Search

Background: Non-sustained ventricular tachycardia (NSVT) can occur asymptomatically and can be incidentally detected in the internal records of pacemakers (PM). The clinical value of NSVT in the population of PM patients is still uncertain.Our aim was to assess the prevalence of NSVT detected by remote PM control, to describe the clinical and demographic characteristics of patients with NSVT, and to assess the prognostic significance of NSVT in terms of both overall and cardiovascular mortality. Methods: Consecutive patients followed with PM remote interrogations from September 2010 to December 2015 were included. The transmissions pertaining to the first 12 months of remote control were analysed and the patients were divided by those presenting NSVT and those without NSVT. The two groups were compared in terms of total mortality and cardiovascular mortality based on the administrative data provided by the regional administration of the Italian National Health System. Results: The prevalence of NSVT in 408 patients (62% males, mean age 75.6; SD 10.6 years old) was 21% in a year. During a mean follow-up duration of 44 months, NSVT did not emerge as independently associated with overall mortality, but was associated with cardiovascular mortality in a competing risk regression model with older age, male gender, diabetes, chronic renal insufficiency, ischemic cardiomyopathy and chronic obstructive pulmonary disease. Conclusions: We show that NSVT episodes recorded by remote control in a PM population are independently associated with cardiovascular mortality with possible implications for risk stratification and therapeutic options. Keywords: Pacemaker, Remote interrogation, Non-sustained ventricular tachycardia, Cardiovascular mortality

Electrical storm (ES) is defined as three or more episodes of ventricular fibrillation (VF) or ventricular tachycardia (VT) within 24 h and is associated with an increased cardiac and all-cause mortality. ES is a full arrhythmic emergency, its prevalence steadily increasing along with the number of implantable cardioverter-defibrillator implanted every year in developed countries. Nowadays, little evidence exists regarding clinical predictors of ES and their potential association on mortality and heart failure (HF), nor optimal pharmacological and non-pharmacological treatment has ever been codified. The intErnationaL eLeCTRicAl storm registry (ELECTRA) is a multicentre, observational, prospective clinical study with two major aims. First, to create an international database on ES encompassing clinical features, pharmacological management, and interventional treatment strategies. Second, to describe mortality and rehospitalization rates in patients with ES over a long follow-up. The primary endpoint is all-cause mortality 3 years after the ES index event. The main secondary endpoint is hospitalization for all causes 3 years after the ES index event. Other secondary endpoints includes ES recurrences, unclustered VTs/VFs recurrences, and hospitalizations for HF worsening. A minimum of 500 patients will be included in the registry, and all patients will be followed-up for a minimum of three years. The present paper describes the background and current rationale of the ELECTRA study and details the study design, from enrolment strategy to data collection methods to planned data analysis. A brief overview of the expected results and their potential clinical and research implications will also be presented (NCT02882139). Keywords: Arrhythmia, Catheter ablation, Electrical storm, Implantable cardioverter-defibrillator, Ventricular fibrillation, Ventricular tachycardia

Spontaneous coronary artery dissection (SCAD) is increasingly recognized as an important cause of myocardial infarction (MI). Currently there is little knowledge about prognostic factors for unfavorable outcome at long term follow-up; furthermore, there is also little knowledge about the genetics of these patients., Aims: This observational and retrospective study describes long-term cardiovascular outcomes of a population affected by SCAD and assesses predictors of recurrent de novo SCAD and major adverse cardiovascular events (MACE). Furthermore, a correlation between genotype and adverse events at follow-up was sought., Methods: Baseline characteristics, angiographic features, use of medication and long-term cardiovascular events were systematically ascertained between 2000 and 2019. Next generation sequencing was performed with a panel consisting of twenty genes of interest. Variants found were filtered based on their frequency and only frequencies <1% in the general population were considered as "positive"., Results: Seventy patients were enrolled and followed for a median time of 39.1 months. Median age was 52 years and the majority were women (86%). Use of hormone therapy (HT) (OR 3.64, p = 0.041) and presence of malignant ventricular arrhythmias (VAs) at onset (OR 7.03, p = 0.0073) were associated with a greater risk of recurrent de novo SCAD. Proximal type SCAD (OR 8.47, p < 0.0001) and presence of VAs at onset (OR 9.97, p = 0.047) were associated with a greater risk of MACE. A potential SCAD-associated mutation was detected in 27 patients (44%); 6 patients (22%) defined as genetically "positive" developed MACE vs. 2 patients (6%) defined as "negative" (p = 0.06 at univariate analysis). MACE at follow-up is reached earlier in genetically positive patients (7.9 vs. 42.5 months)., Conclusion: use of HT and VAs at SCAD onset are prognostic factors for recurrent de novo SCAD. Proximal SCAD site and VAs at SCAD onset were prognostic factors for MACE. Analysis by molecular genetics seems to be a promising tool for the possible additional role it could play in MACE prediction., Competing Interests: Declaration of Competing Interest None declared., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Danon disease is characterized by hypertrophic cardiomyopathy, skeletal myopathy, and intellectual disability due to deficiency of the lysosome-associated membrane protein-2 (LAMP-2). Although heart transplantation is considered an option for end stage Danon cardiomyopathy, scarce information is available about long term follow up. We report on long term follow up (14.7 years, IQ range 9-21 years) of 4 patients, transplanted for Danon disease cardiomyopathy, showing two LAMP-2 gene variants, the novel c.815T > C and the previously reported c.294G > A. We have also analysed previous published paper on this topic comparing available data from different follow up. Being a skeletal and cardiac muscle disease, with systemic effects, long term results about HTx are indispensable to justify any treatments in this subset of patients., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Background: Although octogenarians constitute a fast-growing portion of cardiovascular patients, few data are available on the outcome of patients aged ≥85 years with ST-Elevation Myocardial Infarction (STEMI)., Methods and Results: We analyzed 126 consecutive patients aged ≥85 years (age 88±2 years) with STEMI, undergoing primary percutaneous coronary intervention (pPCI) within 12 hours from symptoms onset. Long-term follow-up (median 898 days) was obtained for the 102 patients surviving the index-hospitalization. In-hospital mortality rate was 19%. Nonagenarians, diabetes mellitus, severe left ventricular systolic dysfunction and intra-aortic balloon pumping were significantly and independently correlated to in-hospital mortality at the multivariate analysis. A low rate of complications was detected. Among patients surviving the index hospitalization, 32 (31%) patients died during follow-up. 55 patients (54%) had re-hospitalization due to cardiovascular causes. The univariate analysis identified chronic renal failure, Killip class ≥ 3, TIMI Risk Score >8 and very high risk of bleeding as predictors of long-term overall mortality. At the multivariate analysis only chronic renal failure and very high risk of bleeding were significantly and independently correlated to long-term all-cause mortality. Renal function and anterior myocardial infarction were significantly and independently associated with the combined end-point of cardiac mortality and re-hospitalization due to cardiovascular disease at the multivariate analysis., Conclusions: PPCI in patients ≥85 years old is relatively safe. In this population, pPCI is associated with a good long-term survival, although still worse than in younger patients, despite a considerable incidence of re-hospitalization due to cardiovascular events., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Background: Recent studies have suggested that para-Hisian atrial tachycardia (AT) can be successfully ablated from the right atrium (RA), left atrium (LA), or noncoronary cusp (NCC) in the aorta; however, the best approach remains unclear., Objective: This study aimed to compare different approaches to radiofrequency catheter ablation (RFCA) of para-Hisian AT., Methods: We retrospectively analyzed 68 consecutive patients (49[72%] women; mean age 61 ± 13 years) treated with RFCA for this type of AT. Mapping of the RA (n = 68), LA (n = 21), and NCC (n = 52) was performed during AT to identify the earliest activation site and to apply RFCA., Results: RFCA successfully terminated AT in the RA in 13 of 28 patients (46.4%), in the LA in 4 of 16 patients (25.0%), and in the NCC in 46 of 52 patients (88.5%) after 1 procedure (P < .05). Atrioventricular block occurred only during RFCA in the RA in 4 of 28 patients (14.3%). After a mean follow-up of 33.5 ± 25.4 months, AT recurrence was observed in 5 of 13 patients (38.5%) ablated in the RA, 1 of 4 (25.0%) ablated in the LA, and 2 of 46 (4.4%) ablated in the NCC (P < .05). RFCA in the LA was effective only if the local activation time (LAT) was earlier than that in the RA. RFCA in the NCC was successful regardless of the LAT (P < .05)., Conclusion: Mapping and ablation in the NCC should be always considered in cases of AT originating from the His bundle region, regardless of the LAT., (Copyright © 2017 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Cardiovascular involvement is common in acromegaly and can lead to development of acromegalic cardiomyopathy, characterized by concentric biventricular hypertrophy with a progressive impairment of diastolic and systolic function. The onset of heart failure and arrhythmias are related to poor prognosis. We report on a case of a 48-year-old man with acromegalic cardiomyopathy caused by pituitary adenoma. Despite the successful trans-sphenoidal resection of the tumour, the patient was rehospitalized for ventricular arrhythmic storms that led to cardiogenic shock, which required mechanical hemodynamic support with intra-aortic balloon pump, venoarterial extracorporeal membrane oxygenation, and urgent heart transplantation., (Copyright © 2017 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Background: Device follow-up is mandatory in the care of patients with a pacemaker. However, in most cases, device checks appear to be mere technical, time-consuming procedures. The aim of this research is to evaluate whether remote follow-up can replace in-clinic device checks by assessing clinical outcomes for pacemaker patients followed only via remote follow-up., Methods and Results: Consecutive pacemaker patients followed with remote monitoring were prospectively included by 6 Italian cardiology centers in an observational investigation. The workflow for remote monitoring included an initial assessment by nursing staff and, when necessary, by a responsible physician for medical decisions. No in-person visits were scheduled after the start of remote monitoring. One-thousand and two-hundred and fifty one patients (30% female, 75±11years old) were followed for a median observation period of 15months. Out of 4965 remote transmissions, 1882 (38%) had at least one clinically relevant event to be investigated further, but, only after 137 transmissions (2.8%), the patients were contacted for an in-clinic visit or hospitalization. Sixty-nine patients died and 124 were hospitalized for various reasons. Atrial fibrillation episodes were the most common clinical events discovered by remote transmissions, occurring in 1339 (26%) transmissions and 471 (38%) patients., Conclusions: Our experience shows that remote monitoring in a pacemaker population can safely replace in-clinic follow-up, avoiding unnecessary in-hospital device follow-up., (Copyright © 2016. Published by Elsevier Ireland Ltd.)

Background: Cardiac resynchronization therapy defibrillator can terminate ventricular tachycardia (VT) and fast VT (FVT) via antitachycardia pacing (ATP)., Objectives: We evaluated efficacy and safety of ATP, whether ATP induces ventricular arrhythmias after inappropriate ATP or atrial fibrillation (AF) after appropriate ATP, and whether ATP is associated with mortality., Methods: A total of 1404 patients with a cardiac resynchronization therapy defibrillator were followed in a prospective multicenter observational research. All-cause mortality rates were estimated in patient subgroups in order to uncouple the trigger (VT/FVT or other rhythms causing inappropriate detections) from ATP therapy., Results: Over a median follow-up of 31 months, 2938 VT/FVT episodes were treated with ATP in 360 patients. The adjusted ATP success rate was 63% (95% confidence interval [CI] 57%-69%) on FVTs and 68% (95% CI 62%-74%) on VTs. Acceleration occurred in 55 (1.87%) and syncope in 4 (0.14%) of all ATP-treated episodes. In 14 true VT/FVT episodes in 5 patients, AF followed ATP therapy. In 4 episodes in 2 patients, VT followed ATP inappropriately applied during AF. Death rate per 100 patient-years was 5.6 (95% CI 4.3-7.5) in patients with appropriate ATP and 1.5 (95% CI 0.4-6.1) in patients with inappropriate ATP (P = .045)., Conclusion: ATP was effective in terminating VT/FVT episodes and displayed a good safety profile. ATP therapies by themselves did not increase death risk; prognosis was indeed better in patients without arrhythmic episodes, even if they received inappropriate ATP, than in patients with ATP on VT/FVT episodes. Adverse outcomes observed in patients receiving implantable cardioverter-defibrillator therapies are probably related to the arrhythmia itself, a marker of disease progression, rather than to adverse effects of ATP., (Copyright © 2016 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Objectives: The purpose of this study is to determine whether, in patients with atrial fibrillation (AF) undergoing cardiac resynchronization therapy (CRT), atrioventricular junction ablation (AVJA) is associated with a better outcome than treatment with rate-slowing drugs., Background: Different trials have demonstrated that CRT is effective in treating heart failure (HF) patients who are in sinus rhythm (SR). No trials have addressed whether CRT confers similar benefits on AF patients, with or without AVJA., Methods: The clinical outcomes of CRT for patients with permanent AF undergoing CRT combined with either AVJA (n = 443) or rate-slowing drugs (n = 895) were compared with those of SR patients (n = 6,046)., Results: Median follow-up was 37 months. Total mortality (6.8 vs. 6.1 per 100 person-years) and cardiac mortality (4.2 vs. 4.0) were similar for patients with AF+AVJA and patients in SR (both p = NS). In contrast, the AF+drugs group had a higher total and cardiac mortality than the SR group and the AF+AVJA group (11.3 and 8.1, respectively; p < 0.001). On multivariable analysis, AF+AVJA had total mortality (hazard ratio [HR]: 0.93, 95% confidence interval [CI]: 0.74 to 1.67) and cardiac mortality (HR: 0.88, 95% CI: 0.66 to 1.17) similar to that of the SR group, independent of known confounders. The AF+drugs group, however, had a higher total mortality (HR: 1.52, 95% CI: 1.26 to 1.82) and cardiac mortality (HR: 1.57, 95% CI: 1.27 to 1.94) than both the SR group and the AF+AVJA group (both p < 0.001)., Conclusions: Long-term survival after CRT among patients with AF+AVJA is similar to that observed among patients in SR. Mortality is higher for AF patients treated with rate-slowing drugs., (Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Transcatheter aortic valve implantation (TAVI) has become a feasible therapeutic option for the management of high-risk patients with severe degenerative aortic stenosis. Recently it has been extended to high-risk patients with severe aortic regurgitation. Degenerative aortic valve disease is generally uncommon in heart transplant recipients. We report the case of a 75-year-old man in whom severe degenerative aortic regurgitation developed 14 years after heart transplantation (HTx). Because of multiple comorbidities and high surgical risk, TAVI was preferred. A 29-mm CoreValve prosthesis (Medtronic Inc, Minneapolis, MN) was successfully implanted using a transfemoral approach., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Aims: Several trials demonstrated the life saving role of implantable cardioverter-defibrillators (ICD) in primary prevention of sudden cardiac death (SCD). The aim was to evaluate the clinical characteristics and 4-year outcome of consecutive patients treated in clinical practice by prophylactic ICD implantation on the basis of class I recommendations and up-to-date ICD programming., Methods and Results: IRIDE multi-center, prospective and observational study enrolled 604 consecutive patients (mean age: 66 ± 10 years) treated by ICD between 01/01/2006 and 30/06/2010. Main characteristics were similarly distributed among the inclusion criteria of MADIT II (24%), SCD-HeFT (24%), COMPANION (26%) and MADIT-CRT (18%) trials, while a small number of patients met the MUSTT and MADIT (7%) inclusion criteria. Single-chamber ICDs were implanted in 168 (28%) patients, dual-chamber in 167 (28%) and biventricular in 269 (43%) patients. ATP programming was activated in 546 (90%) patients. Overall survival and rate of appropriate ICD intervention by ATP and/or shock at 12-24-36-48 months of follow-up were 94%, 89%, 80%, 75% and 16%, 28%, 37% and 50%, respectively. No difference in mortality rate between the groups who received or did not receive appropriate ICD interventions was demonstrated (p=ns)., Conclusions: The IRIDE study confirms the effectiveness in real world practice of ICD implantation in patients at risk of SCD. The life saving role of ICD therapy increases as the duration of follow-up is prolonged and the survival benefit is similar in patients who received or did not receive appropriate device treatment, thus suggesting a beneficial effect of up-to-date device programming., (Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.)

Pheochromocytoma (PCC) is a challenging and life-threatening neoplasm. Herein, the authors report an interesting and unexpected solution for a clinical case concerning a patient with a PCC, who developed delayed ectopic adrenocorticotropic hormone Cushing syndrome originating from the PCC. In addition, after a misleading I-labeled metaiodobenzylguanidine single-photon emission computed tomography/computed tomography, an F-fluorodeoxyglucose positron emission tomography/computed tomography, executed to confirm the diagnosis of PCC, showed a silent pulmonary nodule that unexpectedly turned out to be a lung nocardiasis.

Background and Methods: In a prospective protocol for noninvasive diagnosis of acute cardiac rejection, 83 routine endomyocardial biopsies, followed each time by the analysis of signal-averaged electrocardiography and by a cardiac Doppler echocardiographic study, were performed in 18 heart transplant recipients. The follow-up time was 5 +/- 3.6 months. To detect noninvasively acute cardiac rejection, we compared biopsy findings with the presence of late potentials at signal-averaged electrocardiography and with two diastolic indexes, pressure half-time, and isovolumic relaxation time obtained from Doppler echocardiographic study., Results: Thirteen acute rejection crises requiring modification of immunosuppression were diagnosed by means of endomyocardial biopsy. This clinically relevant acute cardiac rejection was associated with the presence of late potentials in 69% of cases and with the presence of pressure half-time < or = 55 msec and isovolumic relaxation time < or = 60 msec in 69% and 62% of cases, respectively. Sensitivity and specificity were as follows: for late potentials, 69% and 71%; for pressure half-time < or = 55 msec, 69% and 76%; for isovolumic relaxation time < or = 60 msec, 62% and 83%, respectively. The presence in a single patient of at least one abnormal parameter showed a sensitivity of 100% and a specificity of 60% in detecting important rejection., Conclusions: These data support the use of combined signal-averaged electrocardiography and Doppler echocardiographic study of the left ventricular diastolic function in the screening of acute cardiac rejection. Such results can suggest when endomyocardial biopsy should be performed, with the reliance that a normal noninvasive study highly excludes the presence of acute cardiac rejection requiring intensified immunosuppression.