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The aim of this study is to identify echocardiographic predictors of transient left ventricle dysfunction after pulmonary valve balloon dilatation (PVBD), in neonates with pulmonary valve stenosis (PVS) and atresia with intact septum (PAIVS) at birth. The study includes patients admitted at the Bambino Gesù Children Hospital from January 2012 to January 2017. Clinical, echocardiographic and cardiac catheterization data before and after PVBD were retrospectively analyzed. Twenty-nine infants were included in the study (21 male and eight female). The median age was 5.8 ± 7.1 days. Eight patients developed transient LV dysfunction (three PAIVS and five PVS) and comparing data before and after the procedure, there was no difference in right ventricle geometrical and functional parameters except for evidence of at least moderate pulmonary valve regurgitation after PVBD. Moderate to severe degree pulmonary valve regurgitation was significant associated to LV dysfunction ( < 0.05) in PVS and PAIVS patients.

Patients with repaired Tetralogy of Fallot (rToF) typically report having preserved subjective exercise tolerance. Chronic pulmonary regurgitation (PR) with varying degrees of right ventricular (RV) dilation as assessed by cardiac magnetic resonance imaging (MRI) is prevalent in rToF and may contribute to clinical compromise. Cardiopulmonary exercise testing (CPET) provides an objective assessment of functional capacity, and the International Physical Activity Questionnaire (IPAQ) can provide additional data on physical activity (PA) achieved. Our aim was to assess the association between CPET values, IPAQ measures, and MRI parameters. All rToF patients who had both an MRI and CPET performed within one year between March 2019 and June 2021 were selected. Clinical data were extracted from electronic records (including demographic, surgical history, New York Heart Association (NYHA) functional class, QRS duration, arrhythmia, MRI parameters, and CPET data). PA level, based on the IPAQ, was assessed at the time of CPET. Eighty-four patients (22.8 ± 8.4 years) showed a reduction in exercise capacity (median peak VO2 30 mL/kg/min (range 25–33); median percent predicted peak VO2 68% (range 61–78)). Peak VO2, correlated with biventricular stroke volumes (RVSV: β = 6.11 (95%CI, 2.38 to 9.85), p = 0.002; LVSV: β = 15.69 (95% CI 10.16 to 21.21), p < 0.0001) and LVEDVi (β = 8.74 (95%CI, 0.66 to 16.83), p = 0.04) on multivariate analysis adjusted for age, gender, and PA level. Other parameters which correlated with stroke volumes included oxygen uptake efficiency slope (OUES) (RVSV: β = 6.88 (95%CI, 1.93 to 11.84), p = 0.008; LVSV: β = 17.86 (95% CI 10.31 to 25.42), p < 0.0001) and peak O2 pulse (RVSV: β = 0.03 (95%CI, 0.01 to 0.05), p = 0.007; LVSV: β = 0.08 (95% CI 0.05 to 0.11), p < 0.0001). On multivariate analysis adjusted for age and gender, PA level correlated significantly with peak VO2/kg (β = 0.02, 95% CI 0.003 to 0.04; p = 0.019). We observed a reduction in objective exercise tolerance in rToF patients. Biventricular stroke volumes and LVEDVi were associated with peak VO2 irrespective of RV size. OUES and peak O2 pulse were also associated with biventricular stroke volumes. While PA level was associated with peak VO2, the incremental value of this parameter should be the focus of future studies.

Marfan syndrome (MIM: # 154700; MFS) is an autosomal dominant disease representing the most common form of heritable connective tissue disorder. The condition presents variable multiorgan expression, typically involving a triad of cardiovascular, eye, and skeletal manifestations. Other multisystemic features are often underdiagnosed. Moreover, the disease is characterized by age related penetrance. Diagnosis and management of MFS in the adult population are well-described in literature. Few studies are focused on MFS in the pediatric population, making the clinical approach (cardiac and multiorgan) to these cases challenging both in terms of diagnosis and serial follow-up. In this review, we provide an overview of MFS manifestations in children, with extensive revision of major organ involvement (cardiovascular ocular and skeletal). We attempt to shed light on minor aspects of MFS that can have a significant progressive impact on the health of affected children. MFS is an example of a syndrome where an early personalized approach to address a dynamic, genetically determined condition can make a difference in outcome. Applying an early multidisciplinary clinical approach to MFS cases can prevent acute and chronic complications, offer tailored management, and improve the quality of life of patients.

has been sporadically reported in Asia and South America but not in Europe. This study reports the first European outbreak of in a paediatric hospital, outlining diagnostic challenges and containment measures. Fifteen isolates were recovered from blood cultures of consecutive pediatric patients admitted to intensive care units between April and August 2025. Identification was performed using MALDI-TOF MS, chromogenic media, Fourier-transform infrared (FT-IR) spectroscopy, and whole-genome sequencing (WGS). Antifungal susceptibility testing was performed by broth microdilution. All isolates were initially misidentified as by MALDI-TOF MS and undetected by the FilmArray BCID2 panel. WGS confirmed , and FT-IR analysis revealed clonally related strains, indicating an outbreak. Colonies displayed a distinct deep-blue color on chromogenic CHROMagar™ medium. Elevated fluconazole minimum inhibitory concentrations were observed, while isolates remained susceptible to echinocandins and amphotericin B. A multidisciplinary infection-control response halted transmission within four weeks. This investigation documents the first outbreak in Europe, highlighting diagnostic limitations of current commercial tools and the need for updated databases. Integration of FT-IR spectroscopy and WGS facilitated outbreak detection and containment, underscoring the importance of advanced diagnostics and surveillance for emerging fungal pathogens.

Background: Adults with congenital heart disease (ACHD) are a growing population needing ongoing care. The aim of this study was to investigate if a dedicated ACHD team impacted the timing and indication of invasive cardiology procedures in these patients at our hospital. Methods: Our retrospective single-center study enrolled adult patients with moderate or complex congenital heart disease and with at least one cardiac catheterization between January 2010 and December 2021. According to the period, procedures were labeled as group A (2010 to 2015) or group B (2016 to 2021) and further divided into diagnostic (DCC) and interventional cardiac catheterizations (ICC). Results: 594 patients were eligible for the study. Both DCC (p < 0.05) and ICC increased between groups A and B (p < 0.05). In group B: Fontan patients accounted for the majority of DCC (p < 0.001), while DCC decreased in arterial switch repair (p < 0.001). In Fontan patients, conduit stenting was prevalent (p < 0.001), while fenestration closures dropped (p < 0.01). In patients with tetralogy of Fallot and native outflow tract, percutaneous pulmonary valve implantations (PPVI) increased, with a concurrent reduction in pulmonary valve replacements (p < 0.001 vs. surgical series). In right ventricular conduits, ICC increased (p < 0.01), mainly due to PPVI. Among Mustard/Senning patients, baffle stenting increased from Group A to Group B (p < 0.001). In patients with pulmonary atresia and biventricular repair, ICC often increased for pulmonary artery stenting. Conclusions: A dedicated working group could improve ACHD patients’ indications for interventional procedures, leading to tailored treatment, better risk stratification and optimizing time until heart transplantation.

Background In the last few years trio‐whole exome sequencing (WES) analysis has demonstrated its potential in obtaining genetic diagnoses even in nonspecific clinical pictures and in atypical presentations of known diseases. Moreover WES allows the detection of variants in multiple genes causing different genetic conditions in a single patient, in about 5% of cases. The resulting phenotype may be clinically discerned as variability in the expression of a known phenotype, or as a new unreported syndromic condition. Methods Trio‐WES was performed on a 4‐month‐old baby with a complex clinical presentation characterized by skeletal anomalies, congenital heart malformation, congenital hypothyroidism, generalized venous and arterial hypoplasia, and recurrent infections. Results WES detected two different homozygous variants, one in CEP57, the gene responsible for mosaic variegated aneuploidy syndrome 2, the other in DYNC2H1, the main gene associated with short‐rib thoracic dysplasia. Conclusion The contribution of these two different genetic causes in determining the phenotype of our patient is discussed, including some clinical signs not explained by the detected variants. The report then highlights the role of WES in providing complete and fast diagnosis in patients with complex presentations of rare genetic syndromes, with important implications in the assessment of recurrence risk. Whole exome sequencing (WES) allows the detection of variants in multiple genes causing different genetic conditions in a single patient. The resulting phenotype may be clinically discerned as variability in the expression of a known phenotype, or as a new unreported syndromic condition. Trio‐WES was performed in a 4 months patient with complex clinical presentation, and detected two different homozygous variants, one in CEP57, the gene responsible of mosaic variegated aneuploidy syndrome 2, the other in DYNC2H1, the main gene associated with short‐rib thoracic dysplasia. The contribution of these two different genetic causes in determining the phenotype of our patient is discussed, including some clinical signs not explained by the detected variants.

The ideal aortic valve substitute for young and middle-aged adults with aortic valve disease is still debated. The Ross procedure (pulmonary autograft replacement) is the only operation that allows replacement of the diseased aortic valve with a living substitute. During the years, the use of this procedure has declined significantly due to concerns over increased intraoperative risk and especially potential long-term failure of the operation. However, several recent publications have shown that, after appropriate learning curve and in specialized centers, the Ross procedure can be performed safely in appropriately selected patients. Moreover, comparative studies suggest that the Ross procedure is associated with better long-term outcomes compared to conventional aortic valve replacement in young and middle-aged adults.

The massive, pragmatic and intellectual work by Peter Stein on the Oresteia of Aeschylus perfectly summarizes the German director’s conception of ‘classic’. This essay reviews the twenty-years period which started with the first experimental approach to ancient tragedy by the Schaubühne to the staging of the trilogy in Moscow in 1994. In his work, Stein investigates the origins of western theatre, compares the role of gestures and words at the dawn of theatre with that of the contemporary age, and then moves, in line with the evolution of the Aeschylus trilogy plot, to the study of the purely political connotation of the text. Precisely the post-soviet Russia represented the ideal setting where to reproduce the rite of dismissal of high hierarchies, whether of divine nature or not, and the affirmation of the new democratic system.L’imponente lavoro pratico e intellettuale di Peter Stein sull’Orestea di Eschilo riassume in maniera esemplare il pensiero del regista tedesco riguardo all’idea di ‘classico’. Nel saggio si traccia il percorso lungo i vent’anni che vanno dal primo sperimentale approccio alla tragedia antica da parte del gruppo della Schaubühne fino alla messinscena moscovita della trilogia del 1994. Nel suo lavoro Stein riflette sulle origini del teatro in Occidente, sul ruolo del gesto e della parola agli albori del teatro e in epoca contemporanea, spostando poco a poco lo sguardo, in linea con l’evoluzione dell’intreccio della trilogia eschilea, sul significato prettamente politico del testo. Proprio la Russia postcomunista si qualifica allora luogo ideale in cui ripetere il rito di destituzione di ogni ordine superiore (ove il termine sia da intendere nel senso più lato, non solo divino) e affermazione del nuovo sistema democratico.

The massive, pragmatic and intellectual work by Peter Stein on the Oresteia of Aeschylus perfectly summarizes the German director’s conception of ‘classic’. This essay reviews the twenty-years period which started with the first experimental approach to ancient tragedy by the Schaubühne to the staging of the trilogy in Moscow in 1994. In his work, Stein investigates the origins of western theatre, compares the role of gestures and words at the dawn of theatre with that of the contemporary age, and then moves, in line with the evolution of the Aeschylus trilogy plot, to the study of the purely political connotation of the text. Precisely the post-soviet Russia represented the ideal setting where to reproduce the rite of dismissal of high hierarchies, whether of divine nature or not, and the affirmation of the new democratic system. L’imponente lavoro pratico e intellettuale di Peter Stein sull’Orestea di Eschilo riassume in maniera esemplare il pensiero del regista tedesco riguardo all’idea di ‘classico’. Nel saggio si traccia il percorso lungo i vent’anni che vanno dal primo sperimentale approccio alla tragedia antica da parte del gruppo della Schaubühne fino alla messinscena moscovita della trilogia del 1994. Nel suo lavoro Stein riflette sulle origini del teatro in Occidente, sul ruolo del gesto e della parola agli albori del teatro e in epoca contemporanea, spostando poco a poco lo sguardo, in linea con l’evoluzione dell’intreccio della trilogia eschilea, sul significato prettamente politico del testo. Proprio la Russia postcomunista si qualifica allora luogo ideale in cui ripetere il rito di destituzione di ogni ordine superiore (ove il termine sia da intendere nel senso più lato, non solo divino) e affermazione del nuovo sistema democratico.

Aortic reconstruction at the time of the comprehensive stage II (CSII) procedure can be complicated by compression within the aortopulmonary space resulting in airway or pulmonary artery narrowing. We describe our experience with 2 patients with hypoplastic left heart syndrome and pulmonary artery stenosis after the CSII procedure. Both patients underwent an aortic extension with a Hemashield interposition graft to open up the aortopulmonary space. The patients were discharged from the hospital. In all cases the aortopulmonary space was enlarged, and the pulmonary arteries and airway were free from compression. Aortic extension is an option to be considered in children with pulmonary artery compression who previously had a CSII procedure.