Multiphysiologic State Computational Fluid Dynamics Modeling for Planning Fontan With Interrupted Inferior Vena Cava.

Background: Single ventricle (SV) patients with interrupted inferior vena cava (iIVC) and azygos continuation are at high risk for unbalanced hepatic venous flow (HVF) distribution to the lungs after Fontan completion and subsequent pulmonary arteriovenous malformations (AVMs) formation.

Objectives: The aim of the study was to utilize computational fluid dynamics (CFD) analysis to avoid maldistribution of HVF to the lungs after Fontan surgery.

Methods: Four SV subjects with iIVC were prospectively studied with a 3-dimensional (3D) modeling workflow with digital 3D models created from segmented magnetic resonance images or computer tomography scans, virtual surgery, and CFD analysis over multiple physiologic states for the evaluation of operative plans to achieve balanced HVF to both lungs.

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.

Evolving Role of Three-Dimensional Echocardiography for Right Ventricular Volume Analysis in Pediatric Heart Disease: Literature Review and Clinical Applications.

Accurate knowledge of right ventricular (RV) volumes and ejection fraction is fundamental to providing optimal care for pediatric patients with congenital and acquired heart disease, as well as pulmonary hypertension. Traditionally, these volumes have been measured using cardiac magnetic resonance because of its accuracy, reproducibility, and freedom from geometric assumptions. More recently, an increasing number of studies have described the measurement of RV volumes using three-dimensional (3D) echocardiography.

Prospective randomized controlled trial of the safety and feasibility of a novel mesenchymal precursor cell therapy in hypoplastic left heart syndrome.

Objective: To assess the safety and feasibility of low-dose, novel, allogenic mesenchymal precursor cell (MPC) therapy as an adjunct to left ventricular (LV) recruitment for patients with hypoplastic left heart syndrome (HLHS) and borderline left ventricles. MPC injections into the hypoplastic left ventricle may stimulate neovascularization and beneficial LV remodeling and may improve the likelihood of achieving biventricular (BiV) or 1.5 ventricle (1.

Staged ventricular recruitment and biventricular conversion following single-ventricle palliation in unbalanced atrioventricular canal defects.

Objective: Restoration of biventricular circulation is an alternative management strategy in unbalanced atrioventricular canal defects (uAVCDs), especially in patients with risk factors for single-ventricle palliation (SVP) failure. When ventricular volume is inadequate for biventricular circulation, recruitment procedures may accommodate its growth. In this study, we review our uAVCD experience with biventricular conversion (BIVC) after prior SVP.

Case report: Active clinical manifestation of endocardial fibroelastosis in adolescence in a patient with mitral and aortic obstruction-histologic presence of endothelial-to-mesenchymal transformation.

This is the first description of active clinical manifestation of endocardial fibroelastosis (EFE) and remodeling of the endocardium endothelial-to-mesenchymal transformation (EndMT) in an adolescent with Shone's variant hypoplastic left heart complex (HLHC) and a genetic heterozygous ABL1 variant. While EFE has not been typically associated HLHC or Shone's syndrome, in this patient flow alterations in the left ventricle (LV), combined with genetic alterations of intrinsic EndMT pathways led to active clinical manifestation of EFE in adolescence. This case emphasizes that new therapies for EFE might need to focus on molecular factors influenced by intrinsic and extrinsic stimuli of EndMT.

Recommendations for the Use of Echocardiography in the Evaluation of Rheumatic Heart Disease: A Report from the American Society of Echocardiography.

Acute rheumatic fever and its chronic sequela, rheumatic heart disease (RHD), pose major health problems globally, and remain the most common cardiovascular disease in children and young people worldwide. Echocardiography is the most important diagnostic tool in recognizing this preventable and treatable disease and plays an invaluable role in detecting the presence of subclinical disease needing prompt therapy or follow-up assessment. This document provides recommendations for the comprehensive use of echocardiography in the diagnosis and therapeutic intervention of RHD.

Symmetric bicuspidizing repair for patients with congenital aortic or truncal valve disease.

Objective: Symmetric bicuspidizing repair has been shown to be safe and effective in the short term in adults and children with unicuspid aortic valve. Outcomes of extending this technique to patients with other forms of aortic and truncal valve disease have not been reported.

Methods: We performed a retrospective review of patients who underwent the symmetric bicuspidizing repair at Boston Children's Hospital between December 2019 and June 2022 with a contemporary comparator group of patients who underwent other forms of bicuspidization.

Residual Lesion Severity Predicts Midterm Outcomes After Congenital Aortic Valve Repair.

Background: We sought to validate the technical performance score (TPS) as a predictor of midterm outcomes after congenital aortic valve repair.

Methods: This was a single-center, retrospective review of consecutive patients who underwent aortic valve repair between January 1, 2011, and December 31, 2019. Predischarge echocardiograms were used to assign a TPS for each index operation as class 1, no aortic valve residua; class 2, minor aortic valve residua; or class 3, major aortic valve residua or predischarge reintervention for major residua.

Contemporary outcomes of the double switch operation for congenitally corrected transposition of the great arteries.

Objective: To determine the contemporary outcomes of the double switch operation (DSO) (ie, Mustard or Senning + arterial switch).

Methods: A single-institution, retrospective review of all patients with congenitally corrected transposition of the great arteries undergoing a DSO.

Results: Between 1999 and 2019, 103 patients underwent DSO with a Mustard (n = 93) or Senning (n = 10) procedure.

Anomalous Left Coronary Artery Arising from the Pulmonary Artery (ALCAPA): The Critically Important Role of Color Flow Doppler in Identifying a Rare Intramural Course.

• ALCAPA must be considered in an infant with ischemia and/or heart failure. • A rare variant with intramural aortic course may appear normal on 2D echo. • Retrograde color flow in the LCA is highly suspicious for this lesion.

Single-Leaflet Aortic Valve Reconstruction Utilizing the Ozaki Technique in Patients With Congenital Aortic Valve Disease.

Evaluate outcomes of single leaflet aortic valve reconstruction using Ozaki sizer and template. Single institute retrospective analysis between August 2015 and August 2019. Thirty-three patients, median age 9.

Management of Congenitally Corrected Transposition of the Great Arteries With Intact Ventricular Septum: Anatomic Repair or Palliative Treatment?

[Figure: see text].

The Association of Age and Repair Modification with Outcome after Cone Repair for Ebstein's Malformation.

Cone repair (CR) uses native tissue for tricuspid valve (TV) repair and provides potential for growth. Results after CR were investigated in different age groups including several surgical modifications. Single institution retrospective analysis of all CR excluding neonatal procedures.

Congenital aortic and truncal valve reconstruction using the Ozaki technique: Short-term clinical results.

Objectives: Aortic valve reconstruction (AVRec) with neocuspidization or the Ozaki procedure with complete cusp replacement for aortic valve disease has excellent mid-term results in adults. Limited results of AVRec in pediatric patients have been reported. We report our early outcomes of the Ozaki procedure for congenital aortic and truncal valve disease.

Early Infant Symptomatic Patients With Tetralogy of Fallot With Absent Pulmonary Valve: Pulmonary Artery Management and Airway Stabilization.

Background: Tracheobronchomalacia and airway obstruction from severely dilated pulmonary arteries in tetralogy of Fallot with absent pulmonary valve (TOF-APV) has been associated with high rates of respiratory failure and mortality (15% to 25%). It is not known whether aggressive pulmonary artery (PA) or direct airway intervention during early definitive cardiac repair improves outcomes.

Methods: A retrospective observational study was made of all patients undergoing surgical repair for TOF-APV at our center between 2006 and 2018.

Mechanical Mitral Valve Replacement: A Multicenter Study of Outcomes With Use of 15- to 17-mm Prostheses.

Background: The aim of this study was to evaluate early and mid-term outcomes (mortality and prosthetic valve reintervention) after mitral valve replacement with 15- to 17-mm mechanical prostheses.

Methods: A multicenter, retrospective cohort study was performed among patients who underwent mitral valve replacement with a 15- to 17-mm mechanical prosthesis at 6 congenital cardiac centers: 5 in The Netherlands and 1 in the United States. Baseline, operative, and follow-up data were evaluated.

Speckle tracking echocardiographically-based analysis of ventricular strain in children: an intervendor comparison.

Background: Strain and synchrony can be calculated from a variety of software packages, but there is a paucity of data with inter-vendor comparisons in children. To test the hypothesis that different packages may affect results, independent of acquisition, we compared values obtained using two commercially available analysis tool (QLAB and TomTec), with several different settings.

Methods: The study population included 108 children; patients were divided into three groups: (1) normal cardiac structure and conduction; (2) ventricular paced rhythm; and (3) flattened ventricular septum (reflecting right ventricular pressure or volume load lesions).

A geometrically adaptable heart valve replacement.

Congenital heart valve disease has life-threatening consequences that warrant early valve replacement; however, the development of a growth-accommodating prosthetic valve has remained elusive. Thousands of children continue to face multiple high-risk open-heart operations to replace valves that they have outgrown. Here, we demonstrate a biomimetic prosthetic valve that is geometrically adaptable to accommodate somatic growth and structural asymmetries within the heart.

[Current surgical options and outcomes for newborns with hypoplastic left heart syndrome].

Since the first successful palliation was performed by Norwood et al. in 1983, there have been substantial changes in diagnosis, management, and outcomes of hypoplastic left heart syndrome, Survival for stage 1 palliation has increased to 90% in many centres, with patients potentially surviving into adulthood. However, the associated morbidity and mortality remain substantial.

Flow disturbances and the development of endocardial fibroelastosis.

Objectives: Endothelial-to-mesenchymal transition (EndMT) has been identified as the underlying mechanism of endocardial fibroelastosis (EFE) formation. The purpose of this study was to determine whether hemodynamic alterations due to valvar defects promote EndMT and whether age-specific structural changes affect ventricular diastolic compliance despite extensive surgical resection of EFE tissue.

Material And Methods: We analyzed EFE tissue from 24 patients with hypoplastic left heart syndrome (HLHS) who underwent left ventricular (LV) rehabilitation surgery at Boston Children's Hospital between December 2011 and March 2018.

Three-dimensional transesophageal echocardiography-guided transcatheter closure of multiple mitral paravalvular leaks demonstrating real time avoidance of device-induced valve malfunction.

Transcatheter closure of mitral paravalvular leaks has become an accepted alternative to surgical repair, especially in patients at high risk for reoperation. Device placement using three-dimensional (3D) transesophageal echocardiography (TEE) guidance allows en-face views of the valve and provides detailed information for device sizing and placement. We present a case demonstrating hybrid transapical transcatheter 3D TEE-guided closure of two mitral paravalvular leaks.