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.

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.

Aortic Valve Neocuspidalization May Be a Viable Alternative to Ross Operation in Pediatric Patients.

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.

Aortic valve neocuspidalization in paediatric patients with isolated aortic valve disease: early experience.

Objectives: There is growing interest in the aortic valve (AV) neocuspidalization technique for the treatment of aortic valve disease (AVD). We report our medium-term results with this procedure performed in a paediatric patient population.

Methods: Between July 2016 and May 2020, 22 patients with both congenital and acquired isolated AVD were treated with neocuspidalization.

Atypical cardiac defects in patients with RASopathies: Updated data on CARNET study.

Background: RASopathies are a set of relatively common autosomal dominant clinically and genetically heterogeneous disorders. Cardiac outcomes in terms of mortality and morbidity for common heart defects (such as pulmonary valve stenosis and hypertrophic cardiomyopathy) have been reported. Nevertheless, also Atypical Cardiac Defects (ACDs) are described.

Outcome for Conservative Surgery for the Correction of Severe Mitral Valve Regurgitation in Children: A Single-Center Experience.

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.

Midterm Echocardiographic Assessment of Right Ventricular Function After Midline Unifocalization.

Background: Patients with an open ventricular septal defect (VSD) after repair of pulmonary atresia (PA), VSD, and major aortopulmonary collaterals (MAPCAs) are the most vulnerable subgroup. We analyzed the impact of concomitant versus delayed VSD closure on survival and intermediate-term right ventricular (RV) function.

Methods: Between October 1996 and February 2017, 96 patients underwent a pulmonary flow study-aided repair of PA/VSD/MAPCAs.

Data on cardiac defects, morbidity and mortality in patients affected by RASopathies. CARNET study results.

A comprehensive description of morbidity and mortality in patients affected by mutations in genes encoding for signal transducers of the RAS-MAPK cascade (RASopathies) was performed in our study recently published in the International Journal of Cardiology. Seven European cardiac centres participating to the CArdiac Rasopathy NETwork (CARNET), collaborated in this multicentric, observational, retrospective data analysis and collection. In this study, clinical records of 371 patients with confirmed molecular diagnosis of RASopathy were reviewed.

Long-term outcomes following transatrial versus transventricular repair on right ventricular function in tetralogy of Fallot.

Background And Aim Of The Study: Outcomes after repair of tetralogy of Fallot (TOF) are good with either a transventricular (TV) or transatrial (TA) approach. We sought to determine if there is a relationship between the TV or TA approach and right ventricular (RV) function, and the role of residual pulmonary regurgitation (PR) on the long-term outcomes.

Methods: This was a retrospective cohort multicentric study on survivors after surgical repair of TOF (TA versus TV approach, ±transannular patch) between 1990 and 2004.

Long-Term Outcomes After Extracardiac Fontan Takedown to an Intermediate Palliative Circulation.

Background: Acute failure of the Fontan circulation is rare but remains associated with high morbidity and mortality rates. Little is known about the long-term outcome of patients who underwent Fontan takedown to an intermediate palliative circulation and their potential candidacy for redo Fontan completion.

Methods: Patients followed up at a single institution who underwent takedown of a Fontan circulation to an intermediate palliative circulation within 2 months of extracardiac Fontan completion were reviewed.

Impact of Pulmonary Flow Study Pressure on Outcomes After One-Stage Unifocalization.

Background: The purpose of this study was to evaluate the accuracy of the pulmonary flow study in (1) predicting the feasibility of concomitant intracardiac repair after one-stage unifocalization; and in (2) predicting long-term survival and the onset of right ventricular dysfunction after surgery.

Methods: Between October 1996 and July 2015, a flow study was obtained in 95 patients undergoing complete one-stage unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals. The ability to achieve 100% flow (approximately 2.

Cardiac defects, morbidity and mortality in patients affected by RASopathies. CARNET study results.

Background: RASopathies are developmental disease caused by mutations in genes encoding for signal transducers of the RAS-MAPK cascade. The aim of the present study was to provide a comprehensive description of morbidity and mortality in patients with molecularly confirmed RASopathy.

Methods: A multicentric, observational, retrospective study was conducted in seven European cardiac centres participating to the CArdiac Rasopathy NETwork (CARNET).

Intraoperative coronary revision but not coronary pattern is associated with mortality after arterial switch operation.

Objectives: We sought to determine differences in baseline characteristics and clinical outcomes in a consecutive series of patients undergoing arterial switch operation (ASO), assessing the effect of coronary anatomy on postoperative mortality, both overall and adjusted for surgical era.

Methods: From January 2000 to May 2015, 283 consecutive patients underwent ASO for transposition of the great arteries. A total of 103 patients (36.

Outcome of Standard and Bicuspidalized Cryopreserved Homografts for Primary Right Ventricular Outflow Tract Reconstruction.

Background And Aim Of The Study: The study aim was to analyze the safety and longevity of cryopreserved homografts used for primary right ventricular outflow tract reconstruction (RVOTR), and to compare the outcome using either standard or bicuspidalized allografts.

Methods: Between February 2000 and September 2014, a total of 53 patients underwent primary RVOTR using either a standard (n = 40) or a bicuspidalized (n = 13) cryopreserved homograft. The median age at surgery was 15.

Airway complications after single-stage unifocalization for pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries.

Objective: We analyze the incidence of postoperative severe airflow limitation after single-stage unifocalization in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) and comment on the treatment performed.

Methods: From 1994 until 2014, 118 patients with diagnosis of PA, VSD, MAPCAs underwent surgical treatment. Four patients (3.

Vacuum-assisted closure system in newborns after cardiac surgery.

Objective: To analyze the effectiveness and the results of the use of a vacuum-assisted closure (VAC) system for the treatment of complex sternal wounds in newborns after cardiac surgery.

Methods: From May 2008 until December 2012, six patients developed post-sternotomy wound problems (large defects of epithelialization or mediastinitis), which were treated with a VAC system. Median age at the time of institution of VAC was 24.

Identification of TBX5 mutations in a series of 94 patients with Tetralogy of Fallot.

Tetralogy of Fallot (TOF) (OMIM #187500) is the most frequent conotruncal congenital heart defect (CHD) with a range of intra- and extracardiac phenotypes. TBX5 is a transcription factor with well-defined roles in heart and forelimb development, and mutations in TBX5 are associated with Holt-Oram syndrome (HOS) (OMIM#142900). Here we report on the screening of 94 TOF patients for mutations in TBX5, NKX2.

"Baby Heart Project": the Italian project for accreditation and quality management in pediatric cardiology and cardiac surgery.

Optimization of the relationship between the supply and the demand for medical services should ideally be taken into consideration for the planning within each national Health System. Although government national health organizations embrace this policy specifically, the contribution of expert committees (under the scientific societies' guarantee in any specific medical field) should be advocated for their capability to collect and analyze the data reported by the various national institutions. In addition, these committees have the competence to analyze the need for the resources necessary to the operation of these centers.

[Informed consent in pediatric cardiology and cardiac surgery].

Background: A multidisciplinary study group was established to review and approve the informed consent froms in Pediatric Cardiology and Cardiac Surgery.

Methods: The work was carried out in several stages, starting with an analysis of what was already in use in several Italian Centers. Subsequently, shared forms for pediatric cardiac surgery and interventional catheterization procedures were developed, pointing to clarity of information, prediction of therapeutic options, quantification for verbal categories of risk associated with cardiac surgery procedures, and provision of information also to young patients.

One-stage repair of aberrant left brachiocephalic artery and coarctation of the aorta in right aortic arch.

The combination of right aortic arch with aberrant left brachiocephalic artery and aortic coarctation is very rare. Here, we report the case of a 3.1-kg neonate with multiple malformations who received detailed preoperative anatomical definition by chest computed tomography (CT) scan and eventually underwent one-stage repair at the age of 17 days.

Repair of incompetent truncal valves: early and mid-term results.

Objectives: To analyse the factors associated with in-hospital mortality and mid-term significant neoaortic valve regurgitation (AR) after truncal valve (TV) repair.

Methods: Eleven children underwent TV repair at our institution from July 1999 to March 2012. All children presented significant preoperative TV regurgitation.

Early and late failure of tissue-engineered pulmonary valve conduits used for right ventricular outflow tract reconstruction in patients with congenital heart disease.

Objectives: To identify factors associated with the surgical outcome in patients undergoing right ventricular outflow tract reconstruction (RVOTR) using decellularized tissue-engineered pulmonary valve conduits (TEPVc) and to study their safety and longevity.

Methods: From April 2006 to April 2010, 93 patients underwent either palliative or corrective RVOTR using Matrix P (37) and Matrix P Plus (56) xenogenic decellularized TEPVc (size range 11-27 mm). Median age and weight at operation were 20 (0.

Determinants of outcome after surgical treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.

Objectives: Identification of variables influencing surgical outcome in patients treated for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.

Methods: A total of 90 consecutive patients (median age, 12 months; range, 20 days to 35 years), who had primarily undergone either 1-stage unifocalization (n = 69) or palliation to promote native pulmonary arterial development (n = 21), were studied. Chromosome 22q11 deletion had occurred in 37% of the cases.

Unifocalization and repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.

Aim: To correlate anatomic and genetic features of paediatric patients with pulmonary atresia, ventricular septal defect (VSD) and multiple aortopulmonary collateral arteries with surgical outcome.

Methods: 44 consecutive patients aged 33 +/- 40 mo underwent either primary one-stage unifocalization (n = 32) or palliative right ventricular outflow tract reconstruction (n = 12) followed by secondary unifocalization and repair (n = 10) based on preoperative morphometric and functional evaluation of pulmonary blood sources. Chromosome 22q11.

Multiresolution analysis of heart rate variability as investigational tool in experimental fetal cardiac surgery.

Multiresolution analysis of heart rate variability derived from aortic blood pressure, acquired before and after (30 and 60 min) experimental fetal cardiac bypass performed on five ewe's fetuses, was used to investigate the physiological response to an invasive clinical approach. Tachograms were implemented and analyzed by wavelet transform in order to verify the existence of a quantitative relationship between arterial blood gases and time series in the very-low (0.021 View Article and Find Full Text PDF