Risk stratification and subclinical phenotyping of dilated and/or arrhythmogenic cardiomyopathy mutation-positive relatives: CVON eDETECT consortium.

In relatives of index patients with dilated cardiomyopathy and arrhythmogenic cardiomyopathy, early detection of disease onset is essential to prevent sudden cardiac death and facilitate early treatment of heart failure. However, the optimal screening interval and combination of diagnostic techniques are unknown. The clinical course of disease in index patients and their relatives is variable due to incomplete and age-dependent penetrance. Several biomarkers, electrocardiographic and imaging (echocardiographic deformation imaging and cardiac magnetic resonance imaging) techniques are promising non-invasive methods for detection of subclinical cardiomyopathy. However, these techniques need optimisation and integration into clinical practice. Furthermore, determining the optimal interval and intensity of cascade screening may require a personalised approach. To address this, the CVON-eDETECT (early detection of disease in cardiomyopathy mutation carriers) consortium aims to integrate electronic health record data from long-term follow-up, diagnostic data sets, tissue and plasma samples in a multidisciplinary biobank environment to provide personalised risk stratification for heart failure and sudden cardiac death. Adequate risk stratification may lead to personalised screening, treatment and optimal timing of implantable cardioverter defibrillator implantation. In this article, we describe non-invasive diagnostic techniques used for detection of subclinical disease in relatives of index patients with dilated cardiomyopathy and arrhythmogenic cardiomyopathy.

Novel use of repolarization parameters in electrocardiographic imaging to uncover arrhythmogenic substrate.

BACKGROUND: Measuring repolarization characteristics is challenging and has been reserved for experienced physicians. In electrocardiographic imaging (ECGI), activation-recovery interval (ARI) is used as a measure of local cardiac repolarization duration. We hypothesized that repolarization characteristics, such as local electrogram morphology and local and global dispersion of repolarization timing and duration could be of significance in ECGI. OBJECTIVE: To further explore their potential in arrhythmic risk stratification we investigated the use of novel repolarization parameters in ECGI. MATERIALS AND METHODS: We developed and compared methods for T-peak and T-end detection in reconstructed potentials. All methods were validated on annotated reconstructed electrograms (EGMs). Characteristics of the reconstructed EGMs and epicardial substrate maps in IVF patients were analyzed by using data recorded during sinus rhythm. The ECGI data were analyzed for EGM morphology, conduction, and repolarization. RESULTS: We acquired ECGI data from 8 subjects for this study. In all patients we evaluated four repolarization parameters: Repolarization time, T-wave area, Tpeak-Tend interval, and T-wave alternans. Most prominent findings were steep repolarization time gradients in regions with flat EGMs. These regions were also characterized by low T-wave area and large differences in Tpeak-Tend interval. CONCLUSIONS: Measuring novel repolarization parameters in reconstructed electrograms acquired with ECGI is feasible, can be done in a fully automated manner and may provide additional information on underlying arrhythmogenic substrate for risk stratification. Further studies are needed to investigate their potential use and clinical application.

Catheter ablation in children and young adults: is there an additional benefit from remote magnetic navigation?

PURPOSE: Although rare, children and young adults can suffer from significant cardiac arrhythmia, especially in the context of congenital malformations and after cardiac surgery. METHODS: A total of 62 patients (32 female, median age 20 years) underwent an invasive electrophysiology study between 2008-2011: half had normal cardiac anatomy, whereas the remaining patients had various types of congenital heart disease. All patients were treated using either conventional techniques (CVN) or remote magnetic navigation (RMN). RESULTS: Patients treated with the RMN system differed substantially from patients in the CVN group with respect to presence of congenital heart disease (67 % vs. 37 %), previous cardiac surgery (59 % vs. 20 %) or failed previous conventional ablation (22 % vs. 9 %), respectively. Although these more complex arrhythmias resulted in longer median procedure duration (180 vs. 130 min, p = 0.034), the median overall fluoroscopy exposure in the RMN group was significantly lower (4.1 vs. 5.2 min, p = 0.020). Clinical outcome was comparable in both groups without complications caused by the ablation. CONCLUSIONS: Catheter ablation using remote magnetic navigation is safe and feasible in children and young adults and is especially valuable in patients with abnormal cardiac morphologies. RMN resulted in significantly lower radiation exposure compared with the conventional technique.