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ICD Interventions and Mortality Background Patients with severe structural heart disease have increased mortality after implantable cardioverter-defibrillator (ICD) shocks. Whether this is limited to ICD shock therapy only or extends also to no-shock therapies, such as antitachycardia pacing (ATP), is unclear. We investigated the impact of different ICD therapies on long-term mortality. Methods We enrolled 573 patients who underwent ICD implantation at our institution from 2004 to 2011. The population was divided into 3 groups: no device interventions (group 1), ATP interventions (group 2), and shock interventions (group 3). The endpoint was the all-cause mortality. Results Over a follow-up period of 48 months (range 1–110), 447 (78%) had no device interventions, 71 (12%) had ATP therapy only, and 55 (10%) had at least one shock intervention. All-cause mortality occurred in 94 patients in group 1 (21%), 23 patients (43%) in group 2, and 21 patients (38%) in group 3. At multivariable Cox regression analysis, ATP intervention (HR: 1.8; 95% CI 1.1–3; P < 0.001), shock intervention (HR: 1.39; 95% CI 1.09–1.77; P = 0.008), age (HR: 1.05; 95% CI 1.02–1.07; P < 0.001), and LVEF (HR: 0.95; 95% CI 0.93–0.98; P = 0.001) were predictors of all-cause mortality. No significant difference in mortality was found between group 2 and 3. Conclusion Patients with ICDs who receive appropriate interventions are at increased risk of mortality. Such risk is not dependent on different types of ICD therapy, such as shocks or ATP. Our data suggest that sustained ventricular arrhythmias per se have a negative impact on prognosis rather than modality of ICD therapy.

Positron emission tomography-computed tomography (PET-CT) with (18)F-fluorodeoxyglucose (FDG) has emerged as a rapidly evolving diagnostic tool for infectious diseases. However, the optimal imaging time in this clinical setting is not clear yet. The aim of this study is to investigate whether delayed (3 hours) FDG PET-CT could increase the diagnostic accuracy of this technique compared to standard (1 hour) imaging in the detection of septic foci involving the pocket and/or pacing leads in patients with suspected cardiovascular implantable electronic device (CIED) infection scheduled for device removal.Twenty-seven patients underwent standard and delayed imaging. PET-CT results were compared to bacteriological cultures after CIED removal. Fifteen controls free of infection underwent PET-CT imaging as part of investigation of malignancy. The diagnostic accuracy of delayed imaging was significantly higher than 1-hour scan for lead infection (70% vs 51%, P = .024). No significant difference was found between standard and delayed diagnostic accuracy for pocket or device infection. Semi-quantitative analysis showed that mean pocket and lead target-to-background ratio were significantly higher on delayed compared to standard imaging (3.7 ± 1.9 vs 1.6 ± 1.1, P = .0002; 3.0 ± 1.3 vs 0.7 ± 1.0, P = .01).Delayed FDG PET-CT imaging should be considered at least in patients with negative 1-hour scan and founded suspicion of pacing lead infection.

The research in the field of sleep medicine has increased during the whole twentieth century, principally for the involvement of sleep-related disordered breathing (SDB) in cardiovascular disease. If sleep encompasses about a third of one’s life, the reasons are mostly linked to its effects on the cardiovascular and respiratory systems. Sleep is a physiological phenomenon characterized by changes in the human body leading to a state of quiescence of the cardiovascular, respiratory and metabolic systems [1]. The importance of these events becomes more evident if we Review Article British Journal of Medicine & Medical Research, 4(1): 34-45, 2014 35 consider what happens in their absence, that is, during SDB syndromes. These syndromes include habitual snoring, sleep apnea, Cheyne-Stokes breathing syndrome and sleep hypoventilation syndrome [2]. Sleep apnea syndromes are characterized by several apneic events during the night, which consist in absence of the airflow or its reduction by more than 90% lasting more than 10 seconds, with consequent oxyhemoglobin desaturation and arousal [2]. These events provoke microawakening and sleep fragmentation that represent, along with hypoxemia, important harmful triggers on the cardiovascular system. In fact, SDB presents as a highly prevalent comorbidity in patients with heart failure (HF); both diseases are related to each other in a bidirectional way through multiple mechanisms: apneic events raise cardiac afterload, and at the same time impaired cardiac function itself may contribute to the development of sleep apnea. HF is a clinical syndrome characterized by signs or symptoms due to the inability of the heart to provide a normal tissue perfusion: the failing cardiac pump is not able to maintain an adequate output for this task. Typical features of HF are represented by shortness of breath, resting or exertion dyspnea, fatigue, fluid retention leading to pulmonary congestion or ankle swelling, and objective evidence of a structurally or functionally abnormal heart at rest [1,3].

Background Islets of myocytes within fibrofatty scars represent the substrate for reentrant ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy (ARVC). Electroanatomic mapping can reliably identify such areas. Objective To prospectively test the association between late and fragmented electrograms within scar and arrhythmic events in patients with ARVC. Methods High-density right ventricle electroanatomic mapping was performed in 32 patients with ARVC without history of cardiac arrest or sustained ventricular arrhythmias. Standard definitions of electroanatomic scars and fragmented, isolated, and very late potentials were used. All patients received an implantable cardioverter-defibrillator for the primary prevention of sudden death. Results After a mean follow-up of 25 ± 7 months, 12 (38%) patients received appropriate implantable cardioverter-defibrillator shock for sustained ventricular arrhythmias. With the exception of a higher rate of previous syncope ( P = .053), patients with arrhythmic events at follow-up did not differ from those who remained free from arrhythmic events in terms of other clinical variables, including cardiac magnetic resonance findings. Electroanatomic scars were present in all patients. The distribution and extent of electroanatomic scars were similar in the 2 groups (38 ± 25 cm 2 vs 33 ± 20 cm 2 ; P = .51). However, patients with implantable cardioverter-defibrillator shock had a higher prevalence of fragmented electrograms (92% vs 20%; P P = .004), and of very late potentials (67% vs 25%; P = .030). Fragmented electrograms were the only variable independently associated with arrhythmic events at follow-up (hazard ratio 21; P = .015). Conclusion The presence of fragmented and delayed electrograms within the scar predicts arrhythmic events in ARVC.

Background— The differential diagnosis between idiopathic and cardiomyopathy-related right ventricular outflow tract (RVOT) ventricular arrhythmias (VAs) is crucial. Signal-averaged ECG (SAECG) abnormalities are frequent in cardiomyopathy-related RVOT-VAs, although their pathophysiologic basis and diagnostic value in this setting are undefined. We tested the association between SAECG and the myocardial substrate underlying RVOT-VAs. Methods and Results— Twenty-four consecutive patients (median age, 50 years [42–59]; 12 men) with RVOT-VAs (10 with frequent [>1000/24 hours] premature ventricular contractions, 14 with ventricular tachycardias) underwent SAECG with 40-Hz filtering and electroanatomic mapping (EAM) with EAM-guided biopsy for characterization of the RVOT-VAs substrate. A filtered averaged QRS (fQRS) was obtained and analyzed for fQRS duration, low amplitude signal duration P =0.01) and LAS40 (39 ms [36–51] versus 19 ms [18–21], P =0.02), and lower RMS40 (18 µV [9–26] versus 33 µV [32–33], P =0.04). A significant linear correlation was found between the extension (cm2) of the RVOT scar and all 3 SAECG parameters (rs=0.76, P P P 2 . Conclusions— In patients with RVOT-VAs, abnormal SAECG parameters reflect the presence of extensive cardiomyopathic involvement of the RVOT. However, a negative SAECG does not reliably rule out cardiomyopathy-related RVOT-VAs in the presence of a small RVOT scar.

Patients at risk of sudden cardiac death (SCD) after myocardial infarction (MI) can be offered therapy with implantable cardioverter defibrillators (ICDs). Whether plasma biomarkers can help risk stratify for SCD and ventricular arrhythmias (VT/VF) is unclear.The primary objective of the CAMI-GUIDE study is to assess the predictive role of C-reactive protein for SCD or VT/VF in ischaemic patients with the ejection fraction30% and ICDs. Secondary endpoints included all-cause mortality, hospitalizations, and death from heart failure. Additional analyses incorporated cystatin-C and NT-ProBNP in multi-marker approach for the prediction of adverse outcomes. A total of 300 patients were enrolled. All-cause mortality at 2 years was 22.6%, mortality from heart failure was 8.3%. Primary endpoint occurred in 17.3%. At a competing risk multivariable analysis adjusted for baseline variables, no significant difference in primary endpoint was found between patients with C-reactive protein ≤3 vs.3 mg/L [heart rate (HR) 0.91 (0.50-1.64) P = 0.76], while C-reactive protein3 mg/L was strongly associated with mortality due to heart failure [HR: 3.17 (1.54-6.54) P = 0.002]. NT-proBNP above median was significantly associated with the primary endpoint [adjusted HR: 1.46 (1.020-2.129) P = 0.042]. A risk function, including the three biomarkers, NYHA class and resting HR, allowed stratification of patient mortality risk from 5 to 50%.C-reactive protein3 mg/L is not associated with SCD or fast VT/VF, however, is a strong predictor of HF mortality. Biomarkers combined with clinical markers allow an excellent risk stratification of mortality at 2 years.

Background: Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is associated with an increased risk of sudden cardiac death (SCD). Risk stratification of ARVC/D patients, however, remains an unresolved issue. In this study we investigated whether heart rate variability (HRV) can be helpful in identifying ARVC/D patients with increased risk of arrhythmic events. Methods and Results: We studied 30 consecutive patients (17 males; 45.4±18 years) with ARVC/D, diagnosed according to guideline criteria; 15 patients (50%) had received an implantable cardioverter defibrillator (ICD) for primary SCD prevention. HRV was assessed on 24-h ECG Holter monitoring. The primary endpoint was the occurrence of major arrhythmic events (SCD, sustained ventricular tachycardia (VT), ICD therapy for sustained VT or ventricular fibrillation (VF)). During the follow-up period (19±7 months), no deaths occurred, but 5 patients (17%) experienced arrhythmic events (4 VTs and 1 VF, all in the ICD group). All HRV parameters were significantly lower in patients with, compared with those without, arrhythmic events. Low-frequency amplitude was the most significant HRV variable associated with arrhythmic events in univariate Cox regression analysis (P=0.017), and was the only significant predictor of arrhythmic events in multivariable regression analysis (hazard ratio 0.88, P=0.047), together with unexplained syncope (hazard ratio 16.1, P=0.039). Conclusions: Our data show that among ARVC/D patients HRV analysis might be helpful in identifying those with increased risk of major arrhythmic events. (Circ J 2012; 76: 618-623)

Background: The Seattle Heart Failure Model (SHFM) is a multimarker risk assessment tool able to predict outcome in heart failure (HF) patients. Aim: To assess whether the SHFM can be used to risk-stratify HF patients who underwent cardiac resynchronization therapy with (CRT-D) or without (CRT) an implantable defibrillator. Methods and Results: The SHFM was applied to 342 New York Heart Association class III-IV patients who received a CRT (23%) or CRT-D (77%) device. Discrimination and calibration of SHFM were evaluated through c-statistics and Hosmer-Lemeshow (H-L) goodness-of-fit test. Primary endpoint was a composite of death from any cause/cardiac transplantation. During a median follow-up of 24 months (25th–75th percentile [pct]: 12–37 months), 78 of 342 (22.8%) patients died; seven patients underwent urgent transplantation. Median SHFM score for patients with endpoint was 5.8 years (25th–75th pct: 4.25–8.7 years) versus 8.9 years (25th–75th pct: 6.6–11.8 years) for those without (P < 0.001). Discrimination of SHFM was adequate for the endpoint (c-statistic always ranged around 0.7). The SHFM was a good fit of death from any cause/cardiac transplantation, without significant differences between observed and SHFM-predicted survival. Conclusion: The SHFM successfully stratifies HF patients on CRT/CRT-D and can be reliably applied to help clinicians in predicting survival in this clinical setting. (PACE 2012; 35:88–94)

RV Scar by Electroanatomical Mapping and CMR. Introduction: Assessment of late gadolinium enhancement (LGE) at cardiac magnetic resonance is often used to detect scar in patients with arrhythmias of right ventricular (RV) origin. Recently, electroanatomic mapping (EAM) has been shown to reliably detect scars corresponding to different cardiomyopathic substrates. We compared LGE with EAM for the detection of scar in patients with arrhythmias of RV origin.

Background: The management of implantable cardioverter defibrillators (ICDs) affected by advisories, which often include generator replacement, is complex and the risk of device failure needs to be carefully assessed for each patient. Methods: We analyzed the response to the advisory communication in the Italian centers involved in the recall for the Prizm 1861 and Renewal (Boston Scientific–formerly Guidant—St. Paul, MN, USA) communication. Results: One hundred and thirty-nine of 843 Prizm (16.5%) and 458 of 2,342 Renewal devices (19.6%) were explanted. The total incidence over a 4-year time frame of the failure event was equal to zero of 710 (0%) for Prizm ICDs and eight of 2,342 (0.34%) for Renewal ICDs. A limited percentage of devices affected by recall were definitely explanted following the indications stated by the advisory. The failure rates that resulted from analysis of our data (0% for Prizm and 0.34% for Renewal) were inferior to those already found or projected along the device lifetime globally, as reported in the most recent Company Product Performance Report (0.72% for Prizm and 1.83% for Renewal). Conclusions: In absence of underestimation of the events, a lower incidence than expected could resize the dimension of the problem, justifying the concept of a more frequent follow-up of patient with respect to the choice of an immediate device explant. (PACE 2011; 34:998–1002)

Radiofrequency catheter ablation (RFCA) of supraventricular tachyarrhythmias carries a small but non-negligible radiation risk. Studies in children already showed the feasibility of using three-dimensional mapping systems as the primary guide for catheter visualization and positioning in these RFCAs. We aim to demonstrate the feasibility and safety of such an approach in young and middle-aged patients.Fifty patients (age 34 ± 12) with supraventricular tachyarrhythmias underwent electrophysiological study; of these, 47 patients proceeded to RFCA guided by the EnSite NavX(TM) system (23 with atrioventricular nodal reentry tachycardia, 16 with an accessory pathway, six with typical atrial flutter, and two with right atrial tachycardia).In 38/50 cases (76%), electroanatomical mapping avoided fluoroscopy entirely, including four cases requiring access to the left heart chambers by a retrograde approach. In the remaining 12/50 cases (24%), fluoroscopy use was limited to 122 ± 80 s, with a correspondingly low radiation exposure (dose area product 1.3 ± 1.1 mGy × m(2)). All procedures were acutely successful, with a procedural time of 113 ± 37 minutes, and without incurring in any major complication. Over a mean follow-up of 12 ± 3 months, we observed one recurrence of pre-excitation and one relapse of atrial flutter.Our study shows that non-fluoroscopic RFCA of supraventricular tachyarrhythmias using the EnSite NavX(TM) system is feasible, safe, and effective in a population of relatively young adults. Our experience of a non-fluoroscopic approach in these procedures deserves consideration, particularly in the young or in other patients at higher radiation risk.

Hypertension is one of the most important risk factors and causes of cardiovascular disease (CVD). From some years, renin-angiotensin-aldosterone system (RAAS) inhibitors such angiotensin converting enzyme (ACE) and angiotensin receptor blockade (ARB) have been of interest, not only for better blood pressure (BP) control but also for their involvement in the mechanisms of various organ functions.The aim of this review is to focus on the effectiveness and safety of aliskiren beyond the treatment of hypertension.Aliskiren, the first approved renin inhibitor to reach the market, is a low-molecular-weight, orally active, hydrophilic non-peptide molecule that blocks angiotensin I generation. Because of its mechanism of action, aliskiren may offer the additional opportunity to inhibit progression of atherosclerosis at tissue level and the potential to be useful in a wide spectrum of conditions. However, we will discuss how it might become a reasonable therapeutic choice also in a broad number of clinical conditions, sharing an increased cardiovascular risk as stable coronary artery disease (CAD), microvascular and cardio-renal disease, diabetes, and peripheral arterial disease (PAD).Therapy of hypertension through a better blockade of RAAS may be the first step in also achieving interesting results in the complications that hypertension causes in several organs.

Contact point-to-point electroanatomic mapping (Pt-Map) is a validated tool to evaluate right ventricular (RV) substrate. When using the EnSite NavX system (St. Jude Medical, St Paul, Minnesota), geometry reconstruction by dragging the mapping catheter (Geo-Map) allows for quicker acquisition of a large number of points and better definition of anatomy, but it is not validated for substrate mapping.This study evaluates the feasibility and accuracy of Geo-Map.Thirteen patients (mean age 38 +/- 12 years) with RV arrhythmias and an apparently normal heart underwent cardiac magnetic resonance imaging (MRI), Pt-Map, and Geo-Map. The 2 maps were compared in terms of mapping procedural time, radiation time, and total number of points acquired. We finally compared the number and characteristics of low-potential areas on each patient's Pt-Map, Geo-Map, and cardiac MRI.Geo-Map required significantly shorter mapping and radiation times in comparison to Pt-Map (12.4 +/- 4.6 vs. 31.9 +/- 10.1 and 5.8 +/- 2.1 vs. 12.1 +/- 3.9, P.001). Furthermore, Geo-Map was based on a significantly higher density of points in comparison to Pt-Map (802 +/- 205 vs. 194 +/- 38, P.001). Taking into consideration the total number of RV regions analyzed, the Pt-Map and Geo-Map disagreed in 2 of 65 (3%) regions (P = NS), which only Geo-Map identified as low-potential areas and indeed corresponded to wall motion abnormalities on MRI.Voltage maps obtained through RV geometry acquisition have accuracy comparable to that of conventional point-by-point mapping in detecting low-voltage areas, have a good correlation with MRI wall motion abnormalities, and allow a significant reduction in procedural time and x-ray exposure.

Hypertension is one of the most important risk factors for, and causes of, cardiovascular disease. The difficulty in achieving a normal blood pressure range in some patients makes the rate of cardiovascular disease high. For some years renin-angiotensin system inhibitors such as angiotensin-converting enzyme (ACE) and angiotensin receptor blockade have been objects of interest for treatment of cardiovascular disease. Aliskiren, the first approved renin inhibitor to reach the market, is a low molecular weight, orally active, hydrophilic nonpeptide molecule, which blocks angiotensin I generation. However it might also become a reasonable therapeutic choice in a broad number of clinical conditions, as stable coronary artery disease, cerebrovascular and cardiorenal disease, diabetes, and peripheral arterial disease. The aim of this review is to describe the effectiveness and safety of aliskerin in the treatment of hypertension.

Objectives We evaluated the diagnostic contribution and the therapeutic and prognostic implications of 3-dimensional electroanatomic mapping (EAM)-guided endomyocardial biopsy (EMB) in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). Background ARVC is a frequent cause of life-threatening ventricular arrhythmias and sudden death in young people. The value of current diagnostic criteria and the role of imaging techniques and EMB are still debated. Methods We studied 30 consecutive patients (17 male, mean age 43 ± 17 years) with a noninvasive diagnosis of ARVC according to current criteria. All patients underwent 3-dimensional EAM-guided EMB. Results Twenty-nine (97%) of 30 patients presented an abnormal voltage map. Histology and immunohistochemistry confirmed the diagnosis of ARVC in 15 patients, and showed active myocarditis according to Dallas criteria in the remaining 15 patients. Patients with ARVC were not distinguishable on the basis of clinical features, presence, and severity of structural and functional right ventricular abnormalities and 3-dimensional EAM findings. On the basis of clinical and histological features, a cardioverter-defibrillator was implanted in 13 patients with biopsy-proven ARVC and in 1 patient only with myocarditis. At a mean follow-up of 21 ± 8 months, 7 (47%) patients with ARVC experienced a recurrence of symptomatic sustained ventricular arrhythmias with appropriate defibrillator intervention in all cases. All patients with myocarditis remained asymptomatic and free from arrhythmic events. Conclusions Right ventricular myocarditis frequently mimics ARVC. Three-dimensional EAM-guided EMB is a safe and effective tool in differential diagnosis and in the selection of the most appropriate therapeutic strategy.

We report the case of a 50 year-old woman referred for extraction of an infected biventricular implantable cardioverter defibrillator (ICD) who underwent device and leads extraction. Due to adhesions at the level of the subclavian–innominate vein angle, manual traction with locking stylet was ineffective. A new mechanical sheath provided with a stainless steel bladed tip, the Evolution™ Mechanical Dilator Sheath (Cook Medical), was successfully used to complete lead extraction. As far as we know, this is the first report on the use of this new tool; its safety should be ascertained by increasing the number of cases but its simple use and its low cost make the Evolution™ Mechanical Dilator Sheath a new interesting tool that can be added to the instruments available for lead extraction.

A 65-year-old man was referred for atrial fibrillation ablation to our center. Routine pre-procedure transthoracic and transoesophageal echocardiography and cardiac computed tomography examinations showed a normal interatrial septum and fossa ovalis anatomy. Access to left atrium was initially planned using a conventional transseptal needle puncture. During the procedure, several consecutive attempts in conjunction with intracardiac echocardiography support, failed to cross the septum. The procedure was then successfully carried out using a specifically designed radiofrequency transseptal catheter.

Objective To assess whether magnetic resonance imaging could detect any cardiac morphological or functional myocardial alterations in healthy children and young adults with ventricular arrhythmias. Methods Twenty-three subjects (14 male, mean age 15.6 +/- 6.5 years) with frequent (> or =30/h) premature ventricular complexes (PVCs) on Holter monitoring and normal echocardiographic and electrocardiographic findings underwent cardiac magnetic resonance (CMR) on a 1.5T scanner and an exercise stress test. Subjects were also followed up for a period of 71 +/- 24 months. Results CMR showed no evidence of structural cardiac abnormalities, but functional assessment revealed significant impairment in 17 subjects (74%): mild to moderate right ventricular enlargement was found in all of these subjects associated with a mild reduction of ventricular function in five cases (22%) and mild free wall and/or apex contraction abnormalities in eight subjects (35%). PVCs persisted during stress test in three subjects (13%) and disappeared in 19 (83%). No serious cardiac event was observed during the follow-up. Conclusions Our study shows that subjects with PVCs without detectable electrocardiographic and echocardiographic abnormalities frequently exhibit functional impairment of the right ventricle at CMR, potentially responsible for ventricular arrhythmias. Although the causes of these abnormalities remain to be elucidated, the long-term outcome of these subjects is excellent.

Aims Electrical storm is an emergency in ‘implantation of a cardioverter defibrillator’ carriers with ischemic dilated cardiomyopathy (DCM) and negatively impacts long-term prognosis. We evaluated the feasibility, safety, and effectiveness of radiofrequency catheter ablation (RFCA) in controlling electrical storm and its impact on survival and ventricular tachycardia/fibrillation recurrence. Methods We enrolled 27 consecutive patients (25 men, age 73.1 ± 6.5 years) with ischemic DCM and an indication to RFCA for drug-refractory electrical storm. The immediate outcome was defined as failure or success, depending on whether the patient's clinical ventricular tachycardia could still be induced after RFCA; electrical storm resolution was defined as no sustained ventricular tachycardia/ventricular fibrillation in the next 7 days. Results Of the 27 patients, 1 died before RFCA; in the remaining 26 patients, a total of 33 RFCAs were performed. In all 26 patients, RFCA was successful, although in 6/26 patients (23.1%), repeated procedures were needed, including epicardial ablation in 3/26 (11.5%). In 23/26 patients (88.5%), electrical storm resolution was achieved. At a follow-up of 16.7 ± 8.1 months, 5/26 patients (19.2%) had died (3 nonsudden cardiac deaths, 2 noncardiac deaths) and 10/26 patients (38.5%) had ventricular tachycardia recurrence; none had electrical storm recurrence. A worse long-term outcome was associated with lower glomerular filtration rate, wider baseline QRS, and presence of atrial fibrillation before electrical storm onset. Conclusion In patients with ischemic DCM, RFCA is well tolerated, feasible and effective in the acute management of drug-refractory electrical storm. It is associated with a high rate of absence of sustained ventricular tachycardia episodes over the subsequent 7 days. After successful ablation, long-term outcome was mainly predicted by baseline clinical variables.

Biventricular implantation procedures require contrast venography of the coronary sinus. The aim of our study was to evaluate the efficacy and safety of contrast venography obtained by direct manual contrast injection into the guiding catheter, compared with venography obtained after occlusion of the coronary sinus by a Swan-Ganz catheter.Eighty-three patients were randomly assigned to direct or occlusive venography technique. The primary endpoint was complication rate. The secondary endpoints were rate of and time required for an adequate venography, total dose of contrast medium and total procedure time.Four dissections of the coronary sinus were observed with the occlusive venography technique group while no complications were observed with the direct venography technique group (p=0.04). Rate of adequate venography was similar in the two groups (p=NS). The time needed for coronary sinus venography and the total dose of contrast medium was significantly lower in the direct venography technique group compared with the alternative (p0.0001 and p=0.003, respectively); the total procedure time was not significantly different between the two groups (p=NS).The direct venography technique shows a significantly lower incidence of complications and should be considered to be the first line approach to coronary sinus venography during biventricular pacemaker implantation.

Biventricular pacing system implantation is a time-consuming and challenging procedure. A critical step in biventricular pacemaker implantation is coronary sinus (CS) cannulation. CS cannulation can be achieved either using dedicated guiding catheters (guiding catheter alone positioning strategy, GCA) or with the aid of an electrophysiology catheter advanced inside the guiding catheter (electrophysiology catheter aided positioning strategy, EPA).To evaluate whether the EPA technique is useful for reducing CS cannulation time compared to a conventional GCA technique.Thirty-four consecutive patients were randomly assigned to the GCA (18 patients) or EPA (16 patients) CS cannulation strategy.Time to successful catheterization of CS was 5.0 +/- 2.4 min in the EPA group versus 10.1 +/- 5.4 min in the GCA group p = 0.004. Fluoroscopy time was 4.6 +/- 2.3 min in the EPA group versus 9.2 +/- 4.9 min in the GCA group p = 0.004. Total contrast dye volume to search and engage the CS ostium was 0.0 ml in the EPA group versus 14.3 +/- 3.4 ml in the GCA group p0.001.Cannulation of CS with the adjunct of an electrophysiology catheter to dedicated delivery systems significantly reduces procedural time, fluoroscopy time and contrast dye volume compared to a conventional strategy.

The term "heart rate turbulence" (HRT) indicates the physiologic changes in the sinus cycle that follow a ventricular premature complex; impaired HRT denotes abnormalities in cardiac autonomic function. To investigate whether HRT is impaired in patients with stable coronary artery disease (CAD), we studied 29 patients with documented CAD and frequent (/=30/hour) ventricular premature complexes on Holter monitoring and 31 patients with frequent ventricular arrhythmias but normal hearts (NH-VA). HRT and heart rate variability analyses were analyzed on 24-hour Holter recordings. HRT variables differed significantly between the 2 groups (turbulence onset -0.20 +/- 1.7% vs -0.67 +/- 2.2%, p = 0.00001; turbulence slope 2.83 +/- 1.9 vs 10.83 +/- 7.4 ms/RR, p = 0.0001 in patients with CAD and NH-VA, respectively). The difference was independent of a history of previous myocardial infarction, left ventricular function, and age. Top quartile turbulence onset values (-0.26%) and bottom quartile turbulence slope values (2.12 ms/RR) had similar predictive power in discriminating between patients with CAD and NH-VA (positive predictive value 86.7%, negative predictive value 64.4% for both). Among heart rate variables, bottom quartile SD of all RR intervals values (96.3 ms) only had the same power of HRT variables in discriminating between patients with CAD and NH-VA. Thus, our data show that HRT variables are impaired in patients with CAD patients versus those with NH-VA, indicating abnormalities in the control of short-term cardiac autonomic mechanisms resulting in decreased vagal activity with likely predominant sympathetic activity.

The treatment of patients with angina pectoris refractory to medical therapy and unsuitable for revascularization procedures has yet not been well standardized. Previous retrospective studies and small prospective studies have suggested beneficial effects of spinal cord stimulation (SCS) in these patients. We created a Prospective Italian Registry of SCS to evaluate the short- and long-term clinical outcome of patients who underwent SCS device implantation because of severe refractory angina pectoris. Overall, 104 patients were enrolled in the registry (70 men, aged 68 ± 17 years), most of whom (83%) had severe coronary artery disease. Average follow-up was 13.2 ± 8 months. Overall, 17 patients (16%) died, 8 (8%) due to cardiac death. Among clinical variables, only age was found to be significantly associated both with total mortality (p = 0.04) and cardiac mortality (p = 0.02) on Cox regression analysis. A significant improvement of anginal symptoms (≥50% reduction of weekly anginal episodes, compared with baseline) occurred in 73% of patients, and Canadian Cardiovacular Society angina class improved by ≥1 class in 80% and by ≥2 classes in 42% of patients, with a relevant reduction in the rate of hospital admission and days spent in the hospital because of angina (p

Should every Patient with Heart Failure be Investigated for Sleep Apnea Syndrome? The increasing interest in the field of sleep medicine during the whole twentieth century is principally due to the involvement of sleep-related disordered breathing (SDB) in cardiovascular disease. Disorders of a physiological phenomenon such as sleep lead to important changes in state of quiescence of the cardiovascular, respiratory and metabolic systems during the night. Consequences of SDB (microawakening, sleep fragmentation, hypoxemia) represent important harmful triggers on the cardiovascular system, above all in patients suffering by inability of the heart to provide an adequate output such as for heart failure (HF) patients. SDB and HF may be related to each other in a bidirectional way from epidemiologic and physiopathologic point of view.

Objectives: The aims of this study were to compare the efficacy of a 2- versus a 3 RF lesions ablation procedure in the RA in patients with paroxysmal AF, and to map right atrial activation during AF. Background: RF catheter-mediated ablation lines mimicking the Maze operation have been proposed as a novel curative approach for AF. The relationship between this type of ablation and right atrial mapping has not been extensively studied. Methods: Twenty-four patients with recurrent, drug-refractory, paroxysmal AF underwent an extensive mapping of the RA before attempting linear lesion RF ablation. Patients were divided into two groups: 15 patients received two linear lesions (Group 1), 9 patients three linear lesions (Group 2). Results: One-month success rate AF did not recur in 40% of Group 1 patients (6/15) and in 66% (6/9) of Group 2 patients. 27% of patients in Group 1 and 11% of patients in Group 2 had recurrences controlled by drugs. No benefit in 33% of Group 1 patients and 22% of Group 2. Long-term follow-up In the absence of any drug treatment, no AF recurrences were observed in 26% of Group 1 patients (FU: 23 to 47 months) and 55% of Group 2 (FU: 14 to 23 months). No complications were reported. Conclusions: Right atrial linear ablation is safe and may be proposed for AF treatment in selected patients.

Background Previous studies reported an impairment of nervous autonomic activity in patients with Duchenne muscular dystrophy (DMD). However, the relationship of the autonomic dysfunction to the impairment of cardiac mechanical function and of respiratory failure is not completely understood. Methods We evaluated cardiac autonomic function by time- and frequency-domain heart rate variability (HRV) analysis on 24-hour Holter recordings in 60 patients with DMD (16.8 ± 4.8 years) and 28 healthy control patients (15.2 ± 4.6 years, P = not significant). The circadian rhythm of R-R interval, low frequency, high frequency, and low-frequency/highfrequency ratio was also assessed. In all patients, left ventricular ejection fraction was measured by 2D echocardiography; respiratory function was assessed by spirometry. Results All HRV parameters were lower in patients with DMD than in control subjects, with the percentage of differences between adjacent R-R intervals >50 ms (11.6% ± 8.5% vs 27.3% ± 14.1%, P =.00001) and high frequency (23.9 ± 10.3 ms vs 36.1 ± 12.2 ms, P =.0001) showing the strongest differences. A significant circadian rhythm of HRV variables was present in both groups, but it was considerably flattened in patients with DMD. There was no correlation between left ventricular ejection fraction and HRV indexes except for a weak correlation with high frequency (r = 0.30, P =.02) and with low-frequency to high-frequency ratio (r = –0.29, P

Aim of the study: The Consistent Atrial Pacing (CAP) algorithm has been designed to achieve a high percentage of atrial pacing to suppress paroxysmal atrial fibrillation. The aim of our study was to compare the impact of DDDR+CAP versus DDDR pacing on paroxysmal atrial fibrillation recurrences and triggers in patients with Brady-Tachy Syndrome. Methods: 61 patients, 23 M and 38 F, mean age 75±9 y, affected by Brady-Tachy Syndrome, implanted with a DDDR pacemaker, were randomized to DDDR or DDDR+CAP pacing with cross over of pacing modality after 1 month. Results: 78 % of patients in DDDR pacing and 73 % in DDDR+CAP pacing (p=n.s.) were free from symptomatic paroxysmal atrial fibrillation recurrences. During DDDR+CAP pacing, the atrial pacing percentage increased from 77±29 % to 96±7 % (p

A 51-year-old man was admitted for burning dysesthesias over the soles. Neurologic examination showed a pansensory loss over both feet associated with weakness of toes dorsiflexion. Motor conduction study of the sural nerve showed a significant reduction of nerve conduction velocity (21.4 m/s) suggesting a demyelinating neuropathy. In the following days he was referred to the cardiologist because of the sudden onset of a rapid atrial tachycardia, which was terminated by adenosine. Echocardiography showed a left atrial mass arising from the atrial septum consistent with the diagnosis of cardiac myxoma. The patient underwent cardiac surgery to remove the tumor, which was confirmed a myxoma by pathology. In the days following cardiac mass removal, neurological symptoms progressively disappeared in the absence of anti-inflammatory and steroid therapy and control motor conduction study showed complete normalization of nerve conduction velocity (54.5 m/s). Peripheral demyelinating neuropathy represented the first clinical presentation of cardiac myxoma in our patient and should be included among the possible paraneoplastic manifestations of this cardiac tumor.

An apparently healthy 43-year-old man was submitted to cardiological evaluation for sport activity. Two-dimensional echocardiography led to suspicion of noncompaction deformity, later confirmed by magnetic resonance imaging (MRI), left ventricular catheterization and endomyocardial biopsies. To exclude life-threatening arrhythmias, the patient was submitted to an electrophysiological study and to a real-time three-dimensional electroanatomic reconstruction of left ventricle. The electroanatomic map revealed extensive area of electrical abnormalities. Extent and localization of scar areas mostly corresponded to the areas of enhancement observed at MRI. The present report is the first on electroanatomic substrate evaluation in a noncompaction left ventricle. Our findings show that ventricular noncompaction is characterized by electrical abnormalities including low voltage and scar areas, mainly related to the presence and extent of myocardial fibrosis rather than noncompacted myocardium. Electroanatomic mapping may contribute to detect and quantify fibrotic areas in patients presenting this rare cardiomyopathy.

Objectives Scleroderma heart disease is a major risk of death in systemic sclerosis (SSc). Mechanisms underlying myocardial damage are still unclear. We performed an extensive study of SSc patients with recent-onset symptoms for heart disease and examined the efficacy of immunosuppressive therapy. Methods A cohort of 181 SSc patients was enrolled. Of these, 7 patients newly developed clinical symptoms of heart disease (heart failure, chest pain, and palpitation); all of them showed mild but persistent increase in cardiac enzymes. These patients underwent Holter ECG, 2D-echocardiography, perfusional scintigraphy, delayed-enhancement-cardiac magnetic resonance (DE-CMR), coronary angiography, and endomyocardial biopsy. Patients were treated for at least 12 months and followed-up for 5 years. Results Ventricular ectopic beats (VEBs) were found in 4 patients, wall motion abnormalities in 3, pericardial effusion in 6, and DE in CMR in 6 with T2-hyperintensity in 2. In all patients, histology showed upregulation of endothelium adhesion molecules and infiltration of activated T lymphocytes, with (acute/active myocarditis in 6) or without (chronic/borderline myocarditis in 1) myocyte necrosis. Parvovirus B19 genome was detected in 3. None showed occlusion of coronary arteries or microvessels. Compared with SSc controls, these patients more often had early disease, skeletal myositis, c-ANCA/anti-PR3 positivity, VEBs, pericardial effusion, and systolic and/or diastolic dysfunction. Immunosuppressive therapy improved symptoms and led to cardiac enzyme negativization; however, 2 patients died of sudden death during follow-up. Conclusions Myocarditis is a common finding in SSc patients with recent-onset cardiac involvement. Its early detection allowed to timely start an immunosuppressive treatment, preventing cardiac damage progression in most cases.

BACKGROUND Catheter ablation of accessory pathways using radiofrequency current has been shown to be effective in patients with Wolff-Parkinson-White syndrome, by using either the ventricular or atrial approach. However, the unipolar electrogram criteria for identifying a successful ablation at the atrial site are not well established. METHODS AND RESULTS One hundred patients with Wolff-Parkinson-White were treated by delivering radiofrequency energy at the atrial site. Attempts were considered successful when ablation (disappearance of the delta wave) occurred in < 10 seconds. In eight patients with concealed pathway, the accessory pathway location was obtained by measuring the shortest V-A interval either during ventricular pacing or spontaneous or induced reciprocating tachycardia. In 92 patients both atrioventricular valve annuli were mapped during sinus rhythm, in order to identify the accessory pathway (K) potential before starting the ablation procedure. When a stable filtered (30-250 Hz) "unipolar" electrogram was recorded, the following time intervals were measured: (1) from the onset of the atrial to the onset of the K potential (A-K); (2) from the onset of the delta wave to the onset of the K potential (delta-K); and (3) from the onset of the K potential to the onset of the ventricular deflection (K-V). During unsuccessful versus successful attempts, A-K (51 +/- 11 ms vs 28 +/- 8 ms, P < 0.0001 for left pathways [LPs]; and 44 +/- 8 ms vs 31 +/- 8 ms, P < 0.02 for right pathways [RPs]) and delta-K intervals (2 +/- 9 ms vs -18 +/- 10 ms, P < 0.0001 for LPs; and 13 +/- 7 ms vs 5 +/- 8 ms, P < 0.02 ms for RPs) were significantly longer. CONCLUSIONS Short A-K interval (< 40 ms), and a negative delta-K interval recorded from the catheter positioned in the atrium are strong predictors of successful ablation of LPs and RPs. Therefore, the identification of the K potential appears to be of paramount importance for positioning of the ablation catheter, followed by analysis of A-K and delta-K unipolar electrogram intervals. However, it appears that the mere recording of K potential is not, per se, predictive of successful outcome, but rather the A-K and delta-K interval.

We discuss a case of transvenous lead extraction (TLE) in a patient with a large vegetation. To prevent embolization, a Dormia basket was placed in the pulmonary artery trunk. After uncomplicated TLE, the basket was withdrawn, and vegetation material was retrieved from it. Our experience confirms that TLE is feasible even with large vegetations, and the pulmonary circulation may be protected with a simple intravascular device.

Background: In the past few years, there has been a relative explosion of activity in the realm of interventional cardiology. The high rate of success of radiofrequency energy ablation have transformed catheter ablation from an investigational procedure into the first-line therapy for symptomatic Wolff-Parkinson-White syndrome. Radiofrequency catheter ablation for preexcitation syndrome is commonly based on a ventricular approach. Such an approach might he associated with the risk of prolonged arterial catheter manipulation, retrograde left ventricular catheterization, and production of multiple, potentially arrhythmogenic, ventricular lesions created during ablation. Potential risks can be avoided using atrial insertion ablation procedures. The transseptal procedure that was developed in the 1950s and 1960s as a diagnostic procedure and then shelved in the 1970s and early 1980s has now come back into prominence as a therapeutic technique in the treatment of valvular heart disease, and then in the ablation of the left accessory atrioventricular connections. Methods: Atrial aspect of mitral annulus is a relatively smooth, nonobstructed surface that simplifies catheter movement, thereby permitting rapid and accurate accessory pathway location. Although primary use of earliest endocardial retrograde atrial activation as a marker of accessory pathway atrial insertion is sufficiently accurate to permit successful ablation, direct recording of an accessory pathway potential is an important predictor of successful ablation site. Moreover, the analysis of the unipolar atrial electrogram, recorded during sinus rhythm from the tip of the ablation catheter, provides further information for localizing the atrial insertion of the accessory pathways. Shortest atrial-accessory pathway and negative delta-accessory pathway intervals have been found to be the best predictors of the successful site. Results: A 90.5% success of the transseptal approach on an overall population of 328 patients, higher for overt than for concealed pathways, is comparable with the results of the retrograde. Complications are 0.5%. Conclusion: In conclusion, the transseptal approach for ablation at the atrial site is very safe and highly effective, and avoids prolonged arterial cannulation and catheter manipulation in the ascending aorta and left ventricle.

Background— Ventricular tachycardia (VT) is a significant therapeutic challenge in patients with myocarditis. This study aimed to assess the efficacy and safety of radiofrequency catheter ablation (RFCA) of VT in patients with myocarditis. Methods and Results— We enrolled 20 patients (15 men; age, 42 [28–52] years) with a history of biopsy-proven viral myocarditis and drug-refractory VT; 5 patients presented with electrical storm. The median left ventricular ejection fraction was 55% (45–60%). All patients underwent endocardial RFCA with an irrigated catheter, using contact electroanatomic mapping. Recurrence of sustained VT after endocardial RFCA was treated with additional epicardial RFCA. Endocardial RFCA was acutely successful in 14 patients (70%) while in the remaining 6 (30%) clinical VT was successfully ablated by epicardial RFCA. In 1 patient, hemodynamic instability required an intra-aortic balloon pump to complete RFCA. No major complication occurred during or after RFCA. Over a median follow-up time of 28 (11–48) months, 18 patients (90%) remained free of sustained VT; 2 patients (10%, both with baseline left ventricular ejection fraction ≤35%) died of acute heart failure unrelated to ventricular arrhythmias. Conclusions— In patients with myocarditis, RFCA of drug-refractory VT is feasible, safe, and effective. Epicardial RFCA should be considered as an important therapeutic option to increase success rate.

Transvenous Lead Extraction. Introduction: As the population ages, the number of elderly patients with implantable cardiac devices referred for transvenous lead extraction will dramatically increase in Western countries. The safety and effectiveness of lead extraction in elderly patients has not been well evaluated. We report the safety and effectiveness of transvenous lead extraction in octogenarians. Methods and Results: From January 2005 to January 2011, we reviewed data from consecutive patients ≥ 80 years referred to our institutions for transvenous lead extraction because of cardiac device infection or lead malfunction. Clinical characteristics, procedural features, and periprocedural major and minor complications were compared between octogenarians and younger patients. Out of 849 patients undergoing lead extraction in the participating institutions during the study period, 150 (18%) patients were octogenarians (mean age 84 years; range 80–96; 64% males). A significantly higher percentage of octogenarians presented with chronic renal failure (55% vs 26%; P < 0.001), history of malignancy (22% vs 6%; P < 0.001), and chronic obstructive pulmonary disease (46% vs 19%; P < 0.001). Complete lead extraction rates were similar in the 2 age groups (97% in octogenarians vs 96% in patients

Heart failure is a growing global epidemic that involves in its pathophysiology a proinflammatory state. Since the first description of elevated cytokine levels in this setting, there has been increasing interest in understanding the role of these molecules in left-ventricular remodeling and function. Over the years, intense research on the 'cytokine theory' of heart failure has allowed evaluation of the role of inflammatory biomarkers not only as pathogenetic mediators, but also as potential tools in the diagnosis and risk stratification of heart failure patients. Whereas current evidence does not support the use of inflammatory biomarkers for the diagnosis of heart failure, the assessment of their levels and the connection between their changes and changes in clinical status and prognosis has been well validated. At present, the utility of anti-inflammatory therapies in heart failure is still debated, since trials of anti-inflammatory agents in this setting have pointed out controversial results. On the contrary, established treatments of heart failure, including β-blockers, renin-angiotensin system antagonists, and aldosterone-receptor blockers seem able to act by modulating cytokine expression, suggesting a new role for these molecules in guiding heart failure therapy. Therefore, the binomial topic of heart failure and inflammation still has a number of fields not completely explored: our aim is to update current knowledge and future perspectives.

Myocarditis is defined as a disease characterized by myocardial inflammation associated with myocyte necrosis. It can be caused by infections, autoimmune response primarily affecting the myocardium or by systemic autoimmune or inflammatory disorders (Aretz et al., 1986). Viral infections are the most frequent cause and account for the vast majority of cases in North America and Europe (Cooper, 2009). Cardiac symptoms that develop during myocarditis may follow after a delay of days to weeks from the beginning of the pathological process; they are quite unspecific and include fatigue, dyspnoea, palpitations, malaise and atypical chest discomfort. Even the clinical cardiac signs may be vague in many patients and generally include cardiac murmurs, gallop rhythms and other signs of heart failure and sometimes pericardial rubs when the pericardium is also involved in the inflammatory process. Myocarditis is often associated with various types of ECG abnormalities (including bundle branch blocks, Q waves resembling those related to myocardial infarction, repolarization abnormalities and QRS prolongation) and rhythm disturbance such as atrio-ventricular blocks, supraventricular tachycardias and ventricular ectopies and tachycardias. Echocardiography may reveal overt systolic dysfunction or a reduction of peak systolic velocities at TDI; moreover, regional wall motion abnormalities and diastolic dysfunction may be found (Cooper, 2009; Feldman et al. 2000). Therefore, this disease should always be considered in patients who present with rapidly progressive cardiomyopathy, chest pain with ECG anomalies that mimic an acute coronary syndrome but with normal coronary arteries or idiopathic ventricular arrhythmias. Furthermore, in young people myocarditis may be frequently responsible for sudden cardiac death, particularly after strenuous physical exertion (Doolan et al., 2004; Corrado D et al. 2001). With this regard it should be highlighted that the recognition of myocarditis in patients presenting with aborted sudden death or major ventricular arrhythmias is actually challenging in everyday clinical practice, as the diagnosis may be difficult and may require the use of invasive procedures. Nevertheless, the detection of myocarditis in patients presenting with ventricular arrhythmias may have a pivotal importance, because the

In this paper we describe the management of ICD (implantable cardioverter defibrillator) carriers who undergo extraction of infected leads and who, in view of the need for sameside reimplantation, are studied with 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography with computed tomography (PET-CT) scan, or with leukocyte technetium-99m hexamethylpropylene amine oxime (99mTc-HMPAO) scan before reimplantation, in order to rule out a residual infection at the site of original implantation.

A 50-year-old recreational futsal player was referred to our sports cardiology outpatient clinic for palpitations and detection of frequent (>5000) ventricular premature beats with bigeminy and runs of nonsustained ventricular tachycardia at 24-hour Holter monitoring. Rest ECG showed normal QRS morphology with negative T waves in precordial lateral (V4 to V6) and inferior leads (Figure 1A). Less-prominent negative T waves in lateral but not inferior leads also were present in previous ECGs obtained during routine sports preparticipation evaluation at age 26 years (Figure 1B) but in the absence of symptoms and arrhythmias at Holter monitoring; no other diagnostic test was performed at that time. Figure 1. Rest ECGs. A , Rest ECG obtained in 1984 showing negative T waves in lateral precordial leads (V4 to V6). B , Rest ECG obtained in 2010 showing more-prominent negative T waves in lateral precordial leads. A stress ECG failed to reveal ST-segment changes diagnostic for myocardial ischemia, whereas frequent polymorphic ventricular premature beats with right bundle branch block morphology and a short run of nonsustained ventricular tachycardia were observed during the recovery phase. Two-dimensional echocardiography showed a mild reduction of ejection fraction with a diffuse apical a-dyskinesia of the left ventricle (LV), whereas the right ventricle (RV) presented normal dimensions and global function but hypokinesia of the apex and …

Background The diagnosis of subtle structural heart disease in competitive athletes with ventricular arrhythmias (VAs) and an apparently normal heart is challenging. Three-dimensional electroanatomic mapping (EAM) has been demonstrated to reliably identify low-voltage areas that correspond to different cardiomyopathic substrates. Objective The purpose of this study was to test whether EAM may help in the diagnosis of concealed cardiomyopathies in athletes with VAs and an apparently normal heart. Methods We studied 13 consecutive competitive athletes (12 males, age 30 ± 13 years) who had documentation of VAs within the previous 6 months on 12-lead electrocardiogram (ECG), 24-hour Holter ECG, or ECG exercise testing and who were judged as having a structurally normal heart after a thorough noninvasive evaluation, including signal-averaged ECG, transthoracic echocardiogram, and cardiac magnetic resonance imaging. Depending on the presumed site of VA origin according to 12-lead ECG criteria, patients underwent right or left ventricular EAM and EAM-guided endomyocardial biopsy. Results Presenting arrhythmias included sustained ventricular tachycardia (n = 3), multiple episodes of nonsustained ventricular tachycardia (n = 7), and frequent ventricular ectopic beats (>1,000 during 24 hours; n=3). Three patients had a history of syncope. Twelve (92%) patients had at least one low-voltage region at EAM, which corresponded at EAM-guided endomyocardial biopsy to the histological diagnosis of active myocarditis in seven patients and of arrhythmogenic right ventricular cardiomyopathy in five. In one patient the histological evidence of contraction band necrosis allowed the unmasking of caffeine and ephedrine abuse. Conclusions Electroanatomical substrate mapping may help diagnose concealed myocardial diseases in competitive athletes presenting with recent-onset VAs and an apparently normal heart. Further studies are warranted to assess the prognostic implications of such subtle myocardial abnormalities.

[Figure][1] [![Graphic][3] ][3][![Graphic][4] ][4] In August 2004, a 59-year-old woman received the diagnosis of idiopathic dilated cardiomyopathy on the basis of normal coronary angiography and magnetic resonance evidence of a hypokinetic, dilated, nonhypertrophic left

Background— The diagnostic reliability and pathophysiologic relevance of different noninvasive diagnostic criteria for arrhythmogenic right ventricular cardiomyopathy (ARVC) are undefined. We tested the association between noninvasive diagnostic criteria for ARVC and the presence of low-voltage areas (LVAs) detected at electroanatomic voltage mapping (EAM). Methods and Results— Noninvasive diagnostic criteria, including ECG, signal-averaged ECG (SAECG), and cardiac magnetic resonance (CMR) criteria, were compared with the presence and location of LVAs detected at right ventricular (RV) EAM in 17 patients (9 men) aged 50±16 years with biopsy specimen-proven ARVC. LVAs were found in 15 (88%) patients. Patients with surface ECG abnormalities showed a higher degree of RV involvement than those without ECG abnormalities (number of LVAs, 1.8±0.5 versus 0.9±0.6, respectively; P P =0.03) but not between SAECG parameters and LVAs in other RV regions. Among CMR findings, RV delayed enhancement was more significantly associated with the distribution of LVAs (free wall, P P P =0.02). Regional RV dysfunction also showed a good correlation with LVAs, with the most significant association being found with the free wall ( P =0.01), whereas RV fat infiltration at CMR was not correlated with LVAs. Conclusion— In patients with ARVC, SAECG abnormalities correlate with the presence of LVAs selectively in the RV outflow tract, whereas surface ECG abnormalities are associated with a more diffuse RV involvement. Myocardial delayed enhancement is the CMR finding more strongly associated with LVAs, thus supporting the appropriateness of its inclusion among diagnostic criteria for ARVC.

In this report we describe the case of a 56-year-old man with a medical history of onset of asthenia, palpitations and dyspnoea for mild efforts. After a negative ergometric test for myocardial ischaemia and 24-h Holter monitoring showing frequent ventricular premature beats, but an echocardiogram with significant dilation and dysfunction of the left ventricle, coronary angiography was performed and did not show haemodynamically significant stenosis. Regarding the anamnesis of snoring, daytime sleepiness and the relationship between sleep-related breathing disorder and cardiovascular disease, we performed a cardiorespiratory sleep study that indicated a diagnosis of sleep-related breathing disorder with prevalent obstructive apnoea. In addition to optimal medical therapy for cardiovascular disease, the patient began therapy with continuous positive airway pressure during the night. This showed a considerable decrease in ventricular premature beats (VPBs) during the night, a better control of ventricular ectopy during the day and a better compliance with medical therapy.

Objective Myotonic dystrophy type 1 (DM1) is the most frequent muscular dystrophy in adults. DM1 is a multisystem disorder also affecting the heart with an increased incidence of sudden death, which has been explained with the common impairment of the conduction system often requiring pacemaker implantation; however, the occurrence of sudden death despite pacemaker implantation and the observation of major ventricular arrhythmias generated the hypothesis that ventricular arrhythmias may play a causal role as well. The aim of the study was to assess the 2-year cumulative incidence and the value of noninvasive and invasive findings as predictive factors for sudden death, resuscitated cardiac arrest, ventricular fibrillation, sustained ventricular tachycardia and severe sinus dysfunction or high-degree atrioventricular block. Methods/design More than 500 DM1 patients will be evaluated at baseline with a clinical interview, 12-lead ECG, 24-h ECG and echocardiogram. Conventional and nonconventional indications to electrophysiological study, pacemaker, implantable cardioverter defibrillator or loop recorder implantation have been developed. In the case of an indication to electrophysiological study, pacemaker, implantable cardioverter defibrillator or loop recorder implant at baseline or at follow-up, the patient will be referred for the procedure. At the end of 2-year follow-up, all candidate prognostic factors will be tested for their association with the endpoints. Trial registration ClinicalTrials.gov ID NCT00127582. Conclusion The available evidence supports the hypothesis that both bradyarrhythmias and tachyarrhythmias may cause sudden death in DM1, but the course of cardiac disease in DM1 is still unclear. We expect that this large, prospective, multicenter study will provide evidence to improve diagnostic and therapeutic strategies in DM1.