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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.)

Rapid dehydration of maize grain is one of the main characteristics of cultivar selection for mechanical grain harvest; however, the dominant driving forces and mechanisms of grain dehydration before physiological maturity remain disputable and obscure. This study compared the grain moisture content and dehydration rate of coated treatment (no surface evaporation) and control grains. Meanwhile, the xylem-mobile dye was infused from stem and cob, and its movement was observed in cob, ear-stalk and stem xylem. The development dynamics of husk, grain and cob were analyzed to determine the mechanism of grain dehydration. The results showed that, from grain formation to 5-10 days before physiological maturity, the main driving force of grain dehydration of the early and middle-maturity maize cultivars was filling, followed by surface evaporation. In the dye movement experiment, the movement of the stem-infused xylem-mobile dye through the pedicel xylem was observed during but not after the grain formation period. Moreover, the cob-infused xylem-mobile dye moved to the ear- stalk and the stem via the xylem. There was a significantly positive correlation between grain filling rate and dehydration rate from grain formation to physiological maturity. According to these results, we proposed that in the grain dehydration phase driven by filling, the surplus water in the grain flows back to the cob via the pedicel xylem, of which some flowed back to the plant via the cob and ear- stalk xylem. This provides a new theoretical basis for selecting and breeding maize cultivars suitable for mechanical grain harvesting., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The reviewer JR declared a shared affiliation with the authors to the handling editor at the time of review, (Copyright © 2022 Zhang, Marasini, Li and Zhang.)

We present a case of aortopulmonary window in which the diagnosis of anomalous left coronary artery originating from pulmonary artery was made intra-operatively even if the coronary arteries anatomy was correctly studied pre-operatively with echocardiography. No evidence of coronary anomalies or indirect sings of coronary anomalies has been noted. Should we improve our pre-operative diagnostic accuracy and how?

Objectives: This study aims to determine a combination of third-trimester echocardiographic parameters for improving the prenatal prediction of coarctation of the aorta (CoA) after birth., Methods: We included all cases of suspected CoA during fetal echocardiography performed in the second and/or third trimester of pregnancy at Gaslini Children's Hospital between January 2010 and December 2020. The last prenatal ultrasound evaluation was reviewed considering most of the echocardiographic criteria were already published for prenatal CoA diagnosis. Associated minor cardiac anomalies, such as a ventricular septal defect, persistent left superior vena cava (PLSCV), and redundant foramen ovale (FO) membrane, as well as postnatal outcomes, were reported. Initial perinatal management was defined based on the risk stratification of CoA during prenatal echocardiography. Neonates were divided into two groups depending on the presence or absence of CoA after birth., Results: A total of 91 fetuses with CoA suspicion were selected, of which 27 (30%) were confirmed with CoA after birth and underwent surgical repair. All cardiac parameters except redundant FO membrane and PLSCV showed a significant correlation with CoA. Statistical analysis confirmed that cardiovascular disproportion with right predominance carries an increased risk for occurrence of CoA, especially if already evident during the ultrasound evaluation in the second trimester. Aortic valve (AV) z-score and distal transverse aortic arch (TAA) z-score resulted as the best predictors of CoA after birth. The best cutoff point for CoA discrimination with ROC analysis was an AV z-score of -1.25 and a distal TAA z-score of -0.37. A total of 46% of those without CoA were diagnosed with a cardiac defect, which was not diagnosed in utero, pulmonary hypertension, or a genetic syndrome., Conclusion: The current criteria for diagnosing CoA in utero allow accurate diagnosis of most severe cases but the rate of false positives remains relatively high for milder cases. A combination of anatomic and functional echocardiographic parameters might be used in stratifying the risk of CoA. We proposed the AV and the TAA diameter z-scores as the best predictors of CoA after birth. In addition, neonates without CoA deserve proper monitoring at birth because prenatal evidence of a significant cardiovascular discrepancy between the right and left cardiac structures has an inherent risk for additional morbidity postnatally., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Tuo, Paladini, Marasini, Buratti, De Tonetti, Calevo and Marasini.)

Background: A multidisciplinary study group involving physicians and jurists was established to review and approve an informed consent about the most frequent interventional procedures for congenital heart diseases., Methods: The authors worked together with representatives of the Italian Society of Pediatric Cardiology and Congenital Heart Disease (SICP) Council and Jurist's expert in the field of health case-law. The final draft was shared with the major Italian centers involved in congenital interventional procedures and with AICCA, the Italian Patients Association of Congenital Heart Diseases - Adults and Children., Results: At the end of this review process, a final informed consent form was developed for the most frequent procedures performed in our catheterization laboratories. All of them consist of two parts: a general statement and a procedure-related one., Conclusions: The work performed by this multidisciplinary study group, under the supervision of the SICP, resulted in a new dedicated informed consent about interventional procedures in the field of congenital cardiology, taking into account the new legal requests. This informed consent is intended to be both a document that can be used as such and a document from which to derive a specific document for each center. We believe that using similar informed consents in all Congenital Heart Disease Centers or at least have informed consents all inspired by the same setting, could be a further improvement in taking care of the patients and their families.

Cardiac involvement in multisystem inflammatory syndrome in children (MIS-C) associated with coronavirus-19 disease is often observed with a high risk of heart failure. The aim is to describe cardiovascular involvement, management and early outcome in MIS-C by comparing cardiovascular manifestations in children younger and older than 6 years old. This retrospective observational study included 25 children with MIS-C, admitted to a single pediatric center between March 2020 and September 2021. The median age was 5 years (13 patients under 6 years and 12 over 6 years); coronary artery abnormalities were observed in 77% of preschoolers, with small and medium aneurysms in half of the cases and two cases of mild ventricular dysfunction. School-age children presented myopericardial involvement with mild to moderate ventricular dysfunction in 67% of cases, and two cases of transient coronary dilatation. There was a significant NT-pro-BNP and inflammatory markers increase in 25 of the patients, and mild elevation of troponin I in 9. All patients were treated with intravenous immunoglobulin and corticosteroids, and 8 with anakinra. None of the patients needed inotropes or intensive care unit admission. Our study shows the frequent cardiovascular involvement in MIS-C with a peculiar distribution, according to different age group: coronary artery anomalies were more frequent in the younger group, and myopericardial disease in the older one. A prompt multitarget, anti-inflammatory therapy could probably contribute to a favorable outcome.

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.)

Background: The Cocoon septal occluder (CSO) is a new generation double disk occluder device for catheter closure of the secundum atrial septal defect (ASD). Initial clinical evaluations with the use of this device have shown quite satisfactory results but large follow-up studies are missing. In this international multicenter study, we present procedural and follow-up data from 4008 patients with secundum ASD who underwent catheter closure with the use of CSO., Methods: The study cohort consisted of 1853 pediatric and 2155 adult patients with secundum ASD treated with the CSO. Patients were enrolled retrospectively from 11 international centers and were followed for a mean period of 43 months (range 12-84 months), postprocedural. Clinical, electrocardiographic, echocardiographic, procedural, and follow-up data were collected from each collaborating hospital., Results: The CSO was permanently implanted in 3983 patients (99.4%). Echocardiographic evaluation at one month follow-up revealed complete closure in 99.6% of those patients who had a device implanted. Thrombus formation in one adult patient was the only major device related to procedural complication. During the follow-up period, no patient developed cardiac erosions, allergic reactions to nickel, or other major complications., Conclusions: Implantation of CSO provided satisfactory procedural and follow-up results with high success and no device-related cardiac erosions and nickel allergy., Competing Interests: Declaration of interest None., (Copyright © 2021 Hellenic Society of Cardiology. All rights reserved.)

Objective: The aim of the study is to describe a delivery room intensive care unit (DRICU) model and evaluate its effectiveness in preventing morbidity and mortality in high-risk newborns. Design: This retrospective case series includes all DRICU procedures performed from 2016 to 2020. Setting: Gaslini Children's Hospital is a major pediatric tertiary care center where high-risk pregnancies are centralized. The Neonatal and Pediatric Intensive Care Unit admits every year about 100 high-risk newborns. Patients: The selected patients are newborns at risk of critical conditions immediately after birth for respiratory or cardiovascular congenital disorders. Interventions: The perinatal plan is defined by the multidisciplinary team of Fetal and Perinatal Medicine. The DRICU procedure provides highly specialized care through a protocol that includes logistics, personnel, equipment, and clinical pathways. Main Outcome Measures: The primary outcome is the prevention of acute complications and mortality in the delivery room and early neonatal period. Results: From 2016 to 2020, 40 DRICU procedures were performed. The main prenatal diagnoses included congenital heart disease with a high risk of life-threatening events immediately after birth (38%), congenital diaphragmatic hernia (35%), and fetal hydrops/hydrothorax (23%). Mean gestational age was 35.9 weeks (range: 31-39), and mean birth weight was 2,740 grams (range: 1,480-3,920). DRICU assistance completed in all patients by neonatal intensivists included tracheal intubation and arterial and central venous cannulation; complex procedures such as ex-utero intrapartum technique and extracorporeal membrane oxygenation cannulation are described. No deaths nor severe acute complications occurred in the delivery room or in the immediate postnatal period. Conclusions: The outcome in critical newborns is potentially affected by planned assistance strategies and specialized competencies through the implementation of a DRICU protocol., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Buratti, Lampugnani, Faggiolo, Buffoni, Paladini, De Tonetti, Tuo, Marasini, Mattioli and Moscatelli.)

Coronary ostial stenosis is a rare congenital cardiac anomaly, frequently associated with hypoplasia of the proximal coronary artery. This condition is potentially life-threatening, as it may present with myocardial ischemia and sudden death. We present a case of left coronary ostial stenosis in a 48-day-old infant symptomatic for sudden cardiac arrest, who successfully underwent surgical angioplasty. Any cardiac arrest in a neonate or young infant should raise suspicion of coronary ostial stenosis/atresia, considering the difficulty in diagnosing this congenital heart defect.

Background: Despite recent technical advances, interventional cardiac catheterization is still challenging in neonatal age and no specific data concerning early outcome are so far published in literature., Methods: Neonatal trans-catheter cardiac interventions performed in high-volume Italian referral centers were retrospectively analyzed. Primary outcomes were procedural major adverse events, in-hospital mortality and procedural failure. Secondary outcomes were minor adverse events and need for blood transfusion., Results: From January 2000 to December 2017, 1423 newborns (mean weight 3.0 ± 0.6 kg, range 1.0-5.8; median age 2.0 days) underwent interventional cardiac catheterization. Overall, global procedure adverse event rate and in-hospital mortality were 10.2% and 5.2%, respectively. At multi-variable analysis, primary composite outcome was significantly related to low-weight (<2.5 kg) (p < 0.01) and younger age (≤7 days) (p < 0.01) at the procedure, prematurity (p < 0.01), uni-ventricular physiology (p < 0.01), associated genetic syndromes (p < 0.01) and procedure risk category (p < 0.01). No relationship between volume of activity of any single center and procedure outcome was found. Over time, a trend toward an increased number of procedures and their complexity was recorded. Trans-catheter management of cardiac malformations with critical, duct-dependent pulmonary blood flow by arterial duct stenting or right ventricular outflow tract stenting showed the highest increase., Conclusions: Interventional cardiac catheterization is relatively safe and feasible in neonatal age. Peri-natal age, low weight, uni-ventricular physiology and genetic syndromes still significantly contribute to procedural morbidity and in-hospital mortality of this approach., Competing Interests: Declaration of competing interest None of the authors declares any conflict of interest regarding this paper, (Copyright © 2020 Elsevier B.V. All rights reserved.)

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. The aim of the present study was to report both prevalence and cardiac outcome of ACDs in patients with RASopathies., Methods: A retrospective, multicentric observational study (CArdiac Rasopathy NETwork-CARNET study) was carried out. Clinical, surgical, and genetic data of the patients who were followed until December 2019 were collected., Results: Forty-five patients out of 440 followed in CARNET centers had ACDs. Noonan Syndrome (NS), NS Multiple Lentigines (NSML) and CardioFacioCutaneous Syndrome (CFCS) were present in 36, 5 and 4 patients, respectively. Median age at last follow-up was 20.1 years (range 6.9-47 years). Different ACDs were reported, including mitral and aortic valve dysfunction, ascending and descending aortic arch anomalies, coronary arteries dilation, enlargement of left atrial appendage and isolated pulmonary branches diseases. Five patients (11%) underwent cardiac surgery and one of them underwent a second intervention for mitral valve replacement and severe pericardial effusion. No patients died in our cohort until December 2019., Conclusions: Patients with RASopathies present a distinct CHD spectrum. Present data suggest that also ACDs must be carefully investigated for their possible impact on the clinical outcome. A careful longitudinal follow up until the individuals reach an adult age is recommended., (© 2020 Wiley Periodicals, Inc.)

A comprehensive description of morbidity and mortality as well as risk factors of interventional cardiac catheterization performed in neonatal age was reported in our paper recently published on the International Journal of Cardiology ( IJCA28502; PII: S0167-5273(20)30384-3; DOI: 10.1016/j.ijcard.2020.04.013 ). Eight Italian high-volume centres of Paediatric Cardiology were involved in this observational, retrospective data collection and analysis. In this dataset, clinical and procedural characteristics of 1423 newborns submitted to 1551 interventional cardiac catheterization procedures were analyzed. Primary outcomes were considered procedure and in-hospital mortality as well as major adverse event and procedural failure rates. Secondary outcomes were considered minor adverse events and need for blood transfusion. Targets of this data analysis were: 1) to evaluate the overall major risk factors of interventional cardiac catheterization; 2) to identify the most hazardous interventional procedures; 3) to assess possible trends of individual procedures as well as their outcome over time; 4) to find possible relationships between the volume activity of any centre and the procedure and follow-up outcome. In particular, this Data in Brief companion paper aims to report the specific statistic highlights of the multivariable analysis (binary logistic regression) used to assess the impact of any potential risk factors on the type of procedure over a short-term follow-up., (© 2020 The Authors.)

Background and Objectives: Stem cell therapy is a promising strategy for treating neurological diseases but its effectiveness is influenced by the route of administration and the characteristics of the stem cells. We determined whether neural induction of mesenchymal stem cells (MSCs) was beneficial when the cells were delivered intra-arterially through the carotid artery., Methods and Results: MSCs were neurally induced using a retroviral vector expressing the neurogenic transcription factor neurogenin-1 (Ngn1). The LacZ gene encoding bacterial β-galactosidase was used as a control. Ischemic stroke was induced by transluminal occlusion of the middle cerebral artery and 3 days later the MSCs were delivered intra-arterially through the internal carotid artery. Magnetic resonance imaging analysis indicated that compared to MSCs expressing LacZ (MSCs/LacZ), MSCs expressing Ngn1 (MSCs/Ngn1) exhibited increased recruitment to the ischemic region and populated this area for a longer duration. Immunohistochemical analysis indicated that compared to MSCs/LacZ, MSCs/Ngn1 more effectively alleviated neurological dysfunction by blocking secondary damage associated with neuronal cell death and brain inflammation. Microarray and real-time PCR analysis indicated that MSCs/Ngn1 exhibited increased expression of chemotactic cytokine receptors, adherence to endothelial cells, and migration ability., Conclusions: Neural induction with Ngn1 increases the homing ability of MSCs, enhancing their engraftment efficiency in the ischemic rat brain. Intra-arterial delivery of neurally induced MSCs/Ngn1 3 days after ischemic injury blocks neuronal cell death and inflammation, and improves functional recovery. Thus, intra-arterial administration of stem cells with neural properties may be a novel therapy for the treatment of ischemic stroke.

Background: Double-outlet right atrium (DORA) is a rare congenital cardiac defect, where the right atrium (RA) is connected to both the right ventricle (RV) and the left ventricle (LV). Double-outlet right atrium is classified into two types, each containing two subtypes: malaligned atrial septum with common or single atrioventricular (AV) valve and malaligned ventricular septum (VS) with adequate or inadequate RV. The VS type is characterized by straddling right AV valve (RAVV) with intact VS, resulting in two RAVV orifices., Methods: We report a case of "acquired DORA" in a 17-year-old male patient previously treated with subaortic VSD closure. At admission, we diagnosed DORA with VS malalignment and adequate RV. The patient had 2 orifices within the RAVV, connecting the RA to both the RV (via RAVV orifice 1) and the LV (via RAVV orifice 2). The latter was insufficient, with severe LV to RA shunt. A review of the literature indexed in PubMed and Scopus databases was undertaken., Results: The patient underwent biventricular repair through closure of the RAVV orifice 2 with a pericardial patch. Pacemaker implantation for complete AV block was necessary. Postoperative course and follow-up were regular. The literature review showed 39 cases of DORA, of which 8 had a malaligned VS and an adequate RV, as in our case., Conclusion: Acquired DORA is an interesting post-surgical variant of a rare congenital heart defect. Biventricular repair is a feasible and viable option, which involves closing the third AV orifice. Careful attention is required in order to avoid injury to the conduction system.

IntroductionPulmonary atresia, ventricular septal defect, major aorto-pulmonary collateral arteries, and pulmonary arteries hypoplasia are rare and complex congenital defects that require early interventions to relieve cyanosis and enhance the growth of native pulmonary arteries. The treatment of these patients is still controversial. Surgical techniques require cardiopulmonary bypass which is poorly tolerated by small infants. Percutaneous techniques such as radiofrequency perforation can be challenging. The hybrid technique consists of perventricular stenting of the right ventricle outflow tract through medial sternotomy, to restore native pulmonary flow., Methods: We retrospectively reviewed the cardiovascular database of our centre in order to analyse our experience in hybrid procedure. We detected six patients who underwent hybrid first approach between November 2007 and December 2015. We report our early results and mid-term outcomes., Results: Median age at the procedure was 26 days, median weight was 3150 g, and median Nakata index was 52 mm2/m2. All procedures were successful except for one: this patient underwent a surgical shunt. No immediate and early deaths or major complications occurred and oxygen saturation levels increased in all the patients. Patients were followed up for a period of 12-103 months, and four of them underwent a procedure of unifocalisation at the mean age of 12.5 months., Conclusions: We reported data from the largest series of patients who underwent this hybrid procedure. Our experience demonstrated encouraging results to expand the use of this approach to bridge high-risk patients with diminutive pulmonary arteries to a second step of surgical repair.

Tart cherries are increasingly popular due to purported health benefits. This Prunus cesarus species is cultivated worldwide, and its market has increased significantly in the last two decades due to improvements in agricultural practices and food processing technology. Tart cherries are rich in polyphenols, with a very specific profile combining anthocyanins and flavonols (berries-like) and chlorogenic acid (coffee-like). Tart cherries have been suggested to exert several potentially beneficial health effects including: lowering blood pressure, modulating blood glucose, enhancing cognitive function, protecting against oxidative stress and reducing inflammation. Studies focusing on tart cherry consumption have demonstrated particular benefits in recovery from exercise-induced muscle damage and diabetes associated parameters. However, the bioconversion of tart cherry polyphenols by resident colonic microbiota has never been considered, considerably reducing the impact of in vitro studies that have relied on fruit polyphenol extracts. In vitro and in vivo gut microbiota and metabolome studies are necessary to reinforce health claims linked to tart cherries consumption.

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.

Objectives: The aims of this study were to review systematically literature on and describe the sonographic features and associated anomalies of total (TAPVC) and partial (PAPVC) anomalous pulmonary venous connection and scimitar syndrome (SS)., Methods: A retrospective cohort study was carried out of cases of TAPVC, PAPVC and SS that underwent comprehensive ultrasound examination, seen over a 20-year period at two tertiary referral centers. Assessed variables included TAPVC subtype, gestational age at diagnosis, area behind the left atrium, ventricular disproportion, vertical vein, pulmonary venous obstruction, mode of diagnosis, association with cardiac and extracardiac conditions, and pregnancy and fetoneonatal outcomes. The outcome was considered favorable if the individual was alive and well (no functional impairment from surgery or cardiac or extracardiac conditions). Cases associated with right isomerism were excluded from the analysis, as TAPVC in these cases was only one of several major cardiac anomalies affecting sonographic signs. A systematic review was performed in order to obtain a synthesis of characteristics associated with TAPVC, PAPVC and SS. The literature search of PubMed and EMBASE (1970-2016) included reviews, case series and case reports. A meta-analysis was conducted only for TAPVC. Random-effects models were used to obtain pooled estimates of the frequencies of clinical characteristics and sonographic features., Results: For TAPVC, a total of 15 studies involving 71 patients (including 13 from the current cohort study) were included in the systematic review and meta-analysis. The pooled estimate for the association of TAPVC with congenital heart disease was 28.3% (95% CI, 18.1-41.3%) and with extracardiac anomalies it was 18.5% (95% CI, 10.5-30.6%). Of TAPVC cases, obstructed venous return was observed in 34.1% (95% CI, 22.7-47.7%), a favorable outcome in 43.8% (95% CI, 24.0-65.8%), ventricular disproportion in 59.2% (95% CI, 45.1-72.0%), increased area behind the left atrium in 58.1% (95% CI, 41.1-73.5%) and a vertical vein in 59.3% (95% CI, 41.1-75.3%). Diagnosis was established by using color or power Doppler in 84.9% (95% CI, 67.3-93.9%) of cases. For SS, there were only three studies describing eight cases, to which the current study added another five. Ventricular disproportion was present in three out of nine SS cases for which data were available, but for two of these, there was a concurrent heart anomaly. Color Doppler was used for all SS diagnoses, and four-dimensional echocardiography was useful in two out of six cases in which it was used. Outcome for SS cases was generally good. For PAPVC, there were only five studies describing five cases, to which the current study added another two. Major cardiac anomalies were associated in four out of seven of these cases, and extracardiac anomalies in three out of six cases for which data were available., Conclusions: TAPVC can be associated with other cardiac and extracardiac anomalies in a significant percentage of cases. Leading sonographic signs are ventricular disproportion, increased area behind the left atrium and the finding of a vertical vein. Color/power Doppler is the key mode for diagnosis of TAPVC. Obstructed venous return can be expected in roughly one-third of cases of TAPVC and outcome is favorable in less than half of cases. Data for SS and PAPVC are too few to synthesize. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd., (Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.)

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. Cardiac defects, crude mortality, survival rate of patients with 1) hypertrophic cardiomyopathy (HCM) and age <2 years or young adults; 2) individuals with Noonan syndrome and pulmonary stenosis carrying PTPN11 mutations; 3) biventricular obstruction and PTPN11 mutations; 4) Costello syndrome or cardiofaciocutaneous syndrome were analysed. Mortality was described as crude mortality, cumulative survival and restricted estimated mean survival. In particular, with this Data In Brief (DIB) paper, the authors aim to report specific statistic highlights of the multivariable regression analysis that was used to assess the impact of mutated genes on number of interventions and overall prognosis.

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.

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). Clinical records of 371 patients with confirmed molecular diagnosis of RASopathy were reviewed. Mortality was described as crude mortality, cumulative survival and restricted estimated mean survival. Multivariable regression analysis was used to assess the impact of mutated genes on number of interventions and overall prognosis., Results: Cardiac defects occurred in 80.3% of cases, almost half of them underwent at least one intervention. Overall, crude mortality was 0.29/100 patients-year. Cumulative survival was 98.8%, 98.2%, 97.7%, 94.3%, at 1, 5, 10, and 20years, respectively. Restricted estimated mean survival at 20years follow-up was 19.6years. Ten patients died (2.7% of the entire cohort; 3.4% of patients with cardiac defect). Patients with hypertrophic cardiomyopathy (HCM) and age <2years or young adults, as well as subjects with biventricular obstruction and PTPN11 mutations had a higher risk of cardiac death., Conclusions: The risk of intervention was higher in individuals with Noonan syndrome and pulmonary stenosis carrying PTPN11 mutations. Overall, mortality was relatively low, even though the specific association between HCM, biventricular outflow tract obstructions and PTPN11 mutations appeared to be associated with early mortality, including immediate post-operative events and sudden death., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Background . Fresh vegetables such as tomato should have low microbial population for safe consumption and long storage life. The aerobic bacterial count (ABC) and coliform bacterial count (CBC), yeast, and mold population are the most widely used microbial indicators in fresh vegetables which should be lower than 4 log CFU g -1 for safe consumption. The stages of the supply chain, postharvest handling methods, and crop varieties had significant effects on microbial population. ABC, CBC, yeast, and mold population were significantly highest ( P < 0.05) at retail market (5.59, 4.38, 2.60, and 3.14 log CFU g -1 , resp.), followed by wholesale market (4.72, 4.71, 2.43, and 2.44 log CFU g -1 , resp.), and were least at farm gate (3.89, 3.63, 2.38, and 2.03 log CFU g -1 , resp.). Improved postharvest practices (washing in clean water and grading and packaging in clean plastic crate) helped to reduce ABC, CBC, and mold population by 2.51, 32.70, and 29.86 percentage as compared to the conventional method (no washing and no grading and packaging in mud plastered bamboo baskets). Among varieties, Pusa ruby had the lowest microbial load of 2.58, 4.53, 0.96, and 1.77 log CFU g -1 for ABC, CBC, yeast, and mold count, respectively. Significantly negative correlation ( P < 0.05) was observed between fruit pH & ABC and pH & mold count. Although the microbial quality of fresh tomato is safe in the local market of western Terai of Nepal both in conventional and in improved practices however still it is essential to follow improved postharvest handling practices in production and marketing of newly introduced tomato cultivars (high-pH cultivars) for ensuring the safe availability of fresh tomato in the market.

Introduction: Neonates with D-transposition of the great arteries (dTGA) may die at birth because of the inadequate intracardiac mixing due to a misdiagnosed restrictive foramen ovale. We reviewed our experience in echocardiographic assessment and perinatal management of fetuses with dTGA searching for new features that may predict the need for urgent balloon atrial septostomy (BAS) immediately after birth., Patients and Methods: We included fetuses diagnosed with dTGA between January 2000 and December 2014. We assessed pre- and postnatal appearance of the foramen ovale, ductus arteriosus and pulmonary veins. Both the diagnostic findings at the time of last prenatal echocardiogram and those findings deriving from a retrospective reevaluation of stored videos were considered. BAS was defined as urgent if performed in neonates with restrictive foramen ovale and severe hypoxemia., Results: We reviewed 40 fetuses with dTGA. 20/40 fetuses received urgent BAS at birth. Not only the restrictive but also the hypermobile and the redundant appearance of the foramen ovale was significantly associated with urgent BAS (p < 0.0001, p = 0.002 and p = 0.0001, respectively)., Conclusions: Prenatal evaluation of the foramen ovale appearance in fetuses with dTGA is still challenging. Based on our experience, also the redundant foramen ovale appearance may need urgent BAS at birth., (© 2016 S. Karger AG, Basel.)

Background. Stents have become the treatment of choice for native aortic coarctation in adults and adolescents, but in pediatric patients insufficient data are currently available to identify the best therapeutic option. Methods. To compare the outcomes of pediatric and adolescent patients, we retrospectively evaluated early and long-term results of stenting for aortic coarctation in 34 patients divided into 2 groups (A and B) composed, respectively, of 17 children (mean age 8.2 ± 2.3, weight ≤30 kg) and 17 adolescents (mean age 14.3 ± 1.7, weight >30 kg). Results. No significant differences in outcome were found between groups immediately after the procedure. In all of our patients, peak systolic gradient pressure significantly decreased after stenting from 43.7 ± 12 to 1.7 ± 3.1 mmHg in group A and from 39.4 ± 16.8 to 1.6 ± 3 in group B (p < 0.0001). We observed early and late adverse events in both groups: early femoral vessel injury or thrombosis was more frequent in younger patients, as well as restenosis due to vessel growth requiring stent redilatations, often complicated by stent fractures. Data from long-term follow-up showed that, in younger patients, stress-related hypertension was more frequent. Conclusions. The procedure was immediately safe and effective in both groups. Pediatric patients must be accurately selected before stenting because they could probably need reinterventions and stents could impact on their future therapeutic perspectives.

Background: Veno-venous extracorporeal membrane oxygenation (ECMO) is probably the preferable configuration to assist children with respiratory failure who do not respond to maximized conventional therapies. The single-vessel, double-lumen approach through the internal jugular vein is extremely advantageous, especially in infants, where femoral access presents limitations related to the small dimensions of the veins. In case of emergencies, ECMO might need to be started at the bedside, without the availability of fluoroscopic guidance. To our knowledge, a completely percutaneous approach has not been reported before in children younger than 1 year and weighing less than 5 kg., Methods: We describe 3 cases of emergency bedside, percutaneous, bicaval double-lumen cannulation under real-time transthoracic ultrasound control in 2 neonates and 1 infant., Results: In our experience, this approach proved to be safe, effective and time saving, while minimizing bleeding from the cannula insertion site. Cannulation times, from decision making to the beginning of ECMO flow, were 30, 28, 25 minutes respectively, from patient 1 to 3. We do not report any cannula-related injury to vessels and heart structures., Conclusions: Our preliminary data suggest that, with the described precautions, percutaneous, echo-guided, bicaval double-lumen cannulation in neonates and infants could be effective and free from major complications. Further evaluation should be warranted in the neonatal population.

Background: Fluoroscopic catheter ablation of cardiac arrhythmias in pediatric patients exposes the patients to the potential risk of radiation considering the sensitivity of this population and its longer life expectancy. We evaluated the feasibility, safety, and efficacy of accessory pathway (AP) ablation guided by CARTO3 electroanatomic mapping (EAM) system with both cryoenergy and radiofrequency (RF) energy in order to avoid x-ray exposure in pediatric patients., Methods: We included 44 patients (mean age: 13.1 ± 3.3 years); nine of 44 presented concealed AP. An electrophysiological study with a three-dimensional EAM reconstruction was performed in every patient with a venous transfemoral direct right atrium approach or an arterial transfemoral retrograde approach to reach the mitral annulus. In two patients with left-sided AP, the ablation was performed via a patent foramen ovale., Results: A total of 47 APs were present, left sided in 45% (21/47) of cases (15 lateral, one anterior, three posteroseptal, and two posterolateral) and right sided in 55% (26/47; one anterior, three anterolateral, one posterolateral, three lateral, five para-Hisian, 12 posteroseptal, and one anteroseptal). Ablation without the use of fluoroscopy was successfully performed in every patient (33 with RF and in 11 with cryoenergy). No complication occurred. At a mean follow-up of 16.0 ± 11.7 months, we observed seven recurrences, three of them successfully re-ablated without fluoroscopy. In one case cryoablation of a para-Hisian AP was ineffective in the long term., Conclusions: Three-dimensional EAM allowed a safe and effective fluoroless AP ablation procedure in a pediatric population both with RF and cryoenergy., (© 2015 Wiley Periodicals, Inc.)

Objective: To define the prevalence and characteristics of associated congenital heart diseases (CHDs) in patients with Hirschsprung's disease (HSCR)., Method: All patients with a histological diagnosis of HSCR admitted to our hospital between January 2010 and December 2013 were included in this prospective observational study and underwent cardiovascular screening. Cardiac anatomy was assessed by a segmental echocardiographic approach. Measurements of aortic root and left ventricular dimensions, wall thickness, and function were obtained. CHDs requiring a percutaneous or surgical intervention were described as major heart diseases., Results: One hundred thirty-three consecutive patients were enrolled at median age of 2.3 years. Eleven patients (8.3%) presented an associated heart disease. Moreover, five patients had mild dilatation of aortic root. Six out of 11 (4.5%) patients had a major CHDs requiring surgical repair., Conclusion: Prevalence of associated CHDs was slightly higher than in previous papers, and mostly represented by septal defects. Four out of six patients with major heart disease had also a chromosomal anomaly. If we do not consider the subpopulation of patients with a chromosomal anomaly, cardiac defects were present in 3.8% of the patients. Based on these results, we suggest to perform routine echocardiogram in all Hirschsprung patients, with or without associated chromosomal syndromes.

Objective: To describe the antenatal and neonatal echocardiographic morphology and flow pattern of the ductus arteriosus in patients with tetralogy of Fallot., Patients and Methods: We included patients with a prenatal diagnosis of tetralogy of Fallot between January 2006 and December 2012., Results: Among the 52 fetuses with tetralogy of Fallot the severity of right ventricular outflow obstruction was considered mild in 32, moderate in 14, and severe in 6. In the mild right ventricular outflow obstruction group (n = 32) all had normal ductal morphology and flow pattern, eight (25%) elected for termination of pregnancy and two died in the neonatal period from extracardiac causes. In the moderate right ventricular outflow obstruction group (n = 14) the fetuses had a small ductus arteriosus with antegrade but abnormal flow velocity, one (7%) elected for termination of pregnancy. Immediately after birth the ductus arteriosus was very small or already closed at echocardiographic examination. Two out of 13 patients (15%) developed severe hypoxic spells and underwent modified Blalock-Taussig shunt during the neonatal period. Six fetuses were considered to have severe right ventricular outflow obstruction with flow reversal in the ductus arteriosus, three (50%) of whom elected for termination of pregnancy. The other three newborns underwent modified Blalock-Taussig shunt., Conclusion: In fetuses with tetralogy of Fallot, ductal diameter can be reduced even up to prenatal closure. Prenatal ductal morphology assessment may be useful for improving management of patients with moderate right ventricular outflow obstruction and small ductus arteriosus who may become cyanotic at birth., (© 2013 Wiley Periodicals, Inc.)

Background: In adult patients with d-transposition of the great arteries after atrial switch operation, dysfunction of the systemic right ventricle (RV) is a well-known complication. Echocardiographic variables may provide adequate estimation of subpulmonary RV function, but their applicability to the subaortic RV is not straightforward. We evaluate the concordance between tricuspid annular plane systolic excursion (TAPSE) and magnetic resonance imaging-derived ejection fraction of the RV (MRI-RVEF) in these patients., Methods: Patients were recruited from those evaluated at the adult congenital clinic of our department between 2010 and 2012. All patients who had an echocardiographic assessment within 6 months of their MRI examination were selected. Patients clinically unstable, not in sinus rhythm, with a prosthetic systemic atrioventricular valve, permanent pacemaker, or more than moderate systemic atrioventricular valve regurgitation were excluded., Results: Eighteen Mustard-operated patients aged 22 ± 3.7 years were studied. The mean values of TAPSE and RVEF were 13.22 ± 1.7 mm and 49.7 ± 6%, respectively. TAPSE and RVEF were normal in 1 (5.5%) and 10 (55.5%) patients, respectively. Seventeen (94.4%) patients showed reduced TAPSE (12.9 ± 1.3 mm): RVEF was reduced in eight (47%) of these subjects, and normal in nine (53%). In patients with normal RVEF, both the MRI-RV end-diastolic and the MRI-RV end-systolic volumes were significantly lower than in patients with reduced RVEF. There were no other statistically significant differences between these patients. No correlation was found between TAPSE and both the MRI-RV end-diastolic and the end-systolic volumes. Globally, agreement between TAPSE and RVEF was slight (K = 0.09 ± 0.089)., Conclusions: Our results indicate that in these patients TAPSE is not a useful measure of RV function., (© 2013 Wiley Periodicals, Inc.)

Two cases of ultrasound diagnosis of Holt-Oram syndrome are described. Both were characterized by significant right atrial enlargement that was not due to concurrent tricuspid regurgitation or other cardiac anomalies. In both cases the associated skeletal anomaly was subtle and barely visible using ultrasound. Interestingly, despite the fact that Holt-Oram syndrome is also called atriodigital dysplasia, unexplained right atrial enlargement has not been described in this context in the fetus before. When such a finding is detected, we believe a thorough search for upper limb abnormalities should be carried out and genetic testing for Holt-Oram syndrome should be discussed with the parents., (Copyright © 2013 ISUOG. Published by John Wiley & Sons Ltd.)

Background: Anatomical considerations and risks related to x-ray exposure make atrioventricular nodal reentrant tachycardia (AVNRT) ablation in pediatric patients a concerning procedure. We aimed to evaluate the feasibility, safety, and efficacy of performing fluoroless slow-pathway cryoablation guided by the electroanatomic (EA) mapping in children and adolescents., Methods: Twenty-one consecutive patients (mean age 13.5 ± 2.4 years) symptomatic for AVNRT were prospectively enrolled to right atrium EA mapping and electrophysiological study prior to cryoablation. Cryoablation was guided by slow-pathway potential and performed using a 4-mm-tip catheter., Results: Sustained slow-fast AVNRT was inducible in all the patients with a dual AV nodal physiology in 95%. Acute success was achieved in 100% of the patients with a median of two cryo-applications. Fluoroless ablation was feasible in 19 patients, while in two subjects 50 seconds and 45 seconds of x-ray were needed due to difficult progression of the catheters along the venous system. After a mean follow-up of 25 months, AVNRT recurred in five patients. All the recurrences were successfully treated with a second procedure. In three patients, a fluoroless cryoablation with a 6-mm-tip catheter was successfully performed, while in the remaining two patients, a single pulse of 60 seconds of radiofrequency energy was applied under fluoroscopic monitoring. No complications occurred., Conclusions: Combination of EA mapping systems and cryoablation may allow to perform fluoroless slow-pathway ablation for AVNRT in children and adolescents in the majority of patients. Fluoroless slow-pathway cryoablation showed a high efficacy and safety comparable to conventional fluoroscopy guided procedures., (©2013, The Authors. Journal compilation ©2013 Wiley Periodicals, Inc.)

Background: Associated anomalies have been reported in around 20% of Hirschsprung patients but many Authors suggested a measure of underestimation. We therefore implemented a prospective observational study on 106 consecutive HSCR patients aimed at defining the percentage of associated anomalies and implementing a personalized and up-to-date diagnostic algorithm., Methods: After Institutional Ethical Committee approval, 106 consecutive Hirschsprung patients admitted to our Institution between January 2010 and December 2012 were included. All families were asked to sign a specific Informed Consent form and in case of acceptance each patient underwent an advanced diagnostic algorithm, including renal ultrasound scan (US), cardiologic assessment with cardiac US, cerebral US, audiometry, ENT and ophthalmologic assessments plus further specialist evaluations based on specific clinical features., Results: Male to female ratio of our series of patients was 3,4:1. Aganglionosis was confined to the rectosigmoid colon (classic forms) in 74,5% of cases. We detected 112 associated anomalies in 61 (57,5%) patients. The percentage did not significantly differ according to gender or length of aganglionosis. Overall, 43,4% of patients complained ophthalmologic issues (mostly refraction anomalies), 9,4% visual impairment, 20,7% congenital anomalies of the kidney and urinary tract, 4,7% congenital heart disease, 4,7% hearing impairment or deafness, 2,3% central nervous system anomalies, 8,5% chromosomal abnormalities or syndromes and 12,3% other associated anomalies., Conclusions: Our study confirmed the underestimation of certain associated anomalies in Hirschsprung patients, such as hearing impairment and congenital anomalies of the kidney and urinary tract. Subsequently, based on our results we strongly suggest performing renal US and audiometry in all patients. Conversely, ophthalmologic assessment and cerebral and heart US can be performed according to guidelines applied to the general population or in case of patients with suspected clinical features or chromosomal abnormalities. This updated diagnostic algorithm aims at improving overall outcome thanks to better prognostic expectations, prevention strategies and early rehabilitation modalities. The investigation of genetic background of patients with associated anomalies might be the next step to explore this intriguing multifactorial congenital disease.

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 1.05 years (range, 1 day-41 years). Thirty-three patients (75%) had an isolated form; 11 patients (25%) had associated congenital heart diseases. Twenty-two patients (50%) were symptomatic at diagnosis, including respiratory symptoms (n=20) and congestive heart failure (n=6). Patients with associated congenital heart defects had a higher prevalence of congestive heart failure (4 of 11 [36.4%] vs 2 of 33 [6.1%]; P=.027), pulmonary arterial hypertension (7 of 11 [63.6%] vs 2 of 33 [6.1%]; P=.027) than patients with isolated forms. Ten patients (22.7%) underwent correction of associated cardiac defects, leaving the anomalous pulmonary venous drainage intact. The median length of follow-up after diagnosis was 6.4 years (range, 0.2-27.5 years). Two patients died, both with associated cardiac defects and severe pulmonary arterial hypertension. Of 42 survivors, 39 (92.8%) were asymptomatic at the last follow-up visit; 3 patients still complained respiratory symptoms. There was no difference between isolated and associated forms of the disease., Conclusions: In most patients, scimitar syndrome presented as an isolated lesion with a benign outcome. Nonetheless, when associated with other cardiac defects and pulmonary arterial hypertension, there was an increased risk of congestive heart failure and mortality. Correction of associated cardiac defects (transforming "associated" into "isolated" forms), together with the therapeutic occlusion of anomalous arterial supply to the lung, led to a benign outcome comparable to that in primarily isolated forms., (Copyright © 2013 Sociedad Española de Cardiología. Published by Elsevier Espana. All rights reserved.)

Objectives: To describe our experience in the management of coronary artery anomalies both in an adult and in a paediatric population and to compare the two groups for finding out differences in terms of angiographic incidence and treatment., Patients and Methods: Databases at the Department of Cardiology of San Martino Adult's Hospital and of Gaslini Children's Hospital were searched for all patients with a diagnosis of coronary artery anomaly who underwent coronary angiography between 1994 and 2006., Results: Coronary anomalies were diagnosed in 76 (1%) adult patients. Anomalous left circumflex artery was the commonest coronary anomaly (25%). Anomalous left coronary artery from pulmonary artery and myocardial bridges were the only anomalies responsible for angina-like symptoms. No patients except the one with anomalous left coronary artery from pulmonary artery needed surgical intervention. In the paediatric population, we found 28 (0.9%) patients with coronary anomalies. Anomalous left coronary artery from pulmonary artery was the most common anomaly (48%) and always required emergency surgical treatment; in addition there were two patients with stenosis of the left main coronary artery., Conclusion: Coronary artery anomalies may be associated with very acute, even life-threatening symptoms in children, whereas they are usually clinically silent and detected by accident on coronary angiography in adults. Recognition of coronary artery anomalies enables early treatment or close follow-up in children, whereas it could be useful in case of cardiac surgery in adults.

Background: Pulmonary atresia and intact ventricular septum is a complex congenital heart disease with great morphological variability. Approximately two-thirds of patients may be suitable for transcatheter pulmonary valvotomy. We reviewed our experience in the use of two different percutaneous approaches to evaluate the impact on fluoroscopy time and morbidity of a new technique to perform transcatheter radiofrequency perforation and valvotomy in newborns with pulmonary atresia and intact ventricular septum., Methods and Results: In all, 31 patients underwent radiofrequency perforation of the pulmonary valve. The first 14 infants were treated using a 5 French Judkins right coronary catheter, which was manoeuvred directly underneath the atretic pulmonary valve (Group A). The others were treated using a telescopic system consisting of Northstar Lumax Flex and White Lumax Guiding Catheters (Cook; Group B). In both groups, after radiofrequency perforation of the pulmonary valve, a 0.014-inch superfloppy guidewire was advanced into the descending aorta and balloon dilations were performed. Required fluoroscopy time was significantly lower in Group B (48.5 ± 28.1 versus 24.9 ± 14.4 minutes, respectively; p < 0.01). A higher incidence of unfavourable events including the need for early surgery was found in Group A., Conclusion: In our experience, telescopic catheter proved to be a valid option able to decrease the fluoroscopy time of percutaneous radiofrequency perforation of pulmonary valve and consequently patients’ exposure to procedure-related risks.

Acquired pulmonary vein occlusion is a rare complication of surgical correction of complex congenital heart diseases. Associated vascular remodeling of arterial and venous vessels includes medial thickening, intimal fibrosis, and progressive development of aorto-pulmonary collaterals. The consequent pulmonary perfusion changes impact more importantly on a low pressure circulation such as the Fontan circuit. The management of these patients is still controversial. We describe this condition in two patients with single ventricle who underwent staged cavopulmonary connection. In both cases, we found acquired occlusion of one pulmonary vein, poor antegrade flow in the involved hypoplastic pulmonary arteries due to a widespread thin collateral circulation producing a backward washout of unopacified blood flow with competitive mechanism. Percutaneous embolization of a segmental pulmonary artery was performed with restoration of a more homogeneous perfusion of both main pulmonary arteries. Extracardiac total cavopulmonary connection was successfully performed a few months later in both patients., (Copyright © 2011 Wiley Periodicals, Inc.)

Objectives: To determine the impact of fetal echocardiography on the management of pregnancy and of newborns affected by pulmonary atresia and intact ventricular septum (PAIVS) and to evaluate the outcome of infants with and without prenatal diagnosis of PAIVS., Methods: We searched our database for cases of PAIVS prenatally and postnatally diagnosed during the period January 1993-December 2009. Postnatal follow-up was available in all cases included in the study. Karyotyping and fluorescent in situ hybridization analysis for the DiGeorge critical region (22q11.2) were performed in all but one case., Results: The study comprised 60 cases of PAIVS: 36 with (Group A) and 24 without (Group B) prenatal diagnosis. In Group A, there were two intrauterine deaths, six postnatal deaths (five early after birth) and one termination of pregnancy. In this group, radiofrequency (RF) perforation was successfully performed in 25 cases; 20/25 infants had a biventricular (BV) repair, without further operation in 13 of them. No patient of Group B died. In this group, RF perforation was successfully performed in 22 cases; 20/22 had a BV repair without further procedure in 15 of them., Conclusions: Prenatal diagnosis of PAIVS allows a reliable prognosis of severity and planning of proper surgical repair strategies. Fetuses that are prenatally diagnosed present a more severe spectrum of the disease; for the cases capable of getting through the neonatal period, the mortality rate and the need for further intervention were not significantly different when compared with babies with only postnatal diagnosis.

Objectives: To analyze fetal two-dimensional (2D) echocardiographic characteristics of interrupted aortic arch (IAA) and its different types, to explore whether the use of 4D ultrasound with B-flow imaging and spatiotemporal image correlation (STIC) can improve prenatal diagnostic accuracy, and to describe associations and outcome., Methods: The study comprised IAA fetuses examined exclusively by 2D conventional echocardiography during the period from 1994 to 2003, and those identified by conventional echocardiography and examined further by 4D ultrasound with B-flow imaging and STIC during the period January 2004 to July 2008, identified among fetuses examined at two referral centers for congenital heart defects (CHD). Postnatal follow-up was available in all cases. Karyotyping and fluorescent in-situ hybridization (FISH) analysis for the DiGeorge critical region (22q11.2) were performed in all cases., Results: Twenty-two cases of isolated IAA (15 Type B and seven Type A, seven and three of which, respectively, underwent B-flow imaging and STIC) were detected among 2520 cases of fetal CHD. In seven of the 15 Type B cases, a right subclavian artery arose anomalously (ARSA). 2D echocardiography failed to distinguish the type of IAA in only two cases and the ARSA in five of the seven cases. B-flow imaging and STIC successfully identified IAA types in all 10 cases examined and clearly visualized the origin and course of the ARSA, including cervical ones. FISH detected 22q11.2 microdeletion in 10 of the 15 Type B cases and an unusual association with Type A in one of the seven cases. Fetal/neonatal outcome included: eight terminations of pregnancy, one intrauterine death and four postoperative deaths in the neonatal period, and nine neonates were alive after surgery at a mean follow-up time of 58 months (range, 4 months-13 years)., Conclusion: Our results confirm the feasibility of prenatal characterization of IAA and its different types based on 2D echocardiographic examination, albeit with some limitations in the thorough assessment. 4D ultrasound with B-flow imaging and STIC can apparently facilitate visualization and detailed examination of the anatomical features of the IAA types, including visualization of the neck vessels, thus supplying additional information with respect to 2D sonography. As for the known association with microdeletion 22q11.2, our data indicate that Types A and B are distinct, there being a close association only with IAA Type B., ((c) 2009 ISUOG. Published by John Wiley & Sons, Ltd.)

Objective: To review the outcome of patients with pulmonary atresia with intact ventricular septum after interventional perforation of the pulmonary valve, to assess the capability of this procedure to avoid neonatal or late intervention and to obtain a long-term biventricular repair., Design: Retrospective interventional study and clinical follow-up study., Setting: Tertiary referral centre., Patient Population: Between November 1994 and December 2007, 40 neonates underwent radiofrequency perforation. Median age at pulmonary valvotomy was 28 hours (range 1-147 hours) and median weight was 2925 g (range 1900-4400 g)., Main Outcome Measures: Procedural success and complication rates; early-term and long-term follow-up results., Results: The procedure was successful in 39 patients but 16 of them needed neonatal surgery. The overall mortality was 7.5%. At a median follow-up of 82 months, four patients underwent a bidirectional Glenn procedure, whereas all the other patients achieved a biventricular circulation without any further intervention in 19 of them. Patients who died or needed additional intervention with or without biventricular circulation failure had a higher incidence of bipartite right ventricular (65% vs 15.8% of those not needing additional intervention; p = 0.004) and a lower median tricuspid Z value (-2 (range -3.5 to 1) vs -0.5 (range -2 to 1); p = 0.004))., Conclusions: The results confirm that percutaneous interventional perforation is an effective first-stage procedure in patients with pulmonary atresia with intact ventricular septum. The right heart appeared to be adequate to maintain a long-term biventricular circulation in the large majority of cases.

The purpose of this study was to describe our experience in the ultrasound visualization and management of prenatally diagnosed isolated vascular rings. From January 2002 to December 2007, a total of 19 fetuses had a sonographic diagnosis of isolated vascular rings in 2 reference centers at a mean gestational age of 23 weeks. There were 8 cases of left aortic arch with aberrant right subclavian artery, 5 cases of double aortic arch, and 6 cases of right aortic arch with aberrant left subclavian artery. Two fetuses had associated trisomy 21 and 1 had a 22q11 microdeletion. Parents chose to terminate the pregnancy in all cases. Four patients successfully underwent surgical correction, and in 1 patient, tracheoplasty was also performed. In conclusion, isolated vascular rings can be accurately diagnosed prenatally by using the "3-vessel and trachea view" and "supra-aortic-branch view" that allow detection of vascular structures running around the trachea. Karyotyping and prenatal testing for 22q11 microdeletions should be offered to all parents. Affected children should undergo surgical correction as soon as symptoms of tracheal compression appear, avoiding tracheomalacia. Associated congenital tracheal stenosis should be excluded before surgery.

Objective: To report our data on selected patients with previous paradoxical embolism who underwent transcatheter patent foramen ovale (PFO) closure., Methods: Between July 2001 and July 2007, percutaneous PFO closure was performed on 128 patients (65 women, mean age: 46 +/- 12.8 years). Patent foramen ovale closure was recommended for secondary prevention in patients with previous transient ischemic attacks (52.5%), stroke (46%), or peripheral embolism (1.5%)., Results: Implantation was successful in all patients, and at the end of intervention, complete PFO closure was achieved in 70.3% of them. There were no "major" complications (ie, deaths, device embolization or thrombosis, need for cardiac surgery). The overall incidence of complications (mostly hemorrhagic) was 7%. The mean follow-up period was 32 months. Complete closure had been achieved in 78.4% and in 82.5% of patients at the third month of transesophageal echocardiography examination and at the sixth month of transcranial Doppler examination, respectively. There were no recurrent thromboembolic events during the follow-up period., Conclusions: Percutaneous closure of PFO is a feasible procedure, but it is not a risk-free technique. However, in correctly selected patients (ie, large PFO and those at risk for neurologic relapse), nearly complete PFO closure seems to provide protection from future neurologic ischaemic events at midterm follow-up.

Objective: Coronary arterial abnormalities can be one of the few negative prognostic indicators in transposition of the great arteries (TGA), and their occurrence is related to the type of spatial relationship of the great arteries. The main objective of this study was to assess whether the use of the reconstructed en-face view with color Doppler imaging of the four cardiac valves can demonstrate the different types of spatial relationship of the arterial trunks in fetuses with TGA, in order to derive the risk of coronary abnormalities. A secondary end-point was the evaluation of the type of coronary arterial branching pattern., Methods: Twenty-three fetuses with a confirmed diagnosis of TGA underwent four-dimensional (4D) echocardiography at 19-33 gestational weeks. The en-face view of the four cardiac valves and color Doppler with high persistence were employed to assess the spatial relationships of the great arteries. In all cases, confirmation of the vessels' arrangement and coronary arterial distribution was obtained at neonatal echocardiography and/or surgery., Results: The spatial relationships of the great vessels was identified correctly in 20/23 (87%) cases. The aorta was found to be located anterior to and to the right of the pulmonary trunk in 13/23 (56.5%) cases and just anterior to the pulmonary artery in 6/23 (26.1%) cases; in the remaining four (17.4%) cases, the two vessels were side by side. With respect to the association between the spatial relationship of the great arteries and the occurrence of an unusual pattern of coronary arterial branching, five of the TGA fetuses had abnormal coronary arterial distribution., Conclusions: Using 4D echocardiography with color Doppler, it is possible to define the spatial relationships of the great arteries in fetuses with TGA with a high degree of accuracy. This information can be used during counseling to predict the likelihood of abnormal coronary arterial distribution., ((c) 2008 ISUOG. Published by John Wiley & Sons, Ltd.)

Objective: To describe clinical findings in a case of premature ductal constriction associated with maternal use of nimesulide, a cyclo-oxygenase type-2 inhibitor., Methods: Case report., Results: A mother self-administered nimesulide at 39 weeks of gestation due to lower back pain. Less than 24 h later premature ductal constriction was diagnosed by echocardiography following a suspicious fetal heart rate recording with baseline tachycardia and absent accelerations., Conclusion: In a term pregnancy, nimesulide administration can rapidly cause premature ductal constriction. This condition may induce abnormalities in the fetal heart rate trace., (Copyright 2008 S. Karger AG, Basel.)

Loeys-Dietz syndrome is a recently described autosomal dominant disorder with cardinal manifestations in cardiovascular, craniofacial and skeletal systems. Although the disease has some phenotypic overlap with Marfan syndrome, the disease, that is caused by mutations in the transforming growth factor beta-receptor 1 (TGFBR1) or transforming growth factor beta-receptor 2 (TGFBR2) genes, presents many distinctive features and a particularly aggressive cardiovascular course. We describe prenatal identification of an aortic root aneurysm in a fetus of 19 week of gestation as an early marker of Loeys-Dietz syndrome.

Objective: To describe the anatomy, associated anomalies and outcome of 30 cases of congenitally corrected transposition of the great arteries (ccTGA) detected prenatally., Methods: This was a retrospective observational study of the 30 cases of ccTGA confirmed at autopsy or postnatal echocardiography seen at one of three referral centers from 1994 to 2003. The following data were considered: gestational age at diagnosis, cardiac anatomy, associated cardiac and extracardiac anomalies and fetoneonatal outcome. All fetuses underwent fetal echocardiography and a detailed anomaly scan, with follow-up scans at 3-4-week intervals until delivery. The diagnosis was confirmed at autopsy or after delivery. Follow-up data were retrieved from the clinical files of the patients., Results: The mean gestational age at diagnosis was 25.5 weeks. Intracardiac defects associated with the ccTGA included a ventricular septal defect in 21 cases, pulmonary outflow obstruction in 12 cases, an abnormal tricuspid valve in 10 cases, ventricular hypoplasia in five cases and dextro/mesocardia in five cases. The karyotype was normal in all 24 newborns, and unknown in the cases which resulted in termination of pregnancy (n = 5) or intrauterine death (n = 1). There were associated extracardiac anomalies in four cases only. Three of the four cases of atrioventricular block (AV block) developed in the third trimester, while the fourth appeared after birth. There were nine deaths (five terminations, two perinatal deaths and two infant deaths). The remaining 21 (70%) newborns were alive at a median follow-up time of 32 months, 11 of them after various surgical procedures., Conclusions: Our data suggest that in fetuses with ccTGA the risk of chromosomal and extracardiac anomalies is low, in accordance with postnatal data. The spectrum of associated cardiac lesions is consistent with that reported in the pediatric literature. These data may be of use during prenatal counseling since no figures regarding survival and/or outcome of ccTGA in the fetus have been reported so far., (2006 ISUOG. Published by John Wiley & Sons, Ltd.)

Familial recurrence of congenitally corrected transposition of the great arteries (CCTGA) is considered uncommon. Most of the previous familial studies involved a small number of patients and referred to all situs and looping anomalies including single ventricle, heterotaxia, and other cardiac defects different from CCTGA. We performed a large, consecutive clinical case series study in order to detect the recurrence of congenital heart defects in families of children with the classic form of CCTGA. From January 1997 through December 2004, 102 consecutive patients with CCTGA were evaluated in four institutions. There were 59 male (57.8%) and 43 female (42.2%). Mean age was 8.6 +/- 7.8 years. Eighty-eight patients (86.3%) had situs solitus of the atria, 14 (13.7%) situs inversus. The cardiac and extracardiac anomalies among relatives and the patterns of familial recurrence were investigated. Relatives with congenital heart defects were found in 16/102 families (15.7%). Transposition of the great arteries (TGA) was the most common recurrent defect (6/102 families). Consanguinity was identified in the parents of three probands. Six probands had an unaffected twin-sib. Recurrence risks for congenital heart defects were calculated at 5.2% (6/116) for siblings. In conclusion, CCTGA is not always sporadic in families. The pattern of inheritance, the presence of consanguinity among parents and the recurrence of situs inversus could suggest, in some families, an autosomal recessive mechanism with similarities with that occurring in some pedigrees with heterotaxia. The recurrence of TGA and CCTGA in the same family suggests a pathogenetic link between these two anatomically different malformations., ((c) 2005 Wiley-Liss, Inc.)