Patients with SS have a high overall survival. Survival probability was lower in patients with associated CHDs and in patients with pulmonary hypertension. Surgical treatment of SS is beneficial in reducing symptoms, however, given the significant risk of post-operative scimitar drainage stenosis/occlusion, it should be tailored to a comprehensive haemodynamic evaluation and to the patient's age.

Chest ultrasonography is a useful tool to assess extravascular lung water at bedside. In presence of interstitial-alveolar imbibition, vertical artifacts arising from the pleura are detected; these are called B-lines. Although a positive linear correlation between B-lines and extravascular lung water has been shown in symptomatic heart failure patients, the subclinical phase of pulmonary imbibition and the clearance of B-lines after rapid body fluid removal have been less investigated. The aim of this study was to assess if chest ultrasound could detect lung water imbibition and its variations induced by dialysis, an experimental model of controlled rapid fluid loss. Forty-one patients undergoing hemodialysis were studied. Total number of B-lines from ultrasound chest scanning and vena cava diameters were measured before and after treatment. Before dialysis, most of the patients presented ultrasound signs of pulmonary imbibition despite the absence of dyspnea; the number of B-lines was associated with the accumulated weight before treatment (p < 0.05) as well as with the residual weight after dialysis (p < 0.01); B-lines and end-inspiratory and end-expiratory vena cava diameters were also significantly reduced after dialysis. Moreover, B-lines reduction was significantly related to weight loss. Ultrasound performed at the bedside can detect lung water and intravascular overload and their reduction after dialysis in yet asymptomatic patients. These observations add further evidence regarding the use of lung ultrasound and inferior vena cava measurement in estimating volume overload and monitoring the response to therapy both in hemodialysis and congestive heart failure patients.

Congenital heart diseases (CHDs) and cardiovascular abnormalities are one of the pillars of clinical diagnosis of 22q11.2 deletion syndrome (22q11.2DS) and still represent the main cause of mortality in the affected children. In the past 30 years, much progress has been made in describing the anatomical patterns of CHD, in improving their diagnosis, medical treatment, and surgical procedures for these conditions, as well as in understanding the underlying genetic and developmental mechanisms. However, further studies are still needed to better determine the true prevalence of CHDs in 22q11.2DS, including data from prenatal studies and on the adult population, to further clarify the genetic mechanisms behind the high variability of phenotypic expression of 22q11.2DS, and to fully understand the mechanism responsible for the increased postoperative morbidity and for the premature death of these patients. Moreover, the increased life expectancy of persons with 22q11.2DS allowed the expansion of the adult population that poses new challenges for clinicians such as acquired cardiovascular problems and complexity related to multisystemic comorbidity. In this review, we provide a comprehensive review of the existing literature about 22q11.2DS in order to summarize the knowledge gained in the past years of clinical experience and research, as well as to identify the remaining gaps in comprehension of this syndrome and the possible future research directions.

Background Pediatric patients with atrioventricular valve disease have limited options for prosthetic valve replacement in sizes <15 mm. Based on successful experience with the stented bovine jugular vein graft (Melody valve) in the right ventricular outflow tract, the prosthesis has been modified for surgical valve replacement in pediatric patients with atrioventricular dysfunction with the intention of subsequent valve expansion in the catheterization laboratory as the child grows. Methods and Results A multicenter, retrospective cohort study was performed among patients who underwent atrioventricular valve replacement with Melody valve at 17 participating sites from North America and Europe, including 68 patients with either mitral (n=59) or tricuspid (n=9) replacement at a median age of 8 months (range, 3 days to 13 years). The median size at implantation was 14 mm (range, 9-24 mm). Immediately postoperatively, the valve was competent with low gradients in all patients. Fifteen patients died; 3 patients underwent transplantation. Nineteen patients required reoperation for adverse outcomes, including valve explantation (n=16), left ventricular outflow tract obstruction (n=1), permanent pacemaker implantation (n=1), and paravalvular leak repair (n=1). Twenty-five patients underwent 41 episodes of catheter-based balloon expansion, exhibiting a significant decrease in median gradient ( P<0.001) with no significant increase in grade of regurgitation. Twelve months after implantation, cumulative incidence analysis indicated that 55% of the patients would be expected to be free from death, heart transplantation, structural valve deterioration, or valve replacement. Conclusions The Melody valve is a feasible option for surgical atrioventricular valve replacement in patients with hypoplastic annuli. The prosthesis shows acceptable short-term function and is amenable to catheter-based enlargement as the child grows. However, patients remain at risk for mortality and structural valve deterioration, despite adequate early valvular function. Device design and implantation techniques must be refined to reduce complications and extend durability. Clinical Trial Registration URL: https://www.clinicaltrials.gov. Unique identifier: NCT02505074.

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Additive and logistic EuroSCORE algorithms performed similarly, and cumulative evidence suggests comparable performance in CABG and OPCAB procedures; both risk models, however, significantly overestimated mortality.

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Background: The aim of this prospective study is to evaluate the role in the onset of surgical site infections of bilateral internal thoracic arteries harvesting in patients with decompensated preoperative glycemia.