Repetitive syncopal episodes in a child with documented ventricular tachycardia, early repolarization pattern in leads I an aVL, Brugada syndrome, and fever

Ann Noninvasive Electrocardiol. 2019;00:e12698. wileyonlinelibrary.com/journal/anec | 1 of 6 https://doi.org/10.1111/anec.12698 1 | INTRODUCTION Brugada syndrome (BrS) is an inherited arrhythmic disorder that may result in sudden cardiac death (SCD), and it is estimated to account for 4% of all SCDs and for 20% of all SCDs in patients with an appar‐ ently normal heart. The first arrhythmic event (AE) typically occurs in male patients aged 40 to 50 years, but rarely during childhood. According to the Survey on Arrhythmic Events in Brugada Syndrome Received:22May2019 | Accepted:1July2019 DOI: 10.1111/anec.12698 CASE REPORT Repetitive syncopal episodes in a child with documented ventricular tachycardia, early repolarization pattern in leads I an aVL, Brugada syndrome, and fever Humberto Rodriguez-Reyes MD, FACC, FHRS1 | Andrés Pérez-Riera MD2 | Brenda Lucia López MD1 | Maria del Refugio Salinas MD1 | Luz Ma Mayela Muñoz MD1 | Cesar Ivan Laguna MD1 | Nikus Kjell MD3 1Sociedad Cardiovacular y Arritmias (SOCAyA), Aguascalientes, Méxic, o 2Laboratório de Metodologia de Pesquisa e Escrita Científica, Faculdade de Medicina do ABC, São Paulo, Brazil 3Heart Center, Tampere University Hospital and Faculty of Medicine and Health Technology, Tampere University, Tampere, Finland Correspondence Rodríguez Reyes Humberto , Sociedad Cardiovascular y Arritmias (SOCAyA), Rep. de Argentina 123, Fracc. Las Américas, CP 20230, Aguascalientes, México. Email: humbertorodrigu[email protected] Abstract We present a small child with febrile peaks and syncopal episodes secundary to ven‐ tricular tachycardia, in whom it was eventually possible to demostrate the Brugada Syndrome with a special presentation in the ECG; early repolarization pattern in lead I and a aVL and Brugada pattern during fever in V1‐V2. This is, to our knowledge, tha first case with this special ECG presentation in a small child. KEYWORDS Brugada syndrome, early repolarization, fever, syncope, ventricular tachycardia FIGURE 1 Ventricular tachycardia This is an open access article under the terms of the Creative Commons Attribution‐NonCommercial‐NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made. © 2019 The Authors. Annals of Noninvasive Electrocardiology published by Wiley Periodicals, Inc. 2 of 6 | HUMBERTO ET al. (SABRUS) data, in only 4.3% the first AE occurs in the pediatric population (Milman et al., 2017). The Brugada syndrome ECG is much more prevalent in patients with fever than in afebrile controls (Michowitz et al., 2018). We present a small child with febrile peaks and syncopal epi‐ sodes, in whom it was eventually possible to demonstrate the Brugada syndrome with a special presentation in the ECG: early re‐ polarization pattern in leads I and aVL and Brugada pattern during fever in V1‐V2. 2 | CASE REPORT Our case is a 2‐year 6 months old boy, weight 14 kg with a family his‐ tory of diabetes mellitus and hypertension in paternal grandparents and uncles. A cousin had arrhythmias of unknown origin, but without syncope or other alarming symptoms. There was no history of sud‐ den death in the first‐degree relatives. At 1 year of age, he presented with a sudden episode (3 hr) of flaccidity, diaphoresis, cyanosis, polypnea, with spontaneous FIGURE 2 Nodalrhythmwithhighprecordialleads(markedasleadsV1‐V6below),noteJ‐waveelevationinI,aVLandhighV2 | 3 of 6 HUMBERTO ET al. recovery. In the emergency medical service, he was diagnosed with an upper respiratory tract infection. In a short period of time, he was diagnosed with a sob spasm, and repeated episodes were observed on at least five occasions within 1 year, and they were accompanied by cyanosis and diaphoresis. Twelve hours before the current admission, he presented a new episode of flaccidity, diaphoresis, cyanosis, polypnea, seizures, and prolonged hypothermia (approximately 3 hr) with spontaneous im‐ provement. In the following morning, the parents noticed prolonged symptoms (several hours) of diaphoresis, flaccidity, and polypnea. The attending physician directed him to the intensive care unit, where the ECG‐1 (Figure 1) was performed. It showed ventricular tachycar‐ dia. Propranolol 30 mg orally was administered. After 30 min, during the placement of a central catheter the ECG‐2 (Figure 2) was per‐ formed, and it showed reversal of the tachyarrhythmia observed in ECG‐1. The nodal rhythm lasted for 8 hr, probably due to the effect of the beta‐blocker. After that, sinus rhythm ensued (Figure 3). Rx thorax and echocardiogram were considered normal. In the after‐ noon, a 12 lead 24‐hr Holter was performed during a febrile peak of 38 degrees Celsius (Figure 4). FIGURE 3 Sinusrhythmwithhighprecordialleads(below),noteJ‐waveelevationinaVLandhighV2 4 of 6 | HUMBERTO ET al. 3 | DISCUSSION BrS is an inherited arrhythmic disorder that may result in SCD. The first AE of malignant ventricular arrhythmias are documented either at patient presentation because of aborted sudden cardiac arrest (SCA) or after prophylactic implantable cardioverter‐de‐ fibrillator (ICD) implantation. The BrS ECG is much more preva‐ lent in patients with fever than in afebrile controls with a high FIGURE 4 TwelveleadHoltermonitoringduringfebrilepeak(38°C),noteJ‐waveelevationinleadsIandaVLandthepresenceofan alternant Brugada pattern in V2 | 5 of 6 HUMBERTO ET al. prevalence in children aged 0–5 years (Adler et al., 2013). Although the risk of fever‐related AE in BrS markedly varies according to age group, sex, and ethnicity, with a high prevalence of fever and AEs in the Caucasian population, where there is a high proportion of patients with an SCN5A mutation (77%) (Michowitz et al., 2018). The principal mechanism of AEs during fever is the worsening of the biophysical properties of the defective ion channels at higher temperatures, leading to further loss of function of the sodium channel current (Dumaine et al., 1999). The Brugada type 1 pattern and the early repolarization pat‐ tern (ERP) are two ECG patterns characterized by the existence of Jwaves.Someexpertshavearguedthatbasedontheirsimilarities in terms of demographics, ECG signatures, and response to neuro‐ modulation, ERP and BrS represent parts of a continuous spectrum of phenotypic expression, and classification under the umbrella term of “J‐wave syndrome” was proposed (Antzelevitch & Yan, 2010). Inferolateral ERP occurs relatively frequently spontaneously in BrS, and these patients have a more severe phenotype (Kawata et al., ). Class I antiarrhythmic drug (AAD) administration provokes an inferolateral coved BrS pattern in 4.6% of patients (Sarkosy et al.,2009).OurcaseshowedararepresentationofJ‐waveelevation in leads I and aVL in association with the Br pattern in V1, V2, and in high V2. In young patients, the presence of the spontaneous Brugada type 1 ECG pattern and symptoms at diagnosis are predictors of life‐threatening arrhythmias (LTA). The management of BrS should be age‐specific, and prevention of SCD may involve genetic test‐ ing and aggressive use of antipyretics and quinidine, with risk‐ specific consideration for ICD implantation (Andorin et al., 2016). Provocative drug testing during childhood had a low yield for pre‐ dicting future AEs and was associated with serious complications in some patients. In addition, Conte et al (Conte et al., 2014) found that in 23% of children (aged 11.4 ± 2.5 years) with a negative ajmaline challenge test, BrS will be unmasked when the test is re‐ peated several years later (age, 20.9 ± 4.2 years). Therefore, in as‐ ymptomatic patients with periodical normal ECG, adopting a cutoff of 16 years for sodium channel blocker challenge would be reason‐ able and in agreement with the cutoff of 15 years suggested by Andorin et al (Andorin et al., 2016). Quinidine can be used safely until adult age and posture are achieved. Quinidine also proved to be effective during relatively short follow‐up and can be proposed as a valid alternative or as a bridge to ICD implantation (Probst et al., 2007). Concomitant epicardial right ventricular outflow tract ablation and ICD implantation with epicardial leads can be a safe, feasible, and effective approach for symptomatic BrS patients (de Asmundis et al., 2018). ICD therapy is an effective strategy in young patients with symptomatic BrS, treating potentially lethal arrhythmias in >25% of patients during follow‐up. Appropriate shocks were significantly associated with previously aborted SCD and spontaneous type I ECGs. However, 20% of the patients ex‐ perienced inappropriate shocks. Of these, 29% were secondary to supraventricular tachycardia, 42% to lead fracture and 29% to T‐wave oversensing (Gonzalez Corcia et al., 2018). 4 | CONCLUSIONS We present a young boy, who probably had ventricular tachycardia associated with fever since 1 year of age. The ECG presentation was special with ERP in leads I and aVL and BrS‐type spontaneous ST el‐ evation during fever in V1 and V2. This is, to our knowledge, the first case with this special ECG presentation in a small child. 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