Rare Presentation of Wide QRS Tachycardia in a Patient in Their 40s.

This article describes the case of a 40-year-old individual who presented with fulminant myocarditis. Initial ECG displayed sinus tachycardia with a heart rate of 117 bpm, QS complexes in leads V1-V3, ST-segment depression in leads II, III, aVF, V5-V6, and ST-segment elevation >0.2 mV in leads V1 through V3. The initial clinical assessment suggested an acute anteroseptal myocardial infarction. However, subsequent diagnostic evaluation through coronary angiography disclosed that the coronary arteries were normal. Therefore, clinicians should carefully consider the differential diagnosis between these conditions, as their management strategies differ markedly. Two hours after admission, the patient unexpectedly developed syncope. The ECG findings were consistent with the typical characteristics of bidirectional ventricular tachycardia. Our report described the appearance and morphology as well as mechanism of bidirectional ventricular tachycardia in detail. Additionally, we delineate differential diagnoses for disease that can cause bidirectional ventricular tachycardia, such as aconite poisoning, digoxin overdose, immune checkpoint inhibitor (ICI), myocardial ischemia, and hereditary channelopathies, such as catecholaminergic polymorphic ventricular tachycardia (CPVT) and Andersen-Tawil syndrome. Therefore, clinicians should recognize this ECG finding immediately and initiate appropriate treatment promptly as these measures may be vital in saving the patient's life.

Unmasking the uncommon: bidirectional ventricular tachycardia in two rare paediatric cardiomyopathies.

We present two exceptional cases of 14-year-old girls diagnosed with rare cardiomyopathies (left ventricular non-compaction, and arrhythmogenic right ventricular cardiomyopathy), both presenting with the unusual finding of bidirectional ventricular tachycardia.

Alternating morphology ventricular tachycardia associated with stable coronary artery disease: A case report.

Bidirectional ventricular tachycardia (BVT) is part of the spectrum of polymorphic ventricular tachycardia (VT). This is a case report of a 59-year-old male with stable coronary artery disease and baseline normal left ventricular ejection fraction (LVEF) who was followed for high burden symptomatic monomorphic premature ventricular complexes (PVC). He was admitted for syncope. The ECG showed recurrent non-sustained alternating morphology and double cycle length VT runs associated with deterioration in LVEF but without coronary artery disease progression. The patient had a successful catheter ablation of two distinct left ventricular PVC focus. This is a rare ECG pattern of alternating morphology VT similar to a bidirectional VT.

Bidirectional ventricular tachycardia induced by respiratory alkalosis mediated hypokalemia in a patient with acute ischemic heart failure.

Bidirectional ventricular tachycardia (BVT) is a rare arrhythmia that is generally observed in patients with catecholaminergic ventricular tachycardia or digoxin overdose. Herein, we present a case of BVT and electrical storm (ES) in an acute ischemic heart failure patient that is typically induced by hypokalemia. The patient was in invasive mechanical ventilator (MV) support and hypokalemia was related to acute respiratory alkalosis and that caused refractory hypokalemia despite intravenous (IV) potassium replacement. We also discuss our approach to solve refractory hypokalemia caused by respiratory alkalosis.

Pheochromocytoma - An ECG diagnosis?

Pheochromocytoma is a rare catecholamine-secreting tumor in the adrenal medulla. In some cases, the first symptoms are cardiovascular. We report on two patients with pheochromocytoma, who both presented with bidirectional ventricular tachycardia (BDVT). We elaborate on the mechanisms of BDVT in the setting of pheochromocytoma.

Therapeutic management of ventricular arrhythmias in Andersen-Tawil syndrome.

Andersen-Tawil Syndrome (ATS) is a rare periodic paralysis with typical skeletal and neuromuscular features. Cardiac involvement may range from asymptomatic ventricular arrhythmias to sudden death. Its management remains challenging and the choice between antiarrhythmic drug therapy and implantable cardioverter defibrillator (ICD) is not simple. We present a case of ATS patient with episodes of bidirectional ventricular tachycardia, well controlled by flecainide therapy initially, which in particular conditions of fever and hypokaliemia had a cardiac arrest with ventricular fibrillation, with neurological sequelae and need of an ICD implant. A review of the therapeutic management of this disease is presented.

Every face tells a story-unravelling a case of bidirectional ventricular tachycardia.

Bidirectional ventricular tachycardia is a rare form of tachycardia. We hereby report a case of bidirectional ventricular tachycardia in an 8-year-old boy wherein careful clinical exami-nation led to the diagnosis of Andersen Tawil syndrome. The case also demonstrates the efficacy of flecainide in managing bidirectional ventricular tachycardia in the setting of Andersen Tawil syndrome.

Bidirectional ventricular tachycardia induced by caffeine poisoning.

Bidirectional ventricular tachycardia (BVT) is a tachyarrhythmia characterized by 180-degree beat-to-beat alteration in the QRS axis. BVT is traditionally known as an electrocardiography (ECG) finding pathognomonic of digitalis poisoning and a hallmark of catecholamine-induced ventricular tachycardia. Apart from digitalis poisoning, aconitine poisoning is the only reported cause of poisoning-related BVT, and no report of caffeine-poisoning-related BVT is as yet available. A-27-year-old woman was transported to hospital with cardiac arrest from ventricular fibrillation after taking a massive dose of a caffeine-containing supplement (corresponding to 6 g of caffeine) 6 h before presentation. Return of spontaneous circulation (ROSC) was achieved by defibrillation. She developed BVT after ROSC. Hemodialysis was performed to remove the causative drug from the blood, with subsequent resolution of BVT and hemodynamic stabilization. At presentation, she had a blood caffeine concentration of 232 µg/mL. A suggested mechanism of development of BVT is that increased intracellular calcium concentration causes delayed afterdepolarization, which induces alternate occurrence of triggered activities within different His-Purkinje fibers, and thereby produces characteristic ECG findings. Caffeine acts on the ryanodine receptor to promote calcium release from the sarcoplasmic reticulum, and thus can induce BVT via the same mechanism. Caffeine poisoning can be treated by dialysis. In cases of BVT induced by caffeine poisoning, hemodynamic stabilization can be achieved by emergency dialysis.

Clinical Challenges in Catecholaminergic Polymorphic Ventricular Tachycardia.

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inheritable cardiac disorder associated with exercise- and stress-induced sudden death in young individuals. Although important steps forward have been made in the comprehension and treatment of this disease, several aspects remain unclear. Firstly, from an epidemiological standpoint the actual prevalence of CPVT is still unknown and possibly underestimated. In addition, the diagnostic process remains very challenging and can be supported by genetic analysis in only about half of the cases. Finally, up to one third of CPVT patients continue to present complex arrhythmias despite beta blocker treatment; the role of newer therapeutic options, such as flecainide and left cardiac sympathetic denervation, needs to be further elucidated. All these points constitute challenges for the cardiologist in the management of CPVT patients and fuel research into new diagnostic, prognostic and therapeutic approaches.

Alternans of the Ventricular Electrogram in Patients with an Implanted Cardioverter-Defibrillator.

BACKGROUND: The occurrence and significance of alternans of the ventricular electrogram (VEGM) in patients with an implanted cardioverter-defibrillator (ICD) has been rarely reported. OBJECTIVES AND METHODS: This report describes our observations of VEGM alternans documented in nine patients with an ICD (seven new cases and two previously published cases for comparison). RESULTS: We found seven new cases of near-field VEGM alternans and added two of our previously reported examples. Catecholaminergic polymorphic ventricular tachycardia (CPVT) was diagnosed in one patient based on ICD recordings. Alternans occurred during ventricular tachycardia (VT) in eight patients. A fast sinus tachycardia could not be ruled out in one patient. Stable cycle length alternans was found in five patients. QRS alternans of the left ventricular (LV) electrogram (EGM) was recorded in all five patients who had a device for cardiac resynchronization therapy capable of sensing by the LV channel. These five cases exhibited corresponding alternans of the right ventricular (RV) EGM in three cases, none in one patient, and a questionable recording in another. Alternans of the far-field (FF) VEGM occurred simultaneously with RV EGM alternans in all four patients whose device provided an FF tracing. CONCLUSION: Ventricular alternans may be more common than realized in ICD patients with VT. The correlation of VEGM alternans with the surface electrocardiogram remains unknown. Although QRS alternans itself as an electrical pattern is generally benign, its cause may not be, as illustrated in our patient with CPVT. Furthermore, associated cycle length alternans or undersensing of the smaller alternans component may complicate ICD therapy.

Catecholaminergic Polymorphic Ventricular Tachycardia with QT Prolongation.

The QT interval in patients with catecholaminergic polymorphic ventricular tachycardia (CPVT) is typically normal. However, CPVT patients are sometimes misdiagnosed as concealed long QT syndrome (LQTS), because patients with LQTS also manifest with syncope or sudden death following periods of exertion or extreme emotion. We report a CPVT patient with a pathogenic RyR2 mutation associated with a marked QT prolongation, which normalized after flecainide therapy.

Bidirectional ventricular tachycardia?

A 65-year-old woman was admitted to the hospital because of a syncopal episode with documented transient complete atrioventricular block. A DDD pacemaker was implanted. Post implantation, the patient was diagnosed with bidirectional ventricular tachycardia. Analysis of the arrhythmia and differential diagnosis is performed.

A rare case report of catecholaminergic polymorphic ventricular tachycardia with an uncommon CALM2 mutation.

Background: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a primary arrhythmia disorder characterized by syncope or sudden cardiac death and typically caused by a gain-of-function of the Ryanodine Receptor Type 2 (RyR2) mutation. Calmodulin is a calcium-binding protein responsible for many intracellular signalling pathways and disruptions in function or regulation may lead to potentially fatal arrhythmias. We present a case of a young patient with CPVT found to have an unusual, potentially causative, Calmodulin 2-a protein coding gene (CALM2) mutation. Case summary: A 21-year-old female with autism was brought to the ED following cardiac arrest. Bidirectional ventricular tachycardia was captured on electrocardiogram. Propranolol was initiated, and patient had no further episodes of ventricular arrhythmia. A subcutaneous implantable cardioverter defibrillator (ICD) was implanted, and further genetics testing was done. Rapid Whole Genome Sequencing (PGnome®-RAPID) resulted heterozygous variant of uncertain significance in CALM2 gene NM_001743.5 for variant c.136G>A. Discussion: To the authors' knowledge, this is the third known record of such mutation in accordance with the International Calmodulin Registry (n = 74). Identification of CALM mutations can help advance the understanding of genetic underpinnings of arrhythmias and underscore necessity of genetic screening and personalized treatment strategies. Subcutaneous ICDs offer a promising therapeutic option while minimizing risks associated with traditional transvenous ICDs.

Spatially discordant alternans due to periodic pacing site alternation.

BACKGROUND: Bidirectional ventricular tachycardia (BVT) is a rare type of ventricular tachycardia that is characterized by a beat-to-beat alternation in the QRS axis. Previous studies have shown that it is caused by alternating focal activities from 2 locations. OBJECTIVE: This study proposes a novel mechanism for the formation of spatially discordant alternans (SDA) due to the periodic pacing site alternation that occurs in BVT. METHODS: We used mathematical models of cardiac tissue to understand the dynamic and physiologic mechanisms underlying SDA formation. RESULTS: We found that SDA was formed by periodic pacing site alternation. When tissue was paced from 2 locations alternately, the timing of pacing at distant locations varied, creating a long-short-long-short sequence of pacing periods and thus action potential durations. Importantly, the nodal lines were perpendicular to the wavefront, which is more arrhythmogenic than when nodal lines are parallel to the wavefront. A positive correlation was observed between the separation distance of the 2 sites and the alternans amplitude. SDA patterns can be predicted from the tissue geometry and pacing site locations. CONCLUSION: Periodic pacing site alternation, which occurs in BVT, leads to arrhythmogenic SDA. The nodal lines associated with this phenomenon can be predicted on the basis of tissue geometry and focal locations.

Novel mutation in the KCNJ2 gene is associated with a malignant arrhythmic phenotype of Andersen-Tawil syndrome.

BACKGROUND: Andersen-Tawil syndrome (ATS) is a rare inherited multisystem disorder associated with mutations in KCNJ2 and low prevalence of life-threatening ventricular arrhythmias. Our aim was to describe the clinical course of ATS in a family, in which the proband survived aborted cardiac arrest (ACA) and genetic screening revealed a previously unknown mutation (c.271_282del12[p.Ala91_Leu94del]) in the KCNJ2 gene. METHODS: A cascade family screening was performed in a 5-generation family after identification of the KCNJ2 mutation in the proband. Subsequently, 10 of 21 screened individuals appeared to be mutation carriers (median age 38 [range 10-75] years, 3 female). Mutation carriers underwent clinical examination including biochemistry panel, cardiac ultrasound, Holter ECG, and exercise stress test. RESULTS: (1) At baseline, 2 patients had survived ACA, 3 had syncope or presyncopal attacks, and 2 reported palpitations. Exercise-induced nonsustained bidirectional ventricular tachycardia was documented in 4 patients, 2 received implantable cardioverter-defibrillators (ICD) for primary prevention and 2 for secondary prevention. (2) During follow-up, 1 primary prevention and 1 secondary prevention patient received in total 4 adequate ICD shocks. Life-threatening ventricular arrhythmias were documented during childhood in 5 of 10 mutation carriers. (3) All mutation carriers presented with characteristic mild dysmorphic features. Only 1 patient suffered from periodic paralysis. All had normal serum potassium level at repeated assessments and none had any other extracardiac disease manifestation. CONCLUSION: Our findings suggest that the novel KCNJ2 mutation is associated with a predominantly cardiac phenotype of Andersen-Tawil syndrome with high propensity to life-threatening ventricular arrhythmias presenting from childhood and young adulthood.

Alcohol and caffeine synergistically induce spontaneous ventricular tachyarrhythmias: ameliorated with dantrolene treatment.

Background: Alcohol and caffeine are the 2 frequently consumed substances in the general population, and the 2 substances are frequently co-consumed. Both substances may increase cardiac arrhythmia risk. However, it is unknown whether alcohol and caffeine co-consumption can synergistically enhance cardiac arrhythmogenesis. Objective: The study sought to investigate whether caffeine and binge drinking synergistically affect cardiac arrhythmogenesis. Methods: A binge drinking rat model (alcohol 2 g/kg, intraperitoneal, every other day for 3 times) was used. Rats (4 months old, both sexes) were randomized into the following 4 groups: binge alcohol-only group (A) (n = 8), nonalcohol, caffeine-only (60 mg/kg, intraperitoneal) group (C) (n = 8), binge alcohol plus caffeine group (A+C) (n = 8), and binge alcohol + caffeine + dantrolene group (A+D) (n = 7, treated with dantrolene 10 mg/kg before each alcohol injection). We also investigated whether alcohol induces Ca2+ sparks and dantrolene treatment attenuates alcohol-induced Ca2+ leak in ventricular myocytes. Results: No arrhythmia was induced with caffeine alone (group C, n = 0 of 8) or alcohol alone (group A, n = 0 of 8). However, alcohol + caffeine induced spontaneous ventricular tachyarrhythmias in all rats (group A+C, n = 8 of 8; P < .001 vs group C or A). Dantrolene prevented ventricular tachyarrhythmia induction in all 7 rats (group A+D, n = 0 of 7; P < .001 vs group A+C). In isolated ventricular myocytes, alcohol significantly increased Ca2+ sparks and dantrolene treatment reduced alcohol-induced Ca2+ sparks. Conclusion: Co-consumption of caffeine and binge drinking synergistically promote spontaneous ventricular tachyarrhythmias in rats. Dantrolene treatment can decrease alcohol-enhanced Ca2+ sparks in vitro and prevented alcohol and caffeine induced ventricular tachyarrhythmias in vivo.

Bidirectional Ventricular Tachycardia: Challenges and Solutions.

Bidirectional ventricular tachycardia (BiVT) is a rare form of ventricular tachycardia that manifests on surface electrocardiogram by dual QRS morphologies alternating on a beat-to-beat basis. It was first reported in the 1920s as a complication of digoxin, and since then, it has been reported in other conditions including fulminant myocarditis, sarcoidosis, catecholaminergic polymorphic ventricular tachycardia, and Andersen-Tawil syndrome. The mechanism for BiVT is not as well known as other forms of ventricular tachycardia but appears to include typical mechanisms including triggered activity from afterdepolarizations, abnormal automaticity, or reentry. This review will go beyond the definition, surface electrocardiogram, mechanisms, causes, and treatment of BiVT as per our current understanding.

A Visual Resolution of Cardiotoxicity: A Case Report of Digoxin-Induced Bidirectional Ventricular Tachycardia.

Digoxin is rarely used in modern cardiovascular disease management. Therefore, digoxin toxicity has been infrequently encountered and it is paramount to diagnose in a timely fashion. Bidirectional ventricular tachycardia is an unusual arrhythmia wherein every other beat has a different QRS axis as it travels alternately down different conduction pathways. The arrhythmia can be a manifestation of myocarditis, myocardial infarct, Andersen-Tawil syndrome, arrhythmogenic right ventricular cardiomyopathy, catecholaminergic polymorphic ventricular tachycardia, herbal aconite poisoning, and digoxin toxicity. This case illustrates the importance of clinician awareness of rare electrocardiogram (EKG) patterns of digoxin toxicity and visual resolution of fatal arrhythmia with timely treatment.

Caffeine and dobutamine challenge induces bidirectional ventricular tachycardia in normal rats.

BACKGROUND: Bidirectional ventricular tachycardia (BD-VT) is an intriguing arrhythmia, characterized by a beat-to-beat alternation of the QRS polarity on electrocardiogram. Currently there is no simple BD-VT animal model. OBJECTIVE: We report a simple animal model of BD-VT induced by caffeine and dobutamine (C+D) challenge in normal rats in which the arrhythmia can be attenuated by dantrolene (a ryanodine receptor stabilizer) treatment, but not by the pacemaker channel blocker ivabradine treatment. METHODS: Adult (4-5 months old) Sprague-Dawley rats (both sexes) were randomized into C+D (n = 8, received caffeine 120 mg/kg intraperitoneally [IP] and dobutamine 60 µg/kg IP, sequentially) and control (n = 8) groups. In addition, a group of 7 rats were pretreated with dantrolene (10 mg/kg, IP) 30 minutes before the C+D challenge and another group of 8 rats were pretreated with ivabradine (5 mg/kg, IP) 30 minutes before the C+D challenge. RESULTS: C+D challenge induced spontaneous premature ventricular contractions (PVCs) in 7 of 8 rats and BD-VT (lasted 4.3 ± 2.9 minutes, terminated spontaneously) in 6 of 8 (75%) rats. No ventricular arrhythmia was induced in the control group (P < .05 vs C+D group). Dantrolene treatment significantly decreased BD-VT (1 of 7 rats in the Dantrolene+C+D group vs 6 of 8 rats in C+D group, P < .05). Ivabradine treatment did not affect C+D-induced BD-VT (7 of 8 rats in the Ivabradine+C+D group vs 6 of 8 in the C+D group, P > .05). CONCLUSION: Caffeine and dobutamine challenge induces BD-VT in a majority of normal rats. Stabilizing cardiac ryanodine receptors with dantrolene treatment can significantly decrease the occurrence of BD-VT, but pacemaker channel blocker ivabradine treatment does not have effect in this animal model.