Andersen-Tawil Syndrome: A Comprehensive Review.

Andersen-Tawil syndrome (ATS) is a very rare orphan genetic multisystem channelopathy without structural heart disease (with rare exceptions). ATS type 1 is inherited in an autosomal dominant fashion and is caused by mutations in the KCNJ2 gene, which encodes the α subunit of the K+ channel protein Kir2.1 (in ≈ 50-60% of cases). ATS type 2 is in turn linked to a rare mutation in the KCNJ5-GIRK4 gene that encodes the G protein-sensitive-activated inwardly rectifying K+ channel Kir3.4 (15%), which carries the acetylcholine-induced potassium current. About 30% of cases are de novo/sporadic, suggesting that additional as-yet unidentified genes also cause the disorder. A triad of periodic muscle paralysis, repolarization changes in the electrocardiogram, and structural body changes characterize ATS. The typical muscular change is episodic flaccid muscle weakness. Prolongation of the QU/QUc intervals and normal or minimally prolonged QT/QTc intervals with a tendency to ventricular arrhythmias are typical repolarization changes. Bidirectional ventricular tachycardia is the hallmark ventricular arrhythmia, but also premature ventricular contractions, and rarely, polymorphic ventricular tachycardia of torsade de pointes type may be present. Patients with ATS have characteristic physical developmental dysmorphisms that affect the face, skull, limbs, thorax, and stature. Mild learning difficulties and a distinct neurocognitive phenotype (deficits in executive function and abstract reasoning) have been described. About 60% of affected individuals have all features of the major triad. The purpose of this review is to present historical aspects, nomenclature (observations/criticisms), epidemiology, genetics, electrocardiography, arrhythmias, electrophysiological mechanisms, diagnostic criteria/clues of periodic paralysis, prognosis, and management of ATS.

The Vectorcardiogram and the Main Dromotropic Disturbances.

Until the mid-1980s, it was believed that the vectorcardiogram (VCG) presented a greater specificity, sensitivity and accuracy in comparison to the 12-lead electrocardiogram (ECG), in the cardiology diagnosis. Currently, the VCG still is superior to the ECG in specific situations, such as in the evaluation of myocardial infarctions when associated with intraventricular conduction disturbances, in the identification and location of accessory pathways in ventricular preexcitation, in the differential diagnosis of patterns varying from normal of electrical axis deviation, in the evaluation of particular aspects of Brugada syndrome, Brugada phenocopies, concealed form of arrhythmogenic right ventricular cardiomyopathy and zonal or fascicular blocks of the right bundle branch on right ventricular free wall.VCG allows us to analyze the presence of left septal fascicular block more accurately than ECG and in the diagnosis of the interatrial blocks and severity of some chambers enlargements. The three-dimensional spatial orientation of both the atrial and the ventricular activity provides a far more complete observation tool than the linear ECG. We believe that the ECG/VCG binomial simultaneously obtained by the technique called electro-vectorcardiography (ECG/VCG) brought a significant gain for the differential diagnosis of several pathologies. Finally, in the field of education and research, VCG provided a better and more rational tridimensional insight into the electrical phenomena that occurs spatially, and represented an important impact on the progress of electrocardiography.

Unusual ventricular activation produced by temporary transvenous cardiac pacing: electrovectorcardiographic findings / Activación ventricular inusual producida por estimulación cardíaca transvenosa temporal: hallazgos electrovectorcardiográficos

Abstract Complete heart block (CHB) results from dysfunction of the cardiac conduction system, which results in complete electrical dissociation. The ventricular escape rhythm can have its origin anywhere from the atrioventricular node to the bundle branch-Purkinje system. CHB typically results in bradycardia, hypotension, fatigue, hemodynamic instability, syncope, or even Stokes-Adams syndrome. Escape rhythm originating above the bifurcation of the His bundle (HB) produces narrow QRSs with relatively rapid heart rate (HR) (except in cases of His system disease). We present a middle-aged man with an HR of 34 bpm, progressive fatigue, in whom a temporary pacemaker was implanted in the subtricuspid region. The post-intervention electrocardiogram had unusual features.

Resumen El bloqueo cardíaco completo (BCC) resulta de la disfunción del sistema de conducción cardíaco, lo que ocasiona una disociación eléctrica completa entre aurículas y ventrículos. El ritmo de escape resultante puede tener su origen en cualquier lugar desde el nodo auriculoventricular hasta el sistema His Purkinje. El BCC generalmente produce bradicardia, hipotensión, fatiga, inestabilidad hemodinámica, síncope o incluso el síndrome de Stokes-Adams. El ritmo de escape que se origina por encima de la bifurcación del haz de His produce intervalos QRS estrechos con frecuencia cardíaca no muy lenta (excepto en casos de enfermedad del sistema Hisiano). Presentamos a un hombre de mediana edad con una frecuencia cardíaca de 34 lpm, fatiga progresiva, en el que se implantó un marcapasos temporario en la región subtricuspídea. El electrocardiograma resultante a la intervención presentó características inusuales.

Unusual ventricular activation produced by temporary transvenous cardiac pacing: electrovectorcardiographic findings.

Complete heart block (CHB) results from dysfunction of the cardiac conduction system, which results in complete electrical dissociation. The ventricular escape rhythm can have its origin anywhere from the atrioventricular node to the bundle branch-Purkinje system. CHB typically results in bradycardia, hypotension, fatigue, hemodynamic instability, syncope, or even Stokes-Adams syndrome. Escape rhythm originating above the bifurcation of the His bundle (HB) produces narrow QRSs with relatively rapid heart rate (HR) (except in cases of His system disease). We present a middle-aged man with an HR of 34 bpm, progressive fatigue, in whom a temporary pacemaker was implanted in the subtricuspid region. The post-intervention electrocardiogram had unusual features.

El bloqueo cardíaco completo (BCC) resulta de la disfunción del sistema de conducción cardíaco, lo que ocasiona una disociación eléctrica completa entre aurículas y ventrículos. El ritmo de escape resultante puede tener su origen en cualquier lugar desde el nodo auriculoventricular hasta el sistema His Purkinje. El BCC generalmente produce bradicardia, hipotensión, fatiga, inestabilidad hemodinámica, síncope o incluso el síndrome de Stokes-Adams. El ritmo de escape que se origina por encima de la bifurcación del haz de His produce intervalos QRS estrechos con frecuencia cardíaca no muy lenta (excepto en casos de enfermedad del sistema Hisiano). Presentamos a un hombre de mediana edad con una frecuencia cardíaca de 34 lpm, fatiga progresiva, en el que se implantó un marcapasos temporario en la región subtricuspídea. El electrocardiograma resultante a la intervención presentó características inusuales.

Electrocardiographic "Northwest QRS Axis" in the Brugada Syndrome: A Potential Marker to Predict Poor Outcome.

Conduction delay in the right ventricular outflow tract as manifested in the electrocardiogram constitutes a high-risk predictor of ventricular arrhythmias in patients with Brugada syndrome. We present a case with a right QRS axis between -90° and ±180°. This feature has never been reported in the context of Brugada syndrome. (Level of Difficulty: Advanced.).

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.

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.

The tetrafascicular nature of the intraventricular conduction system.

The existence of a tetrafascicular intraventricular conduction system remains debatable. A consensus statement ended up with some discrepancies and, despite agreeing on the possible existence of an anatomical left septal fascicle, the electrocardiographic and vectorcardiographic characteristics of left septal fascicular block (LSFB) were not universally accepted. The most important criteria requested to confirm the existence of LSFB is its intermittent nature. So far, our group has published cases of transient ischemia-induced LSFB and phase 4 or bradycardia-dependent LSFB. Finally, anatomical, anatomopathological, histological, histopathological, electrocardiographic, vectorcardiographic, body surface potential mapping, and electrophysiology studies support the fact that the left bundle branch divides into three fascicles or a "fan-like interconnected network."

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.

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.

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.

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 in the setting of acute coronary syndrome associated with giant slurring variant J-wave.

We report a case of acute coronary syndrome with transient prominent anterior QRS forces (PAF) caused by proximal subocclusion of the left anterior descending (LAD) coronary artery before the first septal perforator branch. The ECG change indicates left septal fascicular block (LSFB) with associated slurring-type giant J-wave. Currently, this J-wave variant is considered as a lambda-like wave or QRS-ST-T "triangulation". Its presence is indicative of poor prognosis because of the risk for cardiac arrest as a consequence of ventricular tachycardia/ventricular fibrillation (VT/VF).

Brugada phenocopy caused by a compressive mediastinal tumor.

Recently, it has been shown that even experts in electrocardiography cannot differentiate the electrocardiographic pattern of genuine Brugada syndrome (BrS) from Brugada phenocopy (BrP). For this reason, this differentiation depends on the presence of established criteria both for BrS and BrP. In this manuscript, we present a patient with type 1 Brugada electrocardiographic pattern caused by expansive anterior mediastinal non-Hodgkin's lymphoma (NHL) with mechanical compression on the right ventricular outflow tract. The electrocardiographic pattern disappeared rapidly after antineoplastic measures.

Catecholaminergic polymorphic ventricular tachycardia, an update.

Catecholaminergic polymorphic ventricular tachycardia is a rare devastating lethal inherited disorder or sporadic cardiac ion channelopathy characterized by unexplained syncopal episodes, and/or sudden cardiac death (SCD), aborted SCD (ASCD), or sudden cardiac arrest (SCA) observed in children, adolescents, and young adults without structural heart disease, consequence of adrenergically mediated arrhythmias: exercise-induced, by acute emotional stress, atrial pacing, or ß-stimulant infusion, even when the electrocardiogram is normal. The entity is difficult to diagnose in the emergency department, given the range of presentations; thus, a familiarity with and high index of suspicion for this pathology are crucial. Furthermore, recognition of the characteristic findings and knowledge of the management of symptomatic patients are necessary, given the risk of arrhythmia recurrence and SCA. In this review, we will discuss the concept, epidemiology, genetic background, genetic subtypes, clinical presentation, electrocardiographic features, diagnosis criteria, differential diagnosis, and management.

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.