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Contains fulltext : 291532.pdf (Publisher’s version ) (Closed access) BACKGROUND: Idiopathic ventricular fibrillation (iVF) is a diagnosis of exclusion. Systematic diagnostic testing is important to exclude alternative causes for VF. The early use of "high yield" testing, including cardiac magnetic resonance (CMR), exercise testing, and sodium channel blocker provocation, has been increasingly recognized. OBJECTIVES: The purpose of this study was to investigate the importance and consistency of systematic diagnostic testing in iVF. METHODS: This study included 423 iVF patients from 11 large secondary and tertiary hospitals in the Netherlands. Clinical characteristics and diagnostic testing data were ascertained. RESULTS: IVF patients experienced the index event at a median age of 40 years (IQR: 28-52 years), and 61% were men. The median follow-up time was 6 years (IQR: 2-12 years). Over the years, "high yield" diagnostic tests were increasingly performed (mean 68% in 2000-2010 vs 75% in 2011-2021; P < 0.001). During follow-up, 38 patients (9%) originally labeled as iVF received an alternative diagnosis. Patients in whom "high-yield" diagnostic tests were consistently performed during the initial work-up received an alternative diagnosis less frequently during follow-up (HR: 0.439; 95% CI: 0.219-0.878; P = 0.020). Patients who received an alternative diagnosis during follow-up had a worse prognosis in terms of cardiac death (P = 0.012) with a trend toward more implantable cardioverter-defibrillator therapy (P = 0.055). CONCLUSIONS: Although adherence to (near) complete diagnostic testing in this population of iVF patients increased over the years, patients with iVF still undergo varying levels of diagnostic evaluation. The latter leads to initial underdiagnosis of alternative conditions and is associated with a worse prognosis. Our results underscore the importance of early systematic diagnostic assessment in patients with apparent iVF.

The EU Horizon 2020 Framework-funded Standardized Treatment and Outcome Platform for Stereotactic Therapy Of Re-entrant tachycardia by a Multidisciplinary (STOPSTORM) consortium has been established as a large research network for investigating STereotactic Arrhythmia Radioablation (STAR) for ventricular tachycardia (VT). The aim is to provide a pooled treatment database to evaluate patterns of practice and outcomes of STAR and finally to harmonize STAR within Europe. The consortium comprises 31 clinical and research institutions. The project is divided into nine work packages (WPs): (i) observational cohort; (ii) standardization and harmonization of target delineation; (iii) harmonized prospective cohort; (iv) quality assurance (QA); (v) analysis and evaluation; (vi, ix) ethics and regulations; and (vii, viii) project coordination and dissemination. To provide a review of current clinical STAR practice in Europe, a comprehensive questionnaire was performed at project start. The STOPSTORM Institutions' experience in VT catheter ablation (83% ≥ 20 ann.) and stereotactic body radiotherapy (59% > 200 ann.) was adequate, and 84 STAR treatments were performed until project launch, while 8/22 centres already recruited VT patients in national clinical trials. The majority currently base their target definition on mapping during VT (96%) and/or pace mapping (75%), reduced voltage areas (63%), or late ventricular potentials (75%) during sinus rhythm. The majority currently apply a single-fraction dose of 25 Gy while planning techniques and dose prescription methods vary greatly. The current clinical STAR practice in the STOPSTORM consortium highlights potential areas of optimization and harmonization for substrate mapping, target delineation, motion management, dosimetry, and QA, which will be addressed in the various WPs.

The combination of big data and artificial intelligence (AI) is having an increasing impact on the field of electrophysiology. Algorithms are created to improve the automated diagnosis of clinical ECGs or ambulatory rhythm devices. Furthermore, the use of AI during invasive electrophysiological studies or combining several diagnostic modalities into AI algorithms to aid diagnostics are being investigated. However, the clinical performance and applicability of created algorithms are yet unknown. In this narrative review, opportunities and threats of AI in the field of electrophysiology are described, mainly focusing on ECGs. Current opportunities are discussed with their potential clinical benefits as well as the challenges. Challenges in data acquisition, model performance, (external) validity, clinical implementation, algorithm interpretation as well as the ethical aspects of AI research are discussed. This article aims to guide clinicians in the evaluation of new AI applications for electrophysiology before their clinical implementation.

Item does not contain fulltext Active and passive smoking are well-known causes of disease, including respiratory and cardiovascular disease and cancer. In 2004 the Dutch government introduced new legislation to regulate smoking in the workplace. However, smoking is still allowed in hotels, bars and restaurants, despite the fact that two-thirds of the Dutch population support a total ban on smoking in public places. Several other European countries and American states have banned smoking in public places. Studies performed in these regions show that the new smoking regulations have had no negative economic effects. Moreover, various studies have shown that smoking bans have a positive impact on public health, even in the short-term, including a significant decrease in respiratory and cardiovascular disease. There is therefore no reason to continue to exclude hotels, bars and restaurants from the smoking ban in all public places in The Netherlands.

Contains fulltext : 74488.pdf (Publisher’s version ) (Open Access)

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Sudden cardiac death is a major problem in the Western world. In young people, sudden cardiac arrest is often caused by ventricular fibrillation (VF). In most cases of VF, an underlying cause is found. However, in a small part of the patients, no cause for VF can be found. These patients are diagnosed with “idiopathic ventricular fibrillation” (IVF). Extensive diagnostic testing is necessary to exclude any underlying diseases. In this dissertation, we have attempted to further unravel the mystery surrounding IVF. Using a large national IVF database, we unraveled the characteristics of current IVF patients. Although adherence to (near-)complete diagnostic testing increased over the years, patients with IVF still undergo varying levels of diagnostic evaluation. This is problematic, as it leads to underdiagnosing of alternative disease. Unfortunately, as the substrate is absent in IVF, there are limited treatment options besides ICD implantation to prevent sudden cardiac death. In this thesis, we focussed on a specific phenotype in IVF, called short-coupled VF. We showed that this phenotype is prevalent in IVF. This is important, as previous research indicates that these patients can be treated effectively with class IA drugs. Another interesting region of interest in IVF patients is the mitral valve. Abnormalities in this region have been associated with ventricular arrhythmias. We were able to reveal abnormalities around the mitral valve using cardiac magnetic resonance imaging. In particular, we showed that mitral annulus disjunction is more prevalent in IVF patients compared to healthy controls. Patients with mitral valve disease proved to have a higher burden of ectopy than patients without mitral valve disease. We also applied echocardiographic deformation imaging to detect subtle myocardial abnormalities. Interestingly, both global and regional echocardiographic deformation abnormalities are more prevalent in IVF patients compared to healthy control subjects. Our research therefore indicates that subtle myocardial disease is present in a large subset of IVF patients. To search for cardiac abnormalities in family members of IVF patients, we performed familial cascade screening. We hypothesized that family members of IVF patients might reveal heritable cardiac diseases that were not apparent in the IVF patient. However, the yield of family screening in relatives of IVF patients appeared to be low when the patients was comprehensively investigated. This underlines the importance of systematic diagnostic testing in IVF. At last, we created a practical flowchart for diagnostic testing after sudden cardiac death of a young person. This is of utter importance as the current rate of diagnostic testing after sudden cardiac death in a young person is low in the Netherlands.

Despite the progress in diagnostic testing and management of life-threatening arrhythmia's over the last few decades in a small part of the survivors a cause for the arrhythmia remains unknown. This group is known as idiopathic ventricular fibrillation and treatment usually consists of ICD implantation to treat any subsequent ventricular arrhythmias. The research described in this dissertation has focused on the potential of (new) diagnostic tests in this population to discover further methods for treatment and provide insight in the prognosis of both patients and their family members. The phenotypic screening of family members and diagnostic possibilities of electrocardiographic imaging are discussed.