Multigenetic lesions in infant acute leukaemias: correlations with ALL-1 gene status

The large majority of childhood B-precursor cell acute lymphoblastic leukaemia cases present IgH and TCRδ gene rearrangements. These rearrangements have been widely used as specific markers for monitoring minimal residual disease. However, their prognostic value still remains unclear. In order to determine whether IgH and TCRδ gene rearrangements have any influence on relapse and event-free survival (EFS), we analysed the clinical impact of these genetic characteristics in 51 B-precursor acute lymphoblastic leukaemia patients. 46/51 patients (90.2%) showed IgH gene rearrangements by Southern blot and/or polymerase chain reaction (PCR) analysis. No statistically significant associations were found between IgH gene rearrangement pattern and age, sex, WBC count, immunophenotype, risk factor, relapse or EFS. 27/41 patients (66%) showed Vδ₂Dδ₃ recombination by Southern blot and/or PCR analysis. At a median follow-up of 53 months the estimated 5-year EFS probability was 78 ± 3% for the whole group. The EFS probability among patients with a Vδ₂Dδ₃ recombination pattern in the TCRδ locus was 90 ± 3%, whereas for patients without Vδ₂Dδ₃ recombination was 39 ± 13% ( P  < 0.005).
IgH rearrangement patterns do not appear to influence relapse or EFS probability. However, TCRδ gene rearrangement patterns have a relevant impact on the relapse rate and the EFS probability. Patients with Vδ₂Dδ₃ recombination have better clinical outcome than patients without this recombination, independent of any other prognostic factors.     

Natural and Unnatural History of Patients with Severe Carotid Sinus Hypersensitivity: A Preliminary Study

Natural history of patients with symptomatic severe carotid sinus hypersensitivity is not clearly known. In order to evaluate the effectiveness of pacing therapy in these patients we performed a randomized treatment/no-treatment prospective study in 35 patients. They were randomly assigned to two groups: 19 patients received no therapy, 16 patients received a VVI (# 11) or DDD (#) pacemaker implant. During the 8.4 ± 4.3 month follow-up period patients receiving no therapy had recurrence of syncope in 9 cases (47%) and minor symptoms in 13 (68%); at the 16th month, actuarial curve showed absence of syncope in 36% of patients and of any symptoms in 30%. During the 7.2 ±4.1 month follow-up period, the patients receiving the pacemaker implant had no recurence of syncope, minor symptoms in three (19%); at the 16th month, actuarial curve showed absence of syncope in 100% of patients and of any symptoms in 78%. During follow-up, 12 patients in no-treatment group received a pacemaker implant because of the recurrence of severe symptoms; successively they had a strong reduction of symptoms. In conclusion, this study definitively demonstrates that patients with severe symptomatic carotid sinus hypersensitivity had a high rate of recurrence of spontaneous symptoms and that in these patients cardiac pacing is a useful therapy.     

Electrophysiological effects and mechanism of action of oral quinidine in patients with sinus bradycardia and first degree A-V nodal block

The effects of quinidine on sinus nodal and A–V nodalfunction were assessed in 20 patients (age: 60±7 years)with sinus bradycardia and a prolonged A–H interval. Electrophysiologicalstudies were performed twice in each patient. In the first study,the measurements of sinus and A–V node function were evaluatedboth in the basal state and after autonomic blockade (propranolol0.2 mg kg^-1 and atropine 0.04 mg kg^-1). Oral quinidine was administeredfor 3–4 days (1200 mg day^-1) and the study was then repeatedusing the same methods. Comparison of data obtained in the twostudies in the basal state allowed us to evaluate the overalleffect of quinidine. Comparing the results obtained followingautonomic blockade, the direct action of the drug could be assessed.Inthe basal state quinidine did not significantly change the functionof either node. In contrast, after autonomic blockade, significantchanges were noted after quinidine. In 3 patients with sinusrate <50 beats min^-1 and an abnormal intrinsic heart rate,quinidine induced marked depression of sinus automaticity.Thesedata suggest that: (1) in patients with sinus bradycardia andprolongation of the A–H interval, oral quinidine has adirect depressant effect on sinus and A–V nodal function,but this effect is counteracted by autonomically mediated actions;(2) in patients with moderate or severe bradycardia and an abnormalintrinsic heart rate, the drug can induce marked depressionof sinus automaticity.     

Long‐Term Follow‐Up of Coronary Venous Bypass Graft Lesions Treated with a New Generation Drug‐Eluting Stent with Bioabsorbable Polymer

Background

To date, no published data are available regarding long‐term follow‐up of new generation DES implanted in coronary artery bypass graft (CABG) lesions.

Objectives

To assess the long‐term clinical outcome of patients receiving the new generation Biolimus A9‐coated drug‐eluting stent (DES) with biodegradable polymer in saphenous vein grafts (SVG).

Methods

Three thousand sixty‐seven patients were included in the NOBORI 2 registry: 71 patients with a total of 117 lesions received at least 1 biolimus A9 DES in SVG lesions and 2,959 patients received percutaneous coronary intervention in other lesions. Clinical follow‐up was performed at 1, 6, and 12 months, and annually up to 3 years.

Results

Compared to the non‐CABG group, patients with CABG lesions were older (P < 0.001), had a higher Charlson Comorbidity Index (P = 0.004), and presented more often with acute coronary syndrome (P = 0.02). At 3‐year follow‐up, cardiac death occurred in 9.7% versus 2.1% (P < 0.001), myocardial infarction (MI) in 8.3% versus 3.0% (P = 0.02), target lesion failure in 13.9% versus 6.4% (P = 0.03), and major adverse cardiac event in 18.1% versus 8.6% (P = 0.01). No differences were observed in TV‐MI and TLR, nor stent thrombosis (ST) which was generally low in both groups (1.4% vs 0.8%, P = NS).

Conclusion

Albeit 3‐year outcomes were less favorable in the CABG group, the higher cardiac mortality was apparently not driven by ST, target vessel MI, or TLR, but is likely due to advanced disease and age as well as comorbidity. The low TLR rate as well as the absence of late and very late ST suggest that BES are safe and effective for the treatment of CABG lesions. (J Interven Cardiol 2013;26:425‐433)

     

Sleep and time course of consolidation of visual discrimination skills in patients with narcolepsy–cataplexy

The level of procedural skills improves in normal individuals when the acquisition is followed by a period of sleep rather than wake. If sleep plays an important role in the consolidation process the advantage it provides should be reduced or delayed when its organization is altered, as in patients with chronic sleep disorders. To test this prediction in patients with narcolepsy–cataplexy (NC), who usually have a more fragmented organization of sleep than normals, we compared the initial, intermediate and delayed level of consolidation of visual skills . Twenty-two drug-naive NC patients and 22 individually-matched controls underwent training at a texture discrimination task (TDT) and were re-tested on the next morning (after a night spent in laboratory with polysomnography) and after another six nights (spent at home). TDT performance was worse in patients than controls at training and at both retrieval sessions and the time course of consolidation was different in NC patients (who improved mainly from next-day to 7th-day retrieval session) compared with controls. Moreover, the less-improving patients at next-day retrieval had a wider disorganization of sleep, probably because of an episode of rapid eye movement (REM) sleep at sleep onset REM, on post-training night more frequently than more-improving patients. These findings suggest that the time course of the consolidation process of procedural skills may be widely influenced by the characteristics of sleep organization (varying night-by-night much more in NC patients than controls) during post-training night.     

Outpatient Electrical Cardioversion of Atrial Fibrillation: 8 Years' Experience. Analysis of Shock-Related Arrhythmias

Background: Outpatient electrical cardioversion (EC) of atrial fibrillation is currently the standard of care. Shock-related arrhythmias may be particularly deleterious in this setting. Preoperative identification of high-risk patients may be very useful.
Methods: A retrospective analysis was made of 543 consecutive elective EC procedures in 457 outpatients over an 8-year period in a university cardiological institute. The protocol included adequate anticoagulation, intravenous anesthesia, direct current shock, and a direct observation after a shock to detect procedure-related complications. No patients were excluded due to severity of pathology or comorbidities. Clinical characteristics, energy delivered, medications, arrhythmic phenomena, and predictors of success and complications were analyzed.
Results: Of 543 ECs performed, 88.2% restored sinus rhythm, which persisted at discharge in 83.2%. No anesthesia-related complications were detected. No thromboembolic complications were detected. Use of a biphasic cardioverter was the only predictor of success (P = 0.0001). The bradyarrhythmic complication rate was 1.5%. No ventricular arrhythmic events were detected. Atrial flutter was present in five of eight patients who developed complications versus 44 of 535 patients who had no complications (P < 0.0005), and prosthetic heart valves in four of eight complicated versus 40 of 535 uncomplicated cases (P = 0.0044). The combination of atrial flutter and prosthetic heart valve was found in four of eight complicated versus 11 of 535 uncomplicated cases (P < 0.0005).
Conclusion: Shock-related arrhythmias are essentially bradyarrhythmias. Atrial flutter and previous cardiac surgery identify a subgroup of patients at high risk of postshock bradyarrhythmic complications.