Syncope or seizure: that is the question—case report of a young patient with convulsive cardioinhibitory syncope treated with cardioneuroablation (2024)

Rachel M A ter Bekke, Handling Editor, Dmitry Duplyakov, Editor, Peter Calvert, Editor, and Elton Luo, Editor

Associated Data

Supplementary Materials

Data Availability Statement

Abstract

Keywords: Case report, Reflex cardioinhibitory syncope, Seizure, Ictal asystole, Cardioneuroablation

Introduction

Reflex syncope is the most common cause of syncope and a frequent reason for emergency visits. Given its benign nature, education and reassurance are the cornerstones of management. Convulsive movements may appear during the transient loss of consciousness (TLOC), leading in some cases to a misdiagnosis of seizure, which has therapeutic implications. In patients with severe or recurrent forms of syncope, additional treatment may be necessary. Pacemaker implantation should be considered in patients older than 40 years with recurrent cardioinhibitory reflex syncope. However, in well-selected younger patients, cardioneuroablation (CNA) is emerging as an alternative strategy. We present a case of a patient with cardioinhibitory reflex syncope treated with CNA.

Summary figure

Case summary

A previously healthy 24-year-old woman was admitted to our hospital after four episodes of TLOC of unknown origin within 3 months. The episodes were mostly situational, while showering or hair-combing with full alertness and awareness afterwards, suggesting a vagal aetiology. No other syncopal episodes were reported in the history. The patient was not taking any prescribed medication at the time of admission, and the family history was negative for sudden cardiac arrest.

The physical examination revealed normal heart sounds, and the blood pressure and the heart rate were within the normal range. The neurological assessment did not reveal any sensory or motor deficits and an orthostatic challenge was conducted, which showed no significant drop in blood pressure. The electrocardiogram (ECG) and the laboratory findings on admission were unremarkable. The echocardiography showed a structurally normal heart.

Due to shaking arms and tongue biting during the last episode, the patient was also evaluated for possible epilepsy. The brain magnetic resonance imaging did not show any relevant structural abnormalities. The first electroencephalogram (EEG) at rest showed pathological repetitive short epochs of high amplitude generalized slowing. To address this, an EEG under photostimulation was performed. During photostimulation, the simultaneously registered ECG showed a progressive bradycardia, which turned into a complete atrioventricular block and sinus arrest with asystole for 14 s. The patient developed a TLOC followed by three bilateral brachial myoclonia. After the complete atrioventricular block, the EEG showed during the TLOC a generalized slow-fast-slow pattern (Figures 1 and ​22). Thereafter, the patient immediately regained consciousness without sequelae.

The differential diagnoses included epilepsy with ictal asystole or a convulsive reflex syncope. A psychogenic form of TLOC was excluded based on the ECG and EEG findings. We interpreted the episode most likely as a cardioinhibitory convulsive syncope, due to the sequence of asystole and EEG findings typical of cerebral anoxia ending with TLOC. However, due to the repetitive pathological findings in the EEG at rest, an underlying epilepsy could not be fully excluded at the time. Therefore, we started an additional antiseizure therapy with levetiracetam, followed by lamotrigine.

Considering the risk of repetitive asystole, pacemaker implantation was initially discussed. However, this strategy was considered inappropriate for a young patient due to the potential long-term risks such as repeated device replacements, risk for lead failures, and infections. As an alternative, a CNA was discussed with the patient, who agreed to undergo the procedure. This was performed over a biatrial, transseptal approach.

In the left atrium, we targeted the right superior ganglionated plexus (GP) anterior to the right superior pulmonary vein and the right inferior GP adjacent to the interatrial groove. These GPs were re-ablated from the right atrium with additional ablation at the right atrial/superior vena cava junction and of the posteromedial left GP posteriorly to the coronary sinus ostium under close monitoring of the phrenic nerve (Figure 3 and Supplementary material online, Videos S1–S3). A loop recorder was implanted after ablation. After a 72 h observation period without complications, the patient was discharged with a prescription of oral anticoagulation for 2 months and proton pump inhibitor for 6 weeks in addition to the antiepileptic therapy.

A 24 h Holter ECG at 3 months showed a reduced heart rate (HR) variability—index (from 37.7 to 14.8) with a mean HR increase from 70 to 99 b.p.m. suggesting that the ablation was effective in reducing cardiac vagal tone. After 6 and 12 months, the Holter ECGs showed a trend to higher HR variability with a lower mean HR, suggesting a possible reinnervation of some GPs (Figure 4). The EEG at 2.5 months after CNA showed very rarely short epochs of focal slowing in the right parieto-occipital leads. The patient autonomously opted to discontinue the antiseizure medication without medical guidance at 10 months. The EEG follow-up at 11 months continued to display short epochs of paroxysmal generalized slowing without clinical correlations and absence of unambiguous epileptiform discharges. After 1 year of follow-up, the patient remained asymptomatic, without syncopal episodes.

Discussion

Differentiating between convulsive reflex syncope and ictal asystole is fundamental for subsequent treatment decisions. Patient history can provide useful details, like the presence and nature of triggers and prodromes or the duration of unconsciousness and of the confusion after the episode. Myoclonic jerks during syncope can lead to a misdiagnosis of seizure. These movements are usually few, irregular, and asymmetrical in syncope. According to current European guidelines,¹ due to its low diagnostic yield, EEG should not be routinely used in the assessment of TLOC when syncope is the most likely cause, as a normal resting EEG cannot rule out epilepsy and this may trigger other examinations and generate higher healthcare costs. However, when clinical data are equivocal, an EEG can be useful to distinguish among syncope, pseudosyncope, and epilepsy. During seizures, typical EEG-discharges are recorded, whereas during syncope, the EEG generally shows diffuse slow activity and flattening up to an isoelectric EEG,² as was in our case (Figure 1B). Due to the limited number of reported cases of syncope induced by light stimulation, the role of photostimulation during EEG in the context of reflex syncope remains unclear. Tilt testing is also useful to demonstrate susceptibility of the patient to reflex syncope and may be helpful in separating syncope with abnormal movements from epilepsy.³

Usually, medical treatment for reflex syncope is not required and reassuring the patient is sufficient. However, when syncopal events recur and impair quality of life, therapeutic options like counter pressure manoeuvers, salt and fluid intake, midodrine or fludrocortisone may be considered.¹ In case of cardioinhibitory reflex syncope, cardiac pacing should only be considered in highly selected patients i.e. those >40 years of age with recurrent episodes and high risk of injury. In younger patient populations (≤40 year), there is currently insufficient evidence for the use of pacing.⁴

Since 2005, CNA has emerged as a new treatment for vasovagal cardioinhibitory syncope.⁵ By endocardial radiofrequency ablation in the area of GPs, CNA reduces vagal tone and reflectory cardioinhibition. Different ablation strategies, including anatomically guided, electrogram guided, bi-, and only right-atrial have been described, with syncope free survival rates of 6.9–10.6% in observational studies.⁶ Recently, the first randomized control trial compared CNA and optimal non-pharmacological treatment in young patients with cardioinhibitory reflex syncope. After 2 years of follow-up, syncope recurrence in the CNA group was significantly lower than the control group (8% vs. 54%; P = 0.0004).⁷ However, multicentre, long-term studies with sham controls are still needed to investigate the role of vagal reinnervation.

Supplementary Material

Acknowledgements

The authors would like to thank the patient for allowing us to present the case. We also thank PhD Sven Knecht, Prof. Stephan Rüegg, Prof. Michael Kühne, and Prof. Christian Sticherling for useful discussion and comments. We would like to thank Joanne Lee for proofreading the manuscript.

Contributor Information

Andrea Papa, Department of Cardiology and Cardiovascular Research Institute Basel, University Hospital Basel, Basel, Switzerland.

Urs Fisch, Department of Neurology, University Hospital Basel, Basel, Switzerland.

Stefano Bassetti, Division of Internal Medicine, University Hospital Basel and University of Basel, Basel, Switzerland.

Patrick Badertscher, Department of Cardiology and Cardiovascular Research Institute Basel, University Hospital Basel, Basel, Switzerland.

Philipp Krisai, Department of Cardiology and Cardiovascular Research Institute Basel, University Hospital Basel, Basel, Switzerland.

Lead author biography

Andrea Papa is a cardiology resident at the Heart Center of the University Hospital Basel with a broad interest in different subspecialties.

Supplementary material

Supplementary material is available at European Heart Journal – Case Reports online.

Consent: The patient undersigned a consent form for the anonymous publication of images and/or information about the case according to the COPE guidelines. Open and continuous communication with the patient allowed a trusting relationship with the physician and the patient never felt exposed to unnecessary risks.

Funding: This research received no external funding.

Data availability

The data underlying this article are available in the article and in its online Supplementary material.

References