Background—: Pulmonary vein reconnection (PVR) still determines recurrences of atrial fibrillation after contact force (CF)–guided pulmonary vein isolation. We studied whether acute PVR (adenosine and waiting time) and late PVR (at repeat) are explained by incomplete transmurality and contiguity within the deployed radiofrequency circle. Methods and Results—: We analyzed 42 CF-guided ipsilateral pulmonary vein isolation procedures. For each radiofrequency tag within the circle, we collected data reflecting lesion depth (time of application, power, impedance drop [Δ-Imp], CF, force–time integral [FTI], and ablation index [AI]) and contiguity (automated interlesion distance [ILD]). Ablation line contiguity index (ALCI) was developed as a novel automated algorithm combining depth and contiguity into one single criterion. Each circle was subdivided into 10 segments. For each segment, we determined its weakest link by annotating time _min , power _min , Δ-Imp _min , CF _min , FTI _min , AI _min , ILD _max , and ALCI _min . Compared with segments without PVR (n=758), PVR segments (n=44) were characterized by lower Δ-Imp _min (4.8 versus 7.4 Ω), CF _min (8.5 versus 11.8 g), FTI _min (351 versus 473 gs), AI _min (367 versus 408 arbitrary unit [au]), and higher ILD _max (6.8 versus 5.5 mm). ALCI _min was significantly lower in segments with PVR (74% versus 104%; P <0.001) and was associated with the highest accuracy to predict durable segments (area under the curve=0.73). Conclusions—: In CF-guided pulmonary vein isolation, PVR is explained by lack of both lesion depth and contiguity within the deployed radiofrequency circle. ALCI, a novel measure combining contiguity and depth, is the most accurate predictor for durable segments. By avoiding weak links in the ablation chain, ALCI-guided ablation is expected to improve success rate of point-by-point radiofrequency ablation.

Catheter ablation is indicated in patients with symptomatic paroxysmal atrial fibrillation (AF) resistant to antiarrhythmic drug therapy (ADT). We investigated whether continued use of previously ineffective ADT beyond the post-ablation blanking period reduces recurrence of atrial tachyarrhythmia within the 1st year after ablation. This was a multicentre, randomized controlled study in patients undergoing contact force-guided pulmonary vein isolation (PVI) for paroxysmal AF in whom previously ineffective ADT was continued during a blanking period of 3 months. If free of AF at the end of the blanking period, patients were randomly assigned in the ratio of 1:1 to continue ADT (ADT ON group, n = 77) or discontinue ADT (ADT OFF group, n = 76). Patients were followed up until 1 year after PVI, with clinical visits, Holter monitoring, and quality-of-life (QOL) questionnaires at 6 and 12 months post-procedure. Analysis of the primary endpoint (any documented atrial tachyarrhythmia lasting >30 s) was performed according to the modified intention-to-treat principle. Secondary endpoints included repeat ablation, unscheduled visits, and QOL score. Baseline clinical characteristics and initial ablation procedure characteristics were comparable between both groups. Three patients were lost to follow-up in each arm. The primary endpoint was observed in 2 of 74 (2.7%) patients in the ADT ON group vs. 16 of 73 (21.9%) patients in the ADT OFF group (P < 0.001). The ADT ON group had a lower rate of repeat ablation [1.4% vs. 19.2%, hazard ratio (HR) = 0.053; 95% confidence interval (CI) 0.007–0.399; P < 0.01) and less unscheduled arrhythmia-related health care visits (2.7% vs. 20.5%, HR = 0.055, 95% CI 0.007–0.410; P < 0.01). Quality-of-life scores were similar in both groups. In patients free of AF at the end of 3 months of post-ablation blanking period, continued use of previously ineffective ADT significantly reduces the recurrence of atrial tachyarrhythmia in the 1st year after PVI.

Introduction: The revised Ghent nosology presents classical features of Marfan syndrome. However, behind its familiar face, Marfan syndrome hides less well-known features. Areas covered: The German Marfan Organization listed unusual symptoms and clinical experts reviewed the literature on clinical features of Marfan syndrome not listed in the Ghent nosology. Thereby we identified the following features: (1) bicuspid aortic valve, mitral valve prolapse, pulmonary valve prolapse, tricuspid valve prolapse, (2) heart failure and cardiomyopathy, (3) supraventricular arrhythmia, ventricular arrhythmia, and abnormal repolarization, (4) spontaneous coronary artery dissection, anomalous coronary arteries, and atherosclerotic coronary artery disease, tortuosity-, aneurysm-, and dissection of large and medium-sized arteries, (5) restrictive lung disease, parenchymal lung disease, and airway disorders, (6) obstructive- and central sleep apnea, (7) liver and kidney cysts, biliary tract disease, diaphragmatic hernia, and adiposity, (8) premature labour, and urinary incontinence, (9) myopathy, reduced bone mineral density, and craniofacial manifestations, (10) atrophic scars, (11) caries, and craniomandibular dysfunction, (12) headache from migraine and spontaneous cerebrospinal fluid leakage, (13) cognitive dysfunction, schizophrenia, depression, fatigue, and pain, (14) and activated fibrinolysis, thrombin, platelets, acquired von Willebrand disease, and platelet dysfunction. Expert opinion: Future research, nosologies and guidelines may consider less well-known features of Marfan syndrome.

Networks provide a powerful methodology with applications in a variety of biological, technological and social systems such as analysis of brain data, social networks, internet search engine algorithms, etc. To date, directed networks have not yet been applied to characterize the excitation of the human heart. In clinical practice, cardiac excitation is recorded by multiple discrete electrodes. During (normal) sinus rhythm or during cardiac arrhythmias, successive excitation connects neighboring electrodes, resulting in their own unique directed network. This in theory makes it a perfect fit for directed network analysis. In this study, we applied directed networks to the heart in order to describe and characterize cardiac arrhythmias. Proof-of-principle was established using in-silico and clinical data. We demonstrated that tools used in network theory analysis allow determination of the mechanism and location of certain cardiac arrhythmias. We show that the robustness of this approach can potentially exceed the existing state-of-the art methodology used in clinics. Furthermore, implementation of these techniques in daily practice can improve the accuracy and speed of cardiac arrhythmia analysis. It may also provide novel insights in arrhythmias that are still incompletely understood.

Marfan syndrome (MFS) is a heritable systemic connective tissue disease with important cardiovascular involvement, including aortic root dilatation and mitral valve prolapse. Life expectancy in patients with MFS is mainly determined by cardiovascular complications, among which aortic dissection or rupture are most dreaded. In recent years, heart failure and ventricular arrhythmia have drawn attention as extra-aortic cardiovascular manifestations and as additional reported causes of death. Imaging studies have provided data supporting a primary myocardial impairment in the absence of valvular disease or cardiovascular surgery, while studies using ambulatory ECG have demonstrated an increased susceptibility to ventricular arrhythmia. In this paper, current literature was reviewed in order to provide insights in characteristics, pathophysiology and evolution of myocardial function, heart failure and ventricular arrhythmia in MFS.