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Cardiovascular magnetic resonance (CMR) and computed tomography (CCT) are advanced imaging modalities that recently revolutionized the conventional diagnostic approach to congenital heart diseases (CHD), supporting echocardiography and often replacing cardiac catheterization. This is the second of two complementary documents, endorsed by experts from the Working Group of the Italian Society of Pediatric Cardiology and the Italian College of Cardiac Radiology of the Italian Society of Medical and Interventional Radiology, aimed at giving updated indications on the appropriate use of CMR and CCT in different clinical CHD settings, in both pediatrics and adults. In this article, support is also given to radiologists, pediatricians, cardiologists, and cardiac surgeons for indications and appropriateness criteria for CMR and CCT in the most referred CHD, following the proposed new criteria presented and discussed in the first document. This second document also examines the impact of devices and prostheses for CMR and CCT in CHD and additionally presents some indications for CMR and CCT exams when sedation or narcosis is needed., (Copyright © 2024 Italian Federation of Cardiology - I.F.C. All rights reserved.)

We sought to evaluate the potential clinical role of 4D-flow cardiac magnetic resonance (CMR)-derived energetics and flow parameters in a cohort of patients' post-Fontan palliation. In patients with Fontan circulation who underwent 4D-Flow CMR, streamlines distribution was evaluated, as well a 4D-flow CMR-derived energetics parameters as kinetic energy (KE) and energy loss (EL) normalized by volume. EL/KE index as a marker of flow efficiency was also calculated. Cardiopulmonary exercise test (CPET) was also performed in a subgroup of patients. The population study included 55 patients (mean age 22 ± 11 years). The analysis of the streamlines revealed a preferential distribution of the right superior vena cava flow for the right pulmonary artery (62.5 ± 35.4%) and a mild preferential flow for the left pulmonary artery (52.3 ± 40.6%) of the inferior vena cave-pulmonary arteries (IVC-PA) conduit. Patients with heart failure (HF) presented lower IVC/PA-conduit flow (0.75 ± 0.5 vs 1.3 ± 0.5 l/min/m 2 , p = 0.004) and a higher mean flow-jet angle of the IVC-PA conduit (39.2 ± 22.8 vs 15.2 ± 8.9, p < 0.001) than the remaining patients. EL/KE index correlates inversely with VO 2 /kg/min: R: - 0.45, p = 0.01 peak, minute ventilation (VE) R: - 0.466, p < 0.01, maximal voluntary ventilation: R:0.44, p = 0.001 and positively with the physiological dead space to the tidal volume ratio (VD/VT) peak: R: 0.58, p < 0.01. From our data, lower blood flow in IVC/PA conduit and eccentric flow was associated with HF whereas higher EL/KE index was associated with reduced functional capacity and impaired lung function. Larger studies are needed to confirm our results and to further improve the prognostic role of the 4D-Flow CMR in this challenging population., (© 2024. The Author(s).)

Background: Sternal cleft (SC), a rare thoracic malformation, is associated with pectus excavatum (PE) in 2.6-5% of cases. It remains unclear if these conditions are congenitally linked or if SC repair triggers PE. To investigate the potential higher frequency of PE in SC cases, we conducted a retrospective study of our SC patients., Methods: We assessed PE incidence, progression, and management in SC patients treated at our institute from 2006 to 2022. When available, we collected pre-SC repair CT scan data, calculating the Haller Index (HI) and Correction Index (CI) and compared them to a selected control group., Results: Among 8 SC patients, 7 had concomitant PE (87.5%), varying in severity. PE management ranged from observation to thoracoplasty, depending on its degree. We observed a significant pre-operative CI difference between SC and control group patients (p < 0.00001). In the last two SC repair cases, we attempted concurrent PE prevention or treatment., Conclusion: Our findings suggest an underestimated association between PE and SC in the existing literature. SC patients may exhibit a predisposition to PE from birth, which may become more apparent with growth after SC repair. Consequently, PE prevention or treatment should be considered during SC repair procedures., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Managing repaired tetralogy of Fallot (TOF) patients is still challenging despite the fact that published studies identified prognostic clinical or imaging data with rather good negative predictive accuracy but weak positive predictive accuracy. Heterogeneity of the initial anatomy, the surgical approach, and the complexity of the mechanism leading to dilation and ventricular dysfunction explain the challenge of predicting the adverse event in this population. Therefore, risk stratification and management of this population remain poorly standardized. Design: The CMR/CT WG of the Italian Pediatric Cardiology Society set up a multicenter observational clinical database of repaired TOF evaluations. This registry will enroll patients retrospectively and prospectively assessed by CMR for clinical indication in many congenital heart diseases (CHD) Italian centers. Data collection in a dedicated platform will include surgical history, clinical data, imaging data, and adverse cardiac events at 6 years of follow-up. Summary: The multicenter repaired TOF clinical database will collect data on patients evaluated by CMR in many CHD centers in Italy. The registry has been set up to allow future research studies in this population to improve clinical/surgical management and risk stratification of this population.

Following current practice, pediatric patients with treated congenital coronary malformations or acquired coronary disease undergo Cardio-Pulmonary Exercise Test (CPET), stress Echocardiography and Electrocardiography (sEcho, sEKG), and Coronary Angiography (CA). Stress cMRI can assess cardiac function, myocardial viability, and stress/rest perfusion deficit-without radiation exposure, general anesthesia, and hospitalization-in a single non-invasive exam. The aim of our pilot study is to assess the feasibility and diagnostic accuracy of Dobutamine stress cMRI compared to the current procedures (sEcho, CPET, CA). The prospective study is focused on pediatric patients, at risk for or with previously diagnosed coronary artery disease: d-looped TGA after arterial Switch, Kawasaki disease, and anomalous origin of left coronary artery from pulmonary artery (ALCAPA) after coronary artery reimplantation. We have compared the results of MRI coronary angiography, and Dobutamine stress cMRI with traditional tests. All these diagnostic exams were acquired in a timeframe of 3 month, in a blinded fashion. All the 13 patients (age: 12 ± 2 years, median 12,7 y) recruited, completed the study without major adverse events. The mean heart rate-pressure product was 25,120 ± 5110 bpm x mm Hg. The target heart rate of 85% of the maximal theoretical was reached by 10 (77%) patients. The comparison between cardiac MRI coronarography versus the gold standard Coronary Angiography to identify the patency of the origin and the proximal pathway of the coronary arteries shows a sensitivity of 100% (confidence interval: 2,5-100%), specificity 92% (confidence interval: 64-100%). The stress test was well tolerated for the 77% of the patients and completed by the totality of patients (Table 3). Three patients (23%) had mild symptoms: nausea, vomiting, or general discomfort. In pediatric patients with a potential or definite diagnosis of coronary artery disease, stress cMRI combines an effective assessment of proximal coronary arteries anatomy with cardiac function, myocardial perfusion, and viability in a single examination. Stress cMRI can be proposed as alternative, standalone test., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Introduction:  Jeune's syndrome, or asphyxiating thoracic dystrophy (ATD), is a rare autosomal recessive disorder characterized by skeletal dysplasia. Ribs are typically short and horizontal resulting-in lethal variant-in severe lung hypoplasia, progressive respiratory failure, and death. Lateral thoracic expansion (LTE) consists in staggered bilateral ribs osteotomy leading to chest expansion and lung development. Studies on LTE in ATD patients report encouraging data, but the rarity of ATD implies the lack of a standardized surgical path. The aim of this report is to present our experience with LTE, the technical modification we adopted, and patients' clinical outcome., Materials and Methods:  We retrospectively reviewed data of 11 LTE performed in 7 ATD patients with lethal variant. Information regarding pre- and postoperative clinical conditions and surgical details was collected. We adopted a single-stage or a two-stage approach based on patient clinical condition. Computed tomography (CT) scan was performed before and after surgery and lung volume was calculated., Results:  Five patients are alive, while two died in intensive care unit for other than respiratory cause (sepsis). Most patients experienced clinical improvement in terms of decreased respiratory infections rate, need for ventilation, and improved exercise tolerance. Postoperative CT scan demonstrated a median lung volume increase of 88%., Conclusion:  Mortality in ADT patients is high. However, LTE is a feasible and safe surgical approach, which could improve clinical conditions and survival rate. Survived patients showed postoperatively less oxygen requirement and improved clinical conditions., Competing Interests: None declared., (Thieme. All rights reserved.)

Background: Sengers syndrome is characterized by congenital cataract, hypertrophic cardiomyopathy, mitochondrial myopathy, and lactic acidosis associated with mutations in AGK gene. Clinical course ranges from a severe fatal neonatal form, to a more benign form allowing survival into adulthood, to an isolated form of congenital cataract. Thus far few reported cases have survived the second decade at their latest examination, and no natural history data are available for the disease., Case Presentation: Here we provide a 20-year follow-up in two siblings with a benign form of Sengers syndrome, expanding the phenotypical spectrum of the disease by reporting a condition of ovarian agenesis., Conclusion: To our knowledge, this report provides the first longitudinal data of Sengers syndrome patients., (© 2022. The Author(s).)

MIS-C is a multisystem inflammatory syndrome that is characterized by multi-organ failure and cardiac involvement. The aim of this study was to describe the long-term cardiovascular outcome in a cohort of MIS-C pediatric patients, who were admitted to two Italian Pediatric Referral Centers. Sixty-seven patients (mean age 8.7 ± 4.7 years, male 60%) were included; 65 (97%) of them showed cardiac involvement. All of the patients completed one month of the follow-up, and 47% completed 1 year of it. ECG abnormalities were present in 65% of them, arrhythmias were present in 9% of them during an acute phase and it disappeared at the point of discharge or later. Pericarditis were detected in 66% of them and disappeared after 6 months. Coronaritis was observed in 35% of the children during an acute phase, and there were no more instances at the 1-year point. An LV dysfunction was present in 65% of the patients at the beginning of the study, with them having a full recovery at the point of discharge and thereafter. Elevated values of the NTproBNP and hsTp were initially detected, which progressively decreased and normalized at the points of discharge and FU. The CMR at the point of FU, there was a presence of long-term myocardial scars in 50% of the patients that were tested. No deaths that were caused by MIS-C during the FU were recorded. Cardiac involvement in MIS-C patients is almost the rule, but the patients' clinical course was satisfactory, and no additional events or sequelae were observed apart from there being long-term myocardial scars in 50% of the patients that underwent CMR.

Cardiovascular magnetic resonance (CMR) and computed tomography (CCT) are advanced imaging modalities that recently revolutionized the conventional diagnostic approach to congenital heart diseases (CHD), supporting echocardiography and often replacing cardiac catheterization. Nevertheless, correct execution and interpretation require in-depth knowledge of all technical and clinical aspects of CHD, a careful assessment of risks and benefits before each exam, proper imaging protocols to maximize diagnostic information, minimizing harm. This position paper, written by experts from the Working Group of the Italian Society of Pediatric Cardiology and from the Italian College of Cardiac Radiology of the Italian Society of Medical and Interventional Radiology, is intended as a practical guide for applying CCT and CMR in children and adults with CHD, wishing to support Radiologists, Pediatricians, Cardiologists and Cardiac Surgeons in the multimodality diagnostic approach to these patients. The first part provides a review of the most relevant literature in the field, describes each modality's advantage and drawback, making considerations on the main applications, image quality, and safety issues. The second part focuses on clinical indications and appropriateness criteria for CMR and CCT, considering the level of CHD complexity, the clinical and logistic setting and the operator expertise., (© 2022. The Author(s).)

Background: In clinical assessment of Pectus Excavatum (PE), the indication to surgery is based not only on symptoms but also on quantitative markers calculated from Computed Tomography (CT) or Magnetic Resonance Imaging (MRI) scans. According to clinical routine, these indexes are measured manually by radiologists with limited computer support. This process is time consuming and potentially subjected to inaccuracy and individual variability in measurements. Moreover, the existing indexes have limitations, since they are based on linear measurements performed on single slices rather than on volumetric data derived from all the thoracic scans., Results: In this paper we present an image processing pipeline aimed at providing radiologists with a computer-aid tool in support of diagnosis of PE patients developed in MATLAB® and conceived for MRI images. This framework has a dual purpose: (i) to automatize computation of clinical indexes with a view to ease and standardize pre-operative evaluation; (ii) to propose a new marker of pathological severity based on volumetric analysis and overcoming the limitations of existing axial slice-based indexes. Final designed framework is semi-automatic, requiring some user interventions at crucial steps: this is realized through a Graphical User Interface (GUI) that simplifies the interaction between the user and the tools. We tested our pipeline on 50 pediatric patients from Gaslini Children's Hospital and performed manual computation of indexes, comparing the results between the proposed tool and gold-standard clinical practice. Automatic indexes provided by our algorithm have shown good agreement with manual measurements by two independent readers. Moreover, the new proposed Volumetric Correction Index (VCI) has exhibited good correlation with standardized markers of pathological severity, proving to be a potential innovative tool for diagnosis, treatment, and follow-up., Conclusions: Our pipeline represents an innovative image processing in PE evaluation, based on MRI images (radiation-free) and providing the clinician with a quick and accurate tool for automatically calculating the classical PE severity indexes and a new more comprehensive marker: the Volumetric Correction Index., (© 2022. The Author(s).)

Background: Congenital and acquired airway anomalies represent a relatively common albeit diagnostic and therapeutic challenge. Obtaining maximum definition of the abnormality is imperative prior to attempting surgical procedure because some tracheal lesions have a significant risk of mortality. Are Tracheobronchography (TBG) and Tracheobronchofluoroscopy (TBF) valuable or obsolete tecniques?, Methods: We retrospectively reviewed all the diagnostic and interventional TBG-TBF requested by the multidisciplinary conference, during the last 10 years in a tertiary care hospital exclusively dedicated to pediatric patients., Results: A total of 268 procedures performed in 60 pediatric patients (68% male, mean age 4,8 years), were reviewed. 41 diagnostic TBG-TBF were performed in a group of 34 patients with excellent result, without complications. A total of 175 procedures of tracheobronchoplasty guided by TBG-TBF were completed in a group of 25 patients. Seven bioabsorbable self-expanding stents were placed in the airway of 6 children., Conclusions: Diagnostic TBG and TBF are still irreplaceable tools to evaluate pediatric airway disease, with many advantages over the newest imaging techniques. Interventional procedures of pediatric airways under the guide of TBGTBF represent safe and effective treatment options in selected patients, with positive clinical impact.

Background: The sternal lift by Vacuum Bell (VB) is effective, as largely demonstrated by its intraoperative use during surgical procedure to elevate the sternum during the Nuss procedure routinely. Indeed, the thoracic remodelling during VB application is comparable to post-surgical scenario, and suitable to compare cardiovascular parameters of the two different thoracic configurations immediately., Objective: We would quantify and correlate preoperative parameters which determine the severity of the pectus excavatum (PE), and the cardiovascular effects at the baseline. Than we would assess the cardiovascular changes during VB positioning, mimicking the immediate, temporary effect of Pectus-correction., Materials and Methods: We included 26 consecutive patients (mean age is 13,3 +/- 2,2 years) symptomatic and non, with a previous clinical diagnosis of PE. CMR was performed before and during application of VB, using the same imaging protocol. In both conditions, we measured thoracic indexes, and cardiac function as well as flow through main vessels., Results: Mean expiratory Haller Index (HI) was 5,4 (+/-1,4 SD; normal <3). During VB application, all patients showed improvement in the main morphologic parameters of the thorax (mean expiratory HI = 4,7 (+/-1,6 SD, delta -13%, P = 0,01). During VB application, a minimal but not significant increase of Right Ventricle End Diastolic Volume (RVEDVi) (delta +4,6%, P = 0,12), and Right Ventricle Ejection Fraction (RVEF) (delta +1,2%, P = 0,2) was observed., Conclusion: In adolescents affected by PE, cardiacMRI (CMR) demonstrates normal values of biventricular volume and systolic function. During VB application, beside significative improvements in chest wall anatomy, CMR shows a minimal positive variation in right ventricle volume and function. A minority of patients showed some degree of diastolic dysfunction at baseline, unchanged after VB application, with possible correlation between valve inflow and sternal impingement., Competing Interests: Declaration of Competing Interest The authors declare that they have no conflict of interest, (Copyright © 2020 Elsevier Inc. All rights reserved.)

Teamwork is one of the most important trend in modern medicine. Airway team were created in many places to respond in a multidisciplinary and coordinated way to challenging clinical problems which were beyond the possibility of an individual management. In this chapter, we illustrate the historical steps leading to the development of an airway team in a pediatric referral hospital, describe the present teamwork activity defining the key points for the creation of a team and discussing different organization models; finally we delineate possible future directions for the airway teams in the globalized world., (Copyright © 2021. Published by Elsevier Inc.)

Extraosseous Ewing sarcoma of primary cardiac origin is an extremely rare variety among pediatric cardiac neoplasms. We report a case of extraosseous Ewing sarcoma of primary cardiac origin in a 9-year-old girl, treated with debulking surgery, adjuvant chemotherapy, and radiotherapy., Competing Interests: The authors declare no conflict of interest., (Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Pectus Excavatum is the most common deformation of the skeletal chest wall, and the Nuss procedure is used to correct this deformation. Residual pneumothorax is a frequent postoperative complication after thoracoscopy. Primary aim of this prospective observational study was to compare the level of agreement among readers using lung ultrasound and chest X-ray., Methods: This was an inter-reader single-center prospective observational agreement study for lung ultrasound and chest X-ray. Three lung ultrasound evaluations were performed at the end of surgery in the operating room by two Consultant Anesthesiologist (A1 and A2) and by a medical student (A3). Chest X-ray was interpreted by a consultant Radiologist (R1) and a pool of 11 radiologists (collectively named R2). Cohen's kappa was used to evaluate the level of agreement between the two imaging techniques., Results: Sixty-eight pediatric patients were included in the study. The overall agreement among (A = Anesthesiologist) A1, A2, A3, (R = Radiologist) R1, and R2 in assessing pneumothorax was fair (k = 0.32; 95% CI 0.21-0.40). The stratified analysis showed moderate inter-reader agreement among lung ultrasound readers A1, A2, and A3 (k 0.58; 95% CI 0.44-0.71), as opposed to the fair agreement found among chest X-ray readers R1 and R2 (k = 0.39; 95% CI 0.18-0.60)., Conclusions: Our results support the use of lung ultrasound to diagnose pneumothorax after Nuss procedure. We found that the inter-reader accuracy was better with ultrasound compared to X-ray at our institution., (© 2020 John Wiley & Sons Ltd.)

Advances in the treatment have drastically increased the survival rate of congenital heart disease (CHD) patients. Therefore, the prevalence of these patients is growing. Imaging plays a crucial role in the diagnosis and management of this population as a key component of patient care at all stages, especially in those patients who survived into adulthood. Over the last decades, noninvasive imaging techniques, such as cardiac magnetic resonance (CMR) and cardiac computed tomography (CCT), progressively increased their clinical relevance, reaching stronger levels of accuracy and indications in the clinical surveillance of CHD. The current review highlights the main technical aspects and clinical applications of CMR and CCT in the setting of congenital cardiovascular abnormalities, aiming to address a state-of-the-art guidance to every physician and cardiac imager not routinely involved in the field.

Objectives: The aim of our study is to report a case of a child with subglottic thymus that was suspected during diagnostic work-up for severe airway obstruction, excised surgically and confirmed with final histopathological examination. Moreover, we performed a narrative literature review to outline clinical and diagnostic features of this rare condition and to report suggestions for the management of subglottic masses., Methods: We report the case of a 7-month-old boy who was admitted to our Pediatric Airway Team Unit due to a history of worsening biphasic stridor and recurrent episodes of upper airway obstruction. The successful diagnostic work-up and a narrative literature of analogous cases of subglottic thymus were reported., Results: Ectopic thymus is a very rare condition in which thymic tissue is found outside the normal pathway of its embryonic migration. It usually presents as a cystic or, more rarely, solid mass, showing an indolent course toward spontaneous involution. In some cases, however, it becomes symptomatic exerting compression on surrounding vital structures. Due to its rarity, the initial diagnosis is normally mistaken with inflammatory diseases or malignancies and the definitive diagnosis is only achieved after histological examination of the excised specimen. To our knowledge, only four other cases of subglottic ectopic thymic tissue have been reported in the English literature so far and the diagnosis has never been suspected preoperatively., Conclusion: It is mandatory to consider ectopic thymic tissue in the differential diagnosis in children presenting with airways obstruction in order to prevent unnecessary, extensive, and exploratory surgery.

Background: Stents are commonly used to treat aortic coarctation. The objective of this study was to evaluate the post-implantation computed tomography (CT) image quality of different stent types used to treat aortic coarctation., Methods: Adult and paediatric patients with stent-treated aortic coarctation who underwent contrast-enhanced CT were retrospectively included from three tertiary care centres. CT scans were subjectively scored for image quality using a 4-point scale (1 = unacceptable; 2 = poor; 3 = good; 4 = excellent). Furthermore, the amount of stent-induced blooming artefacts was measured as the percentage of the difference between outer and inner stent diameters over the outer stent diameter., Results: A total of 35 children and 34 adults implanted with 71 stents of six different types were included. The most commonly used stent type was the Cheatham Platinum stent (52 stents, 73%). The subjective image quality of the Cheatham Platinum stents was moderate with a score of 2.0±0.8 (mean ± standard deviation) in children and 2.3±0.6 in adults. The image quality in patients with Formula stents was 2.3±1.2. The Cheatham Platinum stents induced 34-48% blooming, the Formula stents 44-55%. The image quality in patients with the less commonly used Atrium Advanta V12, IntraStent, AndraStent and Palmaz stents was scored 3 (good) to 4 (excellent) with less blooming. The electrocardiographic gating and tube voltage (kVp) did not affect image quality., Conclusions: There is a substantial variation in CT image quality and blooming artefacts for different stent types used to treat aortic coarctation.

Stent placement is commonly used to treat aortic coarctation. Although invasive angiography remains the gold standard, follow-up is often performed using computed tomography, which allows rapid, noninvasive assessment of the aorta and surrounding tissues. The goal of this pictorial essay is to provide a guide to the interpretation of these examinations. Normal and abnormal computed tomographic appearance of different stent types is shown along with reconstructions that can help assess stent integrity and the stent position in relation to the aortic wall and branches. Furthermore, imaging findings of complications including aortic wall injuries, restenosis, and intimal hyperplasia are depicted.

Congenital and acquired airway anomalies represent a relatively common albeit diagnostic and therapeutic challenge, even for the most skilled operators in dedicated centers. Airway malformations encompass a wide spectrum of pathologies involving the larynx, trachea and bronchi, esophagus, mediastinal vessels. These developmental lesions are often isolated but the association of two or more anomalies is not infrequent. From the traditional chest X-ray to the newest applications of Optical Coherence Tomography, non- or mini-invasive diagnostic techniques represent useful tools to integrate invasive procedures. Comprehensive knowledge of the characteristics of each diagnostic test is mandatory for its useful application. The aim of our paper is to analyze the clinical indications for Imaging the Airway disease in pediatric population, and describe the diagnostic techniques. Only by a close interaction between all the operators involved in diagnosis and treatment of pediatric airway, as it happens in Multidisciplinary Airway Team, the non- or mini-invasive imaging is effective.

Left ventricle (LV) systolic dysfunction in repaired tetralogy of Fallot (TOF) has been identified as a risk factor for functional status and adverse outcome. The aims of this cross-sectional followed by a prospective study were: (1) to evaluate the prevalence of LV systolic dysfunction in a large cohort of adults with repaired tetralogy of Fallot, (2) to test the relationship between LV systolic dysfunction and other known risk factors and (3) to evaluate the impact of LV systolic dysfunction on adverse cardiac events. In a multicenter study, 237 adults repaired TOF (58 % males, age 30 ± 10 years) were evaluated by cardiac magnetic resonance (CMR). Demographics, surgical history, ECG, Echo-Color Doppler and follow-up data were recorded. LV was dilated (Z value >2) in 16 patients (6 %), however 56 patients (23.6 %) had a reduced LV systolic function left ventricle ejection fraction (LVEF) (Z value <-2). Patients with LV systolic dysfunction were mainly males (82 %), had reduced right ventricle ejection fraction (RVEF), and higher right and left Late Gadolinium Enhanced scores. In a multivariate regression analysis male gender and RVEF resulted to be independent factors associated to LV systolic dysfunction. Atrial arrhythmias were the main adverse cardiac event at the follow-up and were associated to higher biventricular volumes and lower biventricular ejection fraction (EF); however multivariable analysis identified age, right ventricle end-diastolic volume (RVEDVi) and tricuspid regurgitation as independents factors associated to atrial arrhythmias. At long term follow-up at least ¼ of repaired TOF has LV dysfunction. Lower LVEF is associated to male gender and lower RVEF.

Background: Congenital and acquired airway anomalies represent a relatively common albeit challenging problem in a national tertiary care hospital. In the past, most of these patients were sent to foreign Centres because of the lack of local experience in reconstructive surgery of the paediatric airway. In 2009, a dedicated team was established at our Institute. Gaslini's Tracheal Team includes different professionals, namely anaesthetists, intensive care specialists, neonatologists, pulmonologists, radiologists, and ENT, paediatric, and cardiovascular surgeons. The aim of this project was to provide these multidisciplinary patients, at any time, with intensive care, radiological investigations, diagnostic and operative endoscopy, reconstructive surgery, ECMO or cardiopulmonary bypass. Aim of this study is to present the results of the first year of airway reconstructive surgery activity of the Tracheal Team., Methods: Between September 2009 and December 2010, 97 patients were evaluated or treated by our Gaslini Tracheal Team. Most of them were evaluated by both rigid and flexible endoscopy. In this study we included 8 patients who underwent reconstructive surgery of the airways. Four of them were referred to our centre or previously treated surgically or endoscopically without success in other Centres., Results: Eight patients required 9 surgical procedures on the airway: 4 cricotracheal resections, 2 laryngotracheoplasties, 1 tracheal resection, 1 repair of laryngeal cleft and 1 foreign body removal with cardiopulmonary bypass through anterior tracheal opening. Moreover, in 1 case secondary aortopexy was performed. All patients achieved finally good results, but two of them required two surgeries and most required endoscopic manoeuvres after surgery. The most complex cases were the ones who had already been previously treated., Conclusions: The treatment of paediatric airway anomalies requires a dedicated multidisciplinary approach and a single tertiary care Centre providing rapid access to endoscopic and surgical manoeuvres on upper and lower airways and the possibility to start immediately cardiopulmonary bypass or ECMO.The preliminary experience of the Tracheal Team shows that good results can be obtained with this multidisciplinary approach in the treatment of complicated cases. The centralization of all the cases in one or few national Centres should be considered.