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. 2018 Jun 1;39(21):1981-1987.
doi: 10.1093/eurheartj/ehy098.

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

Victor Waldmann  1   2   3 , Wulfran Bougouin  1   2   3 , Nicole Karam  1   2   3 , Florence Dumas  2   3   4 , Ardalan Sharifzadehgan  1   2 , Estelle Gandjbakhch  5   6 , Vincent Algalarrondo  6   7 , Kumar Narayanan  2   8 , Alexandre Zhao  1 , Denis Amet  1 , Daniel Jost  9 , Guillaume Geri  2   10 , Lionel Lamhaut  11 , Frankie Beganton  2 , Bertrand Ludes  12 , Patrick Bruneval  13 , Isabelle Plu  14 , Françoise Hidden-Lucet  5 , Juliette Albuisson  2   3   15 , Thomas Lavergne  1   3 , Olivier Piot  16 , Christine Alonso  17 , Antoine Leenhardt  18 , Nicolas Lellouche  6   19 , Fabrice Extramiana  6   18 , Alain Cariou  2   3   20 , Xavier Jouven  1   2   3 , Eloi Marijon  1   2   3   6 ; Paris-SDEC investigators
Affiliations

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

Victor Waldmann et al. Eur Heart J. .

Abstract

Aims: Recent 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 results: Data 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.

Conclusion: Our 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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Figures

Figure 1
Figure 1
Flow chart of the study. ACS, acute coronary syndrome; ARVC, arrhythmogenic right ventricular cardiomyopathy; CPVT, catecholaminergic polymorphic ventricular tachycardia; IVF, idiopathic ventricular fibrillation; LQTS, long QT syndrome; SCA, sudden cardiac arrest; WPW, Wolff–Parkinson–White syndrome.
Figure 2
Figure 2
Yield of different aetiological examinations. ARVC, arrhythmogenic right ventricular cardiomyopathy; CAD, coronary artery disease; CPVT, catecholaminergic polymorphic ventricular tachycardia; DCM, dilated cardiomyopathy; ECG, electrocardiogram; HCM, hypertrophic cardiomyopathy; SCA, sudden cardiac arrest; TTE, transthoracic echocardiography.
Figure 3
Figure 3
Unexplained sudden cardiac arrest (‘idiopathic ventricular fibrillation’) rate by number of additional examinations after the initial workup (electrocardiogram, echocardiography, and coronary angiography) and during follow-up. *Cardiac MRI, ergonovine challenge, and pharmacological tests (ajmaline or cathecolamine) were considered for the initial evaluation. The three additional diagnoses made during the follow-up were revealed by two ergonovine challenges (coronary vasospasms) and one exercise testing (long QT syndrome). IVF, idiopathic ventricular fibrillation.
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