Right ventricular remodeling in complex congenital heart disease.

In congenital heart diseases (CHD) of moderate to great complexity involving the right ventricle (RV), the morphologic RV can be exposed to significant stressors across the lifespan either in a biventricular circulation in a sub-pulmonary or sub-aortic position, or as part of a univentricular circulation. These include pressure and/or volume overload, hypoxia, ischemia, and periprocedural surgical stress leading to remodeling, maladaptation, dilation hypertrophy and dysfunction. This review examines the macroscopic remodeling of the RV in various forms of CHD and explores remodeling trajectories, along with the effects of surgeries and residual lesion repair, in tetralogy of Fallot, Ebstein anomaly, congenitally corrected transposition of the great arteries, transposition of the great arteries with atrial switch surgery, and single ventricle palliated by Fontan.

The Ross Procedure in Children and Infants: A Systematic Review With Pooled Analyses.

Background: The Ross procedure is a surgical option for congenital aortic stenosis that involves replacing the diseased aortic valve with a pulmonary autograft. Little is known about outcomes in children, particularly those younger than 1 year.

Methods: A systematic review with pooled analyses was conducted according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) criteria.

High Reimplantation of Coronary Arteries After the Switch Procedure and Associated Stenoses.

Surgical repair through the arterial switch operation (ASO) is the only definitive treatment in patients with dextro-transposition of the great arteries (d-TGA). A crucial step during the reimplantation process is transfer of coronary arteries (CA) to the neo-aorta. A potential cause of CA stenosis is the presence of a high implantation of CA (HICA), defined by the presence of coronary ostium located above the sinotubular junction (STJ) of the aorta.

Cardiovascular Outcomes in Fontan Patients With Right vs Left Univentricular Morphology: A Multicenter Study.

Background: There is a paucity of data on long-term outcomes after Fontan palliation in patients with a dominant morphological univentricular right (uRV) vs left (uLV) ventricle.

Objectives: The purpose of this study was to compare the incidence of atrial arrhythmias, thromboembolic events, cardiac transplantation, and death following Fontan palliation in patients with uRV vs uLV.

Methods: The Alliance for Adult Research in Congenital Cardiology conducted a multicenter retrospective cohort study on patients with total cavopulmonary connection Fontan palliation across 12 centers in North America.

Role and Applications of Experimental Animal Models of Fontan Circulation.

Over the last four decades, the Fontan operation has been the treatment of choice for children born with complex congenital heart diseases and a single-ventricle physiology. However, therapeutic options remain limited and despite ongoing improvements in initial surgical repair, patients still experience a multiplicity of cardiovascular complications. The causes for cardiovascular failure are multifactorial and include systemic ventricular dysfunction, pulmonary vascular resistance, atrioventricular valve regurgitation, arrhythmia, development of collaterals, protein-losing enteropathy, hepatic dysfunction, and plastic bronchitis, among others.

The Ross-Konno Procedure With or Without Mitral Valve Surgery: A Systematic Review With Individual Data Pooling.

The Ross-Konno procedure is a technically demanding surgical option to treat multilevel left ventricular outflow tract obstruction. A systematic review with pooled analyses was conducted according to PRISMA criteria on studies published between January 2000 and May 2022 that assessed outcomes following the Ross-Konno intervention in children. Individual patient data were extracted from published Kaplan-Meier curves using digitalization software.

Impact of Fontan Fenestration on Adverse Cardiovascular Outcomes: A Multicentre Study.

Background: Fenestrating a Fontan baffle has been associated with improved perioperative outcomes in patients with univentricular hearts. However, longer-term potential adverse effects remain debated. We sought to assess the impact of a fenestrated Fontan baffle on adverse cardiovascular events including all-cause mortality, cardiac transplantation, atrial arrhythmias, and thromboemboli.

Navigating Arrhythmias in Tetralogy of Fallot Throughout the Lifespan: A Case-based Review.

Arrhythmias are a common complication associated with tetralogy of Fallot (ToF), one of the most prevalent forms of congenital heart disease. As illustrated by this case-based review, various forms of arrhythmias can be encountered across the lifespan of patients with ToF, from infancy to older adulthood. These include atrioventricular block, junctional ectopic tachycardia, and atrial and ventricular arrhythmias.

Long-term outcomes after heart transplantation in adult patients with univentricular versus biventricular congenital heart disease.

Objectives: Heart transplantation (HT) is the only life-extending therapy in adults with congenital heart disease (CHD) and end-stage heart failure. HT is considered at high risk in complex CHD given the anatomical complexity and past medical history. Little is known about long-term outcomes after HT in these patients.

The Ross Operation in Young Patients: A 15-Year Experience Focused on Right Ventricle to Pulmonary Artery Conduit Outcomes.

Background: Data on long-term outcomes of the Ross operation in children and young adult patients are limited. The best pulmonary valve substitute for the right ventricular outflow tract reconstruction remains uncertain. This study aimed to assess the outcomes of right ventricular outflow tract reconstruction in the Ross operation in young patients using various pulmonary valve substitutes at a single institution.

Joint-preserving surgery for idiopathic bilateral osteonecrosis of the distal tibia: A case report.

Introduction: Osteonecrosis (ON) is a serious pathological condition that can affect weight-bearing areas of the lower limbs, including the distal tibia. Although trauma is a common cause of ON, the condition has multiple possible etiologies. ON has been associated with a range of factors, including trauma, medication use, alcoholism, and vascular disease.

Ventricular Fibrillation Is a Sign of Life.

Ventricular fibrillation (VF) is a deadly rhythm problem. With asystole, it represents one of the most extreme emergencies that may engage vital prognosis within only few minutes if appropriated treatment is not instituted. It is learned in all medical schools worldwide that VF is not compatible with consciousness and sustained life.

The medical device development ecosystem: Current regulatory state and challenges for future development: A review.

Background/purpose: There has been increasing emphasis on the development of new technology to mitigate unmet clinical needs in cardiovascular disease. This emphasis results in part from recognition that many devices, although being initially developed in the United States, were studied, and then eventually approved abroad before being returned to the U.S.

The Biomedical Entrepreneurship Skills Development Program (BEEP): Educating a New Generation of Medical Innovators.

Innovative, new technologies are rapidly being introduced into the medical world, as scientists and inventors continually discover solutions to all kinds of health issues. However, comprehensive education in medical product development, business process and strategy is distinctly lacking for science students who aspire to become commercial medical innovators and entrepreneurs. Entrepreneurially minded professionals at the New York University Grossman School of Medicine developed, implemented, and integrated programs to train early scientists in the business side of science to accelerate the pace of commercialisation and encourage individuals to pursue venture creation and entrepreneurship to impact highly relevant healthcare solutions.

Ventricular Arrhythmias in Adults With Congenital Heart Disease, Part I: JACC State-of-the-Art Review.

Patients with congenital heart disease associated with a higher risk for ventricular arrhythmias (VA) and sudden cardiac death (SCD) can be divided conceptually into those with discrete mechanisms for reentrant monomorphic ventricular tachycardia (VT) (Group A) and those with more diffuse substrates (Group B). Part I of this review addresses Group A lesions, which predominantly consist of tetralogy of Fallot and related variants. Well-defined anatomic isthmuses for reentrant monomorphic VT are interposed between surgical scars and the pulmonary or tricuspid annulus.

Management for atrial arrhythmias in adults with complex congenital heart disease.

Introduction: The prevalence of congenital heart disease (CHD) is steadily increasing among adults. Atrial arrhythmias are frequent late complications and are associated with substantial morbidity.

Areas Covered: We discuss key considerations regarding management strategies for atrial arrhythmias in common forms of CHD and offer future perspectives.

Systematic Electrophysiological Study Prior to Pulmonary Valve Replacement in Tetralogy of Fallot: A Prospective Multicenter Study.

Background: Ventricular arrhythmias and sudden death are recognized complications in tetralogy of Fallot. Electrophysiological studies (EPS) before pulmonary valve replacement (PVR), the most common reintervention in tetralogy of Fallot, could potentially inform therapy to improve arrhythmic outcomes.

Methods: A prospective multicenter study was conducted to systematically assess EPS with programmed ventricular stimulation in patients with tetralogy of Fallot referred for PVR from January 2020 to December 2021.

Association of Arterial Blood pH at Cannulation With 1 Year Survival Among Veno-Arterial Extracorporeal Membrane Oxygenation Recipients: The Three Seven Rule.

We aimed to describe the clinical outcomes of veno-arterial extracorporeal membrane oxygenation (VA-ECMO) therapy in our institution considering clinical context and pH at cannulation. All patients treated by VA-ECMO during the 2005-2020 period with 1 year complete follow-up were included. Our cohort was divided in three groups according to the pH level at cannulation: pH <7 (group 1), pH 7-7.

The Supreme Biodegradable Polymer DES in Acute and Chronic Coronary Syndromes: A PIONEER III Substudy.

Background: The PIONEER III trial demonstrated noninferiority of 12-month target lesion failure (TLF) with the Supreme DES (Sinomed), a thin-strut cobalt-chromium, biodegradable polymer, sirolimus-eluting stent, compared with a durable polymer, everolimus-eluting (XIENCE/PROMUS) stent (DP-EES). The relative safety and effectiveness of the Supreme DES in patients with acute coronary syndromes (ACS) and those with chronic coronary syndromes (CCS) is not known.

Methods: PIONEER III was a prospective, multicenter, international, 2:1 randomized trial stratified by clinical presentation.

Geographic Variations on the Safety and Efficacy of the Supreme Biodegradable Polymer DES: Results From PIONEER III.

Background: The PIONEER III trial showed the 12-month safety and efficacy of the Supreme drug-eluting stent (DES) vs the durable polymer everolimus-eluting stent. We sought to assess whether the characteristics and clinical outcomes of the Supreme DES in PIONEER III were consistent among patients by enrollment location.

Methods: This subgroup analysis of the PIONEER III trial compared the characteristics and outcomes of patients recruited from North America, Europe, and Japan and the relative differences in patient outcomes according to the site recruitment volume.