Electrical storm in systemic sclerosis: Inside the electroanatomic substrate

We report the case of a 63-year-old woman affected by a severe form of systemic scleroderma with pulmonary involvement (interstitial fibrosis diagnosed by biopsy and moderate pulmonary hypertension) and cardiac involvement (paroxysmal atrial fibrillation, right atrial flutter treated by catheter ablation, ventricular tachyarrhythmias, previous dual chamber implantable cardioverter defibrillator implant). Because of recurrent electrical storms refractory to iv antiarrhythmic drugs the patient was referred to our institution to undergo catheter ablation. During electrophysiological procedure a 3D shell of cardiac anatomy was created with intracardiac echocardiography pointing out a significant right ventricular dilatation with a complex aneurysmal lesion characterized by thin walls and irregular multiple trabeculae. A substrate-guided strategy of catheter ablation was accomplished leading to a complete electrical isolation of the aneurism and to the abolishment of all abnormal electrical activities. The use of advanced strategies of imaging together with electroanatomical mapping added important information to the complex arrhythmogenic substrate and improved efficacy and safety

Difficult case of a trans-septal puncture: Use of a "safesept" guidewire

A 69-year-old man was admitted to our center to undergo catheter ablation of paroxysmal atrial fibrillation refractory to antiarrhythmic drug therapy. This procedure required access to the left atrium through the interatrial septum. During hospitalization, the patient performed routinely pre-procedure transthoracic echocardiography and gadolinium-enhanced cardiac magnetic resonance showing a normal anatomy of both the fossa ovalis and the interatrial septum. Access to the left atrium proved difficult and several unsuccessful attempts to perform the trans-septal puncture were made under both fluoroscopy and intracardiac echocardiography guidance, even with radiofrequency energy delivery. Finally, trans-septal puncture was successfully carried out using a novel nitinol J-shaped "SafeSept" trans-septal guidewire, designed to cross the interatrial septum through the trans-septal needle thanks to a special sharp tip. Moreover, thanks to its rounded J shape that reduces the risk of atrial perforation, the "SafeSept" guidewire, when advanced into the left atrium, becomes atraumatic

Application of ripple mapping to visualize slow conduction channels within the infarct-related left ventricular scar

Background - Ripple mapping (RM) displays each electrogram at its 3-dimensional coordinate as a bar changing in length according to its voltage-time relationship with a fiduciary reference. We applied RM to left ventricular ischemic scar for evidence of slow-conducting channels that may act as ventricular tachycardia (VT) substrate. Methods and Results - CARTO-3(Biosense Webster Inc, Diamond Bar, CA) maps in patient undergoing VT ablation were analyzed on an offline MatLab RM system. Scar was assessed for sequential movement of ripple bars, during sinus rhythm or pacing, which were distinct from surrounding tissue and termed RM conduction channels (RMCC). Conduction velocity was measured within RMCCs and compared with the healthy myocardium (>1.5 mV). In 21 maps, 77 RMCCs were identified. Conduction velocity in RMCCs was slower when compared with normal left ventricular myocardium (median, 54 [interquartile range, 40-86] versus 150 [interquartile range, 120-160] cm/s; P<0.001). All 7 sites meeting conventional criteria for diastolic pathways coincided with an RMCC. Seven patients had ablation colocating to all identified RMCCs with no VT recurrence during follow-up (median, 480 [interquartile range, 438-841] days). Fourteen patients had \ue2\u89\ua51 RMCC with no ablation lesions. Five had recurrence during follow-up (median, 466 [interquartile range, 395-694] days). One of the 2 patients with no RMCC locations ablated had VT recurrence at 605 days post procedure. RMCCs were sensitive (100%; negative predictive value, 100%) for VT recurrence but the specificity (43%; positive predictive value, 35.7%) may be limited by blind alleys channels. Conclusions - RM identifies slow conduction channels within ischemic scar and needs further prospective investigation to understand the role of RMCCs in determining the VT substrate

Electrical storm: A clinical and electrophysiological overview

Electrical storm (ES) is a clinical condition characterized by three or more ventricular arrhythmia episodes leading to appropriate implantable cardioverterdefibrillator (ICD) therapies in a 24 h period. Mostly, arrhythmias responsible of ES are multiple morphologies of monomorphic ventricular tachycardia (VT), but polymorphic VT and ventricular fibrillation can also result in ES. Clinical presentation is very dramatic in most cases, strictly related to the cardiac disease that may worsen electrical and hemodynamic decompensation. Therefore ES management is challenging in the majority of cases and a high mortality is the rule both in the acute and in the long-term phases. Different underlying cardiomyopathies provide significant clues into the mechanism of ES, which can arise in the setting of structural arrhythmogenic cardiomyopathies or rarely in patients with inherited arrhythmic syndrome, impacting on pharmacological treatment, on ICD programming, and on the opportunity to apply strategies of catheter ablation. This latter has become a pivotal form of treatment due to its high efficacy in modifying the arrhythmogenic substrate and in achieving rhythm stability, aiming at reducing recurrences of ventricular arrhythmia and at improving overall survival. In this review, the most relevant epidemiological and clinical aspects of ES, with regard to the acute and long-term follow-up implications, were evaluated, focusing on these novel therapeutic strategies of treatment

New Imaging Technologies To Characterize Arrhythmic Substrate

The cornerstone of the new imaging technologies to treat complex arrhythmias is the electroanatomic (EAM) mapping. It is based on tissue characterization and in particular on determination of low potential region and dense scar definition. Recently, the identification of fractionated isolated late potentials increased the specificity of the information derived from EAM. In addition, non-invasive tools and their integration with EAM, such as cardiac magnetic resonance imaging and computed tomography scanning, have been shown to be helpful to characterize the arrhythmic substrate and to guide the mapping and the ablation. Finally, intracardiac echocardiography, known to be useful for several practical uses in the setting of electrophysiological procedures, it has been also demonstrated to provide important informations about the anatomical substrate and may have potential to identify areas of scarred myocardium

Managing patients with ICD shocks and programming tachycardia therapies during acute heart failure syndromes

We review the pharmacologic, interventional and device programming treatment options for patients with implantable cardioverter-defibrillators who present with acute heart failure and implantable cardioverter-defibrillator shocks

Arrhythmic risk prediction in arrhythmogenic right ventricular cardiomyopathy : external validation of the arrhythmogenic right ventricular cardiomyopathy risk calculator

Aims: Arrhythmogenic right ventricular cardiomyopathy (ARVC) causes ventricular arrhythmias (VAs) and sudden cardiac death (SCD). In 2019, a risk prediction model that estimates the 5-year risk of incident VAs in ARVC was developed (ARVCrisk.com). This study aimed to externally validate this prediction model in a large international multicentre cohort and to compare its performance with the risk factor approach recommended for implantable cardioverter-defibrillator (ICD) use by published guidelines and expert consensus. Methods and results: In a retrospective cohort of 429 individuals from 29 centres in North America and Europe, 103 (24%) experienced sustained VA during a median follow-up of 5.02 (2.05-7.90) years following diagnosis of ARVC. External validation yielded good discrimination [C-index of 0.70 (95% confidence interval-CI 0.65-0.75)] and calibration slope of 1.01 (95% CI 0.99-1.03). Compared with the three published consensus-based decision algorithms for ICD use in ARVC (Heart Rhythm Society consensus on arrhythmogenic cardiomyopathy, International Task Force consensus statement on the treatment of ARVC, and American Heart Association guidelines for VA and SCD), the risk calculator performed better with a superior net clinical benefit below risk threshold of 35%. Conclusion: Using a large independent cohort of patients, this study shows that the ARVC risk model provides good prognostic information and outperforms other published decision algorithms for ICD use. These findings support the use of the model to facilitate shared decision making regarding ICD implantation in the primary prevention of SCD in ARVC

Linking cell function with perfusion : insights from the transcatheter delivery of bone marrow-derived CD133+ cells in ischemic refractory cardiomyopathy trial (RECARDIO)

Background: Cell therapy with bone marrow (BM)-derived progenitors has emerged as a promising therapeutic for refractory angina (RA) patients. In the present study, we evaluated the safety and preliminary efficacy of transcatheter delivery of autologous BM-derived advanced therapy medicinal product CD133(+) cells (ATMP-CD133) in RA patients, correlating perfusion outcome with cell function. Methods: In the phase I "Endocavitary Injection of Bone Marrow Derived CD133(+) Cells in Ischemic Refractory Cardiomyopathy" (RECARDIO) trial, a total of 10 patients with left ventricular (LV) dysfunction (ejection fraction <= 45%) and evidence of reversible ischemia, as assessed by single-photon emission computed tomography (SPECT), underwent BM aspiration and fluoroscopy-based percutaneous endomyocardial delivery of ATMP-CD133. Patients were evaluated at 6 and 12 months for safety and preliminary efficacy endpoints. ATMP-CD133 samples were used for in vitro correlations. Results: Patients were treated safely with a mean number of 6.57 +/- 3.45 x 10(6) ATMP-CD133. At 6-month follow-up, myocardial perfusion at SPECT was significantly ameliorated in terms of changes in summed stress (from 18.2 +/- 8.6 to 13.8 +/- 7.8, p = 0.05) and difference scores (from 12.0 +/- 5.3 to 6.1 +/- 4.0, p = 0.02) and number of segments with inducible ischemia (from 7.3 +/- 2.2 to 4.0 +/- 2.7, p = 0.003). Similarly, Canadian Cardiovascular Society and New York Heart Association classes significantly improved at follow-up vs baseline (p = 0.001 and p = 0.007, respectively). Changes in summed stress score changes positively correlated with ATMP-CD133 release of proangiogenic cytokines HGF and PDGF-bb (r = 0.80, p = 0.009 and r = 0.77, p = 0.01, respectively) and negatively with the proinflammatory cytokines RANTES (r = -0.79, p = 0.01) and IL-6 (r = -0.76, p = 0.02). Conclusion: Results of the RECARDIO trial suggested safety and efficacy in terms of clinical and perfusion outcomes in patients with RA and LV dysfunction. The observed link between myocardial perfusion improvements and ATMP-CD133 secretome may represent a proof of concept for further mechanistic investigations

Cardiac mesenchymal stromal cells are a source of adipocytes in arrhythmogenic cardiomyopathy

Arrhythmogenic cardiomyopathy (ACM) is a genetic disorder mainly due to mutations in desmosomal genes, characterized by progressive fibro-adipose replacement of the myocardium, arrhythmias, and sudden death. It is still unclear which cell type is responsible for fibro-adipose substitution and which molecular mechanisms lead to this structural change. Cardiac mesenchymal stromal cells (C-MSC) are the most abundant cells in the heart, with propensity to differentiate into several cell types, including adipocytes, and their role in ACM is unknown. The aim of the present study was to investigate whether C-MSC contributed to excess adipocytes in patients with ACM

Arrhythmic risk prediction in arrhythmogenic right ventricular cardiomyopathy: external validation of the arrhythmogenic right ventricular cardiomyopathy risk calculator

Aims Arrhythmogenic right ventricular cardiomyopathy (ARVC) causes ventricular arrhythmias (VAs) and sudden cardiac death (SCD). In 2019, a risk prediction model that estimates the 5-year risk of incident VAs in ARVC was developed (ARVCrisk.com). This study aimed to externally validate this prediction model in a large international multicentre cohort and to compare its performance with the risk factor approach recommended for implantable cardioverter-defibrillator (ICD) use by published guidelines and expert consensus.Methods and results In a retrospective cohort of 429 individuals from 29 centres in North America and Europe, 103 (24%) experienced sustained VA during a median follow-up of 5.02 (2.05-7.90) years following diagnosis of ARVC. External validation yielded good discrimination [C-index of 0.70 (95% confidence interval-CI 0.65-0.75)] and calibration slope of 1.01 (95% CI 0.99-1.03). Compared with the three published consensus-based decision algorithms for ICD use in ARVC (Heart Rhythm Society consensus on arrhythmogenic cardiomyopathy, International Task Force consensus statement on the treatment of ARVC, and American Heart Association guidelines for VA and SCD), the risk calculator performed better with a superior net clinical benefit below risk threshold of 35%.Conclusion Using a large independent cohort of patients, this study shows that the ARVC risk model provides good prognostic information and outperforms other published decision algorithms for ICD use. These findings support the use of the model to facilitate shared decision making regarding ICD implantation in the primary prevention of SCD in ARVC