Cardiac magnetic resonance abnormalities in patients with acute myocarditis proven by septal endomyocardial biopsy.

BACKGROUND: Previous studies suggest low diagnostic sensitivity of cardiac magnetic resonance (CMR) imaging based on Lake Louise criteria (LLC) to identify patients with complicated presentations of acute myocarditis (AM). We evaluated classic and updated LLC in patients with AM proven by right ventricular septal endomyocardial biopsy (RVS-EMB). METHODS: From an initial population of 499 patients with clinically suspected AM from a multicenter retrospective cohort, we included 74 patients with histologically proven myocarditis on RVS-EMB and available CMR within 30 days since admission. The prevalence of total and septal CMR abnormalities [namely, T2-weighted images (T2W), late gadolinium enhancement (LGE), T2 and T1 mapping, and extracellular volume (ECV)] were assessed in patients with complicated vs. uncomplicated AM. RESULTS: Among 74 patients [mean age 38 ± 15 years, 65% males, left ventricular ejection fraction (LVEF) 40 ± 18%] with RVS-EMB-proven AM, 53 (72%) had a complicated presentation. The classic LLC were positive in 56/74 patients (76%), whereas the updated ones were positive in 41/41 of cases (100%). Septal involvement, documented in 48/74 patients (65%) by conventional T2W/LGE and in 39/41 cases (95%) by mapping techniques (p < 0.001), was more common in patients with complicated AM. In the 41 patients undergoing both evaluations, CMR sensitivity for myocarditis was 85% for the classic LLC vs. 100% for the updated LLC (p = 0.006). CONCLUSION: In patients with myocarditis on RVS-EMB, CMR using updated LLC has high sensitivity in the detection of AM when performed within 30 days. Septal abnormalities are more common in patients with complicated AM.

The prevalence of left and right bundle branch block morphology ventricular tachycardia amongst patients with arrhythmogenic cardiomyopathy and sustained ventricular tachycardia: insights from the European Survey on Arrhythmogenic Cardiomyopathy.

AIMS: In arrhythmogenic cardiomyopathy (ACM), sustained ventricular tachycardia (VT) typically displays a left bundle branch block (LBBB) morphology while a right bundle branch block (RBBB) morphology is rare. The present study assesses the VT morphology in ACM patients with sustained VT and their clinical and genetic characteristics. METHODS AND RESULTS: Twenty-six centres from 11 European countries provided information on 954 ACM patients who had ≥1 episode of sustained VT spontaneously documented during patients' clinical course. Arrhythmogenic cardiomyopathy was defined according to the 2010 Task Force Criteria, and VT morphology according to the QRS pattern in V1. Overall, 882 (92.5%) patients displayed LBBB-VT alone and 72 (7.5%) RBBB-VT [alone in 42 (4.4%) or in combination with LBBB-VT in 30 (3.1%)]. Male sex prevalence was 79.3%, 88.1%, and 56.7% in the LBBB-VT, RBBB-VT, and LBBB + RBBB-VT groups, respectively (P = 0.007). First RBBB-VT occurred 5 years after the first LBBB-VT (46.5 ± 14.4 vs 41.1 ± 15.8 years, P = 0.011). An implanted cardioverter-defibrillator was more frequently implanted in the RBBB-VT (92.9%) and the LBBB + RBBB-VT groups (90%) than in the LBBB-VT group (68.1%) (P < 0.001). Mutations in PKP2 predominated in the LBBB-VT (65.2%) and the LBBB + RBBB-VT (41.7%) groups while DSP mutations predominated in the RBBB-VT group (45.5%). By multivariable analysis, female sex was associated with LBBB + RBBB-VT (P = 0.011) while DSP mutations were associated with RBBB-VT (P < 0.001). After a median follow-up of 103 (51-185) months, death occurred in 106 (11.1%) patients with no intergroup difference (P = 0.176). CONCLUSION: RBBB-VT accounts for a significant proportion of sustained VTs in ACM. Sex and type of pathogenic mutations were associated with VT type, female sex with LBBB + RBBB-VT, and DSP mutation with RBBB-VT.

Relation of Prolonged Pacemaker Dependency After Cardiac Surgery to Mortality.

Permanent pacemaker implantation (PPI) represents a rare complication after cardiac surgery, with no uniform agreement on timing and no information on follow-up. A multicenter retrospective study was designed to assess pacemaker dependency (PMD) and long-term mortality after cardiac surgery procedures. Between 2004 and 2016, PPI-patients from 18 centers were followed. Time-to-event data were evaluated with semiparametric regression Cox models and semiparametric Fine and Gray model for competing risk framework. Of 859 (0.90%) PPI-patients, 30% were pacemaker independent (PMI) at 6 months. PMD showed higher mortality compared with PMI (10-year survival 80.1% ± 2.6% and 92.2% +2.4%, respectively, log-rank p-value < 0.001) with an unadjusted hazard ratio for death of 0.36 (95% CI 0.20 to 0.65, p< 0.001 favoring PMI) and an adjusted hazard ratio of 0.19 (95% CI 0.08 to 0.45, p< 0.001 with PMD as reference). Crude cumulative incidence function of restored PMI rhythm at follow-up at 6 months, 1 year and 12 years were 30.5% (95% CI 27.3% to 33.7%), 33.7% (95% CI 30.4% to 36.9%) and 37.2% (95% CI 33.8% to 40.6%) respectively. PMI was favored by preoperative sinus rhythm with normal conduction (SR) (HR 2.37, 95% CI 1.65 to 3.40, p< 0.001), whereas coronary artery bypass grafting and aortic valve replacement were independently associated with PMD (HR 0.63, 95% CI 0.45 to 0.88, p = 0.006 and HR 0.807, 95% CI 0.65 to 0.99, p = 0.047 respectively). Time-to-implantation was not associated with increased rate of PMI. Although 30% of PPI-patients are PMI after 6 months, PMD is associated with higher mortality at long term.

Cardiac and Neuromuscular Features of Patients With LMNA-Related Cardiomyopathy.

Background: Mutations in the LMNA (lamin A/C) gene have been associated with neuromuscular and cardiac manifestations, but the clinical implications of these signs are not well understood. Objective: To learn more about the natural history of LMNA-related disease. Design: Observational study. Setting: 13 clinical centers in Italy from 2000 through 2018. Patients: 164 carriers of an LMNA mutation. Measurements: Detailed cardiologic and neurologic evaluation at study enrollment and for a median of 10 years of follow-up. Results: The median age at enrollment was 38 years, and 51% of participants were female. Neuromuscular manifestations preceded cardiac signs by a median of 11 years, but by the end of follow-up, 90% of the patients had electrical heart disease followed by structural heart disease. Overall, 10 patients (6%) died, 14 (9%) received a heart transplant, and 32 (20%) had malignant ventricular arrhythmias. Fifteen patients had gait loss, and 6 had respiratory failure. Atrial fibrillation and second- and third-degree atrioventricular block were observed, respectively, in 56% and 51% of patients with combined cardiac and neuromuscular manifestations and 37% and 33% of those with heart disease only. Limitations: Some of the data were collected retrospectively. Neuromuscular manifestations were more frequent in this analysis than in previous studies. Conclusion: Many patients with an LMNA mutation have neurologic symptoms by their 30s and develop progressive cardiac manifestations during the next decade. A substantial proportion of these patients will have life-threatening neurologic or cardiologic conditions. Primary Funding Source: None.

Percutaneous Left Atrial Appendage Closure with WATCHMAN™ device: peri-procedural and mid-term outcomes from the TRAPS Registry.

PURPOSE: The WATCHMAN device for Left Atrial Appendage Occlusion (LAAO) has proven to be an effective alternative to oral anticoagulation (OAC) in patients with atrial fibrillation (AF), and has now been adopted in clinical practice. In the present study, we analyzed the safety and efficacy profile of the LAAO procedure at mid-term follow-up. METHODS: The TRAPS Registry is an observational, multicenter registry involving four Italian centers. Consecutive patients who had undergone LAAO with WATCHMAN device were enrolled. Clinical, demographic, and procedural data were collected at the time of implantation, and follow-up data were collected to assess the clinical outcome. RESULTS: A total of 151 patients were included in the Registry from May 2012 to October 2015. Implantation of the device was successful in 150/151 patients, with no or minimal (< 5 mm) leakage as assessed by peri-procedural transesophageal echo. In the remaining patient, early device embolization was reported, with no sequelae. Overall, intra-procedural events were reported in 5 (3.3%) patients. During a median follow-up of 16 months (25th and 75th percentile, 10-25), 5 patients died of any cause. The annual rate of all-cause stroke was 2.2% (95% CI, 0.7-5.1), the rate of transient ischemic attack was 1.3% (95% CI, 0.3-3.8), and that of major bleeding 0.4% (95% CI, 0.01-2.4). CONCLUSIONS: LAAO for stroke prevention was safely and effectively achieved by implantation of the WATCHMAN device in patients with non-valvular AF. Moreover, regardless of the risk profile of the population, we observed low rates of death and thromboembolic and bleeding events over a median follow-up of 16 months. These findings were obtained in an unselected group of consecutive patients who were variably eligible for chronic OAC therapy.

Multiple external electrical cardioversions for refractory neonatal atrial flutter.

This report describes a case of atrial flutter in a fetal/neonatal patient who did not respond to adenosine, a propafenone bolus, and three electrical external cardioversions. A fourth direct current cardioversion after propafenone premedication resolved the atrial flutter. The arrhythmia did not recur with propafenone therapy during a 12-month follow-up period.

Ultra high-density multipolar mapping with double ventricular access: a novel technique for ablation of ventricular tachycardia.

UNLABELLED: Ultra High-Density Multipolar Mapping With Double Ventricular Access. BACKGROUND: Analogous to the use of circular loop catheters to guide ablation around the pulmonary veins, it may be advantageous to use a multipolar catheter in the ventricle for rapid mapping and to guide ablation. We describe a technique using double access into the left ventricle for multipolar electroanatomic mapping and ablation of scar-mediated ventricular tachycardia (VT). METHODS: Double access into the left ventricle was obtained via transseptal technique. Endocardial mapping was performed via the first transseptal sheath using a steerable duodecapolar catheter. Higher density mapping was performed in areas of dense scar (<0.5 mV) and border zone (0.5-1.5 mV). All late potentials (LPs) observed on the 20 poles were tagged and pacemapping was performed at these sites for comparison with the clinical or induced VT 12-lead template. If VT was hemodynamically tolerated, entrainment mapping was attempted at sites demonstrating diastolic activity. Ablation was performed through the second transseptal sheath with an open-irrigated catheter at target sites identified by LPs, pacemapping, and/or entrainment on the duodecapolar catheter. RESULTS: Seventeen patients (88% ischemic cardiomyopathy) underwent electroanatomic mapping and ablation with double transseptal access. The mean number of endocardial mapping points was 819 ± 357 with an average mapping time of 31 ± 7 minutes. The mean number of VTs induced was 2.8 ± 1.6, mean cycle length 418 ms ± 101. LPs were seen in all patients during endocardial mapping with the duodecapolar catheter. Good (56%) and perfect (44%) pacemaps were seen in all patients when performed. Concealed entrainment, guided by the earliest diastolic activity seen on the duodecapolar catheter, was demonstrated in 4 patients (24%). Acute success was achieved in 94% of patients with complete success in 47% and partial success in 47%. The intermediate success rate (free of VT recurrence) was 69%, with an average follow-up of 8 ± 3 months. CONCLUSION: Mapping and ablation of scar-mediated VT using a multipolar catheter results in ultra high-density delineation of the left ventricular substrate. A novel double ventricular access strategy has the potential to facilitate identification of LPs, pacemapping, and entrainment mapping.

Image integration increases efficacy of paroxysmal atrial fibrillation catheter ablation: results from the CartoMerge Italian Registry.

AIMS: The aim of this study was to investigate whether circumferential pulmonary vein (PV) isolation guided by image integration improves the procedural and clinical outcomes of atrial fibrillation (AF) ablation in comparison with segmental PV isolation and circumferential PV isolation guided by three-dimensional (3D) electroanatomical mapping alone. METHODS AND RESULTS: Procedural and clinical outcomes of 573 patients who underwent their first catheter ablation for paroxysmal AF between January 2005 and April 2007 were collected from 12 centres. We evaluated three techniques: segmental ostial PV isolation (SOCA group, 240 patients), circumferential PV isolation guided by electroanatomical mapping (CARTO group, 107 patients), and circumferential PV isolation guided by electroanatomical mapping integrated with magnetic resonance/computed tomographic images of the left atrium (MERGE group, 226 patients). Procedure duration proved to be shorter in MERGE group patients than in CARTO group patients (P < 0.04), but longer than in SOCA group patients (P < 0.0001). During follow-up, atrial tachyarrhythmias relapsed more frequently in SOCA group patients (44.6%) and CARTO group patients (41.7%) than in MERGE group patients (22.6%; P < 0.0001). CONCLUSION: In patients with paroxysmal AF, circumferential PV isolation guided by image integration significantly improves clinical outcome in comparison with both circumferential PV isolation guided by 3D mapping alone and with segmental electrophysiologically guided PV isolation.

Hybrid therapies for ventricular arrhythmias.

In recent years several trials demonstrated the efficacy of implantable cardioverter-defibrillation (ICD) therapy in reducing cardiac and total mortality in patients affected by rapid ventricular tachycardia (VT) and/or ventricular fibrillation. Nevertheless, ICD do not prevent arrhythmia recurrences, thus being a palliative and not a curative treatment modality. The tolerance to ICD therapy varies greatly, and within individuals, this leading to a nonuniform acceptance of this form of therapy. The very frequent occurrence of VT, defined as an arrhythmic storm, may be a life threatening condition. The majority of ICD patients is under antiarrhythmic drug therapy, to reduce episodes of VT or to make antitachycardia pacing more effective by slowing the tachycardia rate. Drug therapy, however, may cause additional problems, and does not represent the optimal solution. The prevention of VT and/or ventricular fibrillation episodes and excessive ICD therapy, remains a worthwhile goal. Radiofrequency catheter ablation (RFCA) is a curative approach, and can be expected to reduce the frequency of recurrent VT episodes in the majority of patients. The combination of these treatment modalities (ICD and RFCA) is often described as hybrid therapy, implying that the two treatments act providing some form of synergism. In experienced centers, RFCA is now performed, regardless of whether the VT rate is rapid and/or is hemodynamically unstable. Newer mapping and ablation techniques are now available, enhancing the acute success rate of the procedure. In this review the most recent application of VT catheter ablation and the use of advanced mapping and ablation techniques will be discussed.