Electrocardiographic Pattern Research Articles

Prognostic parameters predict cardiac events in Brugada syndrome: a meta-analysis

Abstract Background Brugada syndrome (BrS) is recognized as a cause of sudden cardiac death (SCD), ventricular tachycardia or ventricular fibrillation (VT/VF). However, the prognosis varies among the patients with Brugada electrocardiograph (ECG) pattern. Furthermore, there is no prognostic tool to predict the worsening cardiac outcomes in BrS. Objectives Primary outcome was to assess the association between risks including patient history, ECG pattern, electrophysiology study (EPS) findings and cardiac events which is identified as VT/VF or SCD. Method We performed a meta-analysis of prospective and retrospective cohort studies which assessed the risks and cardiac events in patients with BrS. Pooled hazard ratios (HRs) was analyzed by using Random-effect model. Result A total of 27 studies involving 15,990 BrS patients. Of these, 17 were retrospective studies and 10 were prospective studies. In univariate analysis, the risks including male, aborted SCD, QRS duration≥120 msec, fragmented QRS (multiple spikes within the QRS complex in lead V1-V3), late potential (positive terminal filtered QRS complex of signal-averaged ECG), sinus node dysfunction or inducibility of ventricular tachyarrhythmias during EPS associated with a higher risk of cardiac events in BrS. In multivariate analysis, higher risks of cardiac events were associated in BrS patients with history of syncope (HR 2.65, 95% confidence interval (CI): 1.68-4.19, I2 = 54 %, p-value <0.001), family history of SCD (HR 2.64, 95%CI:1.39-5.01, I2 = 0%, p-value = 0.003), prior VF (HR 14.52, 95%CI: 3.36-62.84, I2 = 62%, p-value <0.001), type 1 Brugada ECG pattern (HR 2.07, 95%CI: 1.13-3.79, I2 = 0%, p-value = 0.018), spontaneous type 1 ECG pattern (HR 1.91, 95%CI: 1.01-3.61, I2 = 58%, p-value = 0.046), early repolarization pattern (HR 2.78, 95%CI: 1.9-4.07, I2 = 0%, p-value < 0.001) and history of atrial fibrillation before BrS diagnosis (HR 1.82, 95%CI: 1.03-3.23, I2 = 49%, p-value = 0.039) (Table 1). Conclusion Our findings suggest that patient history, ECG pattern, EPS findings can predict the cardiac events. Introducing a risk prediction model could aid in early management of BrS.

Impact of hypokalemia on Brugada syndrome: case report unveiling mechanisms beyond QT interval prolongation.

Brugada syndrome (BrS) is associated with an increased risk of sudden death caused by ventricular arrhythmias. The characteristic electrocardiographic appearance of ST-segment elevation of more than 2mm with coved-type morphology in more than 1 right precordial lead is seen. Hypokalemia is known to unmask the Brugada type-1 pattern, but its exact role and mechanisms in this context are not well understood. We report a case of first-time diagnosis of BrS in a 51-year-old man with hypokalemia 2.8mmol/L. Despite the normalization of potassium levels with potassium chloride (KCL), the Brugada type-1 pattern persisted on ECG. Interestingly, the corrected QT interval was shorter during hypokalemia (QTc 390ms) compared to when potassium levels were normal (QTc 432ms). This case highlights that hypokalemia can unmask the Brugada type-1 electrocardiographic pattern, but does not alter it once unmasked. The observed shorter QT interval during hypokalemia challenges the assumption that QT prolongation is the sole mechanism by which hypokalemia influences Brugada syndrome. This underscores the need for further research into additional mechanisms by which hypokalemia might trigger ventricular arrhythmias in Brugada syndrome.

Exposure to nicotine and withdrawal in Wistar rats: an electrophysiological study.

Throughout the world, smoking is one of the principal causes of preventable death. Nicotine, the primary active component of tabacco, acts as a psychostimulant and modulates the electrical activity of a number of the areas of the brain involved in addiction. Abstinence from nicotine will also impact the functional state of the brain, which is reflected in symptoms of craving and susceptibility to relapse. In addition, given the increase in the sympathetic tone of the heart and pulse rate promoted by nicotine, its consumption can contribute to tachyarrhythmia. The present study investigated the electroencephalographic (EEG) and electrocardiographic (ECG) patterns of Wistar rats submitted to acute or chronic exposure to nicotine, followed by withdrawal for 24 or 48 hours, and the re-administration (or not) of nicotine, to simulate episodes of relapse. The EEG data revealed an increase in all types of brainwaves, with emphasis on high-frequency (alpha, beta, and gamma) brain oscillations following both acute and chronic exposure to nicotine (14 days), whereas in withdrawal, there was a predominancy of delta waves. When exposure to nicotine was reinstated after withdrawal, the observed EEG profile was similar to that found in chronic exposure. The electrocardiogram reads showed that both acute and chronic exposure to nicotine caused abnormalities in the atrioventricular conduction and that, while these changes improve with substance withdrawal, relapse can worsen these parameters. The results of this study indicate that high-frequency brainwaves are correlated with nicotine dependence, while slow brain oscillations are consistent with drug craving, and episodes of nicotine relapse can reproduce brain activity patterns linked to dependence. Finally, exposure to nicotine predisposes the individual to heart rhythm abnormalities, which are attenuated by withdrawal, but may nevertheless be restored rapidly with re-exposure to the substance. This study demonstrated that nicotine increases high-frequency brain oscillations, which is associated with addiction, whereas withdrawal elevates the delta wave power, suggesting craving. Re-exposure to nicotine following withdrawal restores rapidly the EEG profile of chronic dependence. In addition, nicotine has deleterious impacts on cardiac activity, which are linked to fatal arrhythmias. This implies that stopping smoking is beneficial for the amelioration of the alterations in heart rhythm caused by nicotine addiction. This study elucidates the functional states of the brain and heart during both sporadic and chronic nicotine use, and the electrophysiological explanation for substance dependence and drug relapse after craving episodes.

Shark fin ECG pattern in a patient with acute ascending aortic dissection

The ‘shark-fin’ ECG-sign is a rare electrocardiographic pattern seen in ACS, typically associated with poor prognosis. However, its manifestation in acute aortic syndromes remains largely unexplored. Here, we present a case of a patient diagnosed with Ascending Aortic Dissection, who presented to ED with acute chest pain and shark-fin ECG-pattern.

Premature ventricular contraction patterns associated with nonsustained ventricular tachycardia

BackgroundOccurrence of nonsustained ventricular tachycardia (NSVT) is associated with negative outcomes. It is not clear whether specific electrocardiographic characteristics of premature ventricular contractions (PVCs) are associated with the occurrence of NSVT. The aim of this study was to identify electrocardiographic patterns associated with the presence of NSVT during 24-h electrocardiographic monitoring in patients with >10 PVCs per hour. MethodsThis was a retrospective, observational, cross-sectional study. We reviewed consecutive patients who received 24-h ECG monitoring performed at a single outpatient cardiology center. Patients who received 24-h electrocardiographic monitoring, with a PVC burden ≥10 PVCs/h were included. Occurrence of NSVT during 24-h electrocardiographic monitoring was the main outcome. ResultsA total of 343 patients were analyzed (mean [SD] age, 69.7 [12.5] years; 177 men [51.6 %]). NSVT occurred in 72 patients who were compared with 271 patients without NSVT. The novel term “premature beat ratio”, which aims to correlate the coupling interval and compensatory pause, was introduced; a value >0.5 was independently associated with NSVT according to the multivariate model (OR = 3.73, 95 % CI = 1.57–8.82; P = 0.002). PVC burden (OR = 1.09, 95 % CI = 1.02–1.17; P = 0.006), and triplets (OR = 18.19, 95 % CI = 7.32–45.18 P = 0.0) were also associated with NSVT in the multivariate model. ConclusionThese findings suggest that patients with a high PVC burden, triplets, and a premature beat ratio greater than 0.5 have an increased probability of presenting with NSVT and may benefit from more rigorous follow-up.

Electrocardiographic Patterns and Arrhythmias in Cardiac Amyloidosis: From Diagnosis to Therapeutic Management-A Narrative Review.

Electrophysiological aspects of cardiac amyloidosis (CA) are still poorly explored compared to other aspects of the disease. However, electrocardiogram (ECG) abnormalities play an important role in CA diagnosis and prognosis and the management of arrhythmias is a crucial part of CA treatment. Low voltages and a pseudonecrosis pattern with poor R-wave progression in precordial leads are especially common findings. These are useful for CA diagnosis and risk stratification, especially when combined with clinical or echocardiographic findings. Both ventricular and supraventricular arrhythmias are common in CA, especially in transthyretin amyloidosis (ATTR), and their prevalence is related to disease progression. Sustained and non-sustained ventricular tachycardias' prognostic role is still debated, and, to date, there is a lack of specific indications for implantable cardiac defibrillator (ICD). On the other hand, atrial fibrillation (AF) is the most common supraventricular arrhythmia with a prevalence of up to 88% of ATTR patients. Anticoagulation should be considered irrespective of CHADsVA score. Furthermore, even if AF seems to not be an independent prognostic factor in CA, its treatment for symptom control is still crucial. Finally, conduction disturbances and bradyarrhythmias are also common, requiring pacemaker implantation in up to 40% of patients.

Aslanger's pattern with acute lesion in the left coronary system: A case report

Acute Coronary Syndrome (ACS) is characterized by the suspicion or confirmation of acute ischemia or acute myocardial infarction (MI). The presence of ST segment elevation (STE) consists in the main criterion for indication of immediate reperfusion therapy due to acute coronary occlusion, although significant part of the acute coronary occlusion cases do not fit the ST-segment elevation myocardial infarction (STEMI) criteria.A case report of a 50 year-old male presenting typical anginal pain, whose electrocardiogram (EKG) presented Aslanger's pattern. The patient had severe lesion of the left anterior descending artery (ADA) and chronic occlusion of the right coronary artery on cardiac catheterization, with important collateral flow from branches of the left coronary artery to the right coronary artery.The dichotomy between STEMI and Non-ST segment elevation myocardial infarction (NSTEMI) must be contested, owing to the evidence that one third of the NSTEMI patients presents a total coronary occlusion. There are other electrocardiographic patterns which must be considered to diagnose ACS. Our case report outlines a different clinical presentation of Aslanger pattern, that shows a ADA acute occlusion, instead of the lesion being in the right coronary system.There is evidence that, in addition to the STEMI vs NSTEMI criteria, the Aslanger pattern and other electrocardiographic patterns characterize occlusive ACS. Recognizing those patterns in clinical practice is essential to improve the diagnosis and early treatment of patients with ACS.

The Prevalence of Arrhythmias, Including Premature Supraventricular and Ventricular Beats and Other Electrocardiographic Patterns, in 24-Hour Holter Monitoring in Patients with Overweight and Obesity.

this study aims to evaluate the prevalence of various arrhythmias and other electrocardiographic patterns within the group of individuals with overweight and obesity. One hundred eighty-one adults (90 females and 91 males) were qualified for inclusion in the experimental group. All participants had a body mass index (BMI) exceeding 25 kg/m2 (98 patients with obesity and 83 with overweight). The mean BMI in the obesity group was 33.6 kg/m2, and all participants had class 1 obesity. The control group comprised 69 individuals (56 females and 13 males) with normal BMI. The basic measurements were performed, and the participants filled out questionnaires describing their health conditions and lifestyles. Each participant underwent an electrocardiographic (ECG) examination and a 24 h Holter ECG examination. In patients with class 1 obesity compared to the control patients, the average numbers of premature ventricular beats (PVBs) and premature supraventricular beats (SPBs) were statistically significantly higher (p = 0.030 and p = 0.042). There was a positive correlation between body weight and PVB (p = 0.028) and between body weight and SPB (p = 0.028). Moreover, BMI and waist circumference were correlated with SPB (p = 0.043 and p = 0.031). In the backward stepwise multivariate regression model considering 24 h Holter ECG monitoring, concerning SPB as the dependent variable, it was observed that BMI (especially obesity class 1), type 2 diabetes, and thyroid disease exhibited the highest regression coefficients. obesity, even in class 1, might be a factor in a more frequent occurrence of abnormalities in electrocardiographic tests.

Brugada syndrome, early repolarization syndrome/j-wave syndromes, and a subtype of idiopathic ventricular fibrillation: microstructural cardiomyopathies

Brugada Syndrome is an inherited cardiac channelopathy with a high incidence of ventricular fibrillation and sudden cardiac death in patients with structurally normal hearts. Diagnosis is based on a characteristic electrocardiographic pattern (coved type ST-segment elevation ≥2 mm followed by a negative T-wave in ≥1 in the right precordial leads V1-V2) combined with an absence of gross structural abnormalities and several other criteria. The cornerstone of BrS diagnosis and definition, is its characteristic ECG pattern that can be present spontaneously or unmasked by drugs. This entity was described by the Brugada brothers in 1992 and belongs to a group of diseases known as inherited primary arrhythmia syndromes. The prevalence varies among regions and ethnicities, affecting mostly males. Despite several genes identified, SCN5A seems to be the most affected gene related BrS (≈ 30% of patients). The current main therapy is an implantable cardioverter-defibrillator, but radiofrequency catheter ablation has been recently reported as an effective new treatment.

Brugada syndrome in a patient with AKAP9 mutation: Case report and review of the literature

Brugada syndrome (BrS) is a rare autosomal dominant inherited channel disorder characterized by a specific electrocardiographic pattern of right precordial ST-segment elevation. Clinically, patients may experience polymorphic ventricular tachycardia and ventricular fibrillation, leading to recurrent syncope and sudden cardiac death (SCD) in the absence of structural cardiomyopathy. The A-kinase anchor protein 9 (AKAP9) gene, located on chromosome 7, encodes the AKAP9 protein, which plays a crucial role in regulating the phosphorylation of slowly activating delayed rectifier potassium channels (IKs). Here, we present a rare case of BrS associated with an insertion mutation in AKAP9, resulting in a frameshift mutation.

Prevalence and Predictors of Occlusive Myocardial Infarction in Patients Presenting With Non-ST-Elevation Acute Coronary Syndrome in Duhok, Iraq: A Cross-Sectional Study.

Background and aim Myocardial infarction is a major global health issue and the leading cause of death. Non-ST segment elevation acute coronary syndrome (NSTE-ACS) could behave like ST-segment elevation ACS in terms of causing total or near total occlusion of the coronary artery and leading to occlusive myocardial infarction (OMI). This study aims to assess OMI prevalence and associated factors in NSTE-ACS patients in Duhok, Iraq, to improve diagnosis and treatment outcomes. Materials and methods This prospective cross-sectional study, conducted at Azadi Heart Center and Zakho Teaching Hospital from March 2023 to March 2024, included 189 NSTE-ACS patients undergoing coronary angiography. Data collection encompassed demographics, clinical profiles, electrocardiographic (ECG) patterns, cardiac biomarkers, and angiographic outcomes. Patients were categorized into those with and without occlusive myocardial infarction (OMI). Results A total of 189 NSTE-ACS patients with a mean age of 58.65 (±10.38 SD) years were enrolled in the study. The overall OMI rate was 29.63%. OMI patients were older and had a higher prevalence of hypertension, dyslipidemia, and a family history of ischemic heart disease (IHD). Significant ECG changes associated with OMI included biphasic T-wave inversion and ST depression in specific leads. Marked elevation in troponin levels was also noted in OMI patients. The left anterior descending (LAD) artery was the most common culprit artery. Conclusions About one-quarter of our study cohort exhibited OMI. The condition was linked to clinical, ECG, and elevated troponin levels. The study underscores the importance of promptly recognizing occlusive myocardial infarction (OMI) in NSTE-ACS patients for better outcomes. Regular audits are imperative to augment awareness among healthcare professionals at cardiac centers regarding updated protocols and guidelines.

A novel approach to terminate roof-dependent atrial flutter with epicardial conduction through septopulmonary bundle

Atrial flutter, a prevalent cardiac arrhythmia, is primarily characterized by reentrant circuits in the right atrium. However, atypical forms of atrial flutter present distinct challenges in terms of diagnosis and treatment. In this study, we examine three noteworthy clinical cases of atypical atrial flutter, which offer compelling evidence indicating the implication of the lesser-known Septopulmonary Bundle (SPB). This inference is based on the identification of distinct electrocardiographic patterns observed in these patients and their favorable response to catheter ablation, which is a standard treatment for atrial flutter. Remarkably, in each case, targeted ablation at the anterior portion of the left atrial roof effectively terminated the arrhythmia, thus providing further support for the hypothesis of SPB involvement. These insightful observations shed light on the potential significance of the SPB in the etiology of atypical atrial flutter and introduce a promising therapeutic target. We anticipate that this paper will stimulate further exploration into the role of the SPB in atrial flutter and pave the way for the development of targeted ablation strategies.

Sine-wave pattern in a case of severe hyperkalemia and end-stage renal failure from a hospital in a rural area: A case report and narrative review

Background: A sine-wave pattern in electrocardiography (ECG) is one of the manifestations of severe hyperkalemia and requires rapid medical intervention and urgent hemodialysis. This manifestation has high specificity for detecting hyperkalemia, and rapid medical intervention is required upon first medical contact. Case Description: A 60-year-old woman presents to the emergency room with generalized weakness that started around two days ago, and became increasingly more severe and noticeable to the point that the patient went to the emergency department. The patient initially was stable and experienced no other complaints including chest pain or dyspnea. A 12-lead ECG was obtained which showed the sine-wave morphology. Minutes later, the patient rapidly deteriorated and underwent cardiac arrest while waiting for lab results to confirm the hyperkalemia. The patient was resuscitated successfully and lab results confirmed potassium levels of 9.7mmol/L and a creatinine level of 9.55mg/dL (estimated GFR using CKD-EPI formula of 4 ml/min/1.73m2. The patient underwent potassium correction therapy with fast-acting insulin agents, dextrose and calcium gluconate infusions. A secondary ECG post ROSC and potassium correction therapy was taken, showing a reversal of the sine-wave pattern into an accelerated junctional rhythm with tall T waves. Chest X-ray was performed and showed cardiomegaly, and the patient was admitted into the ICU to undergo urgent hemodialysis. Conclusion: This case further emphasizes the importance of recognizing ECG manifestations of hyperkalemia and rapid management after recognition of said ECG patterns to prevent the clinical deterioration of the patient and potentially fatal outcomes should be undertaken as soon as possible.

First description of a mixed Brugada pattern in inferior leads

Brugada Syndrome is a cardiac channelopathy closely related to lethal arrhythmias and sudden death in people without underlying structural pathology. The diagnosis is usually assumed with electrocardiographic patterns strictly limited to the right precordial leads, V1 or V2; but this is not so at all. In our article, we present the clinical case of a patient with a novel and different electrocardiographic presentation: the typical diagnostic pattern was presented in a lower lead of the electrocardiogram, and next to it, another pattern was presented in a contiguous lead. In addition, we propose the possible association of a structural pathology (non-compaction cardiomyopathy) with Brugada Syndrome, since both share a possible common genetic origin in the SCN5A gene.

Recognition of Brugada type 1 pattern on ECG by Echo State Networks

Abstract Background/Introduction The development of artificial intelligence (AI) and machine learning (ML) techniques, which can identify data patterns in electrocardiography, has led to the development of automated analysis tools for standard 12-lead ECGs. These tools are able to identify specific features associated with different cardiac diseases, which may be helpful in clinical practice [1]. One field of application is represented by Brugada Syndrome (BrS) diagnosis, which, despite characteristic ECG features, can be challenging to diagnose and correctly distinguish from other diseases. Purpose Within the Brugada Syndrome and Artificial Intelligence Applications to Diagnosis system study, we are developing an innovative model for diagnosing Type 1 BrS based on electrocardiographic pattern recognition through the Echo State Networks (ESN), a Recurrent Neural Network (RNN) type of architecture. Methods From patients enrolled in 5 Centers, 306 12-lead ECGs were obtained. The datasets were composed of spontaneous Type 1 pattern (coved) (group A, 98 patients); patients undergoing ajmaline test, classified as positive (group B, 47 patients) or negative (group C, 35 patients) according to test results; 220 controls with no clinical and familial history of arrhythmias (group D). ESN analyzed, as input, data collected from 1 beat of the ECG V2 lead. The datasets were used for the ESN model training and were assessed through a double cross-validation approach. The ESN performance was compared with the evaluation of 4 independent cardiologists, and sensitivity, specificity and accuracy were determined. Results The ESN model correctly classified 280/306 ECGs (91.5%), with a performance of 3.6 points superior to clinicians (269/306, 88.0%). ESN sensitivity (87.0% vs. 86.9%) and specificity (94.5% vs. 89.1%) are described in Table 1; accuracy in each group of the dataset (groups A, B, C, and D), represented in Table 2, were comparable to or greater than those of clinicians. Conclusion(s) In this work, an ESN-based system for diagnosing Type 1 BrS pattern on ECG was developed. Our results show that this ML model can detect ECG patterns associated with Type 1 BrS with accuracy, sensitivity and specificity comparable or superior to clinicians. In the future, we will use a larger dataset to increase the model performance further, with the real possibility of becoming a tool for everyday clinical practice.ESN and clinicians' diagnostic accuracyECG accuracy in each dataset group

Pectus carinatum, small pneumothorax, and Brugada patterns: First description and pathophysiological analysis

Pectus carinatum is a chest wall deformity where the breastbone and ribs protrude outward, sometimes referred to as “pigeon chest” due to the bird-like appearance of the chest. This condition occurs in approximately 1 in 1000 children, more commonly affecting boys than girls. Pectus carinatum tends to worsen as a child grows, especially during puberty, with about 15% of the children experiencing associated conditions like scoliosis. We present a case report of a young patient with pectus carinatum who experienced a small right apex pneumothorax and exhibited Brugada patterns on electrocardiogram tracing. This is the first clinical case reporting Brugada pattern presentation in both a patient with pectus carinatum and a small pneumothorax. We have conducted a clinical anatomical and electrocardiographic analysis to explore a physiopathological explanation for these findings, considering that our patient has no history or clinical signs of Brugada Syndrome. To further elucidate the clinical, anatomical, and electrocardiographic findings observed in our patient, detailed analyses were conducted. These investigations focused on how the pectus carinatum may influence electrocardiographic patterns through structural alterations of the thoracic wall, potentially modifying the transmural electrical field across the myocardium. This analysis aimed to explore a physiopathological explanation for the Brugada patterns observed, considering our patient’s unique thoracic anatomy and the absence of clinical Brugada Syndrome. We conclude that: 1. Pectus carinatum may create a window that facilitates the less resistive measurement of cardiac action potentials; 2. Small pneumothoraces can induce modifications in the cardiac electrical signal detected by surface electrodes, and pectus carinatum could unmask these modifications; and 3. Surface electrodes may measure subepicardial potentials that include and are modified by adjacent pericardial tissues, potentially resulting in alterations in transmural potential differences, thus producing fictitious Brugada patterns. Similarly, there is a possibility that the right ventricular outflow tract may be included within the subendocardial potentials.

Abnormal ST-segment changes in anterior and inferior leads during acute inferior myocardial infarction

Anterior and inferior ST elevation on electrocardiography (ECG) in patients with acute myocardial infarction is uncommon. ST-segment elevation due to right ventricular infarction induced by right coronary occlusion may extend from V1 to V3/V4, resembling the pattern of transmural ischemia of the anterior wall of the left ventricle. In addition, a wraparound left anterior descending (LAD) artery can produce ischemia manifesting as ST-segment elevation in the anterior and inferior leads. Our case report reveals dynamic ST-segment changes in acute inferior myocardial infarction, including the appearance of the shark fin ECG pattern, unlike what has been reported before.

IMPLANTABLE LOOP RECORDER IN BRUGADA SYNDROME: INSIGHTS FROM A SINGLE–CENTER EXPERIENCE

Abstract Brugada syndrome (BrS) is characterized by a “coved” ST segment elevation of ≥2 mm in the right precordial electrocardiographic (ECG) leads, either spontaneously or drug–induced. Risk stratification in individuals with BrS remains challenging. Currently, miniaturized and remotely monitored implantable loop recorders (ILRs) are available, providing physicians with a diagnostic tool that can help guide intervention or therapy decisions for these patients. The objective of this study was to investigate the characteristics and outcomes of patients diagnosed with BrS who underwent ICM insertion during routine clinical activity. This is a retrospective single center cohort study. We screened 147 consecutive patients who were diagnosed with BrS based on the presence of spontaneous or drug–induced type 1 ECG pattern between January 2009 and May 2023. Once this comprehensive diagnostic pathway was completed, we engaged in detailed counseling with patients to discuss the potential need for ICD implantation or ILR insertion, if deemed indicated. Compared to patients who did not receive a device, those who received an ILR had a higher prevalence of suspected arrhythmic syncope (43% vs. 22%, p=0.012) and tended to be younger (median age 38 years, IQR 30–52, vs. 43 years, IQR 35–55, p=0.044) and with a higher presence of SCN5A gene mutations (17% vs. 6%). On the other hand, compared to patients who received an ICD, those with an ICM underwent programmed ventricular stimulation less frequently (40% vs. 85%, p=0.010) and, as a result, had a lower frequency of positive result of this test (0% vs. 91%, p< 0.001). During a median follow–up period of 14.7 months (IQR, 4.7–44.8), no deaths occurred among the patients with ILR. Eight individuals (19%) were diagnosed with arrhythmic findings through continuous ILR monitoring. Among these findings, three patients experienced AF (including one case of new–onset AF), two patients exhibited symptomatic asystolic pauses lasting 16 seconds and 3.6 seconds respectively, two patients had non–sustained ventricular tachycardia, and one patient had episodes of paroxysmal supraventricular tachycardia. Except for a history of AF, no significant differences were observed between patients with arrhythmic findings and those without any events. In the ICD group, the median follow–up period was 32.2 months (IQR, 23.2–95.0). During this period, no deaths occurred and no appropriate or inappropriate ICD therapies were delivered.

EXPLORING THE 12 LEAD ECG CHARACTERISTIC OF PAEDIATRIC AND YOUNG ADULT CARRIERS OF DESMOPLAKIN GENE VARIANTS

Abstract Background and Aim The DSP gene (locus. 6p24.3) encodes desmoplakin, a quintessential desmosomal protein for cellular adhesion, particularly within cardiac tissues. DSP gene variants can cause dilated cardiomyopathy, non–dilated left ventricular cardiomyopathy (NDLVC) and arrhythmogenic righ ventricular cardiomyopathy (ARVC), cardiac conditions known to trigger malignant ventricular arrhythmias and cause sudden cardiac death in young individuals. Nevertheless, the electrocardiographic (ECG) patterns in children carrying DSP variants are underexplored. Methods A retrospective analysis was conducted involving paediatric DSP gene variant carriers (</=18 years), followed at Great Ormond Street Hospital from 2006 to 2023. Their 12–lead ECGs were characterised and compared with those of an age and gender–matched healthy control group (χ2, p–value<0.05). Results 25 DSP carriers (64% females; 12.6±4 years) were identified [including 4 (16%) with an overt cardiomyopathy phenotype] and compared to 25 healthy matched controls (12.7±4.3years). Compared to the control group, DSP carriers exhibited a higher prevalence of low QRS voltages (72% vs 44%, p= 0.041), QRS notching (72% vs 26%, p= 0,014) and flattened T waves (72% vs 52%, p= 0,026). All 4 patients with an overt cardiomyopathy phenotype had widespread low QRS voltages and QRS fractionation, as well as flattened/inverted T waves, particularly in the inferior and lateral leads. Conclusion Predominant ECG features observed in paediatric patients with DSP gene variants included low QRS voltages, QRS notching, and flat or inverted T waves. Our findings suggest that ECG features may precede the development of an overt cardiomyopathy phenotype in children with DSP variants, but further studies with larger cohorts are needed to confirm this and to determine their prognostic significance.

Electrocardiographic Patterns and Ejection Fraction in Patients with Nonischemic Dilated Cardiomyopathy: A Cross-sectional Retrospective Study

Background:: The relationship between ECG changes in dilated nonischemic cardiomyopathy and ejection fraction (EF) is complex and poorly understood. Thus, this study aimed to identify the most common patterns of ECG associated with dilated nonischemic cardiomyopathy and their relationship to EF. Methods:: A retrospective cross-sectional study was conducted between 2019 and 2022, including 100 consecutive patients with nonischemic dilated cardiomyopathy. ECG data were rate, axis, left (LBBB) and right bundle branch block (RBBB), first-degree heart block, premature ventricular contractions, depth of S wave in leads V1 and V2, length of R wave in leads V2 and V6, the ratio of R/S in the lead V4, left ventricular hypertrophy (LVH), maximum R wave length in leads I, II, and III, poor R wave progression, ST-segment elevation, and T wave inversion. Results:: The most common ECG pattern associated with cardiomyopathy was T-wave inversion (47%). The EF was 23.9± 8.87% in patients with LBBB and 25.5± 8.07% in patients without LBBB (P= 0.361). In patients with T-wave inversion, the EF was 23.4± 8.21%, and it was 26.28± 8.35% in patients without T-wave inversion (P= 0.086). In patients with LVH, the EF was 23.89± 7.84%, and it was 25.5± 8.66% in patients without LVH (P= 0.354). A negative nonsignificant correlation existed between SV1, SV2, RV2, RV6, R/S V4, and maximum R in I, II, III, and EF. Conclusions:: The study showed that T-wave inversion was the most common pattern associated with dilated nonischemic cardiomyopathy. The study provided insight into the negative correlation between EF and SV1, SV2, RV2, RV6, R/S V4, and maximum R in I, II, and III, although it did not reach a significant level.