Nitroxyl anion (NO ؊ ) is the one-electron reduction product of nitric oxide (NO ⅐ ) and is enzymatically generated by NO synthase in vitro. The physiologic activity and mechanism of action of NO ؊ in vivo remains unknown. The NO ؊ generator Angeli's salt (AS, Na2N2O3) was administered to conscious chronically instrumented dogs, and pressure-dimension analysis was used to discriminate contractile from peripheral vascular responses. AS rapidly enhanced left ventricular contractility and concomitantly lowered cardiac preload volume and diastolic pressure (venodilation) without a change in arterial resistance. There were no associated changes in arterial or venous plasma cGMP. The inotropic response was similar despite reflex blockade with hexamethonium or volume reexpansion, indicating its independence from baroreflex stimulation. However, reflex activation did play a major role in the selective venodilation observed under basal conditions. These data contrasted with the pure NO donor diethylamine͞NO, which induced a negligible inotropic response and a more balanced veno͞arterial dilation. AS-induced positive inotropy, but not systemic vasodilatation, was highly redox-sensitive, being virtually inhibited by coinfusion of N-acetyl-L-cysteine. Cardiac inotropic signaling by NO ؊ was mediated by calcitonin gene-related peptide (CGRP), as treatment with the selective CGRP-receptor antagonist CGRP-(8 -37) prevented this effect but not systemic vasodilation. Thus, NO ؊ is a redox-sensitive positive inotrope with selective venodilator action, whose cardiac effects are mediated by CGRP-receptor stimulation. This fact is evidence linking NO ؊ to redox-sensitive cardiac contractile modulation by nonadrenergic͞noncholinergic peptide signaling. Given its cardiac and vascular properties, NO ؊ may prove useful for the treatment of cardiovascular diseases characterized by cardiac depression and elevated venous filling pressures.N itric oxide (NO ⅐ )-related species play a crucial role in diverse physiological processes, including blood pressure regulation, neurotransmission, and cytostatic͞cytotoxic signaling (1). Whereas some NO ⅐ -mediated cardiovascular effects are firmly established, its control over myocardial contractility remains controversial in that positive, negative, or neutral effects can be observed. The net result varies with the tissue preparation, NO ⅐ concentration and donor, and myocardial redox state (2, 3). These factors can critically influence the particular NO ⅐ species generated and, thereby, the net contractile response.Among the NO ⅐ -related species is nitroxyl anion (NO Ϫ ), the one-electron reduction product of NO ⅐ that is formed by NO ⅐ synthase in vitro by direct enzyme action or metabolism of the decoupled NO ⅐ synthase product N G -hydroxy-L-arginine (4-8). At high concentrations of 0.1-5 mM, NO Ϫ seems more cytotoxic than NO ⅐ in vitro, causing DNA strand breaks and base oxidation (9, 10). Like NO ⅐ , NO Ϫ induces vasodilation in vivo and in vitro in association with the formation of iron-nitro...

Both X-linked and autosomal dominant EDMD patients risk not only bradyarrhythmia (requiring pacemaker implant) but also atrial fibrillation/flutter, which often anticipates atrial standstill and can cause disabling embolic stroke at a relatively young age. Antithromboembolic prophylaxis has to be recommended in EDMD patients with atrial fibrillation/flutter or atrial standstill. With careful monitoring, survival after pacemaker implant may be long. Heart failure, which seems to occur only in a minority of patients, may be severe.

Battery longevity is significantly different among manufacturers. ICD cost is strictly dependent on device longevity, whereas device up-front cost is of limited clinical meaning. Appropriate assessment of cost-effectiveness should be based on ICD longevity in the real-life scenario.

In patients with a cardiac device, PET/CT increases the diagnostic accuracy of the modified Duke criteria for IE, particularly in the subset of patients with possible IE in whom it may help the clinician manage a challenging situation.

Persistence of a left superior vena cava (LSVC) has been reported in 0.3%-0.4% of candidates for pacemaker (PM) or cardioverter-defibrillator (ICD) implantation. The aim of the study was to evaluate the clinical implications of LSVC persistence for proper device performance. We observed the prevalence of LSVC during a 15-year period. A total of 2077 consecutive patients underwent PM implantation over a 15-year period: 7 had persistent LSVCs (0.34%). Among 599 patients undergoing ICD implantation, 4 LSVCs (0.66%) were observed. Overall LSVC persistence was found in 11/2676 (0.41%) patients. The right superior vena cava was absent in 4/11 (36%) patients. The leads were placed from the left subclavian approach in 5/7 PM patients: 2 received an elective right sided approach due to physician preference. All ICD patients had the device placed left pectoral with a single-coil lead: defibrillation therapy was effective in the long term in all but one patient, who required the addition of a subcutaneous array. Left superior vena cava persistence in PM/ICD patients is similar to the general population (0.41% in our study). The left-sided implant may be skill-demanding during lead placement; however, this task can be accomplished in the majority of cases, with a reliable outcome in the short term and appropriate device performance at follow-up.

Telecardiology may help confront the growing burden of monitoring the reliability of implantable defibrillators/pacemakers. Herein, we suggest that the evolving capabilities of implanted devices to monitor patients’ status (heart rhythm, fluid overload, right ventricular pressure, oximetry, etc.) may imply a shift from strictly device-centered follow-up to perspectives centered on the patient (and patient-device interactions). Such approaches could provide improvements in health care delivery and clinical outcomes, especially in the field of heart failure. Major professional, policy, and ethical issues will have to be overcome to enable real-world implementation. This challenge may be relevant for the evolution of our health care systems.

Automatic verification of stimulation is reliable at long term, and warrants superior safety in the event of pacing threshold changes. It allows a significant longevity increase compared to FOP stimulation that may heavily impact the patients' quality of life and the cost of pacing therapy. Moreover, it is a fundamental technology in a strategy of remote patient and device monitoring, and may enable automatic device follow-up operated by trained, nonmedical personnel.

Patients with IVNC-DCM had greater LV reverse remodelling after CRT than patients with DCM. The greater the area of non-compaction (higher number of IVNC segments) the greater the chance of achieving CRT response and greater LV reverse remodelling.