Anatomic characteristics of the right aortic arch with aberrant left subclavian artery in patients who do and do not undergo vascular ring repair.

Introduction: It is unclear if certain anatomic characteristics in patients with a right aortic arch with aberrant left subclavian artery (RAA ALSCA) are associated with undergoing surgical repair.

Methods: This was a single-center retrospective study of patients with RAA ALSCA and computed tomography or cardiovascular magnetic resonance from July 2013-September 2023. The size of the proximal ALSCA or diverticulum of Kommerell (DoK), thoracic inlet index, angle of the proximal ALSCA/DoK from the aortic arch, the position of descending aorta, location of the DoK, and tracheal size were compared between patients who did or did not undergo surgery.

Left aortic arch and aberrant right subclavian artery in children: spectrum of symptoms and response to surgical intervention.

Background: Children with left aortic arch and aberrant right subclavian artery may present with either respiratory or swallowing symptoms beyond the classically described solid-food dysphagia. We describe the clinical features and outcomes of children undergoing surgical repair of an aberrant right subclavian artery.

Materials And Methods: This was a retrospective review of children undergoing repair of an aberrant right subclavian artery between 2017 and 2022.

Operative and nonoperative outcomes in patients with trisomy 13 and 18 with congenital heart disease.

Objective: To evaluate the short- and long-term outcomes of cardiac repair versus nonoperative management in patients with trisomy 13 and trisomy 18 with congenital heart disease.

Methods: An institutional review board-approved, retrospective review was undertaken to identify all patients admitted with trisomy 13/18 and congenital heart disease. Patients were divided into 2 cohorts (operated vs nonoperated) and compared.

Short-Term Results With Ozaki Valved Conduit-A Simple Solution for Patients Needing Right Ventricle to Pulmonary Artery Conduit in a Low-Resource Setting.

Background: The repair of certain types of complex congenital cardiac defects may require a right ventricle-pulmonary artery (RV-PA) conduit. Using the Ozaki Aortic valve neocuspidization (AVNeo)technique, a valved RV-PA conduit was constructed with an Ozaki valve inside a Dacron graft. This study aims to evaluate the short-term outcome of the Ozaki valved RV-PA conduit.

State of the Art Review: Aortic Valve Repair in Infants and Children.

Background: Congenital aortic and truncal valve disease is challenging in infants and children given the lack of available prostheses in very small sizes and the limited durability of homograft aortic valve replacement.

Methods: A comprehensive literature search was performed using the PubMed database. Studies were included either if the report included patients less than 1 year of age or if the technique was tailored to accommodate for somatic growth.

Clinical and 2D/3D-Echo Cardiography Determinants of Mitral Valve Reoperation in Children With Congenital Mitral Valve Disease.

Background: Congenital mitral valve disease (CMVD) presents major challenges in its medical and surgical management.

Objectives: The purpose of this study was to investigate the value of 3-dimensional echocardiography (3DE) and identify associations with MV reoperation in this setting.

Methods: All children <18 years of age who underwent MV reconstruction for CMVD in 2002 to 2018 were included.

Development of a Simple Analytical Model to Facilitate Preoperative Surgical Planning in Valve-Sparing Aortic Root Replacement.

Purpose: Valve-sparing root replacement (VSRR) is attractive for aortic root dilation as it preserves the native aortic valve (AoV). Low effective height (eH) after reconstruction is a risk factor for repair failure and reoperation. We developed and validated a quantitative AoV repair strategy to reliably restore normal valve proportions to promote long-term function.

Cleft closure and other predictors of contemporary outcomes after atrioventricular canal repair in patients with parachute left atrioventricular valve.

Objectives: Parachute left atrioventricular valve (LAVV) complicates atrioventricular septal defect (AVSD) repair. We evaluate outcomes of AVSD patients with parachute LAVV and identify risk factors for adverse outcomes.

Methods: We evaluated all patients undergoing repair of AVSD with parachute LAVV from 2012 to 2021.

Association of tetralogy of Fallot and complete atrioventricular canal: a single-centre 40-year experience.

Objectives: Outcome data in tetralogy of Fallot (ToF) and complete atrioventricular canal (CAVC) are limited. We report our experience for over 40 years in this patient population.

Methods: Single-centre, retrospective analysis of patients who underwent surgical repair with the diagnosis of ToF-CAVC from 1979 to 2022, divided into 2 different periods and compared.

Transcatheter Palliation With Pulmonary Artery Flow Restrictors in Neonates With Congenital Heart Disease: Feasibility, Outcomes, and Comparison With a Historical Hybrid Stage 1 Cohort.

Background: Neonates with complex congenital heart disease and pulmonary overcirculation have been historically treated surgically. However, subcohorts may benefit from less invasive procedures. Data on transcatheter palliation are limited.

Prognostic utility of a risk prediction model for predischarge major residual lesions or unplanned reinterventions following congenital mitral valve repair.

Objective: We sought to develop a risk prediction model for predischarge major mitral valve (MV) residual lesions or unplanned MV reinterventions following congenital MV repair.

Methods: Patients who underwent congenital MV repair (excluding primary repair, but including secondary repair, of canal-type defects) at a single institution from January 2000 to December 2020 and survived to discharge were retrospectively reviewed. The primary outcome was major MV residua (mean gradient >6 mm Hg or moderate or greater regurgitation on the discharge echocardiogram) or predischarge unplanned MV reintervention.

Systematic Analysis of PTFE Monocusp Leaflet Design in a Patient-Based 3D in-Vitro Model of Tetralogy of Fallot.

Purpose: Pulmonary valve (PV) monocusp reconstruction in transannular patch (TAP) right ventricular outflow tract (RVOT) repair for Tetralogy of Fallot has variable clinical outcomes across different surgical approaches. The study purpose was to systematically evaluate how monocusp leaflet design parameters affect valve function in-vitro.

Methods: A 3D-printed, disease-specific RVOT model was tested under three infant physiological conditions.

Right Heart Remodeling After Pulmonary Valve Replacement in Patients With Pulmonary Atresia or Critical Stenosis With Intact Ventricular Septum.

Background Patients with pulmonary atresia or critical pulmonary stenosis with intact ventricular septum (PA/IVS) and biventricular circulation may require pulmonary valve replacement (PVR). Right ventricular (RV) remodeling after PVR is well described in tetralogy of Fallot (TOF); we sought to investigate RV changes in PA/IVS using cardiac magnetic resonance imaging. Methods and Results A retrospective cohort of patients with PA/IVS who underwent PVR at Boston Children's Hospital from 1995 to 2021 with cardiac magnetic resonance imaging before and after PVR was matched 1:3 with patients with TOF by age at PVR.

The Yasui operation: A single institutional experience over 30 years.

Objective: The Yasui operation was introduced in 1987 for patients with 2 adequate ventricles, a ventricular septal defect, and aortic atresia or interrupted aortic arch. Despite promising early outcomes, left ventricular outflow tract obstruction (LVOTO) remains a long-term concern. The purpose of this study is to report our institutional experience with the Yasui operation.

Right ventricular papillary muscle approximation to the septum as an adjunct technique during tricuspid valve repair in congenital heart surgery.

Objective: Tricuspid regurgitation is often caused by leaflet splaying from displaced papillary muscles or ventricular dilatation. Traditional annuloplasty may not address this mechanism. The present study describes a single institution's experience using right ventricular papillary muscle approximation for tricuspid valve repair.

Transcatheter Pulmonary Artery Banding in High-Risk Neonates: In-Vitro Study Provoked by Initial Clinical Experience.

Purpose: Very high-risk, ductal-dependent or complex two-ventricle patients with associated comorbidities often require pulmonary blood flow restriction as bridge to a more definitive procedure, but current surgical options may not be well-tolerated. An evolving alternative utilizes a fenestrated Micro Vascular Plug (MVP) as a transcatheter, internal pulmonary artery band. In this study, we report a case series and an in-vitro evaluation of the MVP to elicit understanding of the challenges faced with device implantation.

Multidisciplinary approach to vascular rings and vascular-related aerodigestive compression: a clinical practice review.

Vascular rings, including double aortic arch and right aortic arch with aberrant left subclavian and left ligamentum, are part of a larger group of vascular-related aerodigestive compression syndromes that also includes innominate artery compression syndrome, dysphagia lusoria, aortic arch anomalies, and aneurysms of either the aorta or pulmonary artery. Additionally, post-surgical airway compression is a distinct entity in itself. The approach to the diagnosis and management of these varied phenomena has been streamlined by the multidisciplinary team at Boston Children's Hospital.

Pacemaker lead insertion sites contribute to abnormalities of myocardial function and histopathology.

Background: Ventricular pacing can cause myocardial dysfunction, but how lead anchoring to the myocardium affects function has not been studied.

Objective: The purpose of this study was to evaluate patterns of regional and global ventricular function in patients with a ventricular lead using cine cardiac computed tomography (CCT) and histology.

Methods: This was a single-center retrospective study with 2 groups of patients with a ventricular lead: (1) those who underwent cine CCT from September 2020 to June 2021 and (2) those whose cardiac specimen was analyzed histologically.