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Kawasaki disease (KD) can be associated with high morbidity and mortality due to coronary artery aneurysms formation and myocardial dysfunction. Aim of this study was to evaluate the diagnostic performance of non-invasive myocardial work in predicting subtle myocardial abnormalities in Kawasaki disease (KD) children with coronary dilatation (CADL). A total of 100 patients (age 8.7 ± 5 years) were included: 45 children with KD and CADL (KD/CADL) (Z-score > 2.5), 45 age-matched controls (CTRL) and, finally, an additional group of 10 children with KD in absence of coronary dilatation (KD group). Left ventricular (LV) systolic function and global longitudinal strain (GLS) were assessed. Global myocardial work index (MWI) was calculated as the area of the LV pressure-strain loops. From MWI, global Constructive Work (MCW), Wasted Work (MWW) and Work Efficiency (MWE) were estimated. Despite normal LV systolic function by routine echocardiography, KD/CADL patients had lower MWI (1433.2 ± 375.8 mmHg% vs 1752.2 ± 265.7 mmHg%, p < 0.001), MCW (1885.5 ± 384.2 mmHg% vs 2175.9 ± 292.4 mmHg%, p = 0.001) and MWE (994.0 ± 4.8% vs 95.9 ± 2.0%, p = 0.030) compared to CTRL. Furthermore, MWI was significantly reduced in children belonging to the KD group in comparison with controls (KD: 1498.3 ± 361.7 mmHg%; KD vs CTRL p = 0.028) and was comparable between KD/CADL and KD groups (KD/CADL vs KD p = 0.896). Moreover, KD/CADL patients with normal GLS (n = 38) preserved significant differences in MWI and MCW in comparison with CTRL . MWI, MCW and MWE were significantly reduced in KD children despite normal LVEF and normal GLS. These abnormalities seems independent from CADL. Thus, in KD with normal LVEF and normal GLS, estimation of MWI may be a more sensitive indicator of myocardial dysfunction.

Reference hemoglobin (Hb) values for the definition of anemia are still largely based on the 1968 WHO Scientific Group report, which established a cutoff value of <13 g/dL for adult men and <12 g/dL for adult nonpregnant women. Subsequent studies identified different normal values according to race and age. Estimated prevalence of anemia on admission in the setting of an acute coronary syndrome (ACS) is between 10% and 43% of the patients depending upon the specific population under investigation. Furthermore, up to 57% of ACS patients may develop hospital-acquired anemia (HAA). Both anemia on admission and HAA are associated with worse short- and long-term mortality, even if different mechanisms contribute to their prognostic impact. Baseline anemia can usually be traced back to preexisting disease that should be specifically investigated and corrected whenever possible. HAA is associated with clinical characteristics, medical therapy and interventional procedures, all eliciting cardiovascular adaptive response that can potentially worsen myocardial ischemia. The intrinsic fragility of anemic patients may limit aggressive medical and interventional therapy due to an increased risk of bleeding, and could independently contribute to worse outcome. However, primary angioplasty for ST elevation ACS should not be delayed because of preexisting (and often not diagnosed) anemia; delaying revascularization to allow fast-track anemia diagnosis is usually feasible and justified in non-ST-elevation ACS. Besides identification and treatment of the underlying causes of anemia, the only readily available means to reverse anemia is red blood cell transfusion. The adequate transfusion threshold is still being debated, although solid evidence suggests reserving red blood cell transfusions for patients with Hb level <8 g/dL and considering it in selected cases with Hb levels of between 8 and 10 g/dL. No evidence supports the use of iron supplements and erythropoiesis-stimulating agents in the setting of ACS.

The Occlutech Atrial Flow Regulator (AFR) is a self-expandable double-disc nitinol device with a central fenestration. Its use has been approved in the adult population with heart failure and described for pulmonary hypertension (PH). Only case reports and small series have been published about its use in the paediatric population and for congenital heart disease (CHD). The authors sought to investigate the feasibility, safety, and short-term follow-up of AFR implantation in patients with CHD or children with PH or cardiomyopathy. This is a multicenter retrospective study involving 10 centers worldwide. Patients of any age with CHD or patients aged < 18 years with PH or cardiomyopathy needing AFR implantation were included. A total of 40 patients underwent AFR implantation. The median age of the population at the time of the procedure was 58.5 months (IQR: 31.5-142.5) and the median weight was 17 kg (IQR: 10-46). A total of 26 (65.0%) patients had CHD, nine (22.5%) children, a cardiomyopathy, and five (12.5%), a structurally normal heart. The implantation success rate was 100%. There were two early and one late device thrombosis. Two patients (5.0%) with dilated cardiomyopathy on extracorporeal membrane oxygenator (ECMO) died during the hospital stay. At a median follow-up of 330 days (IQR: 125-593), 37 (92.5%) patients were alive. At follow-up, 20 patients improved their New York Heart Association (NYHA) class, 12 patients did not change their NYHA class, and one patient with idiopathic PH worsened. AFR implantation in patients with CHD and children with severe PH or cardiomyopathy is promising and seems to have beneficial effects at short-term follow-up.

This international multi-center study aimed to demonstrate the feasibility and reliability of non-invasive myocardial work (MW) parameters in the pediatric population, and to provide normal reference ranges for this useful echocardiographic tool in this specific subset of patients. In this retrospective multi-center study involving three pediatric laboratories, 150 healthy children and adolescents (mean age of 10.6 ± 4.5, 91 males) were enrolled. A complete echocardiographic examination has been performed, including global longitudinal strain (GLS) assessment. The following parameters of non-invasive MW have been obtained through a dedicated software: global work index (GWI), global constructive work (GCW), global work waste (GWW), and global work efficiency (GWE), using left ventricular (LV) strain loops and non-invasive brachial artery cuff pressure values. The lowest expected values were for GWI 1,723 mmHg% in males and 1,682 mmHg% in females, for GCW 2,089 and 2,106 mmHg%, for GWE 95.9 and 95.5% whereas the highest expected value for GWW was 78 mmHg% in men and 90 mmHg% in women. The univariable and multivariable analysis showed significant associations between either GWI or GCW with SBP (β coefficient = 0.446, < 0.001; β coefficient = 0.456, < 0.001, respectively) and LV GLS (β coefficient = -0.268, = 0.001; β coefficient = -0.233, = 0.003, respectively). Inter- and intra-observer variability showed good reproducibility of non-invasive MW parameters. Non-invasive MW parameters were feasible and reliable in the pediatric population. This study provided normal reference ranges of these useful echocardiographic indices.

The Atrial Flow Regulator (AFR) is a self-expandable double-disk device with a central fenestration, intended to maintain a calibrated communication across the interatrial septum. Only case reports and small case series have been published about its use in the pediatric and congenital heart disease (CHD) population. We described AFR implantation in three congenital patients with different anatomies and indications. In the first case, the AFR was deployed to create a stable fenestration in a Fontan conduit, while in the second, it was used to reduce a Fontan fenestration. In the third case, we implanted an AFR to decompress the left atrium of an adolescent with complex CHD in natural history, with complete mixing, ductal-dependent systemic circulation and combined pulmonary hypertension. This case series demonstrates the great potential of the AFR device in the CHD field, showing versatility, efficacy, and safety in establishing a calibrated and stable shunt, with promising hemodynamic and symptomatic benefits.

Transcatheter closure of perimembranous ventricular septal defect is an alternative to surgery in selected small to medium defects with a reasonable distance between the defect and the aortic or tricuspid valves. Surgical closure is generally preferred for large defects with an inlet to outlet extension, aortic malalignment, or deficient aortic rim. We report two successful cases of percutaneous closure of large perimembranous ventricular septal defects with inlet to outlet extension using an Amplatzer Vascular Plug-II via a retrograde approach. Transcatheter closure of large perimembranous ventricular septal defect with inlet to outlet extension is feasible and facilitated by the characteristics of the Amplatzer Vascular Plug-II device and the specific implantation technique.

The Atrial Flow Regulator (AFR) is a self-expandable double-disc device with a central fenestration, intended to maintain a calibrated communication across the interatrial septum. We reported for the first time a stent implantation across an AFR device in an adolescent born with complex congenital heart disease with duct-dependent systemic circulation and severe combined pulmonary hypertension.

Access to complex stenotic pulmonary arteries can be challenging due to their anatomy or secondary to prior multiple surgeries and interventions. Two techniques have been previously described to address this issue: the telescopic catheter-in-long sheath parallel to a stiff guidewire technique and the use of a microcatheter in a telescopic scope. We integrated and modified these techniques creating a super telescopic system with a SuperCross® microcatheter-in-catheter-in-long sheath, parallel to a contralateral stiff guidewire to access a previously repaired and stented left pulmonary artery. The stiff wire support and the 90° flexiblity of the Supercross® microcatheter assembled coaxial to the diagnostic catheter and the long sheath contributed to the successful ballooning and stenting-in-stent of the pulmonary artery.

Objective Prior studies have suggested that the principal determinants of arterial stiffening are age, BP and others CV risk factors such as dyslipidemia and diabetes. However, scanty data are available on the role of psychological factors on arterial stiffness. The aim of the current cross-sectional study was to evaluate the association between depression, anxiety, perceived stress, Type A personality, and Type D personality and Pulse Wave Velocity (PWV) in a cohort of hypertensive patients, using baseline examination data of the TIPICO project. Methods A total of 259 outpatients (ages 18–80 years) followed by the Hypertension Unit of S. Gerardo Hospital (Monza, Italy) affected by essential hypertension were recruited. Aortic stiffness was evaluated by c-f PWV. Moreover, anamnestic data, clinical BP, and laboratory data were evaluated. Patients were asked to complete a battery of psychological questionnaires under the guidance of a psychologist. Results At T0 mean age was 55.9 ± 10.1years, SBP/DBP were 135.6 ± 17.7/82.5 ± 9.1 mmHg and PWV was 8.6 ± 2.1m/s. The multivariate stepwise linear regression analysis showed that age (beta = 0.284, p < 0.001), pulse pressure (beta = 0.369, p < 0.001), dyslipidemia (beta = 0.130, p = 0.012), family history of CV disease (beta = −0.123, p = 0.017), and depression (beta = 0.126, p = 0.014) were significantly and independently associated with PWV. Conclusion Among psychological factors, higher levels of depression is related to higher PWV, while anxiety, perceived stress, Type-A personality and Type-D personality are not. Depression assessment and target intervention to reduce it should be recommended in hypertensive patients.

Percutaneous closure of secundum atrial septal defect (sASD) in children with interrupted inferior vena cava is challenging, especially in case of deficient aortic rim. Trans-jugular access is generally preferred in this scenario. Patients with situs inversus and sASD also carry technical difficulties for transcatheter closure because of the orientation of the atrial septum. We report a successful case of percutaneous closure of a sASD with deficient aortic rim using an occlutech figulla flex II ASD device through the left internal jugular vein in a child with situs inversus, dextrocardia, and interrupted IVC. This case was facilitated by the absence of left-sided hub of the Occlutech device to provide stable opening of the device into the left atrium, whereas the ball-connection of the delivery system allowed an angle of almost 180 degrees between the device and the atrial septum.