True idiopathic ventricular fibrillation in out-of-hospital cardiac arrest survivors in the Swiss Canton Ticino: prevalence, clinical features, and long-term follow-up

Conte, Giulio; Caputo, Maria Luce; Regoli, François; Marcon, Serena; Klersy, Catherine; Adjibodou, Boris; Bufalo, Alessandro Del; Moccetti, Tiziano; Auricchio, Angelo

doi:10.1093/europace/euv447

Cited by 25 publications

(41 citation statements)

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“…Although its exact incidence is unknown and will probably decline with advancements in diagnostic testing and the discovery of new primary arrhythmia syndromes, similar rates from 5 to 10% have been reported in the literature. 47 , 48 It represents, for example, a higher proportion of SCA than identified electrical primary disorders in our study [15 (2.1%) patients], although, as discussed above, inadequate medical investigations probably underestimate the true proportion of channelopathies. We also identified several clinical characteristics of cases labelled IVF.…”

Section: Discussionmentioning

confidence: 63%

Characteristics and clinical assessment of unexplained sudden cardiac arrest in the real-world setting: focus on idiopathic ventricular fibrillation

Waldmann

Bougouin

Karam

et al. 2018

European Heart Journal

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AimsRecent studies have shown that in more than half of apparently unexplained sudden cardiac arrests (SCA), a specific aetiology can be unmasked by a careful evaluation. The characteristics and the extent to which such cases undergo a systematic thorough investigation in real-life practice are unknown.Methods and resultsData were analysed from an ongoing study, collecting all cases of out-of-hospital cardiac arrest in Paris area. Investigations performed during the index hospitalization or planned after discharge were gathered to evaluate the completeness of assessment of unexplained SCA. Between 2011 and 2016, among the 18 622 out-of-hospital cardiac arrests, 717 survivors (at hospital discharge) fulfilled the definition of cardiac SCA. Of those, 88 (12.3%) remained unexplained after electrocardiogram, echocardiography, and coronary angiography. Cardiac magnetic resonance imaging yielded the diagnosis in 25 (3.5%) cases, other investigations accounted for 14 (2.4%) additional diagnoses, and 49 (6.8%) patients were labelled as idiopathic ventricular fibrillation (IVF) (48.7 ± 15 years, 69.4% male). Among those labelled IVF, only 8 (16.3%) cases benefited from a complete workup (including pharmacological testing). Younger patients [odds ratio (OR) 6.00, 95% confidence interval (CI) 1.80–22.26] and those admitted to university centres (OR 3.60, 95% CI 1.12–12.45) were more thoroughly investigated. Genetic testing and family screening were initiated in only 9 (18.4%) and 12 (24.5%) cases, respectively.ConclusionOur findings suggest that complete investigations are carried out in a very low proportion of unexplained SCA. Standardized, systematic approaches need to be implemented to ensure that opportunities for specific therapies and preventive strategies (including relatives) are not missed.

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Section: Discussionmentioning

confidence: 63%

Characteristics and clinical assessment of unexplained sudden cardiac arrest in the real-world setting: focus on idiopathic ventricular fibrillation

Waldmann

Bougouin

Karam

et al. 2018

European Heart Journal

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“…Comprising approximately 1% of out-of-hospital cardiac arrest survivors presenting with a shockable rhythm, IVF can often be challenging to diagnosis. 141, 142 Further, in the setting of a normal ECG, the affected status of an individual can only be known following an arrhythmic event, which makes genetic studies challenging. Traditionally associated with mutations in the SCN5A -encoded Nav1.5, the first IVF-associated mutations were often described in sporadic cases presenting with VF and had significant clinical overlap with a group of Nav1.5-mediated channelopathies known as Brugada syndrome (BrS).…”

Section: Arrhythmias Caused By Heritable Defects In Calcium-handling mentioning

confidence: 99%

Calcium Signaling and Cardiac Arrhythmias

Landstrom

Dobrev

Wehrens

2017

Circulation Research

415

331

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There has been significant progress in our understanding of the molecular mechanisms by which calcium (Ca2+) ions mediate various types of cardiac arrhythmias. A growing list of inherited gene defects can cause potentially lethal cardiac arrhythmia syndromes, including catecholaminergic polymorphic ventricular tachycardia, congenital long QT syndrome, and hypertrophic cardiomyopathy. In addition, acquired deficits of multiple Ca2+-handling proteins can contribute to the pathogenesis of arrhythmias in patients with various types of heart disease. In this review article, we will first review the key role of Ca2+ in normal cardiac function - in particular, excitation-contraction coupling and normal electrical rhythms. The functional involvement of Ca2+ in distinct arrhythmia mechanisms will be discussed, followed by various inherited arrhythmia syndromes caused by mutations in Ca2+-handling proteins. Finally, we will discuss how changes in the expression of regulation of Ca2+ channels and transporters can cause acquired arrhythmias, and how these mechanisms might be targeted for therapeutic purposes.

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“…Sudden cardiac arrest is a major public health problem, with the leading cause being ventricular fibrillation (VF) in the context of coronary heart disease 1 . Out-of-hospital cardiac arrest (OHCA) due to VF in the absence of overt structural or electrical cardiac abnormalities is rare, occurring in 1.2% of all OHCAs presenting with a shockable rhythm 2 . Aetiology is identifiable in up to 90% of OHCA survivors when a complete diagnostic work-up including electrocardiogram (ECG), cardiac imaging (echocardiography and/or cardiac magnetic resonance), coronary angiography, and pharmacological challenges are undertaken 3 , 4 .…”

Section: Introductionmentioning

confidence: 99%

Out-of-hospital cardiac arrest due to idiopathic ventricular fibrillation in patients with normal electrocardiograms: results from a multicentre long-term registry

et al. 2019

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Aims To define the clinical characteristics and long-term clinical outcomes of a large cohort of patients with idiopathic ventricular fibrillation (IVF) and normal 12-lead electrocardiograms (ECGs). Methods and results Patients with ventricular fibrillation as the presenting rhythm, normal baseline, and follow-up ECGs with no signs of cardiac channelopathy including early repolarization or atrioventricular conduction abnormalities, and without structural heart disease were included in a registry. A total of 245 patients (median age: 38 years; males 59%) were recruited from 25 centres. An implantable cardioverter-defibrillator (ICD) was implanted in 226 patients (92%), while 18 patients (8%) were treated with drug therapy only. Over a median follow-up of 63 months (interquartile range: 25–110 months), 12 patients died (5%); in four of them (1.6%) the lethal event was of cardiac origin. Patients treated with antiarrhythmic drugs only had a higher rate of cardiovascular death compared to patients who received an ICD (16% vs. 0.4%, P = 0.001). Fifty-two patients (21%) experienced an arrhythmic recurrence. Age ≤16 years at the time of the first ventricular arrhythmia was the only predictor of arrhythmic recurrence on multivariable analysis [hazard ratio (HR) 0.41, 95% confidence interval (CI) 0.18–0.92; P = 0.03]. Conclusion Patients with IVF and persistently normal ECGs frequently have arrhythmic recurrences, but a good prognosis when treated with an ICD. Children are a category of IVF patients at higher risk of arrhythmic recurrences.

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True idiopathic ventricular fibrillation in out-of-hospital cardiac arrest survivors in the Swiss Canton Ticino: prevalence, clinical features, and long-term follow-up

Abstract: Idiopathic ventricular fibrillation is rare occurring in 1.2% of OHCA survivors presenting with a shockable rhythm. The initial diagnosis can change in up to 27% of cases. Patients with IVF and no ER pattern or AV conduction disturbances have a good prognosis during a long-term follow-up.

Cited by 25 publications

References 20 publications

Characteristics and clinical assessment of unexplained sudden cardiac arrest in the real-world setting: focus on idiopathic ventricular fibrillation

Characteristics and clinical assessment of unexplained sudden cardiac arrest in the real-world setting: focus on idiopathic ventricular fibrillation

Calcium Signaling and Cardiac Arrhythmias

Out-of-hospital cardiac arrest due to idiopathic ventricular fibrillation in patients with normal electrocardiograms: results from a multicentre long-term registry

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