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Brugada Syndrome is a controversial disease whose pathophysiology is still far from being fully understood. Unlike other cardiological disorders, a definite etiology has not yet been established so that it could be summarized under two main chapters: "functional" or "organic", "repolarization" or "depolarization" disorder. Despite initial descriptions leaned towards the organic substrate and delayed depolarization features, functional and repolarization theories have attracted most of the Cardiological attention for many years. Data from electrocardiography, endocavitary tracings, electroanatomic mapping and histopathology, however, demonstrated that Brugada Syndrome is mainly characterized by structural myocardial changes mostly at the right ventricular outflow tract, but also at the right ventricle and by delayed conduction at the same sites. Conduction disorders at different levels may also be present and identify patients at high risk for major arrhythmic events. The aim of the present review is to provide the current state of art of the pathophysiology of BrS, focusing on electro-vectorcardiography and electrophysiological features, histopathology, echocardiography and cardiac magnetic resonance imaging. This article is protected by copyright. All rights reserved.

Several epidemiological studies found an association between acute exposure to fine particulate matter of less than 2.5 &mu, m and 10 &mu, m in aerodynamic diameter (PM2.5 and PM10) and cardiovascular diseases, ventricular fibrillation incidence and mortality. The effects of pollution on atrial fibrillation (AF) beyond the first several hours of exposure remain controversial. A total of 145 patients with implantable cardioverter-defibrillators (ICDs), cardiac resynchronization therapy defibrillators (ICD-CRT), or pacemakers were enrolled in this multicentric prospective study. Daily levels of PM2.5 and PM10 were collected from monitoring stations within 20 km of the patient&rsquo, s residence. A Firth Logistic Regression model was used to evaluate the association between AF and daily exposure to PM2.5 and PM10. Exposure levels to PM2.5 and PM10 were moderate, being above the World Health Organization (WHO) PM2.5 and PM10 thresholds of 25 &mu, g/m3 and 50 &mu, g/m3, respectively, on 26% and 18% of the follow-up days. An association was found between daily levels of PM2.5 and PM10 and AF (95% confidence intervals (CIs) of 1.34&ndash, 2.40 and 1.44&ndash, 4.28, respectively) for an increase of 50 &micro, g/m3 above the WHO threshold. Daily exposure to moderate PM2.5 and PM10 levels is associated with AF in patients who are not prone to AF.

This is the case of a 28-year-old woman with sustained supraventricular tachycardia induced only by monomorphic ventricular couplets but not by isolated extrasystoles. The careful examination of electrocardiogram (ECG) was able to identify the mechanism of the tachycardia in the absence of electrophysiologic investigation. The tachycardia resulted sustained and symptomatic only during periods.

Aims Internet-based remote interrogation systems have been shown to reduce emergency department and in-office visits in patients with implantable cardioverter defibrillators (ICDs), resulting in increased efficiency for healthcare providers. Nonetheless, studies sized to demonstrate the impact of remote monitoring on patients' outcome have been lacking. Methods and results The EFFECT study was a multicentre clinical trial aimed at measuring and comparing the outcome of ICD patients conventionally followed-up by means of in-clinic visits (Standard arm) or by remote monitoring (Remote arm) in the clinical practice of 25 Italian centres. From 2011 to 2013, 987 consecutive patients were enrolled and followed up for at least 12 months. The primary endpoint was the rate of death and cardiovascular hospitalizations. Remote monitoring was adopted by 499 patients. Patients in the Standard and Remote arms did not differ significantly in terms of baseline clinical characteristics, except for a more frequent use of ICD with cardiac resynchronization therapy (CRT-D) in the Remote arm (48 vs. 36%, P < 0.001). One-year rates of the primary combined endpoint were 0.27 events/year for patients in the Standard arm and were 0.15 events/year for those in the Remote arm (incident rate ratio, 0.55; 95% CI, 0.41–0.73; P < 0.001). The endpoint rates in the Standard and Remote arms were 0.27 and 0.08 events/year, respectively, among CRT-D recipients ( P < 0.001), and 0.28 vs. 0.21 among ICD patients ( P = 0.094). The rates of in-office visits were 1.9 per year in the Standard arm and 1.7 per year in the Remote arm. Conclusion Compared with the standard follow-up through in-office visits, remote monitoring is associated with reduced death and cardiovascular hospitalizations in patients with ICD in clinical practice. Clinical Trial Registration URL: Identifier: [NCT01723865][1]. [1]: /lookup/external-ref?link_type=CLINTRIALGOV&access_num=NCT01723865&atom=%2Feuropace%2F17%2F8%2F1267.atom

Background Permanent atrial pacing usually involves lead placement in the right atrial appendage (RAA). Anatomical studies addressing features predisposing to complications are scanty. Objective To assess the morphology of RAA in the perspective of pacing, including the morphology of tenia sagittalis (TS) and the spatial relationship with the aorta. Methods The gross anatomy of the RAA has been analyzed in a consecutive series of 100 hearts following a case of iatrogenic perforation of the aorta by active fixation lead located in the RAA. Transmural RAA sections were taken from 40 hearts to assess the wall thickness at the level of pectinate muscles (PMs) and of inter-PMs spaces and the distance between adjacent PMs. Results The TS was present in 90% of cases (single trunk, 76%; double trunk, 13%; and triple trunk, 1%), demarcating the proximal antral RAA region (facing the adjacent aorta) from the distal saccular RAA region (facing the pulmonary infundibulum). The RAA free wall in the inter-PMs spaces is usually paper-thin and translucent. Histomorphometric analysis reveals that the RAA wall mean thickness was 1.38 ± 0.05 mm (range 0.64–4.25 mm) at the level of PMs and 0.39 ± 0.23 mm (range 0.09–1.05 mm) at the level of inter-PMs spaces. The mean distance between adjacent PMs was 0.88 ± 0.99 mm (range 0.04–4.12 mm). Conclusion In 90% of hearts, a well-defined TS separates the distal saccular from the proximal antral RAA, the latter being closely adjacent to the ascending aorta. The paper-thin wall between PMs is potentially at risk of perforation, and aortic injury could occur when active fixation leads are anchored in the antral RAA, as demonstrated in an iatrogenic fatal case.

Summary Background Although the effects of air pollution on mortality have been clearly shown in many epidemiological and observational studies, the pro-arrhythmic effects remain unknown. We aimed to assess the short-term effects of air pollution on ventricular arrhythmias in a population of high-risk patients with implantable cardioverter-defibrillators (ICDs) or cardiac resynchronisation therapy defibrillators (ICD-CRT). Methods In this prospective multicentre study, we assessed 281 patients (median age 71 years) across nine centres in the Veneto region of Italy. Episodes of ventricular tachycardia and ventricular fibrillation that were recorded by the diagnostic device were considered in this analysis. Concentrations of particulate matter of less than 10 μm (PM 10 ) and less than 2·5 μm (PM 2·5 ) in aerodynamic diameter, carbon monoxide, nitrogen dioxide, sulphur dioxide, and ozone were obtained daily from monitoring stations, and the 24 h median value was considered. Each patient was associated with exposure data from the monitoring station that was closest to their residence. Patients were followed up for 1 year and then scheduled to have a closing visit, within 1 more year. This study is registered with ClinicalTrials.gov, number NCT01723761. Findings Participants were enrolled from April 1, 2011, to Sept 30, 2012, and follow-ups (completed on April 5, 2014) ranged from 637 to 1177 days (median 652 days). The incidence of episodes of ventricular tachycardia and ventricular fibrillation correlated significantly with PM 2·5 (p 10 . An analysis of ventricular fibrillation episodes alone showed a significant increase in risk of higher PM 2·5 (p=0·002) and PM 10 values (p=0·0057). None of the gaseous pollutants were significantly linked to the occurrence of ventricular tachycardia or ventricular fibrillation. In a subgroup analysis of patients with or without a previous myocardial infarction, only the first showed a significant association between particulate matter and episodes of ventricular tachycardia or ventricular fibrillation. Interpretation Particulate matter has acute pro-arrhythmic effects in a population of high-risk patients, which increase on exposure to fine particles and in patients who have experienced a previous myocardial infarction. The time sequence of the arrhythmic events suggests there is an underlying neurally mediated mechanism. From a clinical point of view, the results of our study should encourage physicians to also consider environmental risk when addressing the prevention of arrhythmic events, particularly in patients with coronary heart disease, advising them to avoid exposure to high levels of fine particulate matter. Funding There was no funding source for this study.

Rationale, aims and objectives Heart failure (HF) is a concerning public health burden in Western society because, despite the improvement of medical treatments, it is still associated with adverse outcomes (high morbidity and mortality), resulting in one of the most expensive chronic disease in Western countries. Hospital admission particularly is the most expensive cost driver among the several resources involved in the management of HF. The aim of our study was to investigate the cost of hospitalization before and after the enrolment to a new strategy (GISC) in the management of patients with HF. Methods We enrolled a cohort of 90 patients. Patients were eligible to the study if they were hospitalized with a new diagnosis of HF or a diagnosis of decompensated HF. The enrolment to the study corresponded to the enrolment to the GISC intervention. We calculated the cost for every hospital admission at 6 and 12 months before and after the enrolment using the tariff paid for the diagnosis-related group. Results Comparing per-patient cumulative cost before and after the enrolment, we showed that patient's hospitalization was less expensive after the enrolment to the GISC intervention. The strategy resulted in an average cumulative estimated saving of €439 322.00 (95% CI €413 890.70; €464 753.40) at 6 months and of €832 276.80 (95% CI €786 863.70; €877 690.00) at 12 months after the enrolment. Conclusions We found out that the intervention was a cost-saving strategy for follow-up of the patients suffering from HF at 6 and 12 months after the enrolment compared with hospitalizations' cost before the recruitment.

Manpower of Cardiac Device Home MonitoringBackground This study aimed to assess manpower and resource consumption of the HomeGuide workflow model for remote monitoring (Biotronik Home Monitoring [HM], Biotronik SE & Co. KG, Berlin, Germany) of cardiac implantable electronic devices in daily clinical practice. Methods The model established a cooperative interaction between a reference nurse (RN) for ordinary management, and a responsible physician (RP) for medical decisions in each outpatient clinic. RN reviewed remote transmissions and alerts, addressing critical cases to the RP. Results A total of 1,650 patients were enrolled in 75 sites: 25% pacemakers (PM), 22% dual-, 27% single-chamber implantable defibrillators (ICD), 2% PM with cardiac resynchronization therapy (CRT), and 24% ICD-CRT. During a median follow-up of 18 (10–31) months, 3,364 HM sessions were performed (74% by the RN, 26% by the RP) to complete 18,478 remote follow-ups. Median duration of remote follow-ups was 1.2 (0.6–2.0) minutes, corresponding to a manpower of 43.3 (4.2–94.8) minutes/month every 100 patients for nurses and 10.2 (0.1–31.1) for physicians (P < 0.0001). RN submitted 15% of remote transmissions to RP, who decided unscheduled follow-ups in 12% of the cases. The median manpower for phone calls was 1.9 (0.8–16.5) minutes/month every 100 contacted patients. There were 2.84 in-hospital visits/patient, 0.46 of which triggered by HM findings. A cumulative per-patient HM follow-up time of 15.4 minutes (20% of total follow-up time) allowed remote detection of 73% of actionable events. Conclusions HM implemented in the HomeGuide workflow model required

Objective The purpose of this study was to evaluate the impact of adhering to a strict follow up regimen on mid-term efficacy of intraoperative radiofrequency ablation in maintaining sinus rhythm for patients with atrial fibrillation undergoing cardiac surgery. Methods We enrolled 52 patients with atrial fibrillation and valve disease divided in 2 groups: group 1 (26 patients) underwent intraoperative radiofrequency ablation and was followed by a team of skilled cardiologists who treated recurrences with amiodarone and electrical cardioversion; group 2 (26 patients) was treated with intraoperative radiofrequency ablation but patients were thereafter treated by their local hospital or personal cardiologist. Results There were no intraoperative deaths or complications related to radiofrequency ablation. Twenty four months after the operation, freedom from atrial fibrillation was 60% in group 1 and 32% in group 2 (p = 0.02). Group 1 also showed a reduction in atrial volume (p ≤ 0.05). Conclusion Postoperative follow up with electrical and pharmacologic cardioversion combined with radiofrequency ablation achieves higher rate of sinus rhythm maintenance. After ablation, electrical cardioversion is effective even in patients with enlarged left atrium, long history of atrial fibrillation, or previous failed electrical cardioversion.

Aim: We assessed the efficacy, reliability and cost of an in-home control service, devoted to debilitated patients with pacemakers, during long-term follow up. Methods: We admitted 802 patients (mean age 87.8 ± 6.9 years) with pacemakers into our program, which were monitored periodically during in-home visits by nurses. During these controls, patients underwent a standard electrocardiogram with and without magnet, during which stimulation intervals were measured. In-home pacemaker assessments were performed by a portable programmer only for patients with Biotronik pacemakers. Electrocardiograms and technical data were evaluated at hospital, and a report was sent by mail to the patient. Moreover, we compared the costs for the hospital, patients and society between in-home and ambulatory assessments. Results: During 80 months of follow up, 2418 controls were performed. The monthly average of patients who were followed at home was 283.9 ± 23.9. The mean number of controls per month was 30.2 ± 10 (range 4–51). One hundred and twenty-three pacemakers were replaced due to battery exhaustion, after a mean device longevity of 7.4 years (range 4.9–12.4 years). Four hundred and sixty-eight patients died (8.7% annual mortality). Fifteen patients (annual incidence 0.3%) were invited to in-hospital visits for reprogramming. The cumulative cost for in-home assessment were 20.5% lower than conventional in-hospital controls and 66.5% lower if patients are transported by ambulance. Conclusion: Elderly debilitated patients can be monitored by effective and reliable pacemaker assessments at home, alleviating them and their families from the issues that are associated with their transfer from the home to the hospital and concurrently reducing overall costs. Geriatr Gerontol Int 2012; 12: 30–35.

Aims The remote monitoring of pacemakers and implantable cardioverter defibrillators (ICDs) promotes accurate supervision of the patient and device. Alert settings appear to be a crucial parameter of its efficacy. The purpose of our study was to evaluate various settings for alerts and alert message management in patients with pacemakers and ICDs. Methods and results We prospectively evaluated 282 patients (181 males, 101 females, mean age 72 ± 11 years) who were referred to nine electrophysiological centres in the Veneto region of Italy for a pacemaker (76 patients) or ICD (206 patients) implant in which remote monitoring was activated (Biotronik Home Monitoring®, Biotronik, Berlin, Germany). During a mean follow-up of 382 ± 261 days, we received 106 039 transmissions. In the pacemaker group, the alerts that were most frequently activated were those concerning battery exhaustion, surveying impedances, sensing and threshold measurements, as well as missing transmissions. In the ICD/ICD-cardiac resynchronization therapy group, the alerts nearly always activated were those concerning a detection setoff, battery exhaustion, critical values of impedance, or ineffective maximum energy shock. In both groups, the alarms for heart rate monitoring and supraventricular arrhythmia were activated in fewer cases at higher variability among centres. Conclusions Our study demonstrates that more attention is paid to critical technical data than to patients’ clinical profiles, probably to limit an excessive flow of data into the centre. Accurate alert settings, personalized to the patients' features, are essential for easier and more effective management of patients who are followed remotely.

There is definitive clinical evidence that the implantable defibrillator (ICD) is the most effective therapy for both primary and secondary prevention of sudden cardiac death (SCD) in patients with coronary artery disease. However, there are few available data on efficacy and safety of such a treatment in patients with nonischemic cardiomyopathies, mostly because of the relatively low disease prevalence and the relatively low event rate in affected patients. Arrhythmogenic cardiomyopathy (AC) has become an emerging indication for ICD implantation because its natural history is more strongly related to ventricular electrical instability, which can precipitate SCD mostly in young people, whereas heart failure is uncommon and occurs later during the disease course as a result of right ventricular (RV) disease progression and left ventricular (LV) involvement. In the past, indications for ICD implantation in ACwere empiric and based widely on the experience gained by different centers using analogies with coronary artery disease. Because clinical variables predicting clinical outcomewere undetermined, there was a tendency to implant an ICD once the disease

The aims of the present study were to investigate the incidence and characteristics of conduction disorders (CDs) after transcatheter aortic valve implantation (TAVI), to analyze the predictors of permanent pacemaker (PPM) implantation, and to evaluate the outcomes of CDs over time. In particular, we sought to investigate whether the depth of deployment and other technical aspects of valve implantation might predict the need for PPM implantation after TAVI. TAVI has been reported to favor the onset or worsening of CDs often requiring PPM implantation. A total of 70 patients with aortic stenosis due to dystrophic calcification underwent TAVI with third-generation CoreValve Revalving System from May 2007 to April 2009. We collected electrocardiograms at baseline, during TAVI, during hospitalization and at the 1-, 3-, 6-, and 12-month follow-up visits thereafter. The clinical, anatomic, and procedural variables were tested to identify the predictors of PPM implantation. The PPM dependency at follow-up was analyzed. Six patients were excluded from the analysis because of a pre-existing PPM. Of the 64 patients, 32 (50%) had one or more atrioventricular-intraventricular CDs at baseline. TAVI induced a worsening in the CDs in 49 (77%) of the 64 patients, with 25 (39%) requiring in-hospital PPM implantation. On multivariate analysis, the independent predictors of PPM implantation were the depth of the prosthesis implantation (p = 0.039) and the pre-existing right bundle branch block (p = 0.046). A trend in the recovery of the CDs over time was recorded, although 2 patients required PPM implantation 1 month after discharge for late complete atrioventricular block. In conclusion, TAVI often induces or worsens CDs, requiring PPM in more than one third of patients, although a trend in the recovery of CDs during the midterm was recorded. The independent predictors of PPM implantation were the depth of prosthesis implantation and pre-existing right bundle branch block.

The aim of our study was to identify specific age-related hemodynamic changes during upright tilt test in patients with vasovagal syncope.We studied 115 subjects who were referred to us for unexplained syncope during 65 degrees tilt with pharmacological challenge (s.l. nitrate) by noninvasive monitoring of blood pressure (Finometer). Two derived variables were also considered: total peripheral resistance and stroke volume. The patients were divided into 3 groups by age: Group 1, 10-30 yrs; Group 2, 31-50 yrs; and Group 3,51 yrs.The test was positive in 65 patients (57%). During the first 20 min of the test, older patients experienced lower increases in heart rate (p0.00001) and premature reduction of total peripheral resistance (p0.005) and systolic blood pressure (p0.005). Group 1 showed more pronounced decreases in stroke volume (p0.01). No differences emerged between negative and positive patients. In the 5 min that preceded syncope, younger subjects experienced a distinct, transient increase in heart rate immediately before symptom onset (p0.005). Blood pressure was prematurely and progressively decreased in Group 3, whereas it decreased only in the 90 s prior to syncope in Groups 1 and 2 (p0.01).Our results suggest that blunted sympathetic activation occurs during passive orthostatism in older patients. On the other hand, hemodynamic changes before symptom onset seem to indicate as in younger subjects syncope results from an excessive sympathovagal reaction, whereas in older subjects it is related to an overwhelming, progressive central vagal activity that is associated with a defective peripheral sympathetic activation.

Contains fulltext : 89482.pdf (Publisher’s version ) (Closed access) Cardiovascular remodelling in the conditioned athlete is frequently associated with physiological ECG changes. Abnormalities, however, may be detected which represent expression of an underlying heart disease that puts the athlete at risk of arrhythmic cardiac arrest during sports. It is mandatory that ECG changes resulting from intensive physical training are distinguished from abnormalities which reflect a potential cardiac pathology. The present article represents the consensus statement of an international panel of cardiologists and sports medical physicians with expertise in the fields of electrocardiography, imaging, inherited cardiovascular disease, cardiovascular pathology, and management of young competitive athletes. The document provides cardiologists and sports medical physicians with a modern approach to correct interpretation of 12-lead ECG in the athlete and emerging understanding of incomplete penetrance of inherited cardiovascular disease. When the ECG of an athlete is examined, the main objective is to distinguish between physiological patterns that should cause no alarm and those that require action and/or additional testing to exclude (or confirm) the suspicion of an underlying cardiovascular condition carrying the risk of sudden death during sports. The aim of the present position paper is to provide a framework for this distinction. For every ECG abnormality, the document focuses on the ensuing clinical work-up required for differential diagnosis and clinical assessment. When appropriate the referral options for risk stratification and cardiovascular management of the athlete are briefly addressed. 01 januari 2010

A 63-year-old man presented with recurrent syncopal attacks associated with swallowing and right-sided throat pain. Immediately after admission, he presented a 16-s asystolia. The patient's clinical history was unremarkable except for previous postimplant periodontitis. Several episodes of severe bradycardia and sinus pauses, always associated with painful deglutition, were recorded subsequently. X-ray orthopanthomography and magnetic resonance imaging of the neck confirmed several areas of periodontitis around the previous dental implants and right mastoid inflammation. A barium swallow and fibre-optic endoscopy also revealed a small sliding hiatus hernia and distal chronic oesophageal inflammation. Despite complete dental curettage, antibiotics and antigastro-oesophageal reflux therapy, only partial relief of the pain and incomplete resolution of the arrhythmic disorder were obtained after 3 weeks, and the patient underwent pacemaker implantation. At 1-month follow-up, however, he reported the complete relief of the throat pain; subsequent Holter monitoring showed normal sinus rhythm, without pacemaker-induced electrical activity.

ObjectivesWe tested whether 3-dimensional electroanatomical voltage mapping (EVM) may help in the differential diagnosis between idiopathic right ventricular outflow tract (RVOT) tachycardia and arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D).BackgroundRight ventricular EVM has been demonstrated to reliably identify low-voltage regions (“electroanatomical scar”), which in patients with ARVC/D correspond to areas of fibrofatty myocardial replacement.MethodsThe study population comprised 27 patients (15 men and 12 women, age 33.9 ± 8 years) with RVOT tachycardia and no echocardiographic/angiographic evidence of right ventricular (RV) dilation/dysfunction, who underwent EVM and endomyocardial biopsy (EMB) for characterization of ventricular tachycardia (VT) substrate before catheter ablation.ResultsElectroanatomical voltage mapping was normal in 20 of 27 patients (74%, group A), with electrogram voltage >1.5 mV throughout the RV. The other 7 patients (26%, group B) showed ≥1 (1.4 ± 07) RV electroanatomical scar area(s) (bipolar voltage

Background— Three-dimensional electroanatomic voltage mapping offers the potential to identify low-voltage areas that correspond to regions of right ventricular (RV) myocardial loss and fibrofatty replacement in patients with arrhythmogenic RV cardiomyopathy/dysplasia (ARVC/D). Methods and Results— Thirty-one consecutive patients (22 men and 9 women; mean age, 30.8±7 years) who fulfilled the criteria of the Task Force of the European Society of Cardiology and International Society and Federation of Cardiology (ESC/ISFC) for ARVC/D diagnosis after noninvasive clinical evaluation underwent further invasive study including RV electroanatomic voltage mapping and endomyocardial biopsy (EMB) to validate the diagnosis. Multiple RV endocardial, bipolar electrograms (175±23) were sampled during sinus rhythm. Twenty patients (group A; 65%) had an abnormal RV electroanatomic voltage mapping showing ≥1 area (mean 2.25±0.7) with low-voltage values (bipolar electrogram amplitude 1.5 mV). Low-voltage electrograms appeared fractionated with significantly prolonged duration and delayed activation. In 11 patients (group B; 35%), electroanatomic voltage mapping was normal, with preserved electrogram voltage (4.4±0.7 mV) and duration (37.2±0.9 ms) throughout the RV. Low-voltage areas in patients from group A corresponded to echocardiographic/angiographic RV wall motion abnormalities and were significantly associated with myocyte loss and fibrofatty replacement at EMB ( P P P P =0.02). Conclusions— Three-dimensional electroanatomic voltage mapping enhanced accuracy for diagnosing ARVC/D (1) by demonstrating low-voltage areas that were associated with fibrofatty myocardial replacement and (2) by identifying a subset of patients who fulfilled ESC/ISFC Task Force diagnostic criteria but showed a preserved electrogram voltage, an inflammatory cardiomyopathy mimicking ARVC/D, and a better arrhythmic outcome.

The aim of our study was to assess the arrhythmic profile in patients with primary pulmonary hypertension (PPH) and its correlation with autonomic features, echocardiographic indexes and pulmonary function. We studied 9 subjects with a mean age of 42 +/- 11 years. All underwent echocardiography, 24-hour Holter monitoring, and cardiopulmonary exercise testing. Left ventricle ejection fraction was normal (65 +/- 6%). The right ventricle end diastolic volume was increased (108 +/- 32 ml/m2) with a slight reduction of ejection fraction (49 +/- 5%). Right ventricle systolic pressure was increased (91 +/- 25 mmHg). Heart rate variability analysis showed evidence of a reduced standard deviation of all NN intervals (SDNN) compared with the control group (102.8 +/- 32 versus 156.1 +/- 32, p < 0.005). Patients with significant ventricular arrhythmias had a lower SDNN, and lower baseline and effort PO2 (SDNN: 87.0 +/- 15 versus 115.4 +/- 38; baseline PO2: 63.2 +/- 12% versus 78.8 +/- 7%; effort PO2: 50.7 +/- 13% versus 68.7 +/- 19%). The patients with SDNN lower than 90 ms were characterized by a higher right ventricle systolic pressure (115.0 +/- 22.9 mmHg versus 79.2 +/- 17.8 mmHg, p = 0.035). The patients who experienced syncope had higher SDNN (131.7 +/- 36 versus 88.4 +/- 20, p < 0.05), higher effort PO2 (77.5 +/- 14 mmHg versus 52.3 +/- 14 mmHg, p < 0.03). The patients with PPH evidenced an increased sympathetic activity. Premature ventricular beats were more frequent in those subjects with higher adrenergic drive and lower oxygen saturation. Patients with episodes of syncope seem to have a relatively higher vagal activity, and effective mechanisms of adjustment in blood oxygenation during effort.

The identification of subjects with arrhythmogenic right ventricular cardiomyopathy (ARVC) at higher risk for sudden death is an unresolved issue. An influence of the autonomic activity on the genesis of ventricular arrhythmias was postulated. Heart rate variability (HRV) analysis provides a useful method to measure autonomic activity, and is a predictor of increased risk of death after myocardial infarction. For these reasons, the aim of the study was to evaluate HRV and its correlations with ventricular arrhythmias, heart function, and prognostic outcome in patients with ARVC. The study included 46 patients with ARVC who were not taking antiarrhythmic medications. The diagnosis was made by ECG, echocardiography, angiography, and endomyocardial biopsy. Exercise stress test and Holter monitoring were obtained in all patients. Time-domain analysis of HRV was expressed as the standard deviation of all normal to normal NN intervals (SDNN) detected during 24-hour Holter monitoring. Thirty healthy subjects represented a control group for HRV analysis. The mean follow-up was 10.8 +/- 1.86 years. SDNN was reduced in patients with ARVC in comparison with the control group (151 +/- 36 vs 176 +/- 34, P = 0.00042). Moreover, there was a significant correlation of this index with the age of the patients (r = - 0.59, P0.001), with the left (r = 0.44, P = 0.002) and right (r = 0.47, P = 0.001) ventricle ejection fraction, with the right ventricular end diastolic volume (r = - 0.62, P0.001), and with the ventricular arrhythmias, detected during the same Holter record used for HRV analysis (patients with isolated ventricular ectopic beats1,000/24 hours, 184 +/- 34; patients with isolated ventricular ectopic beats1,000/24 hours and/or couplets, 156 +/- 25; patients with repetitive ventricular ectopic beats (or = 3) and/or ventricular tachycardia, 129 +/- 25; P0.001). During follow-up two patients showed a transient but significant reduction of SDNN and a concomitant increase of the arrhythmic events. In eight patients an episode of sustained ventricular tachycardia occurred, but the mean SDNN of this subgroup did not differ from the mean value of the remaining patients (152 +/- 15 vs 150 +/- 39; P = NS). Only one subject died after heart transplantation during follow-up (case censored). Time-domain analysis of HRV seems to be a useful method to assess the autonomic influences in ARVC. A reduction of vagal influences correlates with the extent of the disease. The significant correlation between SDNN and ventricular arrhythmias confirmed the influences of autonomic activity in the modulation of the electrical instability in ARVC patients. However, SDNN was not predictive of spontaneous episodes of sustained ventricular tachycardia.

Heart failure (HF) is a concerning public health burden in Western society because, despite the improvement of medical treatments, it is still associated with adverse outcomes (high morbidity and mortality), resulting in one of the most expensive chronic disease in Western countries. Hospital admission particularly is the most expensive cost driver among the several resources involved in the management of HF. The aim of our study was to investigate the cost of hospitalization before and after the enrolment to a new strategy (GISC) in the management of patients with HF.We enrolled a cohort of 90 patients. Patients were eligible to the study if they were hospitalized with a new diagnosis of HF or a diagnosis of decompensated HF. The enrolment to the study corresponded to the enrolment to the GISC intervention. We calculated the cost for every hospital admission at 6 and 12 months before and after the enrolment using the tariff paid for the diagnosis-related group.Comparing per-patient cumulative cost before and after the enrolment, we showed that patient's hospitalization was less expensive after the enrolment to the GISC intervention. The strategy resulted in an average cumulative estimated saving of €439322.00 (95% CI €413890.70; €464753.40) at 6 months and of €832276.80 (95% CI €786863.70; €877690.00) at 12 months after the enrolment.We found out that the intervention was a cost-saving strategy for follow-up of the patients suffering from HF at 6 and 12 months after the enrolment compared with hospitalizations' cost before the recruitment.

This study aimed to assess manpower and resource consumption of the HomeGuide workflow model for remote monitoring (Biotronik Home Monitoring [HM], Biotronik SECo. KG, Berlin, Germany) of cardiac implantable electronic devices in daily clinical practice.The model established a cooperative interaction between a reference nurse (RN) for ordinary management, and a responsible physician (RP) for medical decisions in each outpatient clinic. RN reviewed remote transmissions and alerts, addressing critical cases to the RP.A total of 1,650 patients were enrolled in 75 sites: 25% pacemakers (PM), 22% dual-, 27% single-chamber implantable defibrillators (ICD), 2% PM with cardiac resynchronization therapy (CRT), and 24% ICD-CRT. During a median follow-up of 18 (10-31) months, 3,364 HM sessions were performed (74% by the RN, 26% by the RP) to complete 18,478 remote follow-ups. Median duration of remote follow-ups was 1.2 (0.6-2.0) minutes, corresponding to a manpower of 43.3 (4.2-94.8) minutes/month every 100 patients for nurses and 10.2 (0.1-31.1) for physicians (P0.0001). RN submitted 15% of remote transmissions to RP, who decided unscheduled follow-ups in 12% of the cases. The median manpower for phone calls was 1.9 (0.8-16.5) minutes/month every 100 contacted patients. There were 2.84 in-hospital visits/patient, 0.46 of which triggered by HM findings. A cumulative per-patient HM follow-up time of 15.4 minutes (20% of total follow-up time) allowed remote detection of 73% of actionable events.HM implemented in the HomeGuide workflow model required1 hour/month every 100 patients to detect the majority of actionable events with limited administrative workload.

A female infant born at term of a regular pregnancy experienced unexplained episodes of tachycardia. Electrocardiogram revealed junctional ectopic tachycardia (JET) with a frequency of 145–170 beats/min with narrow QRS complex (Figures 1A and 1B). Fifteen days later, JET with a frequency of 4200 beats/min was found (Figure 1C). Medical therapy was not prescribed owing to lack of symptoms; at the age of 1 month, the infant was admitted to the emergency department with a diagnosis of cardiogenic

Background —Patients with the ECG pattern of right bundle branch block and right precordial ST-segment elevation may experience sudden death in the setting of either arrhythmogenic right ventricular cardiomyopathy (ARVC) or a functional electrical disorder such as Brugada syndrome. Methods and Results —Among a series of 273 young (≤35 years) victims of cardiovascular sudden death who were prospectively studied from 1979 to 1998 in the Veneto Region of Italy, 12-lead ECG was available in 96 cases. Thirteen (14%; 12 males and 1 female aged 24±8 years) had right precordial ST-segment elevation, either isolated (9 cases) or associated with right bundle branch block (4 cases). At autopsy, all patients had ARVC (92%) except one, who had no evidence of structural heart disease. Compared with the 19 young sudden death victims with ARVC and no ST-segment abnormalities from the same series, those with AVRC and right precordial ST-segment elevation included fewer competitive athletes (17% versus 58%; P =0.03), more often died suddenly at rest or during sleep (83% versus 26%; P =0.003), and showed serial ECG changes over time (83% versus 0; P =0.015), polymorphic ventricular tachycardia (33% versus 0; P =0.016), and predominant fatty replacement of the right ventricular anterior wall (58% versus 21%; P =0.05), Conclusions —Right precordial ST-segment elevation was found in 14% of young sudden death victims with available ECG. It mostly reflected underlying ARVC with predominant right ventricular anterior wall involvement and characterized a subgroup of patients who share with Brugada patients the propensity to die from non–exercise-related cardiac arrest and to exhibit dynamic ECG changes and polymorphic ventricular tachycardia.

OBJECTIVES We sought to define the clinical picture and natural history of familial arrhythmogenic right ventricular cardiomyopathy (ARVC). BACKGROUND Arrhythmogenic right ventricular cardiomyopathy is a myocardial disease, often familial, clinically characterized by the impending risk of ventricular arrhythmias and sudden death. METHODS Thirty-seven ARVC families of northeast Italy were studied. Probands had a histologic diagnosis of ARVC, either at autopsy (19 families) or endomyocardial biopsy (18 families). Protocol of the investigation included basal electrocardiogram (ECG), 24-hour ECG, signal-averaged ECG, stress test and two-dimensional Doppler echocardiography. Invasive evaluation was performed when deemed necessary. RESULTS Of the 365 subjects, 151 (41%) were affected, 157 (43%) were unaffected, 17 (5%) were healthy carriers, and 40 (11%) were uncertain. Mean age at diagnosis was 31 ± 13 years. By echocardiography, 64% had mild, 30% had moderate, and 6% had severe form. Forty percent had ventricular arrhythmias, 49 were treated with antiarrhythmic drugs, and two were treated with implantable cardioverter defibrillators. Sport activity was restricted in all. Of the 28 families who underwent linkage analysis, 6 mapped to chromosome 14q23-q24, 4 to 1q42-q43, and 4 to 2q32.1-q32.3. No linkage with known loci was found in four families and 10 had uninformative results. During a follow-up of 8.5 ± 4.6 years, one patient died (0.08 patient/year mortality), and 15 developed an overt form of ARVC. CONCLUSIONS Arrhythmogenic right ventricular cardiomyopathy is a progressive disease appearing during adolescence and early adulthood. Systematic evaluation of family members leads to early identification of ARVC, characterized by a broad clinical spectrum with a favorable outcome. In the setting of positive family history, even minor ECG and echocardiographic abnormalities are diagnostic.

We evaluated the incidence of atrial fibrillation in 189 patients (92 males, 97 females, mean age 75 +/- 12 yrs, range 41-100 yrs) with pacemaker, during a mean follow-up of 5.5 yrs (range 1-24 yrs). The indications for implant were: complete AV block (115 pts), second degree Möbitz 2 AV block (51 pts), bifascicular block (5 pts), sick sinus syndrome (14 pts), symptomatic bradycardia (4 pts). The mode of stimulation considered were VVI (105 pt), VVI rate responsive (21 pts), single lead VDD (43 pts), DDD (20 pts). The occurrence of retrograde VA conduction in patients with VVI or VVI rate responsive pacing was also evaluated. Atrial fibrillation occurred in 40 pts (21%). The highest incidence was evidenced in patients with sick sinus syndrome (9 pts, 64%), and in patients with VVI stimulation (28 pts, 27%). On the contrary, the lowest incidence was found in single lead VDD stimulation (4 pts, 9%). The patients with dual chamber pacing showed a relatively high incidence of the arrhythmia (5 pts, 25%). Atrial fibrillation occurred in 9 out of 32 patients with retrograde VA conduction, and in 22 out of 94 patients without retrograde conduction (28% versus 23%, p = NS). In conclusion, it is confirmed that patients with sick sinus syndrome are at high risk for atrial fibrillation. Single lead VDD stimulation seems to be the better mode of pacing in preventing atrial fibrillation, while dual chamber pacing showed minor efficacy. The presence of retrograde VA conduction could not predict the occurrence of the arrhythmia.

Background— Endocardial voltage mapping (EVM) identifies low-voltage right ventricular (RV) areas, which may represent the electroanatomic scar substrate of life-threatening tachyarrhythmias. We prospectively assessed the prognostic value of EVM in a consecutive series of patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D). Methods and Results— We studied 69 consecutive ARVC/D patients (47 males; median age 35 years [28–45]) who underwent electrophysiological study and both bipolar and unipolar EVM. The extent of confluent bipolar (P =0.009). In the remaining patients with normal bipolar EVM (n=16; 23%), the use of unipolar EVM unmasked ≥1 region of low-voltage electrogram affecting 26.2% (11.6–38.2) of RV wall. During a median follow-up of 41 (28–56) months, 19 (27.5%) patients experienced arrhythmic events, such as sudden death (n=1), appropriate implantable cardioverter defibrillator interventions (n=7), or sustained ventricular tachycardia (n=11). Univariate predictors of arrhythmic outcome included previous cardiac arrest or syncope (hazard ratio=3.4; 95% confidence interval, 1.4–8.8; P =0.03) and extent of bipolar low-voltage areas (hazard ratio=1.7 per 5%; 95% confidence interval, 1.5–2; P P Conclusions— The extent of bipolar RV endocardial low-voltage area was a powerful predictor of arrhythmic outcome in ARVC/D, independently of history and RV dilatation/dysfunction. A normal bipolar EVM characterized a low-risk subgroup of ARVC/D patients.

Aims The HomeGuide Registry was a prospective study ([NCT01459874][1]), implementing a model for remote monitoring of cardiac implantable electronic devices (CIEDs) in daily clinical practice, to estimate effectiveness in major cardiovascular event detection and management. Methods and results The workflow for remote monitoring [Biotronik Home Monitoring (HM)] was based on primary nursing: each patient was assigned to an expert nurse for management and to a responsible physician for medical decisions. In-person visits were scheduled once a year. Seventy-five Italian sites enrolled 1650 patients [27% pacemakers, 27% single-chamber implantable cardioverter defibrillators (ICDs), 22% dual-chamber ICDs, 24% ICDs with cardiac resynchronization therapy]. Population resembled the expected characteristics of CIED patients. During a 20 ± 13 month follow-up, 2471 independently adjudicated events were collected in 838 patients (51%): 2033 (82%) were detected during HM sessions; 438 (18%) during in-person visits. Sixty were classified as false-positive, with generalized estimating equation-adjusted sensitivity and positive predictive value of 84.3% [confidence interval (CI), 82.5–86.0%] and 97.4% (CI, 96.5–98.2%), respectively. Overall, 95% of asymptomatic and 73% of actionable events were detected during HM sessions. Median reaction time was 3 days [interquartile range (IQR), 1–14 days]. Generalized estimating equation-adjusted incremental utility, calculated according to four properties of major clinical interest, was in favour of the HM sessions: +0.56 (CI, 0.53–0.58%), P < 0.0001. Resource consumption: 3364 HM sessions performed (76% by nurses), median committed monthly manpower of 55.5 (IQR, 22.0–107.0) min × health personnel/100 patients. Conclusion Home Monitoring was highly effective in detecting and managing clinical events in CIED patients in daily practice with remarkably low manpower and resource consumption. [1]: /lookup/external-ref?link_type=CLINTRIALGOV&access_num=NCT01459874&atom=%2Feuropace%2F15%2F7%2F970.atom

AIMS The majority of patients with pacemakers are very elderly, many being >85-years old. They often suffer from serious illnesses and have great difficulty in walking. The aim of our study was to compare remote pacemaker monitoring with in-home checks of pacemakers, in terms of applicability, efficacy, and cost in a selected population of debilitated elderly patients. METHODS AND RESULTS We selected 72 subjects (mean age 87 ± 8 years) among elderly debilitated patients with Medtronic pacemakers, compatible with the Carelink(®) remote monitoring system (13 patients with DDD pacemaker; 59 patients with single-lead VDD pacemaker). Remote follow-up was compared with in-home checks performed by nurses in 326 patients in similar clinical conditions. A total of 190 transmissions were received by remote monitoring (mean transmissions per month: 7.0; mean per patient: 2.6; range 1-6) during 27 months of follow-up. In this period, seven pacemakers were replaced owing to battery exhaustion, after a mean of 6.7 years from implantation. The occurrence of atrial or ventricular high-rate episodes was reported in 98 transmissions (53%). Nineteen patients died (annual mortality: 11.7%). On comparing the costs borne by the hospital for in-home checks, both for medical personnel and transportation, the estimated average saving was €32 per year per patient. CONCLUSION Our study shows that the remote follow-up of pacemakers is a reliable, effective, and cost-saving procedure in elderly, debilitated patients. Moreover, remote controls provided an accurate and early diagnosis of arrhythmia occurrence.

We evaluated the influence of sympathetic stimulation, induced by mental stress test, on signal-averaged ECG in 30 healthy subjects and in 30 patients with previous myocardial infarction. Both patient and control groups underwent three consecutive signal-averaged ECG: under basal conditions, during a continuous mental stress test, and 5 min after the mental stress test. The signal-averaged ECG parameters, analyzed by mean of a bandpass filter of 25-250 and 40-250 Hz, were: the filtered QRS duration, the duration of the low-amplitude high frequency signals in the terminal portions of the QRS complex below 40 microV (HFLA), and the root mean square voltage in the last 40 ms of the filtered QRS (RMS). The mathematical stress test was considered effective when the heart rate increased by at least 15 beats/min, remaining stable for the total duration of the signal-averaged ECG acquisition. A significant reduction of the filtered QRS duration and HFLA with a simultaneous increase of RMS were found in control group, while no significant changes occurred in patient group. These results indicate that the sympathetic nervous system may affect the results of signal-averaged ECG, probably by influences on intraventricular conduction, in healthy subjects. On the contrary, myocardial alterations in ischemic heart disease induce modification of signal-averaged ECG not influenced by autonomic nervous system activity.

Objectives. This study was designed to evaluate and compare the patterns of arrhythmogenic right ventricular cardiomyopathy in young people and adults. Background. Few data are available on this cardiomyopathy in young people because clinical and morphologic findings considered pathognomonic are normally based on observations in adults. However, a familial occurrence with a probable genetic transmission led to the study of children and to early detection of this disease, in which sudden death has been reported even in young people. Methods. Seventeen young patients with arrhythmogenic right ventricular cardiomyopathy diagnosed at a mean age ± SD of 14.9 ± 4.9 years were studied. Clinical, electrocardiographic, echocardiographic, cineangiographic and biopsy findings were compared with those of 19 adult patients whose condition was diagnosed at a mean age of 38.1 ± 13.4 years. Results. Syncope occurred in 23.5% of the young patients but in none of the adults (odds ratio of familial sudden death 5.54, p = 0.1). Ventricular couplets (odds ratio 16.0, p = 0.004) and subtricuspid bulging on echocardiography (odds ratio 5.95; p = 0.036) were prevalent in the young group. Cineangiographic data in the two groups were similar, except that more hypokinetic areas were found in adults (odds ratio 4.44, p = 0.05). Morphometric analysis of biopsy sections showed a greater amount of fibrous tissue in the young patients (p = 0.04) and a prevalence of fatty tissue in the adults (odds ratio 12, p = 0.005). During an equivalent follow-up time (mean 7 years), two young patients died suddenly, and two had ventricular fibrillation in the absence of antiarrhythmic therapy. Conclusions. The pathognomonic criteria for the diagnosis of arrhythmogenic right ventricular cardiomyopathy in adults are also valid for young people. Sudden or aborted death occurred frequently in young untreated patients.

Objectives The purpose of this study is to assess the effectiveness of defibrillation testing (DT) in patients undergoing implantable cardioverter-defibrillator (ICD) insertion. Background Although DT is considered a standard procedure during ICD implantation, its usefulness has not been definitively proven. Methods The SAFE-ICD (Safety of Two Strategies of ICD Management at Implantation) study is a prospective observational study designed to evaluate the outcome of 2 strategies: performing defibrillation testing (DT+) versus not performing defibrillation testing (DT−) during de novo ICD implants. No deviation from the centers' current practice was introduced. In all, 2,120 consecutive patients (836 DT+ and 1,284 DT−) age ≥18 years were enrolled at 41 Italian centers from April 2008 to May 2009 and followed up for 24 months until June 2011. The primary endpoint was a composite of severe complications at ICD implant and sudden cardiac death or resuscitation at 2 years. Results The primary endpoint occurred in 34 patients: 12 intraoperative complications (8 in DT+ group; 4 in DT− group) and 22 during follow-up (10 in DT+ group; 12 in DT− group). Overall, the estimated yearly incidence (95% confidence interval) was DT+ 1.15% (0.73 to 1.83) and DT− 0.68% (0.42 to 1.12). The difference between the 2 groups was negligible: 0.47% per year (−0.15 to 1.10). Mortality from any cause was similar at 2 years (adjusted hazard ratio: 0.97 [0.76 to 1.23], p = 0.80). Conclusions In this large cohort of new ICD implants, event rates were similar and extremely low in both groups. These data indicate a limited clinical relevance for DT testing, thus supporting a strategy of omitting DT during an ICD implant. (Safety of Two Strategies of ICD Management at Implantation [SAFE-ICD]; NCT00661037 )

Background Approximately 3% to 10% of patients with syncope experience episodes while driving. The aim of our study was to identify their clinical and pathophysiological features, estimate the incidence of recurrence, and correlate these characteristics with recurrence. Method We prospectively studied 40 patients who experienced syncope while driving and 50 subjects who experienced syncope in other situations. All cases underwent upright tilt test (UTT), with simultaneous ECG and beat-to-beat blood pressure recordings, to analyze heart rate variability, total peripheral resistance (TPR), and stroke volume (SV). Results Patients who experienced syncope while driving suffered more frequently from hypertension (40% vs 20%) and vasodepressive reactions during the UTT (52% vs 26%). Moreover, these patients had increased heart rates (p = 0.007) and lower SVs (p = 0.006) during a positive UTT. TPR rose immediately before symptoms appeared only in patients who suffered from nondriving syncope (p = 0.006). During a mean follow-up of 1793 ± 573 days, 8 syncopic patients while driving had recurrences (20%) but never while driving. They were older, experienced more episodes of syncope, and had a higher incidence of vasodepressive reactions during UTT. Conclusions Our study confirms a good prognosis for patients who experience syncope while driving, and indicates that more accurate risk stratification is needed in subjects aged over 50 years, who have had more than 4 episodes of loss of consciousness and vasodepressive reactions during UTT.

Background The diagnostic Brugada-electrocardiogram (Br-ECG) is characterized by "coved-type" ST-segment elevation (type 1) in V 1 to V 2 /V 3 . The sodium-channel blocker test is clinically used to unmask diagnostic Br-ECG in patients with nondiagnostic "saddle-back" Br-ECG (type 2 and type 3). Objective To assess the prognostic value of the sodium-channel blockers test in individuals with a nondiagnostic Br-ECG. Methods We studied 153 consecutive patients (128 men; age 41.7 ± 14.0 years) with a type 2/3 Br-ECG who underwent a sodium-channel blocker test with either flecainide (48%) or ajmaline (52%). Nondiagnostic Br-ECGs were identified during the evaluation of cardiac arrest in 5 patients (3%), syncope in 36 (24%), cascade family screening in 48 (31%), and incidental ECG in 64 (42%). A spontaneous type 1 Br-ECG was systematically excluded by serial ECGs (6.1 ± 0.4) and recording of right precordial leads both at standard and second and third intercostal spaces. Results The sodium-channel blocker test result was positive in 76 (50%) patients. During a follow-up of 59 ± 33 months, 9 (5.9%) patients experienced events such as syncope (n = 4), appropriate interventions of defibrillator (n = 4), or sudden death (n = 1). A positive sodium-channel blocker test was associated with a significantly higher event rate in symptomatic patients ( P = .01) but not in asymptomatic individuals ( P = .18). No events occurred among asymptomatic individuals with an incidental nondiagnostic Br-ECG. Conclusions In asymptomatic individuals with a nondiagnostic Br-ECG, the incidence of events is low regardless of the sodium-channel blocker test result while in symptomatic patients a positive sodium-channel blocker test result is associated with an adverse arrhythmic outcome and may contribute to risk stratification.

We assessed the efficacy, reliability and cost of an in-home control service, devoted to debilitated patients with pacemakers, during long-term follow up.We admitted 802 patients (mean age 87.8 ± 6.9 years) with pacemakers into our program, which were monitored periodically during in-home visits by nurses. During these controls, patients underwent a standard electrocardiogram with and without magnet, during which stimulation intervals were measured. In-home pacemaker assessments were performed by a portable programmer only for patients with Biotronik pacemakers. Electrocardiograms and technical data were evaluated at hospital, and a report was sent by mail to the patient. Moreover, we compared the costs for the hospital, patients and society between in-home and ambulatory assessments.During 80 months of follow up, 2418 controls were performed. The monthly average of patients who were followed at home was 283.9 ± 23.9. The mean number of controls per month was 30.2 ± 10 (range 4-51). One hundred and twenty-three pacemakers were replaced due to battery exhaustion, after a mean device longevity of 7.4 years (range 4.9-12.4 years). Four hundred and sixty-eight patients died (8.7% annual mortality). Fifteen patients (annual incidence 0.3%) were invited to in-hospital visits for reprogramming. The cumulative cost for in-home assessment were 20.5% lower than conventional in-hospital controls and 66.5% lower if patients are transported by ambulance.Elderly debilitated patients can be monitored by effective and reliable pacemaker assessments at home, alleviating them and their families from the issues that are associated with their transfer from the home to the hospital and concurrently reducing overall costs.

The hallmark lesion of arrhythmogenic right ventricular cardiomyopathy (ARVC/D) is the RV myocardial loss with replacement by fibrofatty tissue. Emerging tools offer the possibility to directly visualize fibrofatty ventricular scar. electroanatomic voltage mapping (EVM) by CARTO system has been demonstrated to identify low-voltage myocardial areas (“electroanatomic scar”, EAS) invasively, whereas contrast-enhanced cardiac magnetic resonance CE-CMR has the potential to detect regions of delayed contrast-enhancement (“DCE scar”) non-invasively.

Recent studies have suggested that interlead QT variability on the surface electrocardiogram, defined as QT dispersion, may reflect regional variations in myocardial recovery of excitability.1-4 The finding of a decreased QT dispersion in patients with different heart diseases treated with class III agents, sotalo13 and amiodarone,5T6 further supports this theory. A significant QT dispersion was found both in patients with congenital and acquired long QT interva12,5,7%s and in patients with myocardial infarction. 2,3,g~10 In hypertrophic cardiomyopathy (HC), the presence of electrical instability is a potentially important determinant of sudden death.“-l3 The pathologic substrate of the disease may create the conditions for QT interval prolongation and nonhomogeneous recovery of excitability.14 Dritsas et all5 recently showed a prolonged corrected QT interval (QTc) and increased QTc dispersion in 24 patients with HC compared with normal subjects, but a comparison between patients with and without ventricular arrhythmias was not done. This study assesses QT dispersion in HC patients with and without documented serious ventricular arrhythmias and sudden death. Twenty-six patients with NC (19 males and 7 females, mean age 37 t- 13 years, range 8 to 57) attending our institution for outpatient follow-up evaluation, or referred for risk stratification

Background— The role of implantable cardioverter-defibrillator (ICD) in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia and no prior ventricular fibrillation (VF) or sustained ventricular tachycardia is an unsolved issue. Methods and Results— We studied 106 consecutive patients (62 men and 44 women; age, 35.6±18 years) with arrhythmogenic right ventricular cardiomyopathy/dysplasia who received an ICD based on 1 or more arrhythmic risk factors such as syncope, nonsustained ventricular tachycardia, familial sudden death, and inducibility at programmed ventricular stimulation. During follow-up of 58±35 months, 25 patients (24%) had appropriate ICD interventions and 17 (16%) had shocks for life-threatening VF or ventricular flutter. At 48 months, the actual survival rate was 100% compared with the VF/ventricular flutter–free survival rate of 77% (log-rank P =0.01). Syncope significantly predicted any appropriate ICD interventions (hazard ratio, 2.94; 95% confidence interval, 1.83 to 4.67; P =0.013) and shocks for VF/ventricular flutter (hazard ratio, 3.16; 95% confidence interval, 1.39 to 5.63; P =0.005). The positive predictive value of programmed ventricular stimulation was 35% for any appropriate ICD intervention and 20% for shocks for VF/ventricular flutter, with a negative predictive value of 70% and 74%. None of the 27 asymptomatic patients with isolated familial sudden death had appropriate ICD therapy. Twenty patients (19%) had inappropriate ICD interventions, and 18 (17%) had device-related complications. Conclusions— One fourth of patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia and no prior sustained ventricular tachycardia or VF had appropriate ICD interventions. Syncope was an important predictor of life-saving ICD intervention and is an indication for ICD. Prophylactic ICD may not be indicated in asymptomatic patients because of their low arrhythmic risk regardless of familial sudden death and programmed ventricular stimulation findings. Programmed ventricular stimulation had a low predictive accuracy for ICD therapy.

Right ventricular lead perforation is a rare but serious and potentially life-threatening complication of pacemaker or defibrillator lead implantation. This report describes a patient with Brugada syndrome in whom the diagnosis of asymptomatic right ventricular perforation by an implantable cardioverter defibrillator lead was detected 12 days after implantation, thanks to a report from home monitoring system. The patient was admitted to our institution, where the lead was explanted and replaced. This case illustrates the potential lifesaving benefit of the home monitoring system in patients with implantable cardioverter defibrillator.

Postoperative 24-hour Holter monitoring was performed in 106 patients with atrioventricular septal defect in order to identify the incidence of atrial and ventricular arrhythmias. Of the patients, 72 had separate atrioventricular orifices, including 13 with a small ventricular component to the defect, and 34 patients had a common atrioventricular orifice. Two groups of abnormal patients were found. First, patients with good electrical stability characterized by isolated atrial (9 patients) and ventricular (25 patients) extrasystoles falling in classes I and II of Lown. Second, patients with marked electrical instability characterized in one patient by repetitive atrial extrasystoles, in another by atrial flutter, in 2 by polymorphic ventricular extrasystole and in 8 by couplets or triplets. Electrical instability in individual patients was then compared, by means of logistic regression analysis, with operative, surgical and postoperative variables. There was no incidence of sudden death in our series. After surgical repair, ventricular arrhythmias were more frequent than atrial arrhythmias (33% vs. 10%) and were unrelated to the type of atrioventricular septal defect. Cardiac electrical instability after operation was significantly related to larger operative body size, higher postoperative end diastolic diameter of the right ventricle, larger size of the ventricular septal defect, coexistence of postoperative right bundle branch block and left anterior hemiblock. Conversely, the risk of arrhythmias was reduced by more recent operative data and by greater shortening fraction of the left ventricle.

An asymptomatic 19-year-old top-level athlete had electrocardiographic evidence of intermittent cardiac preexcitation and intermittent left bundle branch block. The electrophysiologic study demonstrated the presence of a direct accessory pathway and enhanced atrioventricular node conduction that resulted in infrahisian and intraventricular conduction disturbances. The echocardiogram disclosed tricuspid valve dysplasia.

Previous studies proposed as one of the main mechanisms involved in neurally mediated syncope, the stimulation of ventricular mechanoreceptors as the final trigger for vagal discharge.This study aimed to verify the presence of a sympathetic driven increase of cardiac contractility before vasovagal syncope.We studied 23 patients with recurrent syncope. All underwent a 60 ° tilt with pharmacologic challenge (sublingual spray nitrate). Two conditions were used to assess autonomic activity by heart rate variability analysis: in a supine position after 5 min of rest and after 15 min of tilt. Simultaneously, cardiac contractility was quantified by tissue-Doppler echocardiography at the base of the free walls of left ventricle. The peak myocardial velocities during systole (Sw) and late diastole (Aw) were considered.Passive tilt induced a significant increase of the low frequency component (LF) as well as a decrease of the high frequency component (HF) in positive patients (LF: from 49 ± 18 to 65 ± 18 nu, p0.05; HF: from 41 ± 21 to 26 ± 16 nu, p0.05). Tissue-Doppler showed a similar increase in Sw in both positive and negative patients but showed a significant decrease of Aw in syncopal subjects (p0.005).Our results do not show an increase in ventricular contractility before tilt-induced syncope, or in presence of a valuable increase of sympathetic activity. Instead, we observe a reduction of atrial contractility, which may be a contributory component in the pathogenesis of vasovagal syncope.

Clinico-pathological findings are described in two patients with typical variant angina who died suddenly during an ischemic attack. In both cases, detailed pathologic examination of the coronary arteries disclosed severe focal atherosclerosis of the anterior descending coronary artery. The only distinctive histological finding was new intimal proliferation of smooth muscle cells enmeshed within mucoid substance, superimposed on the old fibrous cap of the plaque. These findings agree with experimental and clinical data which suggest that coronary vasospasm may be related to growth of atherosclerotic plaques. This study provides histological evidence that progression of an atherosclerotic plaque may underlie variant angina and sudden death.

A 43-year-old woman with progressive external ophthalmoplegia developed a bifascicular block and dilatation of the right ventricle during 4 years of follow-up. Histochemical and electron microscopy studies detected mitochondrial abnormalities in ocular, skeletal muscle and cardiac biopsies. This case registers disease progression from the external ocular to the skeletal and cardiac muscles. Mitochondrial DNA was deleted in relation to the morphological abnormality.

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an inherited heart muscle disease that occurs primarily in young and middle-age individuals. It is characterized by ventricular arrhythmias (VA), sudden death, and by heart failure occurring later in life (1–5). Ventricular electrical instability may occur at any time during the disease depending upon possible different pathophysiologic mechanisms including: a) Inflammation and apoptosis leading to ventricular fibrillation; or b) Fibro-fatty tissue repair leading to scar-related ventricular tachycardia (VT). Heart failure may occur later in life secondary to slow, progressive loss of right and left ventricular myocardium (1–8). The role of pharmacological therapy in controlling VA and preventing sudden death has been evaluated in single-center studies (9), and the results of catheter ablation have been described in small series of patients (10–15). The efficacy and safety of implantable cardioverter/defibrillator (ICD) have also been reported in small, single-center studies (16–19) and recently in larger single and multicenter studies (20–27). The main questions regarding the risk stratification and the therapeutic strategy in ARVC/D are: 1) differential diagnosis with idiopathic VT (right ventricular outflow tract VT) in an apparently normal heart. The prognosis of this latter condition is usually excellent with rare cases of sudden cardiac death; 2) prognostic and therapeutic significance of noninvasive and invasive investigations including electrophysiologic study (EPS); 3) efficacy of antiarrhythmic drugs (AAD) to prevent VT and sudden cardiac death and the adverse effects of these drugs in this population; 4) indications and results of catheter ablation; 5) identification of patients at high risk of sudden cardiac death who need ICD implantation as well as the complications of ICD in a diseased right ventricular myocardium. It is important to recognize that ARVC/D is a progressive disease and risk factors may change during follow-up requiring periodic revaluation of risk as well as of therapy.