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Singleton pregnancy in Fontan patients is burdened by a significant maternal cardiovascular and obstetric risk. The cardiac workload in a twin pregnancy is greater and could place Fontan-palliated patients at an increased risk of complications. We report a case of a woman with Fontan circulation and homozygous MTHFR mutation who had a twin pregnancy., Competing Interests: The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2024 The Authors.)

Background: Multicenter ventricular tachycardia (VT) ablation studies have shown poorer outcomes compared with single-center experiences. This difference could be related to heterogeneous mapping and ablation strategies., Objectives: This study evaluated a homogenous simplified catheter ablation strategy for different substrates and compared the results with those of a single referral center., Methods: This was a multicenter prospective VT ablation registry of patients with the following 4 causes of VT: previous myocardial infarction; previous myocarditis; arrhythmogenic right ventricular dysplasia; or idiopathic dilated cardiomyopathy. The procedural protocol included precise mapping and ablation steps with the combined endpoint of late potential (LP) abolition and noninducibility of VT. The long-term primary efficacy endpoint was freedom from VT., Results: A total of 309 patients were enrolled. LPs were present in 70% of patients and were abolished in 83%. At the end of the procedure 74% of LPs were noninducible. The primary combined endpoint of LP abolition and noninducibility was achieved in 64% of patients with LPs at baseline. Freedom from VT at 12 months was observed in 67% of patients. In the overall study group, VT inducibility was the only predictor of freedom from VT (P = 0.013). In patients with LPs, the VT recurrence rate was lower both for patients with complete LP abolition (P = 0.040) and for patients meeting the composite endpoint (P = 0.035)., Conclusions: A standardized VT mapping and ablation technique reproduced the procedural outcomes of a single referral center in a multicenter prospective study. LP abolition and noninducibility were effective in reducing VT recurrences in patients with 4 causes of cardiomyopathy. (Ventricular Tachycardia Ablation Registry; NCT03649022)., Competing Interests: Funding Support and Author Disclosures This work was supported by Abbott for Contract Research Organisation activities. Dr Della Bella has served as a consultant for Abbott and Biosense; and has received research grants from Abbott, Biosense, Biotronik, and Boston Scientific. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2023 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Endomyocardial biopsy (EMB) is required to make a definite diagnosis of lymphocytic myocarditis (LM), to identify its etiology, and to classify LM into different phases., Objectives: This study aims to characterize and compare clinical and electrophysiological characteristics of different biopsy-proven LM phases, namely acute myocarditis (AM), chronic active myocarditis (CAM), and healed myocarditis (HM)., Methods: All patients with a diagnosis of LM at 3 Italian referral centers were prospectively enrolled. According to EMB findings, LM was classified as AM, CAM, or HM; per-group comparisons of clinical presentations, noninvasive, and invasive findings are reported., Results: Among the 122 enrolled patients (AM, n = 44; CAM, n = 42; HM, n = 36), complex ventricular arrhythmias were very common overall (n = 109, 89%), but ventricular fibrillation was slightly more prevalent in AM (P = 0.028). Cardiac magnetic resonance imaging showed late gadolinium enhancement in more patients with HM and CAM than AM (94.4% vs 92.9% vs 50%; P < 0.001), whereas edema was more common in AM than in CAM, being absent in HM (90.9% vs 50% vs 0%; P < 0.001). Accordingly, edema was the strongest independent clinical predictor of EMB-proven active inflammation. Electroanatomical mapping revealed a lower prevalence of low-voltage areas in AM than in CAM or HM. We observed a strong association between edema at a specific myocardial segment and normal voltages at that site (odds ratio: 0.24; 95% CI: 0.10-0.54; P < 0.01), as well as between late gadolinium enhancement and low-voltage areas (odds ratio: 2.86; 95% CI: 1.19-6.97; P = 0.019)., Conclusions: LM is a highly heterogeneous disease, and its different phases are characterized by diverse clinical, morphological, and electrophysiological features. Further research is required to identify electroanatomical markers of inflammation., Competing Interests: Funding Support and Author Disclosures Dr Natale has received consulting fees from Biosense Webster, Stereotaxis, and Abbott Medical; and has received speaker honoraria/travel from Medtronic, Atricure, Biotronik, and Janssen. Dr Tondo has received honoraria from Abbott Medical; and has served as a member of the advisory board for Medtronic and Boston Scientific. Dr Dello Russo has received consulting fees from Abbott Medical. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2023 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

We present the case of a 17-year-old asymptomatic boy with a diagnosis of arrhythmogenic cardiomyopathy. Merging of cardiac magnetic resonance imaging and three-dimensional electroanatomic mapping provided striking visualization of the association between structural and electrical alterations and guided the decision to implant an implantable cardioverter defibrillator. ( Level of Difficulty: Intermediate. )., Competing Interests: The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2022 The Authors.)

Objectives: This study sought to assess long-term arrhythmic risk in patients with myocarditis who received an implantable cardioverter-defibrillator (ICD)., Background: The arrhythmic risk of patients with myocarditis overtime remains poorly known., Methods: The study enrolled 56 patients with biopsy-proven myocarditis who received an ICD for either primary (57%) or secondary prevention (43%) according to current guidelines. Clinical characteristics, biopsy findings, electrophysiological data from endocardial 3-dimensional electroanatomic voltage mapping, and device interrogation data were analyzed to detect arrhythmic events overtime. Coronary angiography excluded significant coronary artery disease in all patients., Results: At a mean follow-up of 74 ± 60 months (median 65 months), 25 (45%) patients had major ventricular arrhythmias treated by ICD intervention (76% being terminated by ICD shock and 24% by antitachyarrhythmia burst pacing). At multivariable analysis, the presence of sustained ventricular tachycardia on admission (hazard ratio: 13.0; 95% confidence interval: 2.0 to 35.0; p = 0.032) and the extension of the areas of low potentials at the bipolar endocardial mapping (hazard ratio: 1.19; 95% confidence interval: 1.04 to 1.37; p = 0.013) were the only independent predictors of appropriate ICD interventions. A cutoff value of 10% of abnormal bipolar area at electroanatomical ventricular mapping discriminated patients with appropriate ICD interventions with a sensitivity of 89% and a specificity of 85%., Conclusions: The study demonstrates that the prevalence of life-threatening ventricular arrhythmias in patients with myocarditis receiving an ICD according to current guidelines is high and the arrhythmic risk persists late overtime. Electroanatomical ventricular mapping may be a useful tool to identify patients at greater arrhythmic risk., (Copyright © 2020 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)