Electrocardiogram Pattern Research Articles

Wellen’s syndrome caused by severe ostial stenosis of the left main coronary artery: a case report

BackgroundWellen’s syndrome may indicate severe stenosis or even occlusion of the proximal left anterior descending coronary artery. It may progress to acute myocardial infarction. Early recognition and an early invasive strategy are critical to avoiding impending myocardial injury. To our knowledge, only one case of Wellen’s electrocardiogram patterns caused by left main coronary artery (LM) lesions has been reported.Case presentationA 61-year-old man with a history of hypertension and smoking presented to the emergency department complaining of paroxysmal chest pain for the preceding 6 days. In the present case, the T-wave symmetric inversions in the V2 and V3 leads is associated with severe ostial stenosis of LM, which appears similar to Wellen’s type B pattern.ConclusionsPractitioners should familiarize themselves with such uncommon LM lesions patterns and perform emergent reperfusion therapy.

Ferroelectric Perovskite/MoS2 Channel Heterojunctions for Wide-Window Nonvolatile Memory and Neuromorphic Computing.

Ferroelectric materials commonly serve as gate insulators in typical field-effect transistors, where their polarization reversal enables effective modulation of the conductivity state of the channel material, thereby realizing non-volatile memory. Currently, novel 2D ferroelectrics unlock new prospects in next-generation electronics and neuromorphic computation. However, the advancement of these materials is impeded by limited selectivity and narrow memory windows. Here, new concepts of 2D ferroelectric perovskite/MoS2 channel heterostructures field-effect transistors are presented, in which 2D ferroelectric perovskite features customizable band structure, few-layered ferroelectricity, and submillimeter-size monolayer wafers. Further studies reveal that these devices exhibit unique charge polarity modulation (from n- to p-type channel) and remarkable nonvolatile memory behavior, especially record-wide hysteresis windows up to 177V, which enables efficient imitation of biological synapses and achieves high recognition accuracy for electrocardiogram patterns. This result provides a device paradigm for future nonvolatile memory and artificial synaptic applications.

Coronary Angiographic Features of de Winter Syndrome: More Than Just Occlusion of the Left Anterior Descending Artery.

The de Winter electrocardiogram (ECG) pattern is a rare presentation of ST-segment elevation myocardial infarction (STEMI) equivalent. The clinical profile of de Winter syndrome remains to be clarified. Medical records of 1865 consecutive patients with acute myocardial infarction admitted from November 2018 to July 2023 were screened. Twelve patients (0.6%) with de Winter syndrome were included. STEMI patients whose culprit vessel was the left anterior descending artery (LAD) but without de Winter ECG pattern were selected as controls after 1:2 matching for age and sex. The de Winter syndrome patients were all male, aged 49.0 (46.3-52.5) years. The culprit lesion was the proximal LAD in seven patients (58.3%), the middle LAD in three (25%), the left main coronary artery in one (8.3%), and the ramus intermedius artery in the other. All of their culprit lesions had TIMI Thrombus Grade < 4, Cohen-Rentrop Score ≤ 2, and residual stenosis ≥ 80% after pretreatment with thrombus aspiration or balloon predilatation. Intracoronary imaging data were available in four patients, showing severe atherosclerotic stenosis. Compared with STEMI, de Winter syndrome had a higher prevalence of prior recurrent angina (75.0% vs. 37.5%, p = 0.034), better coronary collateralization (Cohen-Rentrop Score: 1 vs. 0, p = 0.001), lower thrombus burden (TIMI grade: 1 vs. 2, p = 0.005) but more severe atherosclerotic stenosis in the culprit lesions (90% vs. 60%, p < 0.001). The culprit lesions of de Winter syndrome have a low thrombus burden, severe atherosclerotic stenosis, and poor collateral circulation protection.

Neurons to heartbeats: spiking neural networks for electrocardiogram pattern recognition

Neurons to heartbeats: spiking neural networks for electrocardiogram pattern recognition

Epicardial ablation in patients with Brugada syndrome: a single centre experience

Abstract Introduction Brugada syndrome (BrS) is a rare disease associated with an increased risk of ventricular fibrillation (VF), ventricular tachycardia (VT), and sudden cardiac death (SCD). The standard prevention of SCD in patients with BrS is the implantation of an implantable cardioverter-defibrillator (ICD), especially in patients who have previously suffered cardiac arrest or arrhythmogenic syncope. Catheter ablation is an effective method to prevent recurrences of VF/VT in these patients. Purpose Retrospective analysis of the results of catheter ablation by epicardial approach and genetic testing in patients with a diagnosis of BrS and repeated adequate ICD interventions. Methods In 2013-2024, catheter ablation by epicardial approach was performed in our centre in 9 patients diagnosed with Brugada syndrome (mean age 41±6 years, nine males, left ventricular ejection fraction 57±4%). All patients had an ICD implanted for secondary prevention of SCD and presented with adequate ICD therapies (mean 9±11 shocks/patient in the last six months before catheter ablation). Before catheter ablation, two patients were treated with quinidine. Patients underwent cardiogenetic testing by next-generation sequencing of 228 genes. Results Type 1 electrocardiogram (ECG) pattern was present at baseline ECG in four (44%) patients; in five (56%) patients, type 1 was induced after ajmaline administration. Genetic testing was performed in seven patients, and only one patient (14%) was found to have a relevant pathogenic mutation in the SCN5A gene. Prior to ablation, sustained VT/VF could be induced by programmed electrical stimulation in three patients (33%). Epicardial ablation was targeted at areas of abnormal electrograms located over the right ventricular outflow tract. No complications were noted. The mean follow-up period was 25±30 months. In one patient (11%), reablation was required to suppress ventricular arrhythmias successfully. After the last ablation, no recurrence of VT/VF was observed in any patient. Conclusions Epicardial ablation is an effective method that leads to the suppression of ventricular arrhythmias in patients with a diagnosis of BrS and repeated ICD interventions. Despite the malignant arrhythmic phenotype, patients in our cohort had a rather low prevalence of signs associated with a high risk of VT/VF, such as spontaneous ECG pattern of BrS, inducibility of VT/VF during electrical programmed stimulation, or the presence of a causative genetic mutation.

Prevalence and associated factors of ECG abnormality patterns indicative of cardiac channelopathies among adult general population of Tehran, Iran: a report from the Tehran Cohort Study (TeCS)

BackgroundThe characteristics of electrocardiogram (ECG) abnormalities related to cardiac channelopathies potentially linked to sudden cardiac death (SCD) are not widely recognized in Iran. We examined the prevalence of such ECG patterns and their related factors among adult residents of Tehran, Iran.MethodsThe clinical characteristics and 12-lead ECGs of Tehran Cohort Study participants were examined. Long QT intervals, short QT intervals, Brugada syndrome (BrS) patterns, and early repolarization (ER) were evaluated using computer-based assessment software validated by cardiologists. Logistic regression models were employed to identify the factors associated with the prevalence of different ECG patterns.ResultsOut of 7678 available ECGs, 7350 were included in this analysis. Long QT interval, ER pattern, BrS patterns, and short QT interval were found in 3.08%, 1.43%, 0.31%, and 0.03% of participants, respectively. The prevalence of long QT interval increased with age, opium consumption, and presence of hypertension. Younger age, lower body mass index (BMI), alcohol use and male sex were independently linked to an elevated prevalence of ER pattern. Most individuals with BrS patterns were men (95%) and had lower BMI, high- and low-density lipoprotein, and total cholesterol compared to those without the BrS pattern. At a mean follow-up of 30.2 ± 5.5 months, all-cause mortality in the group exhibiting abnormal ECG patterns (6.3%) was approximately twice as high as that in the group without such patterns (2.96%).ConclusionAbnormal ECG patterns corresponding to channelopathies were relatively rare among adult residents of the Tehran population, and their prevalence was influenced by various factors.Clinical trial numberNot applicable.

Sudden cardiac death due to adrenal insufficiency masquerading as Brugada syndrome. Case report

Sudden cardiac death in young people in 20% of cases is caused by cardiomyopathies and channelopathies. One of the forms of channelopathies is Brugada syndrome, a hereditary disease characterized by ST segment elevation in the right precordial leads (V1-V3) and an increased risk of sudden cardiac death in the absence of structural heart disease. Brugada phenocopies are also known – clinical situations that are manifested by electrocardiogram (ECG) patterns identical to true Brugada syndrome. They are caused by different clinical circumstances and form a group of heterogeneous conditions that are often difficult to distinguish from true congenital Brugada syndrome due to identical ECG patterns. The formation of Brugada phenocopy due to hyperkalemia is presented in the literature in various conditions: with renal failure, after extensive trauma, application of medications. The article presents a case report demonstrating a rare cause of sudden cardiac arrest in a young patient without a history of cardiovascular pathology: the occurrence of the Brugada pattern on the ECG due to severe hyperkalemia in adrenal insufficiency. The stages of the differential diagnostic search are described, which made it possible to verify the final diagnosis and prescribe effective hormone replacement therapy. Performing a provocative test with novocanamide allowed us to confirm that the patient had a phenocopy, and not Brugada syndrome. Differential diagnosis of phenocopies of Brugada syndrome – a series of often life-threatening cardiac and non-cardiac diseases and conditions, manifested by similar ECG changes in the form of a peculiar ST segment elevation in leads V1-V3, is often a difficult task. This case represents a phenocopy of Brugada in the setting of severe hyperkalemia with development of cardiac arrest due to adrenal insufficiency, which resolved with correction of electrolyte abnormalities and treatment of the underlying disease. Typical ECGs are presented: a graph of the Brugada phenomenon, hyperkalemia, a sinusoidal curve during cardiac arrest, recorded over time in a patient, and the pathogenetic mechanisms causing the formation of the Brugada pattern in adrenal insufficiency are explained.

Abnormal recognition-assisted and onset-offset aware network for pathological wearable ECG delineation

Electrocardiogram (ECG) delineation is essential to the identification of abnormal cardiac status, especially when ECG signals are remotely monitored with wearable devices. The complexity and diversity of cardiac conditions generate numerous pathological ECG patterns, not only requiring the recognition of normal ECG but also addressing an extensive range of abnormal ECG patterns, posing a challenging task. Therefore, we propose an abnormal recognition-assisted network to integrate supplementary information on diverse ECG patterns. Simultaneously, we design an onset-offset aware loss to enhance precise waveform localization. Specifically, we establish a two-branch framework where ECG delineation serves as the target task, producing the final segmentation results. Additionally, the abnormal recognition-assisted network serves as an auxiliary task, extracting multi-label pathological information from ECGs. This joint learning approach establishes crucial correlations between ECG delineation and associated ECG abnormalities. The correlations enable the model to demonstrate sufficient generalization in the presence of diverse abnormal ECG patterns. Besides, onset-offset aware loss focuses intensively on wave onsets and offsets by applying biased weights to various waveform positions. This approach ensures a focus on precise localization, facilitating seamless integration into cross-entropy loss function. A large-scale wearable 12-lead dataset containing 4,913 signals is collected, offering an extensive range of ECG data for model training. Results demonstrate that our method achieves outstanding performance on two test datasets, attaining sensitivity of 94.97% and 94.27% and an error tolerance lower than 20 ms. Furthermore, our method is effective for various aberrant ECG signals, including ST-segment changes, atrial premature beats, and right and left bundle branch blocks.

CardioGuard: AI-driven ECG authentication hybrid neural network for predictive health monitoring in telehealth systems

The increasing integration of telehealth systems underscores the importance of robust and secure methods for patient data management. Traditional authentication methods, such as passwords and PINs, are prone to breaches, underscoring the need for more secure alternatives. Therefore, there is a need for alternative approaches that provide enhanced security and user convenience. Biometric-based authentication systems uses individuals unique physical or behavioral characteristics for identification, have emerged as a promising solution. Specifically, Electrocardiogram (ECG) signals have gained attention among various biometric modalities due to their uniqueness, stability, and non-invasiveness. This paper presents CardioGaurd, a deep learning-based authentication system that leverages ECG signals—unique, stable, and non-invasive biometric markers. The proposed system uses a hybrid Convolution and Long short-term memory based model to obtain rich characteristics from the ECG signal and classify it as authentic or fake. CardioGaurd not only ensures secure access but also serves as a predictive tool by analyzing ECG patterns that could indicate early signs of cardiovascular abnormalities. This dual functionality enhances patient security and contributes to AI-driven disease prevention and early detection. Our results demonstrate that CardioGaurd offers superior performance in both security and potential predictive health insights compared to traditional models, thus supporting a shift towards more proactive and personalized telehealth solutions.

Brugada syndrome precipitated by uncomplicated malaria treated with dihydroartemisinin piperaquine: a case report

BackgroundCardiovascular events following anti-malarial treatment are reported infrequently; only a few studies have reported adverse outcomes. This case presentation emphasizes cardiological assessment of Brugada syndrome, presenting as life-threatening arrhythmia during anti-malarial treatment. Without screening and untreated, this disease may lead to sudden cardiac death.Case presentationThis is a case of 23-year-old male who initially presented with palpitations followed by syncope and shortness of breath with a history of malaria. He had switched treatment from quinine to dihydroartemisinin-piperaquine (DHP). Further investigations revealed the ST elevation electrocardiogram pattern typical of Brugada syndrome, confirmed with flecainide challenge test. Subsequently, anti-malarial treatment was stopped and an Implantable Cardioverter Defibrillator (ICD) was inserted.ConclusionsAnother possible cause of arrhythmic events happened following anti-malarial consumption. This case highlights the possibility of proarrhytmogenic mechanism of malaria infection and anti-malarial drug resulting in typical manifestations of Brugada syndrome.

Significance of Wellens Pattern in Electrocardiogram Revisited – Electrocardiogram and Angiographic Correlation

Abstract Background: In patients with non-ST elevation acute coronary syndrome (NSTEACS), electrocardiogram (ECG) may show deep or biphasic T-wave inversions in precordial leads. Previous reports had linked this ECG change with a lesion in the left anterior descending artery (LAD). However, it is often overlooked in the emergency department and is not included in the major scoring systems for risk stratification of acute coronary syndrome. Objectives: We assessed coronary angiogram (CAG) lesions in patients with biphasic T inversion and/or deep T inversions by ECG. Methods: From July 2019 to November 2021, ECGs of all NSTEACS patients were analyzed and 202 patients who satisfied the criteria were chosen. CAGs were analyzed for the site, severity, and type of lesion. Treatment modality advice was noted. Results: One hundred and ninety-five patients had severe disease in the LAD, of which 178 patients had a lesion proximal to the first septal perforator branch. LAD lesions were discrete in 98, tubular in 68, diffuse in 25, and total occlusion in 11 patients. One hundred and thirty-four patients were advised to have percutaneous coronary intervention (PCI) as treatment. Sixty-three patients with a severe lesion had no recurrent angina and 103 were negative for troponin. Conclusion: 96.5% of patients with NSTEACS and an ECG abnormality had a significant lesion in the LAD. In 88.1% of patients, the lesion was before the first septal perforator branch. Overall, 66.3% of patients were advised to undergo PCI. The sensitivity of troponin in predicting severe lesion was 47.7%, and the sensitivity of recurrent angina was 67.7% only. This ECG abnormality by itself, even without recurrent angina or troponin elevation, should be an indication for invasive evaluation such as CAG.

Dressler - de Winter sign with acute inferoposterior STEMI: An ECG dilemma in artery localization

The Dressler-de Winter sign is an electrocardiogram (ECG) pattern characterized by upsloping ST-segment depression in leads V1-V6 followed by tall, hyperacute T waves, typically indicating an occlusion of the left anterior descending artery (LAD). We present a case involving an inferoposterior ST-segment elevation myocardial infarction (STEMI) with a variant of the de Winter sign, a concept of ST-segment continuum in the precordial leads. Despite initial ECG findings suggesting right coronary artery (RCA) or left circumflex artery (LCX) involvement, coronary angiography confirmed occlusion of the wrap-around LAD distal to the first septal (S1) and diagonal branch (D1) and revealed a left dominant system accompanied by a small non-dominant RCA. This case highlights the diagnostic complexity in accurately localizing the culprit artery in STEMI cases exhibiting the de Winter sign. Understanding such ECG variants is crucial for analyzing the mechanisms of acute ischemia and ensuring accurate assessment of the culprit vessel for effective revascularization.

Brugada Syndrome and Sudden Cardiac Death: An Electrocardiographic History.

A 22-year-old male with a history of anti-neutrophil cytoplasmic antibody vasculitis, renal transplant, hypertension, and no known family history of sudden cardiac death suffered a witnessed cardiac arrest. An initial rhythm strip recorded by emergency medical services revealed ventricular fibrillation. Return of spontaneous circulation was achieved after three rounds of cardiopulmonary resuscitation, defibrillation, and intravenous epinephrine. The patient was brought to the emergency department and admitted to the intensive care unit. He was diagnosed with Brugada syndrome, and an automatic implantable cardioverter-defibrillator (AICD) was placed after discharge. Brugada syndrome is characterized electrocardiographically by ≥2 millimeters (mm) ST-segment elevation in leads V1-V2 with either "coved type" (type 1) or "saddleback" (type 2) ST-segment morphology, or ≤2mm ST-segment elevation in V1-V2 with either "coved" or "saddleback" morphology (type 3). The absence of these patterns on isolated electrocardiograms (ECG) does not exclude the diagnosis, as dynamic fluctuations in ECG patterns are well-documented and can be induced by various physiologic stressors. This case provides an uncommon, complete electrocardiographic history of Brugada syndrome, from out-of-hospital cardiac arrest to AICD placement and depicts dynamic fluctuations between Brugada patterns and normal ECGs. This highlights the importance of serial ECGs in diagnosis, as sudden cardiac death is often the first or only presentation of Brugada syndrome.

Identification of a Novel SCN5A gene variant in a young female with atrioventricular canal defect in the absence of classical Brugada syndrome phenotype.

Brugada syndrome is generally considered a cardiac channelopathy disorder characterized by syncope or sudden cardiac death. The sodium voltage-gated channel alpha subunit 5 (SCN5A) gene is the most commonly mutated gene associated with Brugada syndrome. Recent discoveries of new variants of this gene, along with current guidance of family screening, have identified several asymptomatic carriers with potentially causative mutations. We present the case of a 25-year-old female patient without any family history of Brugada syndrome nor related congenital cardiovascular disorders, with an extensive atrioventricular canal defect, who tested positive for a novel heterozygous variant NM_198056.3: c.3169G>C (p. Asp1057 His) in the SCN5A gene. She had no history of syncope or aborted sudden cardiac death except for recurrent chest infections since her early childhood. Intriguingly, she did not show a type I Brugada electrocardiogram pattern. This report provides a novel heterozygous variant NM_198056.3: c.3169G>C (p. Asp1057 His) in the SCN5A gene, which may have a potential detrimental effect.

Potential preventative impact of aloe-emodin nanoparticles on cerebral stroke-associated myocardial injury by targeting myeloperoxidase: In supporting with In silico and In vivo studies

The present study examined the potential neuroprotective effects of aloe-emodin (AE) nanoparticles on the cerebral stroke-associated target protein myeloperoxidase (MPO). We investigated the binding interactions between AE and MPO through molecular docking and molecular dynamics simulations. Molecular docking results indicated that AE exhibited a binding energy of −6.9 kcal/mol, whereas it was −7.7 kcal/mol for 2-{[3,5-bis(trifluoromethyl)benzyl]amino}-n-hydroxy-6-oxo-1,6-dihydropyrimidine-5-carboxamide (CCl). Furthermore, molecular dynamics studies demonstrated that AE possesses a stronger binding affinity (−57.137 ± 13.198 kJ/mol) than does CCl (−22.793 ± 30.727 kJ/mol), suggesting that AE has a more substantial inhibitory effect on MPO than does CCl. Despite the therapeutic potential of AE for neurodegenerative disorders, its bioavailability is limited within the body. A proposed hypothesis to enhance the bioavailability of AE is its conversion into aloe-emodin nanoparticles (AENP). The AENPs synthesized through a fabrication method were spherical with a consistent diameter of 104.4 ± 7.9 nm and a polydispersity index ranging from 0.525 to 0.586. In rats experiencing cerebral stroke, there was a notable increase in cerebral infarction size; abnormalities in electrocardiogram (ECG) and electroencephalogram (EEG) patterns; a decrease in brain and cardiac antioxidant activities; and an increase in myeloperoxidase levels compared to those in normal rats. Compared with AE treatment, AENP treatment significantly ameliorated cerebral infarction, normalized ECG and EEG patterns, enhanced brain and cardiac antioxidant activities, and reduced MPO levels in stroke rats. Histopathological evaluations revealed pronounced alterations in the rat hippocampus, with pyknotic nuclei, disarray and loosely packed cells, deterioration of cardiac muscle fibers, and extensive damage to cardiac myocytes, in contrast to those in normal rats. AENP treatment mitigated these pathological changes more effectively than AE treatment in both brain and cardiac cells. These findings support that AENP provides considerable protection against stroke-associated myocardial infarction.

Ocimum GratissimumLinn. Leaf Extract and Fractions Pre-Treatments are not Associated With Deleterious Electrocardiogram Changes inTrastuzumab-Intoxicated Wistar Rats

Trastuzumab (TZM) treatment is known to be associated with arrhythmogenic potentialwhich primarily is the basis for its cardiotoxicity. The purpose of this study was to investigate the acute influence of oral pretreatments with 100 mg/kg/day of Ocimun gratissimumethanolic leaf extract (OG) and its fractions (petroleum ether,PEOG; ethyl acetate,EAOG; and ethanolic extract, EOG) as well as valsartan-lisinopril fixed dose combination (VAL-LSP) on electrocardiogram (ECG) of Wistar rats intaperitoneally treated with 2.25 mg/kg/day TZMfor 7 days. Young adult male Wistar rats were randomly allotted into 12groups of 6 rats per group. Therats were subjected to electrocardiograms (ECG) measurement using non-invasive procedures on days 1 and 7 of the experiment. Results showed that oral pretreatment with OGand its fractions (except EOG) as well as VAL-LSP fixed dose combination did not cause any remarkable changes in the ECG patterns of TZM-treated rats indicating that their relative oral safety in TZMchemotherapy. On the other hand, EOGpretreatment caused significant shortening of the OT/QTc interval in the TZM-treated rats highlighting the arrhthymogenic potential of this fraction. Overall, the study highlighted the arrhythmogenic potential of EOG in TZM chemotherapy while OG and its other solvent fractions as well as VAL-LSP could be considered relatively safe for use as adjuvants in TZM chemotherapy

Clinical outcomes in acute coronary syndrome after presentation of unique electrocardiographic findings

BackgroundUnique electrocardiographic findings are rarely observed in acute coronary syndrome (ACS) with a culprit left anterior coronary artery (LAD). The aim of this study was to assess the epidemiological features and prognostic impact. MethodsThis study was designed as an observational study. A total of 641 patients with a culprit lesion in the left main trunk or LAD were extracted from a cohort of 1776 ACS patients. The primary endpoint was mortality, comparing patients presenting with unique electrocardiogram patterns, specifically the de Winter pattern or Wellens' syndrome (type A or B), upon hospital arrival, with those presenting common electrocardiogram patterns. ResultsA unique electrocardiogram was observed in 7.0% (n = 45; 2 with de Winter pattern, 14 with Wellens' type A and 29 with type B). Compared to patients with a common pattern, cardiogenic shock at hospital arrival were rare in patients with a unique pattern (0% vs. 8.4%, P = 0.04), and percutaneous coronary intervention was primary revascularization strategy in all groups (95.6% vs. 98.2%). The mortality rates were similar between the two groups over a median 565 days of observation period (13.3% vs. 15.7%, P = 0.43), with 0% in Wellens' type A, 13.8% in type B, and both patients with the de Winter pattern died. ConclusionThe de Winter pattern or the Wellens syndrome was found in 7.0% of cases with ACS. They had similar mortality rates compared to those with a common pattern, although the de Winter pattern was identified in only 2 patients.

CHARACTERISATION OF THE 12–LEAD ECG IN PAEDIATRIC CARRIERS OF PLAKOPHILLIN–2 GENE VARIANTS

Abstract Background and Aim The PKP2 gene encodes the desmosomal protein Plakophilin–2, vital for cellular adhesion between cardiomyocytes. It is associated with classical Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC), a cardiac disorder that can cause malignant ventricular arrhythmias and sudden cardiac death in the young. The 12–lead electrocardiogram (ECG) is an important tool for diagnosis of ARVC but ECG patterns in paediatric carriers of PKP2 variants have not been previously systematically assessed. Methods The retrospective study enrolled paediatric (&amp;lt;18 years) PKP2 gene carriers referred to Great Ormond Street Hospital between 2005 and 2023. Their ECGs were analysed and compared with an age and gender–matched healthy population using paired two–tailed χ2–test. Results 28 PKP2 variant carriers (54% females) were identified, of which 2 (7%) were diagnosed with ARVC at the time of the ECG, with a mean age of 13.6 +∕– 4.8 years. These were compared to 28 healthy controls (mean age of 12.7 +/– 4.4 years). 17 carriers (60%) had low QRS voltages in the lateral leads, compared to 4 controls (16%). Fractioned QRS complexes were seen in the lateral leads in 19 (67%) and in the septal leads in 20 (71%), compared to 1 and 2 controls (3.5% and 7%, respectively). Abnormal T wave inversion (TWI) for age was found in 54% of the patients (29% in lateral leads) and flat T waves in 22 patients (78%); but these were not statistically–significantly different to controls. Conclusions Paediatric carriers of PKP2 gene variants are more likely to have low QRS voltages and QRS fractionation in the lateral and anterior leads than normal controls, but the presence of abnormally inverted or flat T waves did not discriminate between gene carriers and controls. Our findings suggest that paediatric–specific ECG features may represent early phenotypic expression of PKP2 gene variants.

Syncope and Brugada-like ECG pattern in a patient with syndrome of inappropriate antidiuretic hormone secretion (SIADH)

Syncope is a brief loss of consciousness caused by reduced blood flow to the brain, characterised by sudden onset, short duration and full recovery without intervention. Anamnesis, physical examination and other diagnostic tests such as laboratory analysis and electrocardiogram (ECG) can be conducted to identify the underlying cause of syncope. A Brugada pattern on an ECG in individuals with syndrome of inappropriate antidiuretic hormone secretion (SIADH) who have syncope symptoms may indicate cardiac issues. A 69-year-old man with hypertension and a history of smoking presented with syncope. His vital signs were within normal limits, with no signs of a neurological deficit. The patient met the diagnostic criteria for SIADH, as evidenced by the presence of hyponatraemia (Na 118 mmol/l), a hyperosmolar condition and euvolemia. Upon arrival, a twelve-lead ECG showed ST-segment anomalies that reflected a Brugada ECG pattern. No ventricular arrhythmias were detected during the 24-hour Holter monitoring. Coronary angiography revealed no abnormalities in the coronary arteries. The ECG demonstrated the normalisation of ST elevations and the disappearance of the Brugada ECG pattern after the correction of hyponatraemia. After three months of follow-up the patient, with a normal sodium level, had no episodes of syncope.

Molecular Autopsy With Banked Cord Blood Reveals Brugada Syndrome in Past Sudden Death Case.

Molecular autopsy has recently been gaining attention as a means of postmortem diagnosis; however, it is usually performed using the victim's blood sample at the time of death. Here, we report the first case of a deceased infant with Brugada syndrome whose diagnosis was made with banked cord blood. A seemingly healthy 1-year-old male infant collapsed while having a fever; this collapse was witnessed by his mother. Despite cardiopulmonary resuscitation, he died of ventricular fibrillation. No abnormalities of cardiac structure were identified on autopsy. Genomic samples were not stored at the time because of a lack of suspicion for familial arrhythmia. Five years later, his sister showed Brugada electrocardiogram pattern while febrile from Kawasaki disease. Their father showed a spontaneous type 1 Brugada electrocardiogram pattern. A heterozygous SCN5A p.R893C variant was found by genetic testing in the proband's father and sister. Furthermore, the proband's genetic testing was performed using his banked cord blood, which identified the same variant. Family history of Brugada syndrome with an SCN5A-R893C variant and clinical evidence led to a postmortem diagnosis of Brugada syndrome in the proband. Identification of this variant in this case later contributed to verifying SCN5A-R893C as a pathogenic variant through data accumulation. Banked cord blood may prove useful for conducting molecular autopsies in previously undiagnosed cases of sudden death in which genomic samples were not stored.