Congenital short QT syndrome: A review focused on electrocardiographic features.

Congenital short QT syndrome is a very low prevalence inherited primary arrhythmia syndrome first reported in 2000 by Gussak et al., who described two families with a short QT interval, syncope, and sudden cardiac death. In 2004, Ramon Brugada et al. identified the first genetic type of this entity. To date, a total of nine genotypes have been described. The diagnosis is easy from the electrocardiogram (ECG), not only due to the short QT duration, but also based on other aspects covered in this review. During 24-h Holter monitoring, paroxysmal atrial fibrillation spontaneously converting to sinus rhythm may be found. Even though the T wave may appear symmetric on the ECG, the T loop of the vectorcardiogram confirms that the T wave is constantly asymmetric due to the presence of dashes closer to each other in the efferent branch. In this review, we also describe the minus-plus T wave sign that we have described in a previously published article. In addition to congenital causes, we briefly highlight the existence of numerous acquired causes of short QT interval.

Transient ascending ST-segment depression and widening of the S wave in 3-channel Holter monitoring-A sign of dromotropic disturbance in the right ventricular outflow tract in the Brugada syndrome: A report of five cases.

BACKGROUND: Brugada syndrome (BrS) is somewhat a challenging diagnosis, due to its dynamic pattern. One of the aspects of this disease is a significant conduction disorder located in the right ventricular outflow tract (RVOT), which can be explained as a consequence of low expression of Connexin-43. This decreased conduction speed is responsible for the typical electrocardiographic pattern. Opposite leads located preferably in inferior leads of the electrocardiogram may show a deep and widened S wave associated with ascending ST segment depression. Holter monitoring electrocardiographic (ECG) aspects is still a new frontier of knowledge in BrS, especially in intermittent clinical presentations. METHODS: We describe, as an exploratory analysis, five case series of intermittent type 1 BrS to demonstrate the appearance of ascending ST segment depression and widening of the S wave, during 3-channel 24h-Holter monitoring (C1, C2 and C3) with bipolar leads. RESULTS: In the five cases described, the ST segment depression was observed mainly in C2, but in some cases also in C1 and C3. Only case 1 presented concomitant intermittent elevation of the ST segment in C1. All cases were intermittent. CONCLUSION: The recognition of an ECG pattern with ascending ST-segment depression and widening of the S wave in 3-channel Holter described in this case series should raise a suspicion of the BrS and suggests the counterpart of a dromotropic disturbance registered in the RVOT and/or reciprocal changes.

Northwest axis in the electrocardiogram - A sign of right ventricular remodeling in tetralogy of Fallot. A case report.

The typical ECG changes in tetralogy of Fallot are right axis deviation, large R waves in the anterior precordial leads and large S waves in the lateral precordial leads. We present a patient with extreme deviation of the frontal QRS axis between -90° and ± 180°. The child underwent open heart surgery twice before one year of age and a third time at nine years of age. The axis change persisted into adulthood.

A rare combination of atrial and intraventricular conduction disturbances: Atypical type I advanced interatrial block, left posterior fascicular block and transient right bundle branch block.

According to the first 2012 consensus report about interatrial block, the diagnosis of advanced interatrial block (A-IAB) consists of a P-wave duration ≥120 ms with biphasic "plus-minus" (±) polarity in the three leads of the inferior wall in the electrocardiogram. At the end of 2018, a new concept was introduced: the atypical A-IAB due to changes in the polarity or duration of the P-wave. The prevalence of these atypical patterns in different scenarios is currently unknown, but the patterns should be considered as risk factors of embolic stroke of undetermined source. When the A-IAB pattern is associated with clinical arrhythmic manifestations, it is known as the Bayés' Syndrome. We present a characteristic case of atypical A-IAB, and the rare left posterior fascicular block and transient right bundle branch block.

Relevance of the vectorcardiogram in the Brugada syndrome with "northwest QRS axis".

The outcome in the Brugada syndrome (BrS) is more benign in female than in male individuals. However, outcome could be adversely affected by sinus node dysfunction (SND). Long sinus pauses indicate an overlap between the phenotypes of BrS and SND. We present a 29-year-old woman with syncopal episodes at rest since adolescence.

Transient high-degree right bundle branch block masking the type 1 Brugada ECG pattern associated with possible transient early repolarization syndrome.

The Brugada syndrome (BrS) was the last electrocardiographic syndrome described in the 20th century. The initial description included right bundle branch block (RBBB), persistent ST-segment elevation in the right precordial leads, absence of structural heart disease, and propensity to unexplained syncope and/or sudden death mainly during nocturnal rest. Currently, we know that the first three components are not constant or true since RBBB is present in only 28% of cases, the ST-segment elevation is dynamic, at times absent, and there are discrete structural changes in the right ventricular outflow tract. Additionally, the presence of RBBB can hide the typical type 1 Brugada ECG pattern. We present a very unusual case of spontaneous transient RBBB that revealed a hidden type 1 Brugada ECG pattern that could be seen in the beat with normal ventricular conduction.

Link between Brugada phenocopy and myocardial ischemia: Results from the International Registry on Brugada Phenocopy.

BACKGROUND: Brugada phenocopies clinical entities that have indistinguishable electrocardiographic (ECG) patterns from true congenital Brugada syndrome. However, they are induced by other clinical circumstances such as myocardial ischemia. The purpose of our study was to examine the clinical features and pathogenesis of ischemia-induced Brugada phenocopy (BrP). METHODS: Data from 17 cases of ischemia-induced BrP were collected from the International Registry (www.brugadaphenocopy.com). Data were extracted from these publications and authors were contacted to provide further insight into each case. RESULTS: Of the patients included in this study, 71% were male. Mean age was 59 ± 11 years (range: 38-76). Type-1 Brugada ECG pattern occurred in 15/17 (88%) of the cases, while a type-2 Brugada ECG pattern was observed in the other 2/17 (12%). In all cases, the Brugada ECG pattern resolved upon correction of the ischemia, indicating ischemia as the inducing circumstance. No arrhythmic events have been detected acutely or during the follow-up. Reported time to resolution ranged from 2 minutes to 5 hours. Provocative challenges using sodium channel blocking agents were performed in 7/17 cases (41%), and all failed to induce a Brugada ECG pattern (BrP Class A). The remaining 10/17 cases (59%) did not undergo provocative testing due to various clinical reasons. CONCLUSIONS: Myocardial ischemia is a commonly reported etiology of BrP. Importantly, this study found no association between BrP induced by myocardial ischemia and sudden cardiac death or malignant ventricular arrhythmias.

Electro-vectorcardiographic and electrophysiological aspects of Ebstein's anomaly.

Ebstein's anomaly is a congenital heart disease where the most important anatomic feature is the inferior displacement of the tricuspid valve leaflets. Vectorcardiographic features are mainly forgotten and electrocardiographic features may be unrecognized by cardiologists handling adult patients.

Transient left septal fascicular block and left anterior fascicular block as a consequence of proximal subocclusion of the left anterior descending coronary artery.

The association of left anterior fascicular block (LAFB) with left septal fascicular block (LSFB) characterizes a left bifascicular block subtype rarely described in the literature, probably due to the fact that most researchers are not aware of the existence of the left septal fascicle. We describe a case with this transient intraventricular dromotropic disturbance due to left anterior descending coronary artery subocclusion.

Electro-vectorcardiographic demonstration of bifascicular block associated with ventricular preexcitation.

Down syndrome occurs more frequently in the offsprings of older pregnant women and may be associated with atrioventricular septal defect. This refers to a broad spectrum of malformations characterized by a deficiency of the atrioventricular septum and abnormalities of the atrioventricular valves caused by an abnormal fusion of the superior and inferior endocardial cushions with the midportion of the atrial septum and the muscular portion of the ventricular septum.

Transient left anterior and septal fascicular blocks after self-expandable percutaneous transcatheter aortic valve implantation.

Transcatheter aortic valve implantation (TAVI) is indicated in severe symptomatic aortic stenosis, when there is intermediate-high surgical risk, or a condition considered inoperable, as in the case of "porcelain aorta" that could turn clamping or cannulation of the ascending aorta hazardous in open-heart surgery. Among the complications of this less invasive procedure, intraventricular conduction disorders subsequent to the procedure stand out. TAVI causes worsening of intraventricular dromotropic disorders in more than 75% of the cases, with the presence of preexisting right bundle branch block and first-degree atrioventricular block, deep prosthesis implant, male gender, size of the aortic annulus smaller than the prosthesis, and porcelain aorta being predictive of requirement for permanent pacemaker implant.

Re-evaluating the electro-vectorcardiographic criteria for left bundle branch block.

The criteria for left bundle branch block have gained growing interest in the last few years. In this overview, we discuss diagnostic and prognostic aspects of different criteria. It was already shown that stricter criteria, including longer QRS duration and slurring/notching of the QRS, better identify responders to cardiac resynchronization therapy. We also include aspects of ST/T concordance and discordance and vectorcardiography, which could further improve in the fine-tuning of the left bundle branch criteria.

Left bundle branch block: Epidemiology, etiology, anatomic features, electrovectorcardiography, and classification proposal.

In left bundle branch block (LBBB), the ventricles are activated in a sequential manner with alterations in left ventricular mechanics, perfusion, and workload resulting in cardiac remodeling. Underlying molecular, cellular, and interstitial changes manifest clinically as changes in size, mass, geometry, and function of the heart. Cardiac remodeling is associated with progressive ventricular dysfunction, arrhythmias, and impaired prognosis. Clinical and diagnostic notions about LBBB have evolved from a simple electrocardiographic alteration to a critically important finding affecting diagnostic and clinical management of many patients. Advances in cardiac magnetic resonance imaging have significantly improved the assessment of patients with LBBB and provided additional insights into pathophysiological mechanisms of left ventricular remodeling. In this review, we will discuss the epidemiology, etiologies, and electrovectorcardiographic features of LBBB and propose a classification of the conduction disturbance.

Acute inferior myocardial infarction with right ventricular involvement and several clinical-electrocardiographic markers of poor prognosis.

Right ventricular involvement in inferior myocardial infarction is a marker of poor prognosis. We present a case of a 62-year-old man with very recent onset of acute chest pain and cardiac shock with the triad of elevated jugular venous pressure, distension of the jugular veins on inspiration, and clear lung fields. In addition, the admission electrocardiogram showed a slurring J wave or lambda-like wave and conspicuous ST segment depression in several leads, predominantly in the lateral precordial (V4-V6), all clinical-electrocardiographic features of ominous prognosis.

Electro-vectorcardiographic demonstration of rate-independent transient left posterior fascicular block.

Left posterior fascicular block (LPFB) is a rare intraventricular conduction disorder of rare occurrence, especially as an isolated entity. Its transient form is even rarer and maybe rate-independent or rate-dependent intermittent LPFB (phase 3 block, tachycardia-dependent and phase 4 block or bradycardia-dependent). We present a case of a young adult male whose baseline ECG/VCG showed the typical LPFB pattern. A treadmill stress test revealed rate-independent intermittent LPFB with random occurrence. Imaging exams ruled out structural heart disease. To our knowledge, this is the first case in the literature of a rate-independent intermittent LPFB with no underlying structural heart disease.

Current aspects of the basic concepts of the electrophysiology of the sinoatrial node.

Cardiac pacemaker cells, also named P-cells (pale cytoplasm, pacemaker, phylogenetically primitive), including cells of the sinoatrial node, are heterogeneous in size, morphology, and electrophysiological characteristics. The exact extent to which these cells differ electrophysiologically in the human heart is unclear, yet it is critical for the understanding of normal cellular function. In this review, we describe major ionic currents and Ca2+ clocks acting on Ca2+ release in the sarcoplasmic reticulum. We also explain the external regulation of the heart rate controlled by the two branches of the autonomic (involuntary) nervous system: the sympathetic and the parasympathetic nervous system. Vagal stimulus causes bradycardia, rapid and short-duration modulation, and controls rapid responses, and increases heart rate variability. A typical example is constituted by phasic or respiratory sinus arrhythmia, characterized by pronounced vagal activity, more frequent in children and young individuals.

Transient left septal fascicular block in a patient with stable effort angina and critical proximal obstruction of left anterior descending coronary artery.

Several publications from our group and others have shown that a main cause of middle fiber block or left septal fascicular block of the left bundle of His is proximal obstruction of the left anterior descending coronary artery, before its first septal perforator branch, since this fascicle is the only one of the three fascicles irrigated exclusively by this artery. We present a case in which the phenomenon occurred transiently, causing prominent anterior QRS forces, confirmed by coronary angiography.

Epsilon wave: A review of historical aspects.

The epsilon wave of the electrocardiogram (ECG) together with fragmented QRS (fQRS), the terminal conduction delay, incomplete right bundle branch block (IRBBB) and complete/advanced RBBB (CRBBB) of peripheral origin are part of a spectrum of ventricular depolarization abnormalities of arrhythmogenic cardiomyopathy(AC). Although the epsilon wave is considered a major diagnostic criterion for AC since 2010 (AC Task Force Criteria), its diagnostic value is limited because it is a sign of the later stage of the disease. It would be more appropriate to say that the epsilon wave is a "hallmark" of AC, but is of low diagnostic sensitivity. Although the epsilon wave has high specificity for AC, it can be present in other pathological conditions. In this update we will cover the nomenclature, association with disease states and electrocardiographic aspects of the epsilon wave.

Main artifacts in electrocardiography.

Electrocardiographic artifacts are defined as electrocardiographic alterations, not related to cardiac electrical activity. As a result of artifacts, the components of the electrocardiogram (ECG) such as the baseline and waves can be distorted. Motion artifacts are due to shaking with rhythmic movement. Examples of motion artifacts include tremors with no evident cause, Parkinson's disease, cerebellar or intention tremor, anxiety, hyperthyroidism, multiple sclerosis, and drugs such as amphetamines, xanthines, lithium, benzodiazepines, or shivering (due to hypothermia, fever (rigor due to shaking), cardiopulmonary resuscitation by chest compression (oscillations of great amplitude) and patients who move their limbs during the test, causing sudden irregularities in the ECG baseline that may resemble premature contractions or interfere with ECG wave shapes, or other supraventricular and ventricular arrhythmias. When the skeletal muscles experience shaking, the ECG is "bombarded" by apparently random electrical activity.

Acute coronary syndrome of very unusual etiology.

Aortitis is one of many possible manifestations of tertiary syphilis. Aortic disease is the most common of all cardiovascular syphilitic lesions. Aortic diseases caused by tertiary syphilis include aortitis, aortic root dilation, aneurysm formation, aortic regurgitation and coronary ostial stenosis. A less common manifestation of syphilitic aortitis is coronary artery ostial narrowing related to aortic wall thickening. We report a case of a 40-year-old male patient admitted with a clinical picture of acute coronary syndrome (unstable angina). He had no risk factors for coronary artery disease. The physical examination revealed nothing remarkable. The admission electrocardiogram (ECG) showed ST segment depression in the anterolateral and inferior leads (Figure 1). The coronary angiogram showed critical ostial stenosis of the right (RCA) and left main coronary artery (Figure 2a, b). Cardiac-computed tomography showed aortic wall thickening with involvement of bilateral coronary ostia (Figure 2b, c). The patient was referred for coronary bypass surgery after treatment with two doses of penicillin G. The laboratory test was strongly positive for syphilitic infection. Postoperative treatment with benzathine penicillin, in doses recommended for tertiary syphilis, was implemented.