ER is common, occurring in 1% to 5% of the general population [1]. It is more common in young adults (particularly men) and athletes. The ECG pattern is defined as the presence of a J-point elevation ≥1 mm in ≥2 contiguous inferior and/or lateral leads with or without ST-elevation, either as a QRS slurring (an abrupt change in the slope of the last deflection) or notching (a low-frequency deflection at the end of the QRS complex) [5]. These criteria are fulfilled in our patient as illustrated in Fig. 1a. ER syndrome (ERS) is diagnosed in the presence of this ECG pattern and resuscitation of otherwise unexplained VF or polymorphic VT [5].
Historically ER was considered benign. Population based studies by Tikkanen et al. [1] then showed a higher rate of SCD among patients with ER, case-control studies by Haïssaguerre et al. [2] found an increased prevalence of ER in patients with idiopathic VF (31%–42%) compared with healthy control subjects (5%–13%). This was true for J-point elevations in the inferior leads and leads I to aVL, but not in leads V4–V6, where the frequency was similar. Importantly, subjects with ER and idiopathic VF were more likely to have sustained a cardiac arrest during sleep, which was also the case in our patient. Haïssaguerre et al. [2] noted an increase in the amplitude of ER prior to an arrhythmic period. This finding was consistent with a preliminary analysis [6] of four patients with ER and idiopathic electrical storm, in which an unique electrocardiographic signature was found. A baseline ER pattern with dramatic but transient accentuation of J waves across the precordial and limb leads before the development of the electrical storm, which was precipitated by relatively short-coupled PVCs, was observed in all four patients. The same electrocardiographic signature was seen in our patient: the ER pattern was most pronounced immediately before the electrical storm, with subsequent ECG recordings after the electrical storm demonstrating a progressive decrease in J-wave height and slurring, respectively notching (Fig. 1a vs. b).
With regard to risk stratification, Antzelevitch et al [3] described three subtypes of ER with a varying risk profile: in type 1, the ER pattern is limited to the lateral precordial leads. It is typically seen in healthy male athletes and has the lowest risk of malignant arrhythmias. Type 2 shows ER in the inferior and inferolateral leads and is associated with a greater risk of malignant arrhythmias and type 3 shows ER pattern in all ECG leads and has the highest risk of malignant arrhythmias and electrical storms. The ER pattern of our patient was most likely compatible with type 2. In addition to the location, a J-point elevation of >0.2 mV was previously shown to be linked to a significant risk of death from cardiac arrhythmias [1]. And also distinct T-wave features have been used for the risk stratification in ER patients [7].
Different triggers leading to an electrical storm can be discussed. Recent reports point to an association between ER and the development of VF in the setting of hypothermia [8,9,10]. Current guidelines recommend a targeted temperature management protocol to prevent neurological damage following a cardiac arrest. In our patient, a target temperature of 36 °C was used. Electrical storm started and exacerbated after initiation of cooling and no arrhythmias occurred after it was stopped, suggesting a temporal relationship between hypothermia and increased frequency of ventricular arrhythmias in our patient. Although Brugada syndrome and ER differ in many aspects, a possible relationship to temperature is suspected in both entities, as fever appeared to be proarrhythmic in Brugada patients [11].
The finding of an LAD originating from the right sinus of Valsalva is extremely rare (0.03%) [12]. In general, this subtype of an anomalous origination of a coronary artery from the opposite sinus (ACAOS) is associated with congenital heart disease, e.g. tetralogy of Fallot. The anatomical variant with an interarterial course of the LAD between the ascending aorta and the pulmonary trunk is considered high risk for myocardial ischemia or sudden death as well [13]. The mechanism of sudden death is believed to be due to transient occlusion of the LAD caused by an increase in blood flow through the aorta and the pulmonary artery, resulting in kinking or pinching of the artery [13]. In our case, an interarterial course was present. However, before admission, the patient was asymptomatic without any complaints during daily activity and regular exercise and the VF arrest occurred while sleeping, making ACAOS and resulting myocardial ischemia a very unlikely cause of his VF arrest. Owing to a lack of clear guideline recommendations, the management of patients with ACAOS remains fraught with uncertainty. Of note, there are no controlled studies that have evaluated the outcome of surgical repair in asymptomatic individuals. We decided that further risk stratification with myocardial perfusion imaging stress testing (MPI) was warranted. The MPI was performed four weeks after the hospitalization and showed no signs of ischemia.
Different therapeutic management options for electrical storms in ERS patients can be discussed. For the acute phase, beta-blockers, verapamil and lidocaine/mexiletine are generally ineffective, while amiodarone may be partially effective (3/10 patients) [14]. The most effective strategy in the acute phase of electrical storms however seems the use of isoproterenol infusion or pacing at rapid heart rates to suppress the PVC’s [14]. In addition, Isoproterenol is thought to be effective probably by boosting L-type calcium channel current and thus decreasing electrical gradient [3]. The efficacy of isoproterenol infusion has additionally been reported in a case report of an electrical storm occurring in a patient with Brugada syndrome [15]. For the long-term prevention of recurrent VF in ERS patients, quinidine is the drug of choice [14]. Catheter ablation for idiopathic VF targets the short coupled PVC triggers. In experienced hands, it can have a good long-term success in preventing VF recurrence [16]. However, presence of the PVC’s during the procedure is required for mapping and successful ablation and recurrent VF episodes may be induced during ablation due to triggered automaticity. Therefore, quinidine is often chosen as the first line option for long-term suppression and catheter ablation used in patients with recurrent electrical storms in ERS patients. Importantly, due to the limited data with regard to sample size and follow-up duration [14, 16], neither successful catheter ablation nor quinidine should be considered alternatives to the implantation of an ICD for secondary prevention of SCD. In summary, ER is associated with idiopathic VF and hypothermia appears proarrhythmic in patients with ER and idiopathic VF. It remains uncertain whether additional avoidable triggers of arrhythmogenesis exist in patients with ER. Interventions shown to be effective are isoproterenol and quinidine. Further research of the genetics and mechanisms involved are required.
