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Introduction: Epilepsy with continuous spike-waves during slow sleep syndrome (CSWSS) is characterized by various seizure types, a characteristic EEG pattern and neuropsychological disorders. The main purpose of this study was to evaluate the long-term outcome of CSWSS occurred in childhood and to evaluate the variables that could influence the quality of social adaptation and the personality profile., Material and Methods: This is a prospective study on 24 young adults with previous CSWSS (median age 24.5 yrs) who were enrolled between January and July 2011 at the G. Gaslini Children's Hospital, Genoa, Italy. Patients were divided into two groups: twelve with previous spike-wave index (SWI > 85%) defined as typical CSWSS (T-CSWSS) and twelve with previous SWI = 50-85% defined as atypical CSWSS (A-CSWSS). All the subjects were submitted to Minnesota Multiphasic Personality Inventory-2 (MMPI-2), Psychological General Well-Being Index (PGWBI), and to a structured interview., Results: A correlation was observed with the severity of EEG abnormalities expressed by the SWI and outcome. The T-CSWSS group showed a significantly lower perceived well-being. Similarly in the T-CSWSS group the percentage of MMPI-2 clinical scales with T-scores ≥65 was higher than in the A-CSWSS group. Finally, a significant lower schooling in the T-CSWSS group was observed., Conclusion: There seem to be two forms of the same disease, with similar onset and clinical evolution but a different outcome regarding the social and psychological conditions. The outcome of the social adaptation and of the personality consciousness was related with the severity of the EEG abnormalities: more favorable in patients with less intense SWI activity (A-CSWSS) compared those with a more severe EEG impairment (T-CSWSS)., (Copyright © 2019. Published by Elsevier B.V.)

Background: Although the port-access technique has been shown to be an effective and safe approach for cardiac surgery procedures it has never become routine practice, and it is still limited to few and selected centers. Furthermore, such technique has rarely been applied to treat left ventricle disease. In 1999 we introduced left ventricle aneurysm repair through a left minithoracotomy using the port-access technique. Here we present the results in terms of early and medium-term follow-up using such technique as a routine first choice approach for left ventricle endoplasty., Methods: From 1999 to date, out of 38 patients undergoing left ventricle endoplasty (+/-associate procedures), mini-left thoracotomy and port-access techniques have been used in 32 patients (84%). All patients underwent endoventricular patch-repair with ventricular reshaping and associated procedures were performed in 8 patients., Results: All patients survived the operation and were discharged from the hospital (30 days mortality 0%). Two patients (6.2%) experienced prolonged mechanical ventilations and 3 patients (9.3%) prolonged intensive care unit stay. Mean follow-up was 40+/-34 months (range, 2 to 105). One patient died during follow-up (cumulative mortality 3.3%). Follow-up revealed an improvement of hemodynamic performances (left ventricular ejection fraction 0.44+/-0.09 compared with 0.34+/-0.09 preoperatively, p=0.004) and improved clinical conditions (New York Heart Association class 1.4+/-0.5 compared with 2.3+/-1 preoperatively, p=0.003)., Conclusions: The port-access technique can be safely applied to perform left ventricle endoplasty through a left minithoracotomy. Such approach allows optimal surgical view and therefore optimal surgical correction. Based on our satisfactory experience we support left minithoracotomy as a valuable alternative approach for left ventricle endoplasty in view of an extended use of minimally invasive techniques., (Copyright (c) 2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: A minimally invasive approach through an upper ministernotomy (UMS) has been used in our Division since 1997. On the basis of favorable outcome we have gradually extended this approach from isolated aortic valve replacement (AVR) to more complex cardiac surgery procedures and it is currently our first choice for a variety of procedures. Here we report our 11 years experience., Methods: From 1997 to December 2007, 1,126 procedures were performed at our department, using UMS. Isolated procedures on the aortic valve were performed in 695 patients (61%). Isolated procedures on the aortic valve as redo operation were performed in 77 patients (7%). Complex cardiac surgery procedures (including double valve replacement-repair, ascending aorta-aortic arch replacement, aortic root replacement, aortic dissection, AVR combined with coronary surgery, and complex redo procedures) were performed in 354 patients (32%). Early postoperative outcome was evaluated considering three different groups according to the surgical procedure (first time AVR, redo AVR, and complex procedure)., Results: Overall conversion to full sternotomy was required in 16 patients (1.4%) with no significant differences between isolated AVR (9 patients, 1.3%) and complex or redo procedures (1 patient [1.2%] and 6 patients [1.6%], respectively). Forty-seven patients died in hospital (cumulative in-hospital mortality of 4.1 %). Mortality according to the procedure was 6.7, 3.8, and 2.8% for complex, redo AVR, or isolated AVR procedures, respectively, with a significant difference only for the complex procedures. Similarly, early postoperative outcome in terms of incidence of prolonged mechanical ventilation and ICU stay was significantly different only in the complex procedure group. Incidence of surgical revision (5.1, 2.9, and 2.7% for complex, redo, or isolated AVR procedures, respectively) showed no statistically significant differences regardless the type of procedures., Conclusions: Our experience clearly shows that a minimally invasive approach through upper ministernotomy is feasible and safe not only for isolated AVR but that it can also be utilized for a variety of complex surgical procedures. Minimizing surgical access may be helpful in patients undergoing complex surgical procedures, especially redo procedures, without compromising the surgical result.

Background: Infection is the leading cause of death for left ventricular assist device (LVAD) patients with end-stage heart failure. Decreased infection may be possible with fully implantable LVADs such as the LionHeart, which lacks percutaneous conduits (PCs), a common source of device-related infection (DRI). This sub-study reports infections with the LionHeart and compares these results with historic data from the REMATCH trial, bridge to recovery (BTR) and bridge-to-transplantation (BTT) studies., Methods: Twenty-three patients were implanted with the LionHeart LVAD and followed until death or heart transplant during a non-randomized, multicenter, European trial from October 1999 to April 2004. The nature and incidence of infection were analyzed and adjudicated to definitions similar to, or the same as, the REMATCH definitions., Results: The combined number of implant days was 7,980, with a mean of 347 days (median 112, range 17 to 1,259 days). Survival at 1 year was 39%, with 2-year survival at 22%. Seventy-four percent of patients developed one or more infections, with 30% developing sepsis, and 35% developing pump-pocket infections (PSIs). No patients developed pump-housing or inflow- or outflow-tract infections (PI). For comparison, the prevalence rates of sepsis, PSI and PI in REMATCH were 51%, 35% and 19%, respectively., Conclusions: The patients in the European LionHeart Clinical Utility Baseline Study (CUBS) trial had less sepsis and less overall DRI compared with the REMATCH LVAD group. Therefore, the fully implanted device may cause less infection than PC devices during destination therapy (DT). Although lower for DT, these rates are still higher than for some BTT experiences. Areas for future improvement include miniaturization of controller/battery components to reduce wound complications related to pocket size, and installation of more modern lithium-ion batteries to decrease the need for re-operations due to battery end-of-life.

Background: Neurologic events such as thromboembolic and hemorrhagic strokes are common complications of mechanical circulatory support. We report the neurologic events observed in patients treated for end-stage heart failure with the implantable, pulsatile LionHeart left ventricular assist device (LVAD). This sub-study was part of the LionHeart European Clinical Utility Baseline Study (CUBS)., Methods: Twenty-three male patients were implanted with the LionHeart LVAD in a non-randomized, observational study. Neurologic events were classified into three categories: (1) transient ischemic attacks (TIAs); (2) strokes, including cerebrovascular accidents (CVAs) and intracranial bleeding (ICB); and (3) "other," including hypoperfusion, coma and brain death. Neurologic injuries were also categorized as transient/reversible or permanent/disabling., Results: Thirteen of 23 patients (57%) had a total of 30 neurologic events. Eight patients (35%) had 18 TIAs. Eight patients (35%) also had a stroke, either CVA (n = 5, 22%) or ICB (n = 3, 13%), and 5 of these patients (22%) also had 12 TIAs. Three patients (13%) had 4 "other" neurologic events. Ten patients (43%) had transient/reversible neurologic deficits and 10 (43%) had permanent/disabling events. One patient (4%) had intracranial bleeding as a primary cause of death (anti-coagulation-related hemorrhage). The combined incidence of neurologic events was 1.37 events/patient-year. The incidences of transient and permanent events were 0.91 and 0.46 event/patient-year, respectively., Conclusions: Neurologic events caused morbidity in the CUBS trial, with infrequent mortality. These results are similar to previous experiences with destination therapy and underscore the need for improvements in LVAD design, patient selection and patient management to reduce the incidence of neurologic events.

We evaluated exposure, safety, and efficacy data from an international Phase 3 trial of everolimus in de novo heart transplantation to characterize the longitudinal pharmacokinetics of everolimus and cyclosporine and to identify a therapeutic concentration range for everolimus. We randomized 634 patients to receive either 0.75 mg everolimus twice daily, 1.5 mg everolimus twice daily, or azathioprine in addition to corticosteroids and cyclosporine. At 8 visits during the first 6 months after transplantation, we obtained 2,328 everolimus trough levels (Cmin) and 129 area-under-the-curve (AUC) profiles over the dosing interval in patients treated with everolimus; we collected 3,258 cyclosporine trough concentrations and 174 profiles in all 3 treatment arms. We used median-effect analysis to characterize exposure-response associations between everolimus average Cmin vs freedom from biopsy-confirmed acute rejection; maximum cholesterol, low density lipoprotein, triglyceride, and creatinine levels; and minimum leukocyte and platelet counts. Everolimus Cmins averaged 5.2 +/- 3.8 ng/ml and 9.4 +/- 6.3 ng/ml at the lower and upper dose levels. A 17% underproportionality was noted in Cmins; however, peak exposure and AUC were consistent with dose proportionality. Everolimus exposure was stable during the 6-month period. Interindividual variability was 37% for AUC and 40% for Cmin. The latter parameter was not influenced to a clinically relevant extent by sex, age, or weight. The Cmin was well correlated with AUC (r2 = 0.81). Everolimus Cmin was significantly related to freedom from rejection (p = 0.02) with 3 ng/ml being an informative lower threshold for efficacy. Thrombocytopenia, defined as <75 x 10(9)/liter, was related significantly to Cmin (p = 0.03); however, the incidence in this study was too low to establish an upper end for the therapeutic range. Lower doses of cyclosporine (by 15% to 19%) were used in patients treated with everolimus to achieve cyclosporine Cmins and AUCs similar to those in patients treated with azathioprine. Everolimus exposure was dose proportional and stable during the first 6 months after transplantation. Interindividual pharmacokinetic variability was high but not influenced by common demographic covariates. We observed a significantly increased risk of acute rejection at everolimus trough levels <3 ng/ml, which constitutes the lower therapeutic concentration limit when everolimus is used with conventionally dosed cyclosporine. Everolimus-related adverse events were manageable up to the highest troughs (22 ng/ml) observed in this population. We could not derive a precise upper therapeutic concentration limit from these data.

Background: The connection between the donor and the recipient aorta is a potential source of early and late complications as a result of infection, compliance mismatch, and technical and hemodynamic factors. Moreover, the abrupt change in systolic pressure after heart transplantation involves the entire thoracic aorta in the risk of aneurysm formation. The aim of this study was to analyze the types of aortic complications encountered in our heart transplantation series and to discuss etiology, diagnostic approach, and modes of treatment., Methods: Of the 442 patients having orthotopic heart transplantation and the 11 patients having heterotopic heart transplantation at our center, 9 (2%) sustained complications involving the thoracic aorta. These 9 patients were divided into four groups according to the aortic disease: acute aortic rupture (2 patients); infective pseudoaneurysm (3 patients); true aneurysm and dissection of native aorta (2 patients); and aortic dissection after heterotopic heart transplantation (2 patients). Surgical intervention was undertaken in 8., Results: Five (83%) of 6 patients who underwent surgical treatment for noninfective complications survived the operation, and 4 are long-term survivors. One patient who underwent a Bentall procedure 71/2 years after heterotopic heart transplantation died in the perioperative period of low-output syndrome secondary to underestimated chronic rejection of the graft. One patient with pseudoaneurysm survives without surgical treatment but died several years later of cardiac arrest due to chronic rejection. Both patients operated on for evolving infective pseudoaneurysm died in the perioperative period., Conclusions: Infective pseudoaneurysms of the aortic anastomosis are associated with a significant mortality. In noninfective complications, an aggressive surgical approach offers good long-term results. The possibility of retransplantation in spite of complex surgical repair should be considered in the late follow-up after heart transplantation, due to the increasing incidence of chronic rejection.

Background: Tacrolimus (FK506) has recently become available clinically as an alternative to cyclosporine-based immunosuppression. This study reports the middle-term results of a prospective, randomized trial that compared FK506 with cyclosporine-based immunosuppression in heart transplant recipients., Methods: Twenty-five consecutive patients were randomized at a 2:1 ratio into two groups, one of which received FK506 (15 patients), the other cyclosporine (10 patients). Both groups received similar concomitant immunosuppression. The patients were followed up for 12 months. The following outcome parameters were analyzed: survival, rejection and infection rate, lymphocyte subsets, new-onset diabetes, renal and hepatic function, hypertension, right-sided heart catheterization data, graft coronary artery disease, and neurologic side effects., Results: The mortality rate (two patients) in the FK506 group was 13% versus 0% in the cyclosporine group (p = NS). The two deaths were the consequences of early infections and higher doses of FK506. From the outset, the FK506 group presented a lower prevalence of acute rejection, a lower requirement for rejection treatments and a higher incidence of infections. Accordingly, we reduced overall immunosuppression for the last seven patients in the FK506 group; the decrease in FK506 and prednisone dosage led to a decrease in the early infection rate without an increase in the rejection rate. There was no difference between the two groups in diabetes incidence, renal and hepatic function, right-sided heart catheterization data, or coronary angiograms. Hypertension was less frequent and milder in the FK506 group., Conclusions: This experience suggests that FK506 can be safely used in heart transplantation. It can decrease the frequency of rejection episodes. Low-dose administration allows a lower infection rate without an increase in rejection. With a protocol of delayed starting and low dosing, side effects such as renal toxicity, hypertension, and neurologic toxicity seem to be unlikely. Further studies are needed to establish the exact dosage and therapeutic levels of the drug.

Three patients underwent surgical ablation for ventricular tachycardia resulting from an aneurysm of the membranous portion of the ventricular septum. Two patients had a definite history of cardiac murmur during infancy, and one of them was found at the time of operation to have a left-to-right shunt through the apex of the aneurysm. The earliest ventricular activation sites were located around the neck of the aneurysm and were ablated in 1 patient by encircling the endocardial ventriculotomy and by cryoablation in the remaining 2. After focus resection had been completed, aneurysm resection and ventricular septal reconstruction were performed. All patients were alive and free of ventricular tachycardia and did not need medication as of 61, 66, and 88 months postoperatively. Spontaneous closure of a ventricular septal defect may lead to the formation of an aneurysm in the ventricular septum that may sustain ventricular tachycardias. Such arrhythmias can be effectively treated using electrically guided surgical techniques.

Atrial tachycardia is an infrequent but potentially dangerous arrhythmia which often determines cardiac enlargement. Surgical ablation of the arrhythmia is effective and safe, provided a careful atrial mapping is performed and the surgical technique is tailored to the individual focus location. Eight patients underwent surgical ablation of ectopic atrial tachycardia between 1977 and 1990. Different techniques were adopted for each patient according to the anatomical location of the focus and possibly associated arrhythmias. Whenever possible, a closed heart procedure was chosen. In 1 patient a double focal origin was found and treated by separate procedures. In 1 patient with ostium secundum atrial septal defect and atrial flutter, surgical isolation of the right appendage and the ectopic focus was performed. In all patients ectopic atrial tachycardia was ablated with maintenance of the sinoatrial and atrioventricular nodal function as well as internodal conduction. In follow-up up to December 1991, no recurrency was recorded.

The affinities of selective, pirenzepine and AF-DX 116, and classical, N-methylscopolamine and atropine, muscarinic cholinergic receptor antagonists were investigated in displacement binding experiments with [3H]Pirenzepine and [3H]N-methylscopolamine in membranes from human autoptic tissues (forebrain, cerebellum, atria, ventricle and submaxillary salivary glands). Affinity estimates of N-methylscopolamine and atropine indicated a non-selective profile. Pirenzepine showed differentiation between the M1 neuronal receptor of the forebrain and the receptors in other tissues while AF-DX 116 clearly discriminated between muscarinic receptors of heart and glands. The results in human tissues confirm the previously described selectivity profiles of pirenzepine and AF-DX 116 in rat tissues. These findings thus reveal the presence also in man of three distinct muscarinic receptor subtypes: the neuronal M1, the cardiac M2 and the glandular M3.