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IntroductionThe incidence of adverse short-term outcomes for infants who undergo complex congenital heart disease (CHD) surgery with cardiopulmonary bypass (CPB) is still high. Early identification and treatment of high-risk patients remain challenging, especially because clinical risk factors often fail to explain the different outcomes of this vulnerable population. Metabolomics offers insight into the phenotype of the patient and the complex interplay between the genetic substrate and the environmental influences at the time of sampling. For these reasons, it may be helpful to identify the mechanisms of physio-pathological disruptions experienced in neonates undergoing congenital heart surgery and to identify potential therapeutic targets.MethodsWe conducted a systematic review (PROSPERO: ID 565112) of studies investigating the association between targeted or untargeted metabolomic analysis of infants undergoing elective surgery with CPB for CHD and clinical outcomes. The PRISMA guidelines were followed. We searched MEDLINE via PubMed, EMBASE via Ovid, the Cochrane Central Register of Controlled Trials, the Cochrane Library, ClinicalTrials.gov and the World Health Organization's International Trials Registry and Platform.ResultsSeven studies involving 509 children (aged 1 day to 21.3 months), all of whom underwent cardiac surgery requiring CPB, were included for qualitative analysis. We found associations between metabolomic profiles and various clinical outcomes, such as mortality, acute kidney injury (AKI), and neurological outcomes. Specific metabolites (mainly amino acids, their metabolic products and fatty acids) were identified as potential biomarkers for these outcomes, demonstrating the utility of metabolomics in predicting certain postoperative complications.ConclusionThe quality of the evidence was limited due to heterogeneity in study designs and small sample sizes, but the findings are promising and suggest that further research is warranted to confirm these associations.Systematic Review Registrationhttps://www.crd.york.ac.uk/prospero/, PROSPERO ID 565112.

Aim: Transcatheter Pulmonary Valve Implantation (TPVI), when feasible, is the first-line approach to pulmonary valve replacement. Our aim was to obtain a picture of current TPVI practice in Italy. Methods: After conducting a literature review on TPVI, online surveys were devised by an Advisory Board of 10 experts from the three Italian reference centers for congenital heart diseases and sent electronically to physicians working either in implanting center or in referral non-implanting cardiologic centers. Results: Approximately 450 physicians across Italy were invited to contribute. 82 (18%) physicians answered. EchoColorDoppler, electrocardiogram and cardiac magnetic resonance were considered the first line approach to monitor these patients, before and after TPVI.For non-implanting centers, reasons for non-referral of patients for PVR were: paucisymptomatic disease (67%) and patients’ poor adherence to disease management programs (41%), but also the lack of connections with specialized centers (33%). For implanters, the main reasons for refraining from TPVI were: high risk for coronary compression (67% first rank), the need for concomitant cardiac surgical procedures (39% first rank) and the unsuitable anatomy of the conduit (39% first rank). The availability of new larger valves of a self-expandable nature was indicated as a key technological development for expanding the cohort of patients currently eligible for TPVI. Conclusions: Despite a non-invasive imaging protocol for the follow up and selection of patients candidate to TPVI is well implemented in Italy, there is still a lack in connections between non-implanting and implanting centers.

Percutaneous aortic coarctation treatment with primary stent implantation is the first choice in adult patients. However, current guidelines do not suggest a lower weight limit to perform this procedure safely.The aim of this study was to retrospectively analyze the safety and the mid-term outcome of aortic coarctation stenting in pediatric age at different patients’ weights.We enrolled 47 patients, 8 of them weighting lower than 25 kg, 10 with a weight between 25 and 30 kg and 29 patients with a weight >30 kg.Covered CP stent was used in 32 patients (68.1%), bare CP stent in 6 (12.8%), Andrastent XL in 8 (17.0%) and Palmaz Genesis in one case (2.1%). The balloon mean diameter was 13.8 ± 2.4 mm, range 10–18 mm. The procedure was effective in all patients. The aortic gradient dropped from 28.0 ± 7.8 mmHg to 2.2 ± 2.0 mmHg (p