Diagnostics (Basel)
October 2023
Double-outlet left ventricle (DOLV) is an abnormal ventriculo-arterial connection characterized by the origin of both great arteries from the morphological left ventricle. The aim of our paper is to describe the morphological and imaging features of DOLV and to assess the prevalence of the associated malformations and their surgical outcomes. METHODS From 2011 to 2022, we retrospectively reviewed the electronic case records of patients diagnosed with DOLV at the Bambino Gesu Children's Hospital.
View Article and Find Full Text PDFBackground: Aortic arch malformations (AAMs) should be suspected in the presence of persistent respiratory symptoms despite medical treatment or feeding problems at the pediatric age.
Aim: We report a descriptive cohort of patients with AAMs and the local management protocol applied.
Methods: A total of 59 patients with AAM were retrospectively reviewed.
Constrictive pericarditis is a chronic inflammatory process that can lead to heart failure if not diagnosed and treated correctly. Although Epstein-Barr virus (EBV)-related pericarditis is a very rare condition, it should still be considered for a differential diagnosis. We report the case of an 18-year-old male, who was surgically treated for constrictive pericarditis, in which hybridization to Epstein-Barr virus-encoded RNA (EBER) probe of the excised pericardium led to the subsequent etiological diagnosis of chronic pericarditis caused by EBV.
View Article and Find Full Text PDFTreatment of pediatric aortic valve disease is controversial. In this regard, interest is growing in the neocuspidization of the aortic valve according to Ozaki. In this video tutorial, we illustrate briefly the entire reconstruction technique of a neoaortic valve with pretreated autologous pericardium used in our institution in pediatric patients by describing a case of an incompetent native tricuspid aortic valve.
View Article and Find Full Text PDFEur J Cardiothorac Surg
June 2022
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.
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.
View Article and Find Full Text PDFInt J Environ Res Public Health
October 2021
Aim: This study evaluates the risk factors associated with right ventricular (RV) dilation and dysfunction leading to pulmonary valve replacement (PVR) or adverse cardiac events in repaired Tetralogy of Fallot (rToF) patients.
Methods: Data from all rToF patients who underwent magnetic resonance imaging (MRI) evaluation at our hospital between February 2007 and September 2020 were collected.
Results: Three hundred and forty-two patients (60% males, 42% older than 18 years), with a median age of 16 years (IQR 13-24) at the time of MRI, were included.
We report a unique case of a 6-year-old male child with aborted sudden cardiac death due to ventricular fibrillation. A rare anomalous aortic origin of the right coronary artery was detected and supposed to be the cause of the malignant arrhythmia. Clinical exome sequencing did not reveal any pathogenic variant related to channelopathies nor other known heart-related genes.
View Article and Find Full Text PDFDown syndrome (DS) patients prematurely show clinical manifestations usually associated with aging. Their immune system declines earlier than healthy individuals, leading to increased susceptibility to infections and higher incidence of autoimmune phenomena. Clinical features of accelerated aging indicate that trisomy 21 increases the biological age of tissues.
View Article and Find Full Text PDFEur J Cardiothorac Surg
July 2021
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.
Eur Heart J Cardiovasc Imaging
January 2022
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.
The aim of the study was to evaluate the medium-term results of aortic valve neocuspidalization according to Ozaki compared to Ross procedure for treatment of isolated aortic valve disease in pediatric age. Thirty-eight consecutive patients with congenital or acquired aortic valve disease underwent either Ozaki (n = 22) or Ross (n = 16) operation between 01/2015 and 05/2020. The primary outcome was progression of aortic valve disease and aortic ring and root dimension, whereas secondary outcome was freedom from reintervention or death by type of operation.
View Article and Find Full Text PDFInteract Cardiovasc Thorac Surg
January 2021
About a fifth of all mediastinal masses are primary cysts arising in the absence of other underlying pathology. Bronchogenic cysts, although rare, are the most frequent type responsible for lower airways compression as they often develop in the peripheral branches of the tracheobronchial tree. We report the case of a 6-months-old child admitted for acute respiratory distress and wheezing not responsive to asthma treatment.
View Article and Find Full Text PDFWorld J Pediatr Congenit Heart Surg
January 2020
A strict and rational approach to Fallot's tetralogy with pulmonary atresia and major aortopulmonary collateral arteries allows to achieve optimal results. Rehabilitative and unifocalization strategies do not constitute separate philosophies; instead the surgical strategy should be tailored to each individual patient. Based on our previous experience, the ability to achieve definitive intracardiac repair is the real determinant of both improved survival and adequate systolic right ventricular performance on mid-term follow-up.
View Article and Find Full Text PDFJ Cardiovasc Med (Hagerstown)
December 2019
Background: We evaluated two-dimensional speckle-tracking echocardiography longitudinal strain (L2DSE) in functionally single left ventricles (LV).
Methods: We retrospectively analyzed 21 patients with functionally single LV. We divided patients into two groups according to pre-Fontan cardiac catheterization data: group 1, adequate data for Fontan procedure and group 2, inadequate data.
Evolving reconstructive techniques have progressively become the preferred approach for treatment of pediatric mitral valve regurgitation. We present our experience in a cohort of patients undergoing surgical correction for severe mitral regurgitation. Fifty-five patients (age 1 month-18 years; median 5 years) were included in the present analysis.
View Article and Find Full Text PDFThe thymus plays a fundamental role in establishing and maintaining central and peripheral tolerance and defects in thymic architecture or AIRE expression result in the development of autoreactive lymphocytes. Patients with partial DiGeorge Syndrome (pDGS) and Down Syndrome (DS) present alterations in size and architecture of the thymus and higher risk to develop autoimmunity. We sought to evaluate thymic architecture and thymocyte development in DGS and DS patients and to determine the extent to which thymic defects result in immune dysregulation and T cell homeostasis perturbation in these patients.
View Article and Find Full Text PDFInteract Cardiovasc Thorac Surg
August 2019
Objectives: The population of ageing adults with congenital heart disease (ACHD) is increasing; surgery in these patients presents major difficulties in management. A great debate has developed about whether these patients should be cared for at an adult or paediatric hospital and by an acquired or congenital cardiac surgeon. We analysed data of the surgical treatment of ACHD from the Italian cardiac surgery centres in 2016, focusing on the type of surgery performed, where these patients were operated on and by whom.
View Article and Find Full Text PDFEuropean J Pediatr Surg Rep
January 2018
Lung hypoplasia and pulmonary hypertension (PH) in association with congenital diaphragmatic hernia (CDH) may cause fatal respiratory failure. Lung transplantation (Ltx) may represent an option for CDH-related end-stage pulmonary failure. The aim of this study is to report a patient with CDH who underwent Ltx or combined heart-lung transplantation (H-Ltx).
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