation secondary to myocardial dysfunction or volume load of the left ventricle. Less commonly, it can be due to coronary artery abnormalities leading to mitral valve papillary muscle ischaemia and subsequent dysfunction.

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.

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.

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.

A High-Fidelity Surgical Model and Perfusion Simulator Used to Demonstrate ECMO Cannulation, Initiation, and Stabilization.

Our high-fidelity simulation model provides a realistic example for health-care professionals to experience cannulation, initiation, and hemodynamic stabilization during extracorporeal membrane oxygenation (ECMO) therapy. This educational experience brings a variety of critical care specialties together, in a controlled simulation setting, to develop, master, and maintain clinical skills. This may include perfusionists, ECMO specialists, surgical technicians, registered nurses, physicians, and students.

Myocardial ischemia as presenting manifestation of IgG4-related disease: a case-based review.

Coronary involvement in IgG4-related disease (IgG4-RD) has been scarcely reported, and myocardial ischemia as its presenting feature is even rarer. Here, we describe an additional case with novel and relevant observations. The patient was a previously healthy, middle-aged woman who presented to the clinic with new-onset typical angina.