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There is a paucity of data about mid-term outcome of patients with advanced heart failure (HF) treated with left ventricular assist device (LVAD) in Europe, where donor shortage and their aging limit the availability and the probability of success of heart transplantation (HTx). The aim of this study is to compare Italian single-centre mid-term outcome in prospective patients treated with LVAD vs. HTx. We evaluated 213 consecutive patients with advanced HF who underwent continuous-flow LVAD implant or HTx from 1/2006 to 2/2012, with complete follow-up at 1 year (3/2013). We compared outcome in patients who received a LVAD ( n  = 49) with those who underwent HTx ( n  = 164) and in matched groups of 39 LVAD and 39 HTx patients. Patients that were treated with LVAD had a worse risk profile in comparison with HTx patients. Kaplan–Meier survival curves estimated a one-year survival of 75.5 % in LVAD vs. 82.3 % in HTx patients, a difference that was non-statistically significant [hazard ratio (HR) 1.46; 95 % confidence interval (CI) 0.74–2.86; p  = 0.27 for LVAD vs. HTx]. After group matching 1-year survival was similar between LVAD (76.9 %) and HTx (79.5 %; HR 1.15; 95 % CI 0.44–2.98; p  = 0.78). Concordant data was observed at 2-year follow-up. Patients treated with LVAD as bridge-to-transplant indication ( n  = 22) showed a non significant better outcome compared with HTx with a 95.5 and 90.9 % survival, at 1- and 2-year follow-up, respectively. Despite worse preoperative conditions, survival is not significantly lower after LVAD than after HTx at 2-year follow-up. Given the scarce number of donors for HTx, LVAD therapy represents a valid option, potentially affecting the current allocation strategy of heart donors also in Europe.

Heritable thoracic aortic disease can result from null variants in MYLK, which encodes myosin light-chain kinase (MLCK). Data on which MYLK missense variants are pathogenic and information to guide aortic disease management are limited. Clinical data from 60 cases with MYLK pathogenic variants were analyzed (five null and two missense variants), and the effect of missense variants on kinase activity was assessed. Twenty-three individuals (39%) experienced an aortic event (defined as aneurysm repair or dissection); the majority of these events (87%) were aortic dissections. Aortic diameters were minimally enlarged at the time of dissection in many cases. Time-to-aortic-event curves showed that missense pathogenic variant (PV) carriers have earlier-onset aortic events than null PV carriers. An MYLK missense variant segregated with aortic disease over five generations but decreases MYLK kinase acitivity marginally. Functional Assays fail to identify all pathogenic variants in MYLK. These data further define the aortic phenotype associated with MYLK pathogenic variants. Given minimal aortic enlargement before dissection, an alternative approach to guide the timing of aortic repair is proposed based on the probability of a dissection at a given age.

Heart disease has an impact on patient's identity and self-perception. Taking into account the wide literature about psychological aspects before and after heart transplant, it clearly emerges that there is a lack of data and results for patients up to implantation of ventricular assist devices (VAD). The aim of the present study was to explore quality of life and factors correlated with psychological adjustment in patients supported with VAD. From February 2013 to August 2014, 18 patients (17 male, mean age 57 years) under clinical evaluation before and after VAD implantation were enrolled. During interviews, patients were assessed with EuroQoL-5D questionnaire to monitor improvement of quality of life before implantation and at 3 and 6 months; critical issues, needs and point of views of patients have been described. A significant improvement in the quality of life score was observed at 3 (score 38 [interquartile range 30-40] vs 75 [60-80], p<0.05) and 6 months (38 [30-40] vs 70 [60-80], p<0.05). Overall, patients' psychological state investigated by the test showed a clear and positive trend. All patients need to empower through complete information about the device, related risks and life expectancy. Interdisciplinary approach improved compliance with therapy. Successful treatment and efficient psychological care are closely related to assessment and continuous clinical support. This approach ensures a better selection of patients and improves their compliance. Further data are needed to support our preliminary observations and to explore long-term quality of life.

Razionale. La malattia cardiaca ha un impatto significativo sull’identità e la percezione di sé. A fronte di una vasta letteratura sulle variabili psicologiche in pazienti in fase pre- e post-trapianto di cuore, si è riscontrato che pochi dati sono stati raccolti in pazienti candidati a impianto di dispositivo di assistenza ventricolare (VAD). Materiali e metodi. Da febbraio 2013 ad agosto 2014, 18 pazienti (17 maschi, età media 57 anni) sono stati sottoposti a valutazione psicologica tramite la somministrazione del questionario EuroQoL-5D nella fase precedente l’impianto e successivamente a 3 e 6 mesi al fine di valutare la qualità di vita nel tempo; sono state esaminate variabili psicologiche quali criticità, bisogni e vissuti soggettivi dei pazienti. Risultati. L’aspetto più rilevante che emerge è un netto miglioramento del punteggio relativo alla percezione della qualità di vita a 3 (punteggio 38 [range interquartile 30-40] vs 75 [60-80], p<0.05) e a 6 mesi dall’impianto (38 [30-40] vs 70 [60-80], p<0.05). Anche i punteggi relativi all’item “Ansia e depressione” hanno mostrato nel tempo un miglioramento significativo. Dai colloqui è emerso che tutti i pazienti necessitano di essere coinvolti e motivati attraverso un’informazione esauriente circa il dispositivo, i rischi correlati e la qualità di vita. L’approccio interdisciplinare ha favorito l’aderenza ai trattamenti. Conclusioni. L’outcome clinico dei pazienti e l’efficacia dell’intervento psicologico sono strettamente correlati all’attività di valutazione e di supporto psicologico sul lungo periodo. Riteniamo che ulteriori dati siano utili a supporto delle nostre osservazioni preliminari e per un’analisi più approfondita della qualità di vita a lungo termine.

Acute aortic dissection type A (AADA) is a disease that has a catastrophic impact on a patient's life. Although refinements in perioperative and surgical care have translated into improved outcomes for patients affected by AADA, hospital mortality after surgery still remains very high, ranging from 15% to 30%. The management of AADA is complex and dictated by the modality of presentation, extent and location of the disease. Attempts to formulate consensus statements and relevant guidelines have identified significant gaps in the AADA knowledge with reference to pathogenesis, appropriate management and configuration for clinical services. Several international registries have been created to generate an evidence base that can address current and future management. In this context, the extensive use of surgical/hybrid approaches in treating AADA should be encouraged as well as more extensive interventions to replace the entire dissected aortic root and aortic arch tissues. In addition, the excellent outcome results recently achieved by high-volume surgical centers with focused expertise in AADA pointed out the need for regionalized super-centers offering specific multidisciplinary aortic surgery programs with dedicated high-specialized surgeons.

Heart transplantation (HTx) is considered to be the gold standard treatment for advanced heart failure (HF) but it is available only for a minority of patients, due to paucity of donor hearts (278 HTx were performed in 2011 in Italy). Patients listed for HTx have a prolonged waiting time (that is about 2.3 years in the 2006-2010 time period in Italy) that is superior compared with patients who receive HTx (median time around 6 months), to underline the presence of an allocation system that prioritizes candidates in critical conditions. Patients listed for HTx have a poor quality of life and their annual mortality is around 8-10%. Another 10-15% of HTx candidates are removed from the waiting list each year because they are no longer suitable for transplantation. On the other hand, continuous-flow left ventricular assist devices (LVADs) have been demonstrated to improve survival and quality of life of patients with advanced/refractory HF. LVAD therapy can represent a valid alternative to HTx, and it is recommended for patients with advanced HF in the recent edition of the European Society of Cardiology guidelines on HF management. In the United States, a larger number of centers compared with European ones started to apply a strategy of LVAD implant for many patients who meet clinical criteria for listing for HTx. Data from our center concerning the last 6 years of LVAD implant (51 implants since 2006) reported a 75.5% survival rate at 1 year. In Italian series, as in our center, current HTx survival is only slightly superior (83% survival rate at 1 year), based on data from the Italian National Transplant Center. We report a proposal for updated listing criteria for HTx and indications for LVAD implant in patients with advanced acute and chronic HF. Criteria for identifying suitable patients for HTx and/or LVAD considering the shortage of donors are discussed.

Acute aortic dissection type A (AADA) is a disease that has a catastrophic impact on a patient’s life. Although refinements in perioperative and surgical care have translated into improved outcomes for patients affected by AADA, hospital mortality after surgery still remains very high, ranging from 15% to 30%. The management of AADA is complex and dictated by the modality of presentation, extent and location of the disease. Attempts to formulate consensus statements and relevant guidelines have identified significant gaps in the AADA knowledge with reference to pathogenesis, appropriate management and configuration for clinical services. Several international registries have been created to generate an evidence base that can address current and future management. In this context, the extensive use of surgical/hybrid approaches in treating AADA should be encouraged as well as more extensive interventions to replace the entire dissected aortic root and aortic arch tissues. In addition, the excellent outcome results recently achieved by high-volume surgical centers with focused expertise in AADA pointed out the need for regionalized super-centers offering specific multidisciplinary aortic surgery programs with dedicated high-specialized surgeons.

Background. Heart disease has an impact on patient’s identity and self-perception. Taking into account the wide literature about psychological aspects before and after heart transplant, it clearly emerges that there is a lack of data and results for patients up to implantation of ventricular assist devices (VAD). The aim of the present study was to explore quality of life and factors correlated with psychological adjustment in patients supported with VAD.Methods. From February 2013 to August 2014, 18 patients (17 male, mean age 57 years) under clinical evaluation before and after VAD implantation were enrolled. During interviews, patients were assessed with EuroQoL-5D questionnaire to monitor improvement of quality of life before implantation and at 3 and 6 months; critical issues, needs and point of views of patients have been described. Results. A significant improvement in the quality of life score was observed at 3 (score 38 [interquartile range 30-40] vs 75 [60-80], p<0.05) and 6 months (38 [30-40] vs 70 [60-80], p<0.05). Overall, patients’ psychological state investigated by the test showed a clear and positive trend. All patients need to empower through complete information about the device, related risks and life expectancy. Interdisciplinary approach improved compliance with therapy. Conclusions. Successful treatment and efficient psychological care are closely related to assessment and continuous clinical support. This approach ensures a better selection of patients and improves their compliance. Further data are needed to support our preliminary observations and to explore long-term quality of life.

La dissezione acuta dell’aorta di tipo A (AADA) è una patologia dall’impatto catastrofico sulla vita dei pazienti che ne sono colpiti. Nonostante l’affinamento delle metodiche diagnostiche, dei progressi in campo anestesiologico e chirurgico, la mortalità postoperatoria rimane drammaticamente alta, con un range compreso tra il 15% e il 30%. Il management dell’AADA implica, infatti, un approccio chirurgico complesso, una gestione anestesiologica perioperatoria impegnativa e un follow-up altamente specifico, soprattutto per il monitoraggio di possibili complicazioni tardive che spesso susseguono alla dissezione. Numerosi registri internazionali sono stati creati al fine di delineare attraverso la raccolta meticolosa di dati ed esperienze le basi per offrire un’ottimale gestione clinico-chirurgica del paziente affetto da AADA. Diversi sono, tuttavia, le lacune ed i punti dibattuti sul management appropriato dell’ADAA. Un utilizzo sempre più diffuso della cosiddetta “chirurgia ibrida” e un approccio chirurgico più radicale ed estensivo a livello del bulbo aortico e dell’arco aortico sembrerebbero garantire un migliore outcome per le complicanze tardive della patologia dissecativa. Le recenti evidenze scientifiche riportanti i risultati eccellenti in termini di mortalità operatoria ottenuti nei centri a maggiore volume, sembrerebbero chiaramente suggerire la creazione di super-centri regionali ad alto volume con un team polispecialistico di figure professionali dedicato al trattamento dell’AADA.