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Sacubitril/Valsartan (S/V) is used with optimal results in the therapy of heart failure (HF); reduces mortality and hospitalizations by 20% compared to ACE inhibitor therapy. There is a little data on the use of S/V in adult patients with congenital or acquired pediatric heart disease (GUCH). In these patients, HF is a real challenge for the cardiologist. HF in GUCH often has a different pathophysiology than usual, and interesting univentricular hearts in Fontan circulation, systemic right ventricle and patients undergoing multiple cardiac surgical procedures. The literature is poor in information and the guidelines do not give clear indications. We report our experience on a GUCH population. Starting from November 2019 we started S/V therapy in 13 GUCH patients with severe ventricular dysfunction, all in NYHA class III and already in optimal therapy for HF. Median age was 42.7 years (27–79 years); 8 males and 5 females. Basic heart disease: 4 patients with Fontan circulation, 3 patients with systemic right ventricle (TGA, operated according to Mustard), n. 2 complete CAV operated, n. 1 Ebstein‘s disease, n.1 Patent ductus arteriosus, n. 2 pediatric onset dilated cardiomyopathy. Median duration of follow–up 11.6 months (2–26 months). In n. 1 patient it was necessary to suspend the S/V due to skin itching, which resolved on diuscontinuation. The dosage was reduced in tree patients due to symptomatic hypotension (1 of these was taking Sildenafil). In n. In 1 patient there was a reduction in the glomerular filtration rate, which did not require discontinuation of the drug. After treatment: 6 patients are in NYHA class I, n. 3 pts in NYHA II class and 4 pts remained in NYHA III class. In the year preceding the start of S/V therapy, all patients cumulatively had n. 15 hospitalizations; in the year following the first six months of treatment, the number of hospitalizations decreased to no. 5 (4 for HF and 1 for severe anemia) The ejection fraction, evaluated by Simpson‘s method, remained stable. However, the limitations of the echocardiographic method in patients with such complex anatomy must be considered

DORV accounts for 2–3% of congenital heart disease (prevalence of 1/10,000). It is a cono–truncal anomaly with variable clinic. In 25–42.5% of cases there are chromosomal anomalies such as trisomy 13, 18, 22q11 microdeletion and possible associated anomalies such as facial and skeletal dysmorphisms, brain anomalies and renal agenesis. duplication Xp11.22–p11.23 was described in 2009 by Giorda and is characterized by intellectual disabilities, speech delay, epilepsy, and EEG abnormalities. We report the case of a patient with dupXp11.23 and major anomalies not reported in the literature. C.B. born from 1st pregnancy at 32+4 w, by cesarean section for fetal bradycardia and polyamnios; At first trimester screening, finding of large ventricular defect and high risk for trisomy 18 and 13. Non–resolving fetal DNA and normal 46XY karyotype. At birth weighing 1750 g, APGAR 1‘:3, 5‘:7,10‘: 8, he was intubated and transferred to intensive care. On physical examination: prominent frontal bumps, facial asymmetry with right hypoplasia, anterior plagiocephaly, microphthalmia, low–set left ear, large both pinnae, macroglossia, hypospadias. He underwent surgery for duodenal atresia and Meckel‘s diverticulum. In the following days poor clinical conditions, poor growth and manifestations of heart decompensation. On auscultation II tone of increased intensity, meso–pulmonary systolic murmur, hepatomegaly. The electrocardiogram showed normal sinus rhythm and signs of left ventricular hypertrophy. On echocardiogram Situs solitus –D loop Levocardia. Patent foramen ovale with middle left–right shunt, dilated left atrium, globular left ventricle, preserved systolic function. Poorly aligned arterial outflows emerging from the right ventricle. Large subpulmonary interventricular defect. Hyperflow flowmetry. Aortic arch dilated in the proximal tract. He therefore started therapy with furosemide. Subsequent monitoring showed poor growth for which cardiac surgery was indicated. This case report contributes to broadening the clinical spectrum of duplication syndrome Xp11.22–p11.23.

Sandy beaches are the result of a dynamic interaction among physical conditions, biological processes and the anthropic impact (essentially linked to the natural resource direct or indirect exploitation). Monitoring the health state of coastal areas is a fundamental tool for land-use management. Moreover, integrated sedimentological studies with multidisciplinary methodologies are increasingly needed. This study aims to monitor the evolution of Torre Guaceto beach (Brindisi) over different seasons. The study area is part of a protected marine reserve characterised by a relatively slight human impact and a significant availability of previous data that allows us to observe the natural dynamic effects on the health state of the beach. The research was developed by adopting different techniques in order to investigate the foreshore and the shoreface sector of the beach. The geomorphological investigation, carried out with the terrestrial laser scanner and the optical total station, aimed to quantify the variations of sediment volume of the beach, while the sedimentological and petrographical analyses were conducted to define the sand textural and compositional characteristics throughout different sampling seasons; finally, Delft3d software was applied to analyse the effects of the dominant wave motion on the sedimentary dynamics.

Quaternary uplift is well documented in SE Sicily, a region prone to damaging seismic events, such as the 1693 “Val di Noto” earthquake (Mw7.4), the largest seismic event reported within the Italian Earthquake Catalogue, whose seismogenic source is still debated and, consequently, the long‐term seismic hazard is poorly understood. However, the spatial variation in the timing and rates of uplift are still debated, so it is difficult to link the dominant tectonic process(es) responsible for the uplift and the location of seismogenic sources. To better constrain the uplift rate, we have refined the dating of Late Quaternary marine terraces, using a synchronous correlation approach, driven by both published and newly obtained numerical age controls (234U/230Th dating on corals). This has allowed recalculation of uplift rates along a N‐S oriented transect within the Hyblean Plateau (HP) foreland region. Consequently, we have mapped the geometry of paleoshorelines along a coastline‐parallel transect and hence the rates of uplift. The results suggest increasing uplift rate from south to north across the HP and that uplift rates have remained constant through the Late Quaternary. This spatially changing but temporally constant uplift places constraints on the proportion of uplift produced by regional geodynamic processes versus produced by local faults, such as an offshore east dipping active normal fault. We discuss these new findings in terms of the long‐term seismic hazard for one of the most seismically active regions in the Mediterranean Basin. Differential uplift is mapped in the Hyblean Plateau (SE Sicily, Italy) by looking at raised paleoshorelines over the Late QuaternaryDifferential uplift in the Hyblean Plateau is caused by a combined process between offshore normal faulting and regional processesDeformation rates associated to the offshore normal fault (Western Fault) need to be refined, subtracting the regional processes signal