Outcomes of extracardiac Fontan operation: A single institution experience with 398 patients.

Objective: The study objective was to evaluate the outcomes of the extracardiac Fontan operation at a single institution.

Methods: We conducted a retrospective study of 398 patients from a single institution who underwent their initial extracardiac Fontan operation between 1997 and 2020. We determined the incidence of and risk factors for failure of the Fontan circulation, which includes death, Fontan takedown, heart transplantation, protein-losing enteropathy, plastic bronchitis, and functional status at the last follow-up.

Heart Transplantation after Univentricular Palliation: Improved Outcomes and Increased Complexity.

Aim: Heart transplantation (HT) in patients with failing univentricular circulation is often challenging. This is compounded by the ever-increasing number of patients with prior Norwood-type reconstruction of the aorta, large aortic root, and often dense adhesions from multiple prior operations. We aimed to elucidate differences in outcomes of HT in patients with prior univentricular palliations, with and without prior Norwood-type aortic arch reconstruction (ArchRec).

Long-Term Outcomes of Mechanical Aortic Valve Replacement in Children.

When the options of aortic valve repair or the Ross procedure are not feasible or have been exhausted, mechanical aortic valve replacement (AVR) may provide a reliable and structurally durable alternative, but with the limitations of long-term anticoagulation, thrombosis risk and lack of valve growth potential. In this article, we review the longitudinal outcomes of mechanical AVR in children in our institution and compare them to those recently reported by others. From 1978 to 2020, 62 patients underwent mechanical AVR at a median age of 12.

Surgery for paravalvular abscess in children.

Objective: To investigate the outcomes of surgery in children with paravalvular abscess at our institution.

Methods: A retrospective review of all patients who underwent surgery for paravalvular abscess was performed.

Results: Between 1989 and 2020, 30 patients underwent surgery for paravalvular abscess, of whom 5 (16.

Truncal valve repair in an adolescent with severe annular dilatation.

Approximately 20% of patients with truncus arteriosus might need a truncal valve procedure within 20 years after anatomical repair due to regurgitation. These patients commonly develop valve regurgitation due to root dilatation with a sufficient amount of good quality valvular tissue. Thus, the reduction of the truncal annulus is the single most important factor to achieve durable repair, especially in patients in whom the Ross procedure is not an option.

Bex-Nikaidoh operation and the impact of double root translocation on outcomes.

Objectives: The Bex-Nikaidoh operation can effectively relieve left ventricular outflow tract obstruction. However, if a conduit is used for right ventricular outflow tract reconstruction, a late reoperation can be anticipated. We examined the impact of double root translocation on outcomes.

T-Remodeling of the Pulmonary Artery Bifurcation for Pulmonary Artery Origin Stenosis.

Background: Various surgical techniques are utilized for reconstructing hypoplastic pulmonary arteries (PAs) in patients with conotruncal anomalies and at times, may be susceptible to restenosis and reoperation. We reviewed our experience with a simple technique of T-shaped remodeling of the PA bifurcation.

Methods: Between 2005 and 2019, 31 patients underwent T-remodeling of central PAs by a single cardiac surgeon.

Aortic valve repair in a low-birth-weight neonate with poor ventricular function: staged tricuspidization of the unicuspid valve.

Congenital severe isolated aortic stenosis in neonates requires prompt intervention because left ventricular function may deteriorate rapidly. Surgical repair of a stenotic aortic valve in neonates allows debridement of fibrous material and precision commissurotomy. As such, the child would end up with a much better-quality aortic valve compared to the uncontrolled tear that results following balloon dilatation.

Aortic valve-sparing procedure in the pediatric population.

Background: Aortic valve-sparing procedures have been established as a mainstream treatment option for adult patients with aortic root aneurysms. However, data regarding their use in the pediatric population is limited. This study reports on our experience with aortic valve-sparing procedures in children.

The Ross Procedure in Children: Defining the Optimal Age.

Background: It has been proposed that delaying the Ross procedure to later in childhood, allowing autograft stabilisation and placement of a larger pulmonary conduit, may improve outcomes. However, the effect of age at the time of Ross procedure on outcomes remains unclear.

Methods: All patients who underwent the Ross procedure between 1995 and 2018 were included in the study.

Long-term outcomes of tetralogy of Fallot repair: A 30-year experience with 960 patients.

Objective: This study evaluates the long-term results of tetralogy of Fallot repair and assesses the risk factors for adverse outcomes.

Methods: This retrospective study included 960 patients who underwent transatrial transpulmonary tetralogy of Fallot repair between 1990 and 2020.

Results: A transannular patch was placed in 722 patients, and pulmonary valve preservation was achieved in 233 patients.

Outcomes of total anomalous pulmonary venous drainage repair in neonates and the impact of pulmonary hypertension on survival.

Background: Mortality after repair of total anomalous pulmonary venous drainage (TAPVD) in neonates has remained high. Analysis of risk factors may help identify therapeutic targets to improve survival.

Methods: Retrospective analysis of all neonates who underwent simple TAPVD repair.

Kawashima Procedure: The Impact of Age.

Background: The results of the Kawashima operation are incompletely defined. Furthermore, optimal timing of Kawashima operation, an important consideration when managing desaturated young infants awaiting surgery, remains unclear. We reviewed our outcomes of Kawashima operation, with a focus on the impact of age.

Long-term Outcomes of Transatrial-Transpulmonary Repair of Tetralogy of Fallot With Anomalous Coronary Arteries.

Repair of tetralogy of Fallot (TOF) can be complicated by the presence of an anomalous coronary artery (ACA) crossing the right ventricular outflow tract (RVOT). This study sought to evaluate the late outcomes of a policy of transatrial-transpulmonary repair for this condition. The transatrial-transpulmonary approach was used in 864 consecutive TOF repairs between 1993 and 2018 at a single institution, of which 55 (6%) patients had an ACA.

Outcomes of Mitral Valve Repair in Children With Infective Endocarditis: A Single-Center Experience.

Mitral valve infective endocarditis (IE) in children is rare, and there are few reports on the outcomes of surgery in these patients. This study investigated the long-term outcomes of mitral valve repair in children with IE. Data were retrospectively obtained from medical records and correspondence.

Intrinsic activation of cardiosphere-derived cells enhances myocardial repair.

Objective: Permanent loss of cardiomyocytes after myocardial infarction results in irreversible damage to cardiac function. The present study aims to enhance the cardiomyogenic efficiency of cardiosphere-derived cells (CDCs) to develop into large populations of cardiomyocytes by intrinsic activation of cardio-specific differentiation factors (Gata4, Mef2c, Nkx2-5, Hand2, and Tnnt2) by a CRISPR/dCas9 assisted transcriptional enhancement system.

Methods: Exhaustive screening was performed to identify the specific sequences in endogenous regulatory regions (enhancers and promoters) responsible for transcriptional activation of the target genes, via a CRISPR/dCas9 system fused with transcriptional activator VP64 (CRISPR-dCas9-VP64).

Stem Cell Therapy in Heart Disease: Limitations and Future Possibilities.

Heart diseases are one of the major causes of morbidity and mortality worldwide. Despite major advances in drug and interventional therapies, surgical procedures, and organ transplantation, further research into new therapeutic options is still necessary. Stem cell therapy has emerged as one option for the treatment of a variety of heart diseases.

Impact of Cardiac Progenitor Cells on Heart Failure and Survival in Single Ventricle Congenital Heart Disease.

Rationale: Intracoronary administration of cardiosphere-derived cells (CDCs) in patients with single ventricles resulted in a short-term improvement in cardiac function.

Objective: To test the hypothesis that CDC infusion is associated with improved cardiac function and reduced mortality in patients with heart failure.

Methods And Results: We evaluated the effectiveness of CDCs using an integrated cohort study in 101 patients with single ventricles, including 41 patients who received CDC infusion and 60 controls treated with staged palliation alone.

Transcoronary cell infusion with the stop-flow technique in children with single-ventricle physiology.

Background: Almost all reports on cardiac regeneration therapy have referred to adults, and only a few have focused on transcoronary infusion of cardiac progenitor cells using the stop-flow technique in children.

Methods: Intracoronary autologous cardiosphere-derived cell (CDC) transfer was conducted at Okayama University as a phase 1 clinical trial for seven patients with hypoplastic left heart syndrome between January 2011 and December 2012, and as a phase 2 clinical trial for 34 patients with single-ventricle physiology between July 2013 and March 2015.

Results: A total of 41 patients with single-ventricle physiology underwent transcoronary infusion of CDC with the stop-flow technique.

Intracoronary Cardiac Progenitor Cells in Single Ventricle Physiology: The PERSEUS (Cardiac Progenitor Cell Infusion to Treat Univentricular Heart Disease) Randomized Phase 2 Trial.

Rationale: Patients with single ventricle physiology are at high risk of mortality resulting from ventricular dysfunction. The preliminary results of the phase 1 trial showed that cardiosphere-derived cells (CDCs) may be effective against congenital heart failure.

Objective: To determine whether intracoronary delivery of autologous CDCs improves cardiac function in patients with single ventricle physiology.

Transcoronary infusion of cardiac progenitor cells in hypoplastic left heart syndrome: Three-year follow-up of the Transcoronary Infusion of Cardiac Progenitor Cells in Patients With Single-Ventricle Physiology (TICAP) trial.

Objectives: Our aim was to assess midterm safety and clinical outcomes of intracoronary infusion of cardiosphere-derived cells (CDCs) after staged palliation in patients with hypoplastic left heart syndrome (HLHS).

Methods: In this prospective, controlled study, 14 consecutive patients with HLHS who were undergoing 2- or 3-stage surgical palliations were assigned to receive intracoronary CDC infusion 1 month after cardiac surgery (n = 7), followed by 7 patients allocated to a control group with standard care alone. The primary end point was to assess procedural feasibility and safety; the secondary end point was to evaluate cardiac function and heart failure status through 36-month follow-up.