Investigating the Long-term Durability of Prosthetic Valves in Patients Who Have Undergone Tetralogy of Fallot Surgery with Transannular Patch.

Objectives: This study aimed to determine the longevity of bioprosthetic valves (BPVs) or valved conduits in the pulmonary position and the factors associated with prosthetic valve longevity in patients who underwent surgical repair of tetralogy of Fallot (TOF).

Methods: We performed a single-centre retrospective study in patients undergoing placement of a bioprosthesis or valved conduit in patients operated for TOF with a transannular patch. The primary end-point was freedom from pulmonary valve replacement (PVR) reintervention.

Is There an Ideal Age or Weight for the Warden Procedure in Patients With Partial Anomalous Pulmonary Venous Return?

Background: The Warden procedure is a common surgical approach for repairing partial anomalous pulmonary venous return (PAPVR). However, it may be complicated by cavoatrial anastomosis obstruction. This study evaluates outcomes of the Warden procedure and identifies optimal age and weight thresholds to minimize reintervention risk.

Evaluation of Residual Right Ventricular Outflow Tract Obstruction After Pulmonary Valve-Sparing Repair of Tetralogy of Fallot: An Echocardiographic Study.

BackgroundPulmonary valve-sparing repair (PVSR) of Tetralogy of Fallot (TOF) provides good results in selected patients. However, recurrent right ventricular outflow tract obstruction (RVOTO) can occur requiring surgical revision. We sought to evaluate the course of RVOTO after PVSR by serial echocardiographic (ECHO) assessment.

The impact of tricuspid regurgitation on timing pulmonary valve replacement in ACHD patients after tetralogy of Fallot repair.

Background: The emerging role of tricuspid regurgitation in the long-term outcome of the general population, poses the need for a review of its impact on patients with TOF and the timing for surgical PVR.

Methods: This is a retrospective, single Centre study. Clinical, imaging and surgical data of adults with repaired TOF with a transannular patch repair were collected from our database.

[Focal Segmental Glomerulosclerosis Due to A3243G Point Mutation in the mtDNA Coding for tRNA].

Mithocondropathies are inherited disorders that can result from abnormalities in the mitochondrial or nuclear DNA. Genetic abnormalities impacting the mitochondrial DNA (mtDNA) are consequently passed down through the maternal line. Renal manifestations of mtDNA disorders are often poorly recognized or misdiagnosed for the widely diverse phenotypic expression of this condition.

A Meta-Analysis of Mitral Surgery in Patients Undergoing Surgery for Anomalous Left Coronary Artery: When to Perform Repair?

The present study is the first meta-analysis comparing long-term outcomes in patients undergoing correction for anomalous left coronary artery (ALCAPA) regarding concomitant mitral valve surgery. A systematic literature review was conducted to identify all relevant studies with comparative data on mitral valve surgery performed during surgery for ALCAPA correction. Predefined primary end points included mortality and mitral valve (re)operation.

Postoperative Outcomes of Fontan Operation in a Multicenter Italian Study. How Far Have We Gone? Early Outcomes After Fontan Operation.

Despite the clinical results of the Fontan operation have certainly improved, it still presents with an inherent surgical risk of death and early morbidities. This is a retrospective clinical study of children undergoing Fontan operation in 9 congenital cardiac centers in Italy between 1990 and 2023. Clinical and surgical data were collected via a dedicated RedCap database.

SARS-CoV-2 infection of thymus induces loss of function that correlates with disease severity.

Background: Lymphopenia, particularly when restricted to the T-cell compartment, has been described as one of the major clinical hallmarks in patients with coronavirus disease 2019 (COVID-19) and proposed as an indicator of disease severity. Although several mechanisms fostering COVID-19-related lymphopenia have been described, including cell apoptosis and tissue homing, the underlying causes of the decline in T-cell count and function are still not completely understood.

Objective: Given that viral infections can directly target thymic microenvironment and impair the process of T-cell generation, we sought to investigate the impact of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) on thymic function.

[Denosumab and fracture risk in kidney transplant].

Kidney transplant patients bear a higher risk of bone disease. The monoclonal antibody Denosumab (Den), by binding RANKL, reduces osteoclastic activity and increases mineral density (BMD), thus limiting the risk of bone fractures. We evaluated the efficacy and safety of Den in kidney transplant patients who developed bone fractures.

22q11.2 Deletion Syndrome: Impact of Genetics in the Treatment of Conotruncal Heart Defects.

Congenital heart diseases represent one of the hallmarks of 22q11.2 deletion syndrome. In particular, conotruncal heart defects are the most frequent cardiac malformations and are often associated with other specific additional cardiovascular anomalies.

Late outcome of extracardiac Fontan patients: 32 years of follow-up.

Objectives: We sought to evaluate the long-term survival and risk factors for morbidity and mortality in our cohort of patients after extracardiac (EC) Fontan.

Methods: We collected and analysed follow-up data until September 2020 for all patients who underwent EC Fontan operation at our institution from November 1988 to November 2015.

Results: Out of 406 patients treated with EC Fontan at a mean age of 5.

The Modern Surgical Approach to Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries.

Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals is a complex congenital heart defect that includes a heterogeneous subgroup of patients. Variation in the sources of pulmonary blood flow contributes to the complexity of the lesion and the diversity of approaches to its management. Unifocalization and rehabilitation focus on mobilization of collateral arteries and growth of native pulmonary arteries, respectively, with the ultimate surgical goal of achieving separated systemic and pulmonary circulations with the lowest possible right ventricular pressure.

Predictors of survival in paediatric mitral valve surgery: the impact of age at operation at late follow-up.

Objectives: The aim of this study was to elucidate predictors of death and reintervention after mitral valve (MV) surgery in children.

Methods: A single-centre retrospective study was performed enrolling 142 patients younger than 18 years who underwent primary index surgical mitral repair or replacement at Bambino Gesù Children's Hospital in Rome from July 1982 to April 2020. Patients with complete, transitional or partial atrioventricular septal defect and patients with single ventricle physiology were excluded.

Ascending aortic extension to increase aortopulmonary space after comprehensive stage II palliation.

Aortic reconstruction at the time of the comprehensive stage II (CSII) procedure can be complicated by compression within the aortopulmonary space resulting in airway or pulmonary artery narrowing. We describe our experience with 2 patients with hypoplastic left heart syndrome and pulmonary artery stenosis after the CSII procedure. Both patients underwent an aortic extension with a Hemashield interposition graft to open up the aortopulmonary space.

Use of Transcarotid IMPELLA 2.5 Axial-Flow Pump Device for Left Ventricle Unloading During VA-ECMO Support in Pediatric Acute Heart Failure.

An alternative strategy for left ventricular (LV) venting during short-term mechanical circulatory support is use of Impella axial-flow pump. We present our transcarotid Impella 2.5 implantation technique using a polytetrafluoroethylene graft, in two children with acute heart failure treated primarily with venoarterial ECMO.

Use of carotid artery cannulation during redo sternotomy in congenital cardiac surgery: a single-centre experience.

Objectives: Management of resternotomy is often a difficult challenge in patients with congenital diseases who have undergone multiple sternotomies. Our goal was to report our single-centre experience with carotid cannulation using a graft interposed during redo sternotomy in complex cardiac reintervention procedures.

Methods: We performed a retrospective review of all patients who had undergone complex redo sternotomies between January 2019 and May 2020 utilizing a cervical cannulation technique with a Gore-Tex graft interposed on the carotid artery.

Predictors of survival in paediatric mitral valve replacement.

Objectives: The aim of this study was to identify the predictors of death and of reintervention after mitral valve replacement (MVR) in children.

Methods: A single-centre retrospective study was performed including 115 patients under the age of 18 undergoing MVR between 1982 and 2019. For all patients, the ratio of prosthetic valve size (diameter in mm) to weight (kg) at surgery was calculated and long-term result was assessed.

Blood flow characteristics after aortic valve neocuspidization in paediatric patients: a comparison with the Ross procedure.

Aims: The aortic valve (AV) neocuspidization (Ozaki procedure) is a novel surgical technique for AV disease that preserves the natural motion and cardiodynamics of the aortic root. In this study, we sought to evaluate, by 4D-flow magnetic resonance imaging, the aortic blood flow characteristics after AV neocuspidization in paediatric patients.

Methods And Results: Aortic root and ascending aorta haemodynamics were evaluated in a population of patients treated with the Ozaki procedure; results were compared with those of a group of patients operated with the Ross technique.

Long-term veno-arterial extracorporeal membrane oxygenation as a bridge to heart-lung transplant.

En bloc heart-lung transplantation still represents definitive therapy for end-stage cardiopulmonary failure. However, patients may critically decompensate while awaiting suitable donor organs and necessitate veno-arterial extracorporeal membrane oxygenation. In this article, we describe the combined use of central cannulation with the Berlin Heart EXCOR ventricular assist device cannulae and the CentriMag centrifugal pump as an extended bridge to heart-lung transplantation in three pediatric patients.