Coved-type ST-segment Elevation Research Articles

Provocable coved-type ST-segment elevation in right precordial leads as initial ECG feature after successful resuscitation with developing arrhythmogenic cardiomyopathy

A 54-year old female patient was admitted to hospital after successful resuscitation.

Management of ventricular arrhythmias in suspected channelopathies.

Although structural heart disease remains the predominant substrate for ventricular arrhythmia, channelopathies including long QT syndrome (LQTS), short QT syndrome (SQTS), Brugada syndrome (BrS), catecholaminergic polymorphic ventricular tachycardia (CPVT), and early repolarization syndrome (ERS) are less common but important contributing entities. These etiologies require specific therapies potentially contrary to empirical management of arrhythmias associated with structural heart disease. Conventional therapy including antiarrhythmic drug therapy may not only fail to resolve unstable arrhythmias but worsen them. Additionally, channelopathy patients with implantable cardioverter defibrillators (ICD) and arrhythmic storms represent a major challenge, and the acute care team needs to be cognizant of unique circumstances that require specific acute therapies beyond empirical advanced life support algorithm recommendations.1 Successful and considered acute management of ventricular arrhythmias is contingent on a number of variables, including knowledge of the cardiac substrate or potential substrate; form, mechanism, and precipitants of ventricular arrhythmias; and acute effect of potential therapies. In the longer term, an understanding of the natural history of the channelopathy along with the efficacy of long-term therapy will lead to superior outcomes. This review will present the risk of ventricular arrhythmias associated with these uncommon entities, the evolving understanding of the mechanism of arrhythmia, and the mechanistic basis of therapies along with a clinical approach to summarize the evidence pertaining to acute and long-term management. Patients with a prolonged QT interval are at risk of sudden cardiac death (SCD) due to Torsade de Pointes (TdP; Figure 1). Most patients with congenital LQTS are asymptomatic and diagnosed incidentally on electrocardiogram screening or following family screening. However, syncope, aborted SCD, or SCD may be the first presentation. Most arrhythmic events in congenital LQT1 occur during physical or emotional stress, at rest or in association with sudden auditory stimulation in LQT2, and during sleep or rest in LQT3 …

Suppression of Recurrent Electrical Storms in a Filipino Patient with Brugada Syndrome: A Case Report

Brugada syndrome is an inherited form of arrythmogenic disease associated with a coved-type ST segment elevation in right precordial leads and an increased risk of sudden cardiac death due to ventricular fibrillation (VF) in young, otherwise healthy adults. Electrical storm is malignant but rare in patients with Brugada syndrome. This is the case of a patient who was diagnosed with Brugada syndrome with repetitive discharges of implantable cardioverter-defibrillator during episodes of ventricular tachycardia/VF. The patient was given oral quinidine, which successfully suppressed the recurrence of electrical storms. KEYWORDS Brugada syndrome, bangungut, quinidine.

Early repolarization pattern in patients with provocable Brugada phenocopy: A marker of additional arrhythmogenic cardiomyopathy?

In 2003 in a published manuscript QRS prolongation in inferior leads was described in 22% of cases with arrhythmogenic right ventricular cardiomyopathy [1], meanwhile named arrhythmogenic cardiomyopathy. In a further look at these ECGs it seems that these belong to early repolarization pattern with slurring of the QRS complex. We have recently described a few patients with a definitive diagnosis of arrhythmogenic cardiomyopathy and provocable Brugada pattern by ajmaline testing [2,3]. The extent to which ERP and BrS may overlap remains unclear. ERP could be confirmed in 11–31% in typical BrS cases according to recent publications [4–7]. The question is whether patients with provocable coved-type ST-segment elevation in right precordial leads have ERP when arrhythmogenic right ventricular cardiomyopathy is an additional feature. In 19 patients with provocable coved-type ST-segment elevation in right precordial leads ERP in inferior and lateral leads were analyzed before ajmaline challenge. 17 patients had epsilon potentials and right ventricular conduction abnormalities and echocardiographic, angiographic and magnetic resonance hints of additional arrhythmogenic right ventricular cardiomyopathy (ARVC/D). Magnetic resonance technique and electrocardiographic results of a female patient are shown in Fig. 1a and b. In 17 patients with provocable Brugada pattern and additional ARVC/D ERP was found in 11/17 cases (64%). In 5 cases ERP could be found in lateral leads and in 6 cases in inferior leads. In all patients localized right precordial QRS prolongation with a difference of right to left precordial QRS complexes of more than 1.2 was present. Epsilon potentials were found in 11 cases. Right precordial T wave inversions beyond V1 were present in 6 patients. In 2 patients without additional ARVC/D ERP could be excluded. The number of patients was too small to draw any conclusions. In a large collective of patients with arrhythmogenic cardiomyopathy (ARVC/D) QRS prolongation in inferior leads and slurring of the QRS complex could be found in 22% of the cases [1]. In almost all cases left ventricular function showed inferior and posterolateral hypokinesia beside right ventricular contraction abnormalities. ECG abnormalities are shown in Fig. 2. In typical Brugada syndrome (BrS) early repolarization pattern (ERP) in larger trials are described in 11 up to 31% of the cases [4–7]. The combination of provocable BrS and ERP was more often found in patients with additional hints of ARVC/D (64%). The number of cases without additional ARVC/D was too small to draw any conclusions. The hypothesis is that in patients with provocable BrS and additional ARVC/D the rate of ERP is more pronounced. If ERP and provocable BrS exist in the same patient, screening of additional features of ARVC/D should be performed. The indication in these patients exist for right ventricular angiography, echocardiography and magnetic resonance imaging as listed in the modified diagnostic criteria of ARVC/D published in 2010 [8]. In summary, in all patients with provocable Brugada pattern and early repolarization pattern attempts should be made to diagnose additional arrhythmogenic right ventricular cardiomyopathy by simple ECG analysis and by cardiac imaging techniques. Up to now the risk for ventricular arrhythmias is not clear but theoretically higher as in isolated arrhythmogenic cardiomyopathy.

Low prevalence of Brugada-type electrocardiogram in a prospective large cohort of Egyptians

Abstract Background Brugada syndrome is a cardiac channelopathy that is associated with a high risk of VF and SCD and characterized by ECG pattern of transient or persistent, coved type ST-segment elevation in leads V 1 – V 3 . Aim To prospectively determine the prevalence of Brugada-type ECG pattern (Brugada sign) among unselected non cardiac individuals served at Cairo university teaching-hospital. Methods This study was conducted from October 2011 to September 2012, and included 4000 unselected noncardiac individuals (2078 males 52%, mean age 39 ± 14.44 years) admitted or presented to our hospital for different reasons. A 12-lead ECG was recorded for each of them; these ECGs were reviewed independently by two electrophysiologists for criteria of the three types of Brugada ECG pattern, and diagnosis of Brugada sign was made when both investigators agreed on the classification of the ECG findings according to the criteria of the Consensus Report of the Study Group of the Molecular Basis of Arrhythmia of the European Society of Cardiology. Results Twenty-two (0.55%) subjects had Brugada ECG pattern. All of them were of Mediterranean ethnic group. 18 males (0.45%), and 4 females (0.1%). No subjects showed type-1, while 11 (0.275%) of the 22 subjects had type-2, and the other 11 had type-3 Brugada ECG pattern. Eight cases (0.2%) among the 22 subjects were between 10 and 24 years old. Of the 22 subjects, 4 cases (18.2%) were symptomatic (palpitation and syncope), and 3 cases (13%) had positive F.H of SCD. Conclusion The frequency of type-1 Brugada-type ECG pattern was 0%, while type-2 and type-3 (saddleback type) was 0.55% among Egyptian hospital-based population, and it was more prevalent in middle-aged males.

A case of Brugada syndrome patient undertaken total intravenous anesthesia with remifentanil

A case of Brugada syndrome patient undertaken total intravenous anesthesia with remifentanil

Spectrum and Prevalence of Mutations Involving BrS1- Through BrS12-Susceptibility Genes in a Cohort of Unrelated Patients Referred for Brugada Syndrome Genetic Testing: Implications for Genetic Testing

The aim of this study was to provide the spectrum and prevalence of mutations in the 12 Brugada syndrome (BrS)-susceptibility genes discovered to date in a single large cohort of unrelated BrS patients. BrS is a potentially lethal heritable arrhythmia syndrome diagnosed electrocardiographically by coved-type ST-segment elevation in the right precordial leads (V1 to V3; type 1 Brugada electrocardiographic [ECG] pattern) and the presence of a personal/family history of cardiac events. Using polymerase chain reaction, denaturing high-performance liquid chromatography, and DNA sequencing, comprehensive mutational analysis of BrS1- through BrS12-susceptibility genes was performed in 129 unrelated patients with possible/probable BrS (46 with clinically diagnosed BrS [ECG pattern plus personal/family history of a cardiac event] and 83 with a type 1 BrS ECG pattern only). Overall, 27 patients (21%) had a putative pathogenic mutation, absent in 1,400 Caucasian reference alleles, including 21 patients with an SCN5A mutation, 2 with a CACNB2B mutation, and 1 each with a KCNJ8 mutation, a KCND3 mutation, an SCN1Bb mutation, and an HCN4 mutation. The overall mutation yield was 23% in the type 1 BrS ECG pattern-only patients versus 17% in the clinically diagnosed BrS patients and was significantly greater among young men<20 years of age with clinically diagnosed BrS and among patients who had a prolonged PQ interval. We identified putative pathogenic mutations in ∼20% of our BrS cohort, with BrS genes 2 through 12 accounting for <5%. Importantly, the yield was similar between patients with only a type 1 BrS ECG pattern and those with clinically established BrS. The yield approaches 40% for SCN5A-mediated BrS (BrS1) when the PQ interval exceeds 200 ms. Calcium channel-mediated BrS is extremely unlikely in the absence of a short QT interval.

A Novel &lt;i&gt;SCN5A&lt;/i&gt; Mutation Demonstrating a Variety of Clinical Phenotypes in Familial Sick Sinus Syndrome

Mutations in SCN5A have been reported to cause several types of hereditary arrhythmias (overlap syndrome). We herein report two patients with the overlapping phenotypes of juvenile sick sinus syndrome (SSS) and Brugada syndrome (BrS). The proband was a man who was in his twenties and had been diagnosed with both SSS and ventricular tachycardia (VT). A pilsicainide challenge test revealed a coved type ST segment elevation. His teenage brother also suffered from SSS, but no VT had been documented. A pilsicainide challenge failed to produce a Brugada-type ST elevation, but there was a marked prolongation of the His-ventricle interval. Their electrocardiograms at rest did not display any Brugada-type ST elevations. We identified a novel SCN5A (F1775Lfs*15) mutation in both patients, even though there was a phenotype discrepancy.

Propofol-induced coved-type electrocardiogram during catheter ablation of paroxysmal atrial fibrillation

We report a case of a young man with paroxysmal atrial fibrillation, in whom a characteristic coved-type Brugada pattern developed during catheter ablation performed under sedation with propofol. After immediate discontinuation of the propofol infusion, coved-type ST-segment elevation gradually resolved and no ventricular arrhythmias occurred. An ajmaline challenge failed to unmask a coved-type electrocardiogram and genetic testing was negative for ion channel mutations related to Brugada syndrome.

Diagnostic Dilemmas: Overlapping Features of Brugada Syndrome and Arrhythmogenic Right Ventricular Cardiomyopathy

Arrhythmogenic right ventricular cardiomyopathy (ARVC) and Brugada syndrome are distinct clinical entities which diagnostic criteria exclude their coexistence in individual patients. ARVC is a myocardial disorder characterized by fibro-fatty replacement of the myocardium and ventricular arrhythmias. In contrast, the Brugada syndrome has long been considered a functional cardiac disorder: no gross structural abnormalities can be identified in the majority of patients and its electrocardiographic hallmark of coved-type ST-segment elevation in right precordial leads is dynamic. Nonetheless, a remarkable overlap in clinical features has been demonstrated between these conditions. This review focuses on this overlap and discusses its potential causes and consequences.

Clinical and Electrophysiological Profile of Brugada Syndrome in the Tunisian Population

Most clinical studies of the clinical profile of Brugada syndrome (BS) have been conducted in either Asia, Europe, or America and their applicability to North African populations is largely unknown. The aim of the study was to analyze the clinical profile of BS in Tunisian patients. The clinical and follow-up data of 24 patients (22 men, mean age: 40.8 ± 13.7 years) were collected since 2002. Baseline characteristics, morbidity, and mortality data were obtained from medical records. One patient (4.16%) survived sudden cardiac death (SCD), four patients (16.3%) had syncope, and 19 patients (79.1%) were asymptomatic. Eleven patients (45.8%) had a family history of SCD. Twenty patients showed a spontaneous coved-type ST-segment elevation on electrocardiogram and after medical challenge on the four remnants. An electrophysiological study was performed in 15 of 24 patients (62.5%), during which ventricular fibrillation was induced in six patients (40%); three of the six patients were previously asymptomatic. An implantable cardioverter defibrillator (ICD) was implanted in 14 patients (58.3%). After a mean follow-up of 26 ± 21 months, one patient died from a noncardiac cause and one patient (with a history of aborted SCD) received an appropriate shock from his ICD. None of the asymptomatic and noninducible patients experienced a cardiac event. BS is present in the North African population and is probably under-recognized. Tunisian patients with BS share with their western and Asiatic counterparts similar clinical profile.

Number of electrocardiogram leads displaying the diagnostic coved-type pattern in Brugada syndrome: a diagnostic consensus criterion to be revised

According to the diagnostic consensus criteria, the electrocardiographic (ECG) diagnosis of Brugada syndrome requires coved-type > or =2 mm ST-segment elevation in >1 right precordial lead (RPL) V1-V3 in the presence or absence of a sodium-channel blocker. However, this consensus has not been evaluated. We aimed to assess the distribution of coved-type ST-segment elevation on RPLs in a large patient cohort to reevaluate the appropriateness of the diagnostic consensus criteria. We included 186 individuals with spontaneous and/or drug-induced ECGs of coved-type > or =2 mm ST-segment elevation in at least one RPL. A total of 376 ECGs were analysed for the number, distribution and maximal J-point elevation of diagnostic RPLs. Among all ECGs, 27 (7%) showed a coved-type pattern in 3 RPLs, 205 (55%) in 2 RPLs, and 144 (38%) in only 1 RPL. Leads V1 and V2 were diagnostic in 99% of all ECGs with two diagnostic RPLs. Lead V3 alone was not diagnostic in any ECG. Maximal J-point elevation was significantly higher in lead V2 than V1. Sixty case subjects (32%) had only ECGs with one RPL displaying a coved-type ST-segment elevation. There was no significant difference in clinical presentation and outcome compared with the 126 Brugada patients with ECGs displaying >1 diagnostic RPL. Major arrhythmic events occurred with the same rate (8%) in both groups during a follow-up >5 years. Lead V3 does not yield diagnostic information in Brugada syndrome. Individuals with ECGs displaying only one diagnostic RPL have a similar clinical profile and arrhythmic risk as Brugada patients with ECGs displaying >1 diagnostic RPL. Revision of the consensus criteria should be considered.

Fever-triggered ventricular arrhythmias in Brugada syndrome and type 2 long-QT syndrome.

The risk for lethal ventricular arrhythmias is increased in individuals who carry mutations in genes that encode cardiac ion channels. Loss-of-function mutations in SCN5A, the gene encoding the cardiac sodium channel, are linked to Brugada syndrome (BrS). Arrhythmias in BrS are often preceded by coved-type ST-segment elevation in the right-precordial leads V1 and V2. Loss-of-function mutations in KCNH2, the gene encoding the cardiac ion channel that is responsible for the rapidly activating delayed rectifying potassium current, are linked to long-QT syndrome type 2 (LQT-2). LQT-2 is characterised by delayed cardiac repolarisation and rate-corrected QT interval (QTc) prolongation. Here, we report that the risk for ventricular arrhythmias in BrS and LQT-2 is further increased during fever. Moreover, we demonstrate that fever may aggravate coved-type ST-segment elevation in BrS, and cause QTc lengthening in LQT-2. Finally, we describe molecular mechanisms that may underlie the proarrhythmic effects of fever in BrS and LQT-2. (Neth Heart J 2010;18:165-9.).

A novel microsatellite polymorphism of sodium channel beta1-subunit gene (SCN1B) may underlie abnormal cardiac excitation manifested by coved-type ST-elevation compatible with Brugada syndrome in Japanese

Brugada syndrome (BrS) is a rare sodium channelopathy typically seen in middle-aged, Southeast Asian males conferring high risks of cardiac sudden death. Loss-of-function mutations in SCN5A encoding the alpha-subunit of cardiac sodium channels may account partially for its etiology. We aimed to study whether mutations in the beta-subunits of sodium channel (SCN1B and SCN2B) would also be associated with abnormal cardiac excitation in BrS. 85 Japanese patients suspected to have BrS undertook a diagnostic challenge test with a sodium channel blocker, pilsicainide. Genetic screenings were performed for SCN5A, SCN1B and SCN2B by PCR-SSCP and direct sequence of amplicons in the patients and 50 healthy controls. 30 patients exhibited BrS-like ECG pattern (i.e., a coved-type ST-segment elevation) either at baseline or after the drug challenge. Genetic screenings revealed a sequence variation (p.R190Q) and 3 polymorphisms (p.H558R, p.R1193Q, IVS24+53T > C) in SCN5A, a sequence variation (g.-26G > T) and 2 polymorphisms (IVS1+53G > T and IVS3 +2996(TTA)8-15) in SCN1B and 2 polymorphisms (IVS2+27A > G, IVS2+76G > A) in SCN2B. A logistic analysis revealed that male, middle age (40 - 59 years of age) and IVS3+2996(TTA)8 of SCN1B were significantly (p < 0.05) associated with the development of BrS-like ECG pattern with odds ratios (95% confidence intervals) of 5.9 (1.8 - 19.6), 2.9 (1.4 - 6.1) and 2.3 (1.1 - 4.9), respectively. While the IVS3+2996(TTA)8 allele has not been reported in Caucasians previously, its allelic frequency in the patients exhibiting the BrS-like ECG pattern (0.250) was comparable to that in the healthy controls (0.260). The IVS3+ 2996(TTA)8 allele commonly seen in Japanese would not be pathogenic itself but may render male, middle-aged Japanese more susceptible to BrS.

Possible Contribution of Ischemia of the Conus Branch to Induction or Augmentation of Brugada Type Electrocardiographic Changes in Patients With Coronary Artery Disease

Recent evidence suggests an association between vasospastic angina and Brugada syndrome. Here we present two cases of coronary artery disease who presented with ECG abnormalities which might have been provoked or enhanced by ischemia of the conus branch of the right coronary artery. The 12-lead ECGs demonstrated normal sinus rhythm in these two cases. Interestingly, a saddle back or coved type ST segment elevation in leads V1-V3 was documented either in the percutaneous transluminal angioplasty procedure of the proximal right coronary artery or with an intracoronary acetylcholine (Ach) administration into the right coronary artery. These Brugada type ECG changes were restored to the baseline ECG waveform after improvement in the ischemia. In the second case, vasospasms of the conus branch of the right coronary artery were associated with a coved type ST segment elevation in leads V1 to V2. We discuss the possible interaction between ischemia caused by conus branch lesions and Brugada type electrocardiographic changes.

Epsilon‐Like Electrocardiographic Pattern in a Patient with Brugada Syndrome

Both Brugada syndrome (BrS) and arrhythmogenic right ventricle dysplasia/cardiomyopathy (ARVD/C) can cause repolarization abnormalities in right precordial leads and predispose to sudden cardiac death (SCD) due to ventricular arrhythmias. Although there is controversy over whether BrS is distinct from ARVD/C, it is believed that both are different clinical entities with respect to both the clinical presentation and the genetic predisposition. The coexistence of these two relatively rare clinical entities is also reported, but, some hypothesized that it is more possible that disease of the right ventricular muscle might accentuate the Brugada electrocardiographic pattern. In clinic practice, there may be cases where the dividing line is not so clear. We report a 33-year-old male presenting with recurrent syncope, who has a peculiar pattern of coved-type ST-segment elevation (ST-SE) with epsilon-like wave in right precordial leads.

Pilsicainide‐Induced ST Segment Elevation and ST Segment Depression in Two Patients with Variant Forms of Brugada‐Type Electrocardiographic Abnormalities

In two patients with variant forms of Brugada electrocardiographic abnormalities, ST segment elevation, and reciprocal ST segment depression developed during intravenous administration of pilsicainide. In one patient, pilsicainide accentuated the ST segment elevation in leads I, aV(L), and V(1)-V(3) and caused ST segment depression in leads II, III, and aV(F). Coronary angiograms at the time of ST segment elevation were normal. In the other patient, pilsicainide accentuated the coved-type ST segment elevation in leads II, III, and aV(F) and caused ST segment depression in leads I, aV(L), and V(2)-V(5). Frequent premature ventricular complexes (PVCs) with two different left bundle branch block patterns developed during ST segment elevation. Intravenous isoproterenol returned the ST segment to baseline in both patients and suppressed the PVCs in the second patient. We hypothesize that a wide area of epicardial myocardium with large I(to) current might explain the reciprocal ST segment depression observed at the time of accentuated ST segment elevation.

Idiopathic Ventricular Fibrillation Associated with J Wave and Early Repolarization: A Really Benign Electrocardiographic Sign?

Evaluation of: Sudden cardiac arrest associated with early repolarization. Haiissaguerre M, Derval N, Saccher F et al.: N. Engl. J. Med. 358, 2016-2023 (2008). In patients with idiopathic ventricular fibrillation, several kinds of electrocardiographic findings have been reported. Brugada electrocardiogram (ECG), a coved-type ST-segment elevation in the right precordial leads, is a well-known electrocardiographic sign, which is related to ventricular fibrillation leading to sudden cardiac death. By contrast, J wave and early repolarization are generally considered as benign manifestations on the ECG; however, they are reported as having the potential to cause cardiac arrhythmias at experimental studies. This study revealed that J wave and early repolarization were more frequent in patients with idiopathic ventricular fibrillation compared with control subjects (31 vs 5%; p < 0.0001). Moreover, a higher incidence of recurrent ventricular fibrillation was observed in patients with J wave and early repolarization on the ECG compared with those without such abnormalities (HR: 2.1; 95% CI: 1.2-3.5; p = 0.008). This study raises a question on the general concept that J wave and early repolarization are benign electrocardiographic patterns.

Brugada Syndrome Whose ST-segment Changes were Enhanced by Antihistamines and Antiallergenic Drugs

We describe a case of Brugada syndrome, in which a coved type ST-segment elevation was enhanced by antihistamines and antiallergenic drugs. The patient had been treated with four kinds of antihistamines and antiallergenic drugs. The twelve-lead ECG exhibited a coved type ST-segment elevation in leads V(1) and V(2), and their enhancement was induced by pilsicainide. After discontinuing those drugs, the ST segment elevation in leads V(1) and V(2) became reduced. An ICD implantation was selected for the therapy since ventricular fibrillation was induced. Our report discusses the possible contribution of antihistamines and antiallergenic drugs to the Brugada type ST-segment changes.

Brugada Syndrome Case: Difficult Differentiation Between a Concealed Form and Tricyclic Antidepressant-induced Brugada Sign

We describe a case of Brugada syndrome, in which recurrent syncope with convulsive seizures was induced after antidepressant treatment. The patient had been treated with five kinds of psychotropic drugs. The twelve-lead ECG after the syncope exhibited an RSR'-pattern in the precordial leads, however, a coved type ST-segment elevation was induced by a pilsicainide test. Although ventricular fibrillation was not induced in the electrophysiologic study, an ICD implantation was considered as the recommended therapy since Brugada syndrome unmasked by antidepressants could not be ruled out. The possible contribution of antidepressants to Brugada type ST-segment changes is discussed.