The use of aerosolized - Adrenergic agonist therapy after lung transplantation in pediatric age

It is my opinion the use of aeresolized –adrenergic agonist such as salbutamol may be helpfull&nbsp; not only&nbsp; to reduced the early mortality but also&nbsp; the chronic allograft dysfunction&nbsp; after lung transplantation in children The reduction of pulmonary&nbsp; edema could be prevent the induction of Obstructive Bronchiolites Syndrome (less pneumonia&nbsp; and viral infections etc) such as the primary graft dysfunction.As we know by international literature Lung transplantation continues to suffer from less-than-ideal long-term outcomes, even in pediatric patients.</p

Case report: Coronary allograft vasculopathy: an accurate reflection of the histopathological findings on cardiovascular magnetic resonance imaging

Heart transplant recipients undergo extensive invasive and non-invasive postoperative screening to exclude complications, such as allograft rejection and vasculopathy. Cardiac magnetic resonance imaging is a non-invasive, non-irradiating, diagnostic tool for monitoring graft health and identifying possible tissue rejection or myocardial fibrosis. We describe the case of a 29-year-old female heart transplant recipient admitted to our care center with a worsening clinical condition. The patient underwent clinical evaluation, blood tests, including troponin I and N-terminal pro brain type natriuretic peptide, transthoracic echocardiography, invasive coronary angiography, and cardiovascular magnetic resonance imaging. Cardiovascular magnetic resonance imaging showed widespread sub-epicardial hyperintensity of the myocardial segments along the course of the coronary arteries. T2 mapping sequences showed an elevated value and the myocardial native T1 values and extracellular volume percentage were significantly increased. Late gadolinium enhancement demonstrated a diffuse sub-epicardial hypersignal along the lateral, free, and left ventricular walls. All the sequences evidenced widespread hyper-enhancement of epicardial fat along the course of the thickened main coronary artery walls. One month later, the recipient underwent re-transplantation due to progressive worsening of the clinical condition and refractoriness to intravenous medication. The anatomopathological findings of the explanted heart provided impressive visualization of structural and histopathological changes. These results could guide the tailoring of preventive therapeutic strategies and non-invasive monitoring of cardiac grafts

Incidence of Healthcare-Associated Infections in a Pediatric Population With an Extracorporeal Ventricular Assist Device

During the last decade, ventricular assist devices (VADs) have become a precious tool to support children with end-stage heart failure. However, thromboembolic events, bleeding, and infections may have a considerable impact on outcome. We retrospectively analyzed the incidence of healthcare-associated infections (HAIs) in nine patients supported by EXCOR Pediatric (Berlin Heart [BH]) VAD in a pediatric cardiosurgical intensive care unit between January 1, 2009 and March 31, 2011 (27 months). Median age was 8 months (interquartile range [IQR] 6-11), median weight 7.5kg (IQR 4.5-8.5). Seven patients were supported with a left VAD, two with a biventricular VAD (BiVAD). Six patients with a left VAD underwent heart transplant after 89 days (median, IQR 41-143) of support. One patient is still on the waiting list. All patients with BiVAD died after 12 days of assistance due to VAD malfunction. Sixteen HAIs were reported in five out of nine patients (56%). All infected patients were supported by a left VAD. When compared with noninfected patients, they had a longer mechanical support period (median 131 days, IQR 75-164, vs. 25 days, IQR 11-61, P=0.03), a longer intensive care unit stay (median 159 days, IQR 85-188, vs. 48 days, IQR 17-87, P=0.06) and a longer length of hospital stay (median 186 days, IQR 105-222, vs. 64 days, IQR 34-113, P=0.06). Overall, nine mechanical devices were replaced for thromboembolic issues, most of them (67%) in patients with VAD-related infections. Overall, infection rate was 17.6 per 1000 patients days, 1.3 BH endocarditis per 1000 BH days, 4.0 surgical sites infections per 1000 BH days, 12.5 central line-associated blood stream infections per 1000 central venous catheter days, 5 catheter-associated urinary tract infections per 1000 urinary catheter days, and 13.5 ventilator-associated pneumonia cases per 1000 mechanical ventilation days. Overall, VAD-related infections were 5.4 per 1000 BH days. Of the 17 isolated pathogens, 53% were Gram-negative rods, with a prevalence of Pseudomonas aeruginosa (35.3%). Four bacteria were multidrug resistant (25%), three were carbapenem-resistant P. aeruginosa (50% of all isolated pseudomonads), and one was a methicillin-resistant S.aureus. VADs used as a bridge to cardiac transplantation are associated with a large number of HAIs. Patients with infected VADs were admitted for longer time in intensive care and in hospital with increased healthcare costs but with no impact on surviva

Clinical presentation and natural history of hypertrophic cardiomyopathy in RASopathies

RASopathies are a heterogeneous group of genetic syndromes characterized by mutations in genes that regulate cellular processes, including proliferation, differentiation, survival, migration, and metabolism. Excluding congenital heart defects, hypertrophic cardiomyopathy is the most frequent cardiovascular defect in patients affected by RASopathies. A worse outcome (in terms of surgical risk and/or mortality) has been described in a specific subset of Rasopathy patients with early onset, severe hypertrophic cardiomyopathy presenting with heart failure. New short-term therapy with a mammalian target of rapamycin inhibitor has recently been used to prevent heart failure in these patients with a severe form of hypertrophic cardiomyopathy