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Purpose As Italy faced SARS-CoV-2 outbreak as first country outside China, and our hospital converted most of activities into the ones for COVID-19 patients (pts), we had to manage the need for continuing care of advanced heart failure (HF), heart transplant (HT) and LVAD pts. TM was a possible strategy, but its role in this very sick cohort is unknown. Methods During the lockdown (03-05/2020), we decided to make either a phone (PV) or an in presence (IV) visit, selecting for IV pts listed for HT, with LVAD, recently HT, scheduled for a biopsy within 6 months after HT or a RHC for listing eligibility. In PV, we assessed symptoms, blood pressure, drugs, and programmed a subsequent IV. All pts in IV group were triaged by phone for COVID-19 symptoms or contacts and if scheduled for RHC or biopsy received SARS-CoV-2 swab 48 h before the procedure. Study endpoints were: combined incidence at 6 months of MACE (HF hospitalization, CV death and need for anticipated IV) in HF/VAD group, and MACE, rejection and any cause- hospitalization in HT group. Results Among 448 pts (57±12y, 240 HT, 191 HF, 17 LVAD), 52% were managed by PV and a subsequent IV was scheduled after 3±2 months. Pts managed by PV were healthier: in HF-VAD group they were less frequently listed, had less Afib, LVAD (2/17) (p

Purpose Advanced Heart Failure (A-HF) is a clinical condition which interferes with patient identity and perception. Psychological reactions depend on the nature and severity of patient disease, personality and environmental factors. Our goal was to evaluate quality of life of outpatients with left ventricular assist device (LVAD), heart transplantation (HT) and optimal medical treatment (OMT) for heart failure (HF). We additionally analyzed two cohorts of patients resulting different in terms of objective disease severity, to assess eventual perceived quality of life differences. Methods We included all consecutive adult patients presenting at our outpatient clinic for routine follow-up between January and September 2019. Including criteria were: being subjected to right heart catheterization (RHC), presence of LVAD for at least 2 months, HT for at least 2 months. Clinical severity of HF has been stratified using RHC and 6-minute walk test. Psychological parameters were measured by Kellner Symptom Questionnaire, Brief-Illness Perception Questionnaire, and WHO Quality of Life -BREF. Results Overall population consisted of 179 patients (HT=90, LVAD=15, and OMT-HF =74). OMT-HF patients were stratified into advanced and non-advanced (A-HF/NA-HF). A-HF patients walked significant less meters (p 3 (p=0.89), walked meters (p=0.32), CI (p=0.82). In psychological well-being comparison between OMT, LVAD and HT, patients who underwent advanced surgical therapy showed better results in terms of anxiety (p=0.014), depression (p=0.003), anger (p=0.04) and illness perception (p Conclusion Patients undergoing a major surgical procedure, such as LVAD implantation or Heart Transplantation, showed overall better psychological parameters than patients with chronic heart failure treated with medical therapy.

Purpose Left ventricular assist devices (LVAD) have been developed to support cardiocirculatory function in patients with advanced heart failure, who are refractory to optimal medical treatment. This created the need to identify a professional figure for dedicated management of LVAD patients. Methods In this report, we analyzed all the accesses of all consecutive adult LVAD recipients at our outpatient care unit and the impact of VAD coordinator clinical/technical assessment on patients global management, at the time of COVID-19 pandemic in the period January to August 2020 Results During the study period, 19 LVAD patients had overall 357 contacts with the clinic, for different combinations of: advanced driveline dressing (n=280), log file analysis and technical check-up (n=200), clinical visit (n=102), ramp test (n=17). In the majority of accesses, the patient was seen by the VAD coordinator only, (n=238; 67%), mainly for driveline dressing, which was associated with technical check-ups in 89 cases.Overall, alarms were managed by technical check-ups and log file analyses in 14 cases (6 high priority alarms, 3 cases of high watt or low flow, and 5 cases of low-priority alarms). Ramp tests were performed during right heart catheterization, with contextual echocardiography performed by the VAD coordinator: in 11 out of 17 cases the ramp test led to medical therapy or LVAD settings optimization. Conclusion LVAD patients need frequent outpatients dedicated admissions for proper monitoring. The VAD coordinator is a key healthcare professional representing the main interface for LVAD patients, in support of physicians’ throughout the duration of mechanical support, to preserve optimal outcomes.

Purpose Paradigm HF trial showed that Sacubitril/Valsartan (LCZ696) 97/103 mg bid reduces the risk of death and hospitalizations for heart failure (HF). We tested the hypothesis that this clinical benefit is reflected by changes in hemodynamic profile in a population of patients (pts) affected by advanced HF. Methods Among pts included in our prospective HF Registry, we enrolled those who underwent two right heart catheterizations (RHC) and started LCZ696 in between, from May 2017 to April 2019. Baseline and follow-up RHC were compared in a matched paired fashion. We collected hemodynamic, clinical and echocardiographic data at both RHC. The dose of LCZ696 was expressed as a percentage of the target dose (TD,97/103 mg bid). The endpoint were hemodynamic changes between the two RHC. Changes in symptoms, echo parameters and medical therapy were also assessed. Results 44 pts (88% males; 54±8 yrs; 50% DCM; systolic blood pressure 108±17 mmHg; LVEF: 27±5%; NYHA III-IV: 46%) underwent a baseline RHC, started LCZ696 few days after, then repeated a RHC 178±59 days after the beginning of LCZ696. All pts were on diuretics (including MRAs), ACE-i/ARBs and beta-blockers at baseline. By comparing the two RHC, we observed a significant reduction in right atrial (from 7.5±2.5 to 5.6±2.5 mmHg), mean pulmonary artery (from 31.0±10.4 to 26.5±10.2 mmHg), pulmonary capillary wedge pressures (from 19.8±7.7 to 17.9±8.5 mmHg), transpulmonary gradient (from 10.4±4.6 to 8.6±3.8 mmHg) and pulmonary vascular resistances (from 2.7±1.5 to 2.2±1.3 WU), (p ≤ 0.01 for all), without significant change in systolic and mean systemic pressure. 50% of pts were taking ≤ 37.5% % of the TD; the hemodynamic changes were not influenced by LCZ696 dose. NYHA class improved (III-IV: 40.4 vs 16.7%, p Conclusion Our study shows that LCZ696 improves hemodynamic profile and symptoms in pts with advanced HF by reducing pulmonary and left ventricular filling pressures. This effect seems to be dose-independent. Our results suggest that LCZ696 can lead to a significant clinical and hemodynamic improvement even at low doses.

Purpose Gene expression profiling (GEP) of endomyocardial biopsies (EMB) by the MMDx system has been shown to estimate the probability of T-cell mediated (TCMR) and antibody-mediated rejection (AMR), improving the histology-based diagnoses in heart transplant (HT) recipients. Second generation analysis allowed to identify an additional phenotype characterised by molecules related to tissue injury (INJ), such as de-differentiation pathways, macrophages transcripts, and heart transcripts. The clinical correlates of this novel profile and its association with ISHLT pathology grading is under development. In this study we analyse the association of MMDx novel profiles with cardiac function and with ISHLT grades for TCMR and AMR. Methods 254 EMBs from 121 patients were analysed with MMDx platform. EMBs were performed according to standard protocol during the first 5 years after transplant and by clinical indication later on. Right heart catheretization was performed at the time of EMB, which were graded by 1990 and 2006 ISHLT classifications for TCMR and 2014 guidelines for AMR. Results Prevalence of molecular phenotypes varied during time course: INJ was frequent in the first months, absent during the first 5 years, and rose again in late for-cause EMBs; AMR peaked early and late after transplant, in line with DSA time-course; TCMR probability was meaningful in the early months only. Cardiac function also varied across the three profiles: INJ was significantly associated with low cardiac index (CI) and high right atrial pressure, AMR with elevated wedge pressure and low CI, and TCMR with low CI only. Overall, GEP was in agreement with pathology readings in most cases, being normal in 90% of the 0 graded EMB, and abnormal in over 70% of the 2R or pAMR2. While current 1R grade showed abnormal GEP only in 37% of cases, the old 1B grade had abnormal GEP in 60% of cases, mostly associated with AMR profile. INJ profile was found in 54% of the 2R or greater EMBs. Conclusion GEP reveals variable molecular patterns during transplant time course; INJ phenotype appears associated mainly with low CI in the context of post-operative injury and severe mixed rejection, while AMR seems to increase markers of graft stiffness. Despite good overall agreement with pathology readings, current ISHLT grading is inaccurate, with the “old” 1B grade being highly associated with AMR molecular profiling.

Purpose Little is known about the role of ECG in heart transplanted (HT) patients (pts). In this study, we sought to identify ECG parameters that could help to identify graft dysfunction and to stratify prognosis. Methods Pts enrolled in a prospective study aimed to test a novel immune monitoring test (Quantiferon monitor), consecutively coming at our Clinic (2014-17) constituted study cohort. We created 3 groups: A) normal graft function (HT 5 yrs); C) LVEF Results Among 153 pts (58±14 yrs, 75%M), 79% had normal graft function (51% A, 28% B). Pts with graft dysfunction (21%), compared with groups A and B respectively, had a different distance from HT (1.2±1.6 vs 15.7±5.7 vs 12.9±7.4 yrs), more frequently CAV (8.8% vs 14.9% vs 64%), longer PQ (181±53 vs 163±38 vs 148±25 ms), wider QRS (131±34 vs 105±18 vs 101±18 ms), longer QTc (473±5 vs 445±4 vs 453±3 ms), more conduction disorders (75% vs 51.2% vs 39.2%) and Afib (9.1% vs 0 vs 4.6%), p≤ 0.01 for all; heart rate was intermediate between A and B. Distance from HT (i.e. >5yrs) and, similarly, CAV, carried a longer PQ, wider QRS, more conduction disturbances (p Conclusion ECG parameters in HT are influenced by many factors, reflecting multiple pathways leading to graft dysfunction. QTc may be a marker of subclinical systolic dysfunction (i.e. microvascular disease, chronic rejection) and help in identifying high risk pts.

Resume Objectif. – L’allergie aux Rosacees est peu frequente chez l’enfant en France. A contrario, elle est au 4 e rang des allergies alimentaires de l’enfant en Espagne. Notre etude se propose d’analyser les caracteristiques de l’allergie aux Rosacees dans une population d’enfants francais en milieu mediterraneen et de mettre en evidence ses specificites. Patients et methodes. – Cette etude retrospective a collige 22 cas d’allergie alimentaire pediatrique aux fruits de la famille des Rosacees. Le diagnostic evoque sur l’anamnese clinique a ete confirme par la realisation de prick-tests natifs, de prick-tests commerciaux et de CAP-RAST Pharmacia ® . La sensibilisation au bouleau a egalement ete recherchee par prick-test. Resultats. – Deux groupes ont ete individualises : 12 enfants sensibilises au bouleau et huit enfants non sensibilises. Pour deux enfants cette sensibilisation n’a pu etre determinee. La faible valeur diagnostique du prick-test commercial est commune aux deux groupes. Le groupe bouleau negatif a debute son allergie par la peche et est sensibilise systematiquement a l’extrait natif cuit des fruits auxquels il est allergique. Le nombre de reactions severes a ete plus frequent. Les prick-tests commerciaux ne sont positifs que dans ce groupe. Le groupe bouleau positif est plus frequemment allergique a la pomme et le syndrome oral predomine. Il presente une mediane de sensibilisations aux fruits crus plus elevee. Conclusion. – La region mediterraneenne francaise possede un profil specifique de sensibilisations aux Rosacees avec 60 % de sensibilisations type Europe du Nord (pomme–bouleau) et 40 % de sensibilisation type Espagne–Italie (lipid-transfer-protein) .

The aim of the present study was to evaluate the prevalence of soy allergy (positive skin test and positive challenge test) in a large cohort of atopic children, many of them soy fed early in life for several months. In order to investigate the prevalence of soy allergy, two groups of children were enrolled into the study. The first group comprised a cohort of 505 children with personal history suggestive of food allergy. The second group included 243 children born of atopic parents, who had been soy protein formula fed for the first six months of life for the prevention of cow's milk allergy and who had been prospectively followed up, from birth to 5 years. As regards the prevalence of soy allergy in the cohort of children suffering from allergic disease: 31/505 children (6%) had positive skin prick test to soy, however only six of the 31 children with positive skin prick test to soy had positive challenge test to soy. With regard to the prevalence of soy allergy in the children who had been soy protein formula fed in the first six months of life (second group): 14/243 children (6%) had positive skin prick test to soy, but the double blind placebo control oral food challenge to soy was positive in only one of these 14 children. In conclusion documented soy allergy is not common in atopic children.

UNLABELLED Antibodies directed against human thrombin are exceedingly rare, having only been reported in adult patients with underlying diseases. Consensus on the most appropriate management has not yet been reached. A 12-y-old girl presented with intractable menorrhagia several days after an acute infectious episode. Laboratory tests revealed disturbed clotting tests: prothrombin index 17%, activated partial thromboplastin time >150 s, thrombin time >120 s, and failure to achieve correction with a normal pooled plasma. Further studies demonstrated the presence of an antibody directed against human thrombin. Viral serology revealed a 1/128 titre for adenovirus. Massive haemorrhage was unresponsive to standard treatments, but intravenous administration of recombinant factor VIIa resulted in a successful outcome. CONCLUSION This is the first report of an anti-human thrombin antibody associated with severe bleeding in a child. Recombinant factor VIIa could represent a novel therapeutic approach for such patients.

Objective: To investigate the presence of lower limb entheseal abnormalities in IBD patients without clinical signs and symptoms of SpA and their correlation with IBD clinical variables. Methods: A total of 81 IBD patients [55 Crohn's disease (CD) and 26 ulcerative colitis (UC), 43 females and 38 males, mean age 41.3 (12.4) years, BMI 24 (2)] with low active (12) and inactive (67) disease were consecutively studied with US (LOGIQ5 General Electric 10-MHz linear array transducer) of lower limb entheses and compared with 40 healthy controls matched for sex, age and BMI. Quadriceps, patellar, Achilleon and plantar fascia entheses were scored according to the 0-36 Glasgow Ultrasound Enthesitis Scoring System (GUESS) and power Doppler (PD). Correlations of GUESS and PD with IBD features [duration, type (CD/UC) and activity (disease activity index for CD/Truelove score for UC)] were investigated. The intra- and inter-reader agreements for US were estimated in all images detected in patients and controls. Results: Of the 81 patients, 71 (92.6%) presented almost one tendon alteration with mean GUESS 5.1 (3.5): 81.5% thickness (higher than controls P0.9 intra-class correlation variability). Conclusions: US entheseal abnormalities are present in IBD patients without clinical signs and symptoms of SpA. US enthesopathy is independent of activity, duration and type of gut disease. © The Author 2011. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved