Patient-specific three-dimensional aortic arch modeling for automatic measurements: clinical validation in aortic coarctation.

Aim: A validated algorithm for automatic aortic arch measurements in aortic coarctation (CoA) patients could standardize procedures for clinical planning.

Methods: The model-based assessment of the aortic arch anatomy consisted of three steps: first, machine-learning-based algorithms were trained on 212 three-dimensional magnetic resonance (MR) data to automatically allocate the aortic arch position in patients and segment the aortic arch; second, for each CoA patient (N = 33), the min/max aortic arch diameters were measured using the proposed software, manually and automatically, from noncontrast-enhanced three-dimensional steady-state free precession MRI sequence at five selected sites and compared ('internal comparison' referring to the same environment); third, moreover, the same min/max aortic arch diameters were compared, obtaining them independently, manually from common MR management software (MR Viewforum) and automatically from the model (external comparison). The measured sites were: aortic sinus, sino-tubular junction, mid-ascending aorta, transverse arch and thoracoabdominal aorta at the level of the diaphragm.

Interventional VSD-Closure with the Nit-Occlud Lê VSD-Coil in 110 Patients: Early and Midterm Results of the EUREVECO-Registry.

In August 2010, the Nit-Occlud Lê (EUREVECO) became available for transcatheter coil occlusion of ventricular septal defects (VSDs). Retrospective European Registry for VSD Closure using the Nit-Occlud Lê-VSD-Coil; analysis of the feasibility, results, safety and follow-up of VSD-closure over a 3-year period in 18 European centers. In 102 of 111 patients (female 66), successful VSD closure was performed (mean age 8.

Incidence of Respiratory Disease During the First Two Years of Life in Children with Hemodynamically Significant Congenital Heart Disease in Italy: A Retrospective Study.

Children affected by hemodynamically significant congenital heart disease (HSCHD) experience severe respiratory complications that can increase the frequency of hospitalizations. The aim of the SINERGY study was to describe the incidence of respiratory diseases and to collect information on active and passive immunoprophylaxis in the first 2 years of life. In this retrospective, multicenter, and epidemiologic study, children with HSCHD were enrolled across 11 Italian sites.

MRI as a tool for non-invasive vascular profiling: a pilot study in patients with aortic coarctation.

Aim: While the overall concept of aortic coarctation has changed from one of simple obstruction to one that includes significant vascular dysfunction, this has not yet been translated into the diagnostic and treatment process. To close this gap, we sought to demonstrate the usefulness of an additional non-invasive vascular profile.

Method: During a pilot study in eight coarctation patients, aortic area compliance, aortic distensibility, time phase shift and blood flow (distribution) were calculated from cine-MRI and 2D-/4D-velocity-encoded MRI sequences.

Coronary artery ectasia in Noonan syndrome: Report of an individual with SOS1 mutation and literature review.

Noonan syndrome (NS) is the second most frequent hereditary syndrome with cardiac involvement. Pulmonary valve stenosis and hypertrophic cardiomyopathy are the most prevalent cardiovascular abnormalities. We report on a 14-year-old girl with NS due to SOS1 mutation with pulmonary stenosis and idiopathic coronary ectasia.

Left Ventricular Mass Indexing in Infants, Children, and Adolescents: A Simplified Approach for the Identification of Left Ventricular Hypertrophy in Clinical Practice.

Objective: To determine a simplified method to identify presence of left ventricular hypertrophy (LVH) in pediatric populations because the relationship between heart growth and body growth in children has made indexing difficult for younger ages.

Study Design: Healthy children (n = 400; 52% boys, 0-18 years of age) from 2 different European hospitals were studied to derive a simplified formula. Left ventricular mass (LVM) was calculated according to the Devereux formula.

Endomyocardial biopsy safety and clinical yield in pediatric myocarditis: An Italian perspective.

Objectives: The objective of this investigation is to evaluate the safety, the impact of endomyocardial biopsy (EMB) results in myocarditis management and the incidence of different etiologies of myocarditis in a pediatric population.

Background: Although EMB is an established diagnostic tool to evaluate suspected myocarditis, there is lack of clear diagnostic and management guidelines for myocarditis in pediatric patients, particularly in infants.

Methods: We performed a retrospective database review and subsequent outcomes analysis from five Italian pediatric cardiology centers to identify patients aged 0-18 years who underwent EMB for suspected myocarditis or inflammatory cardiomyopathy (ICMP) between 2009 and 2011.

Optical coherence tomography for characterization of cardiac allograft vasculopathy in late survivors of pediatric heart transplantation.

Background: Optical coherence tomography (OCT) has been shown to reliably detect cardiac allograft vasculopathy (CAV). In recent studies performed in adult heart transplant (HTx) recipients, OCT revealed the presence of vulnerable plaques and complicated coronary artery lesions, thus challenging the current concept that CAV disease is a diffuse concentric and fibrosing vasculopathy. The aim of our study was to characterize CAV by OCT in a young population of HTx recipients.

Tailoring the hybrid palliation for hypoplastic left heart syndrome: A simulation study using a lumped parameter model.

The results of Hybrid procedure (HP) for the hypoplastic left heart syndrome (HLHS) depend on several variables: pulmonary artery banding tightness (PAB), atrial septal defect size (ASD) and patent ductus arteriosus stent size (PDA). A HP complication could be the aortic coarctaction (CoAo). The reverse Blalock-Taussig shunt (RevBT) placement was proposed to avoid CoAo effects.

Advanced Parameters of Cardiac Mechanics in Children with CKD: The 4C Study.

Background And Objectives: Newer parameters of cardiac mechanics provide additional insights on cardiac dysfunction in adult patients with CKD. The aim of this study was to identify prevalence of subclinical abnormalities in cardiac function through the analysis of novel indices of cardiac mechanics in a large population of children with CKD.

Design, Setting, Participants, & Measurements: Between 2009 and 2011, the prospective observational Cardiovascular Comorbidity in Children with CKD Study enrolled patients with CKD ages 6-17 years old with eGFR=10-45 ml/min per 1.

Noninvasive hemodynamic assessment, treatment outcome prediction and follow-up of aortic coarctation from MR imaging.

Purpose: Coarctation of the aorta (CoA) is a congenital heart disease characterized by an abnormal narrowing of the proximal descending aorta. Severity of this pathology is quantified by the blood pressure drop (△P) across the stenotic coarctation lesion. In order to evaluate the physiological significance of the preoperative coarctation and to assess the postoperative results, the hemodynamic analysis is routinely performed by measuring the △P across the coarctation site via invasive cardiac catheterization.

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.

Imaging modalities in children with vascular ring and pulmonary artery sling.

Purpose: Our aim is to compare new non-invasive imaging modalities in the evaluation of vascular ring (VR) and pulmonary artery sling (PAS) and to understand the role of bronchoscopy in comparison with them in assessing tracheobronchial tree.

Methods: We have retrospectively analyzed the data from 41 patients with a VR or a PAS diagnosed at Bambino Gesù Children's Hospital of Rome, between 2008 and 2012. Age, gender, presenting symptoms, clinical history, comorbidities, imaging modalities used for diagnosis (cardiac magnetic resonance [CMR], computed tomography [CT], tracheobronchoscopy [TB]) and surgical treatment were recorded.

"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.

Natural history and clinical outcome of "uncorrected" scimitar syndrome patients: a multicenter study of the italian society of pediatric cardiology.

Introduction And Objectives: To analyze the clinical status of patients with "uncorrected" scimitar syndrome in a multicenter Italian study.

Methods: The natural history of scimitar syndrome was analyzed in 44 affected individuals (from 9 Italian centers).

Results: The median age at diagnosis was 1.

Holt-Oram syndrome with intermediate atrioventricular canal defect, and aortic coarctation: functional characterization of a de novo TBX5 mutation.

Holt-Oram syndrome (HOS) is a rare autosomal dominant disorder characterized by upper limb defects and congenital heart defects (CHD), which are often simple septal and conduction defects, less frequently complex CHDs. We report on a 9 year-old boy with clinical and radiologic features of HOS consisting of bilateral asymmetric hypoplastic thumbs, generalized brachydactyly, limited supination due to radioulnar synostosis, and sloping shoulders, and intermediate atrioventricular canal defect (AVCD) with aortic coarctation. A de novo, previously described mutation, (Arg279ter) was identified in the TBX5 gene.

Early left ventricular abnormality/dysfunction in obese children affected by NAFLD.

Background And Aims: Although it is generally accepted that non alcoholic fatty liver disease (NAFLD) is linked to increased risk of cardiovascular disease, the presence of abnormalities in cardiac function among NAFLD children is limited and controversial. Aim of the study was to detect cardiac abnormalities/dysfunction in a paediatric population of NAFLD.

Methods And Results: Anthropometric, laboratory, cardiovascular fitness, 24 h blood pressure monitoring and Doppler echocardiography parameters were obtained in 50 untreated children (37 males; mean age 12.

[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.

Coronary plaque composition assessed by intravascular ultrasound virtual histology: association with long-term clinical outcomes after heart transplantation in young adult recipients.

Objectives: To assess coronary plaque composition by virtual histology intravascular ultrasound (VH-IVUS) analysis in young adult recipients and to correlate these findings with time from heart transplant (HTx) and long-term outcomes.

Background: Rapid progression of coronary allograft vasculopathy after heart transplantation is a powerful predictor of mortality and clinical events at long-term.

Methods: Forty consecutive young adult recipients transplanted during childhood undergoing VH-IVUS during coronary surveillance have been prospectively included in this study.

Percutaneous treatment of abdominal coarctation in children using a covered stent.

Coarctation of the abdominal aorta is extremely rare. It generally involves a long segment of the descending aorta and causes uncontrolled and unexplainable hypertension in children. The therapeutic choice is very challenging because acute and chronic complications are reported for both the surgical and the percutaneous approaches.

Computational modelling of the right ventricle in repaired tetralogy of Fallot: can it provide insight into patient treatment?

Aims: Pulmonary regurgitation (PR) causes progressive right ventricle (RV) dilatation and dysfunction in repaired tetralogy of Fallot (rToF). Declining RV function is often insidious and the timing of pulmonary valve replacement remains under debate. Quantifying the pathophysiology of adverse RV remodelling due to worsening PR may help in defining the best timing for pulmonary valve replacement.

Change in cardiac geometry and function in CKD children during strict BP control: a randomized study.

Background And Objectives: Left ventricular hypertrophy (LVH) and abnormal systolic function are present in a high proportion of children with CKD. This study evaluated changes in left ventricular (LV) geometry and systolic function in children with mild to moderate CKD as an ancillary project of the Effect of Strict Blood Pressure Control and ACE Inhibition on Progression of Chronic Renal Failure in Pediatric Patients trial.

Design, Setting, Participants, & Measurements: Echocardiograms and ambulatory BP monitoring were performed at baseline and at 1- or 2-year follow-up in 84 patients with CKD and 24-hour mean BP above the 50th percentile and/or receiving fixed high-dose angiotensin converting enzyme inhibition and randomized to conventional or intensified BP control.

Ventricular assist devices as a bridge to heart transplantation or as destination therapy in pediatric patients.

Purpose: Despite the remarkable advances with the use of ventricular assist devices (VAD) in adults, pneumatic pulsatile support in children is still limited. We report on our experience in the pediatric population.

Methods: Retrospective review of 27 consecutive children offered mechanical support with Berlin Heart as a bridge to heart transplant, and Jarvik 2000 as a destination therapy from February 2002 to October 2011.