Abstract:The diagnosis and management of idiopathic ventricular fibrillation is challenging, as it requires extensive diagnostic testing and offers few curative options due to unknown underlying disease. The resulting population is a heterogeneous group of patients with a largely unknown natural history. Structural patient characterisation, follow-up and innovations in diagnostic testing can improve our understanding of the disease mechanisms of idiopathic ventricular fibrillation, detect underlying disease during foll… Show more
“…The need for the Dutch iVF Registry was stated in our first report, in which we introduced the registry in the Netherlands Heart Journal [ 7 ]. With the registry we aim to (1) create a large cohort of patients with an initial iVF diagnosis, (2) focus on additional diagnostic testing (e.g.…”
Section: Methodsmentioning
confidence: 99%
“…1 : Infographic). This progress report focuses on following up on our first report of the registry in 2018 and highlights the output over the years [ 7 ]. Fig.…”
Background
Idiopathic ventricular fibrillation (iVF) is a rare cause of sudden cardiac arrest and, by definition, a diagnosis of exclusion. Due to the rarity of the disease, previous and current studies are limited by their retrospective design and small patient numbers. Even though the incidence of iVF has declined owing to the identification of new disease entities, an important subgroup of patients remains.
Aim
To expand the existing Dutch iVF Registry into a large nationwide cohort of patients initially diagnosed with iVF, to reveal the underlying cause of iVF in these patients, and to improve arrhythmia management.
Methods
The Dutch iVF Registry includes sudden cardiac arrest survivors with an initial diagnosis of iVF. Clinical data and outcomes are collected. Outcomes include subsequent detection of a diagnosis other than ‘idiopathic’, arrhythmia recurrence and death. Non-invasive electrocardiographic imaging is used to investigate electropathological substrates and triggers of VF.
Results
To date, 432 patients have been included in the registry (median age at event 40 years (interquartile range 28–52)), 61% male. During a median follow-up of 6 (2–12) years, 38 patients (9%) received a diagnosis other than ‘idiopathic’. Eleven iVF patients were characterised with electrocardiographic imaging.
Conclusion
The Dutch iVF Registry is currently the largest of its kind worldwide. In this heterogeneous population of index patients, we aim to identify common functional denominators associated with iVF. With the implementation of non-invasive electrocardiographic imaging and other diagnostic modalities (e.g. echocardiographic deformation, cardiac magnetic resonance), we advance the possibilities to reveal pro-fibrillatory substrates.
“…The need for the Dutch iVF Registry was stated in our first report, in which we introduced the registry in the Netherlands Heart Journal [ 7 ]. With the registry we aim to (1) create a large cohort of patients with an initial iVF diagnosis, (2) focus on additional diagnostic testing (e.g.…”
Section: Methodsmentioning
confidence: 99%
“…1 : Infographic). This progress report focuses on following up on our first report of the registry in 2018 and highlights the output over the years [ 7 ]. Fig.…”
Background
Idiopathic ventricular fibrillation (iVF) is a rare cause of sudden cardiac arrest and, by definition, a diagnosis of exclusion. Due to the rarity of the disease, previous and current studies are limited by their retrospective design and small patient numbers. Even though the incidence of iVF has declined owing to the identification of new disease entities, an important subgroup of patients remains.
Aim
To expand the existing Dutch iVF Registry into a large nationwide cohort of patients initially diagnosed with iVF, to reveal the underlying cause of iVF in these patients, and to improve arrhythmia management.
Methods
The Dutch iVF Registry includes sudden cardiac arrest survivors with an initial diagnosis of iVF. Clinical data and outcomes are collected. Outcomes include subsequent detection of a diagnosis other than ‘idiopathic’, arrhythmia recurrence and death. Non-invasive electrocardiographic imaging is used to investigate electropathological substrates and triggers of VF.
Results
To date, 432 patients have been included in the registry (median age at event 40 years (interquartile range 28–52)), 61% male. During a median follow-up of 6 (2–12) years, 38 patients (9%) received a diagnosis other than ‘idiopathic’. Eleven iVF patients were characterised with electrocardiographic imaging.
Conclusion
The Dutch iVF Registry is currently the largest of its kind worldwide. In this heterogeneous population of index patients, we aim to identify common functional denominators associated with iVF. With the implementation of non-invasive electrocardiographic imaging and other diagnostic modalities (e.g. echocardiographic deformation, cardiac magnetic resonance), we advance the possibilities to reveal pro-fibrillatory substrates.
“…In this issue of the Netherlands Heart Journal , Verheul and colleagues present a progress report on the Dutch Idiopathic Ventricular Fibrillation Registry [ 2 ]. The outline of the registry has been published previously in this journal [ 3 ]. Eligible patients ( n = 432 from 11 hospitals across the Netherlands) had VF or polymorphic ventricular tachycardia as the presenting rhythm.…”
mentioning
confidence: 99%
“…During follow-up of the patients in the registry, a definitive diagnosis was established in a mere 38 patients (9%) during 11 years of follow-up, lower than the 22% that was reported in the initial phase of the registry [ 3 ]. Most diagnoses established during follow-up were cardiomyopathies (arrhythmogenic cardiomyopathy in 9 patients (24%), dilated cardiomyopathy in 5 (13%) and hypertrophic cardiomyopathy in 4 (10%)), channelopathies (Brugada syndrome in 5 patients (13%), long QT syndrome in 1 (3%), catecholaminergic polymorphic VT in 5 (13%) and early repolarisation syndrome in 3 (8%)).…”
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
