Recurrent Polymorphic Ventricular Tachycardia Research Articles

Old but not Vain : Two Cases of Refractory Polymorphic Ventricular Tachycardia associated with Coronary Artery Disease successfully treated with Quinidine

Post myocardial infarct polymorphic Ventricular Tachycardia (VT) triggered by injured Purkinje fibres can cause VT storms that are refractory to various treatment even after successful reperfusion or revascularization. We reported such recurrent polymorphic VT in two post myocardial infarct patients after Percutaneous Coronary Intervention. Both of them responded readily to Quinidine. We also discussed the pathophysiology of this arrhythmia and the treatment option of it, namely catheter ablation and antiarrhythmics

Abstract 15535: A Case of Suspected Menstruation Induced QTc Prolongation Leading to Torsades De Pointes

Estrogen is known to produce changes in cardiac electrophysiology specifically in premenopausal women, increasing the risk of arrhythmias. We report a case of Torsades de Pointes (TdP) in a young premenopausal female. A 25-year-old female with a history of anxiety presented to the emergency room (ED) with several days of vomiting. While in the ED, she had an unwitnessed syncopal event, and was found to be in pulseless ventricular fibrillation (V-Fib) . She required one defibrillation to obtain return of spontaneous circulation (ROSC). Initial work up showed calcium 7.6 mg/dl, phosphorous 1.1 mg/dl, magnesium 2.4 mg/dl, potassium 4.0 mg/dl. Remainder of electrolytes were normal. Toxicology screen was positive for cannabinoids. Post ROSC EKG showed sinus tachycardia at a rate of 103bpm with a prolonged QTc of 531ms. Patient was not on any medications outpatient. On further discussion, patient reported no prior history of syncopal episodes, palpitations, and denied any family history of sudden cardiac death. She did report she was presently on her menses. Shortly after admission, she had recurrent polymorphic ventricular tachycardia which degenerated to TdP. She was loaded with magnesium. Echocardiogram showed an ejection fraction (EF) of 35-39% and global hypokinesis. Diagnostic left heart catheterization was performed revealing clean coronaries. Cardiac MRI revealed EF of 43% without any late gadolinium enhancement. Her QTc remained prolonged even with electrolyte normalization. She underwent successful ICD placement and remained event free during the remainder of the hospital course. She will undergo genetic work up for long QT syndrome. Female sex hormones, specifically estrogen, have been described in literature as pro-arrhythmic given its effects on QT prolongation and ion gated channels. Prior cases of menstruation dependent arrhythmias have speculated that the abrupt reduction in estradiol prior to menstruation is associated with increased cyclic adenosine monophosphate dependent arrhythmogenicity. This case highlights the multifactorial etiology of sudden V-Fib arrest in a young female, and the importance of understanding the role that sex hormones play in the underlying pathogenesis.

ОСОБЛИВОСТІ ДІАГНОСТИЧНОГО ТА МЕДИКАМЕНТОЗНО-ІНВАЗИВНОГО МЕНЕДЖМЕНТУ ЧАСТО РЕЦИДИВУЮЧОЇ ПОЛІМОРФНОЇ, ПОЛІТОПНОЇ ШЛУНОЧКОВОЇ ТАХІКАРДІЇ

In today's conditions, given the difficult economic situation in the country and the low adherence of patients to treatment, a difficult and relevant issue is the treatment of post-myocardial infarction patients, especially in the complicated course of the disease. The main reason for the occurrence of complex cardiac arrhythmias is the formation of a focus of ectopic activity in the myocardium or the appearance of a re-entry wave. In case of recurrence of life-threatening tachycardias, despite antiarrhythmic therapy, the choice must be between escalating drug therapy and radiofrequency catheter ablation (RFA). Purpose: a detailed description of the clinical case of the disease in a patient with low compliance to medical treatment, who suffered an acute myocardial infarction and was subsequently hospitalized several times in a specialized cardiac hospital for the development of complex ventricular arrhythmias. The article describes in detail modern approaches to the diagnosis and treatment of life-threatening tachycardias, including emergency care, comparison of escalation of antiarrhythmic therapy with RFA in a patient with post-infarction cardiosclerosis and frequent attacks of recurrent polymorphic ventricular tachycardia, treatment of modern defibrillator, electrophysiological study, RFA. Conclusions: Long-term follow-up of a patient with low compliance to the treatment of complex ventricular arrhythmias showed that ablation of the ventricular tachycardia substrate was more effective than escalation of antiarrhythmic therapy, which led to the remodulation of the heart cavities, improving the quality of life of the patient and preventing the progression of cardiovascular events.

AN INTERESTING CASE OF POLYMORPHIC VENTRICULAR TACHYCARDIA

Introduction: Polymorphic ventricular tachycardia (PMVT) is described as a ventricular rhythm (>100 b/min) characterized by clearly defined QRS complexes with frequent and rapid changes in morphology, axis, or both. The clinical presentation of PMVT can range from a brief, asymptomatic, self-terminating episode to recurrent syncope or sudden cardiac death. Long QT interval is most often implicated. We report a case of a male patient presented to emergency room (ER) with syncope, generalized tonic-clonic seizures(GTCS) and PMVT. Case report: Young male, presented to ER with syncope and GTCS. Patient was initially managed in ER on the lines of status epilepticus. Patient left the hospital against medical advice and then presented again in ER within 8 hours with out of hospital cardiac arrest and had PMVT leading to pulseless electrical activity. He was resuscitated and transferred to Intensive care unit (ICU). He had recurrent PMVT, was DC-cardioverted multiple times and settled with overdrive pacing. ECG showed QTc >500msec. Patient was taking Loperamide approximately 120 tablets/day for last few months. Patient had no further episode of tachyarrhythmia since starting on B-blocker and QTc interval improved gradually. The genetic workup for congenital long QT syndrome was negative. Patient is doing well and is under regular follow-up. Conclusion: Drug induced Long QT is often an unrecognized but a common presentation in patients with ventricular arrhythmias. Arrhythmia recognition in ER/ among general practitioners, strict legislation and dispensing control in pharmacy of prescription only medications can save innumerable lives. Keywords: PMVT, Loperamide, Drug induced Long QT, QTc interval, syncope.

LEFT VENTRICULAR NONCOMPACTION, GARDNER SYNDROME, AND DESMOID TUMORS, A GENETIC CONNECTION?

SESSION TITLE: Monday Fellow Case Report Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: Desmoid tumors are rare, fibrous tumors accounting for only 0.03% of neoplasms. They are locally invasive and associated with high reoccurrence rates. Left ventricular non-compaction (LVNC) is the arrest of normal cardiac compaction during embryonic development characterized by left ventricular trabeculae, intertrabecular recesses and a thin compacted layer. LVNC can result in left ventricular impairment, cardiac arrhythmia and embolic disease. LVNC has a prevalence of 0.05% in the adult population and is associated with genetic syndromes such as Noonan or Barth. CASE PRESENTATION: A 33-year-old male with a history of Gardner’s Syndrome, desmoid tumors, hypertension, non-ischemic cardiomyopathy is evaluated for heart failure. Patient’s symptoms were consistent with NYHA Class III. Patient was prescribed guideline-directed medical therapy (GDMT) and referred for echocardiogram. Echocardiogram revealed an ejection fraction of 40-45% with heterogeneous calcifications within the left ventricular myocardium. Cardiac MRI was ordered for further evaluation. MRI revealed mild dilation and global hypokinesis of left ventricle with ejection fraction of 31%. Trabeculations were seen at the distal aspect of the left ventricular myocardium along with focal areas of perfusion defects consistent with calcifications. Findings were concerning for LVNC. The MRI also detected a large anterior chest wall mass measuring 20x9cm consistent with a desmoid tumor (Figure 1). Patient was continued on GDMT for heart failure and referred to cardiothoracic surgery for evaluation of tumor removal. DISCUSSION: LVNC is diagnosed based on left ventricular morphology. Echocardiogram is the best initial test for evaluation but cardiac MRI provides a better assessment of the severity of LVNC. LVNC predisposes patients to left ventricular systolic failure, malignant arrhythmias, and thrombotic events. Management of LVNC involves goal directed medical therapy for heart failure, anticoagulation, and ICD based on guidelines for nonischemic cardiomyopathy. However no specific guidelines for treatment of LVNC exist. Management of desmoid tumors involve surgical resection to negative margins to minimize reoccurrences. Radiotherapy is used as adjuvant therapy in cases of incomplete resection. CONCLUSIONS: Although LVNC is associated with genetic syndromes, no connection to Gardner’s Syndrome or desmoid tumors could be found in the literature. The presence of these 3 rare genetic diseases in one individual suggest that there may be a genetic link. Reference #1: Akinseye OA, Ibebuogu UN, Jha SK. Left Ventricular Noncompaction Cardiomyopathy and Recurrent Polymorphic Ventricular Tachycardia: A Case Report and Literature Review. Perm J. 2017;21:17-045. Reference #2: Courtney E. Bennett and Ronald Freudenberger, “The Current Approach to Diagnosis and Management of Left Ventricular Noncompaction Cardiomyopathy: Review of the Literature,” Cardiology Research and Practice, vol. 2016, Article ID 5172308, 7 pages, 2016. https://doi.org/10.1155/2016/5172308. Reference #3: Bölke E, Krasniqi H, Lammering G, et al. Chest wall and intrathoracic desmoid tumors: surgical experience and review of the literature. Eur J Med Res. 2009;14(6):240-3. DISCLOSURES: no disclosure on file for Francecso Franchi; No relevant relationships by Aaron Richardson, source=Web Response No relevant relationships by Jose Rivas, source=Web Response

Failure to Pace

4 month old female child diagnosed a case of long QT syndrome with recurrent polymorphic ventricular tachycardia (VT) storm with moderate left ventricular (LV) dysfunction with status epilepticus presented with recurrent seizures in an emergency. Patient was intubated and put on mechanical ventilation. Recurrent seizures subsided with intravenous midazolam and anticonvulsants. In view of unstable rhythm, recurrent polymorphic VT storm and unstable haemodynamic, patient required antiarrhythmic agents such as flecainide and multiple inotropic supports. There was frequent pause dependent VT hence it was decided to put on permanent pacemaker. A repeated pacing attempt failed and on table plan for cardiac sympathectomy was made. However, the parents refused for the later. The patient left back with her own rhythm and parents are prognosticated.

A REALLY LONG QT: VENTRICULAR TACHYCARDIA STORM POST TAKOTSUBO CARDIOMYOPATHY

Takotsubo cardiomyopathy (TTS) is associated with QT prolongation. Torsade de pointe (TdP) has been increasingly reported in TTS cases with severe QT prolongation. A 66 year old female presented with recurrent polymorphic ventricular tachycardia (figure 1K) and severely prolonged QTc 728 ms (figure

Primary hyperaldosteronism presenting as recurrent polymorphic ventricular tachycardia (RCDD code: VI-2)

Primary hyperaldosteronism (PH) is a frequently overlooked cause of secondary hypertension mostly due to an aldosterone-producing adrenal adenoma. We report the case of a 48-year-old female who presented with recurrent episodes of polymorphic ventricular tachycardia before an aldosterone-producing adrenal adenoma was diagnosed. Although her past medical history was remarkable for poorly-controlled hypertension since age 30, she had never been examined for secondary causes. The patient underwent adrenalectomy resulting in stabilisation of her clinical condition, although residual hypertension remained. Causes of atypical presentation and diagnostic pitfalls are discussed. JRCD 2018; 4 (1): 26-30.

The challenges of treating recurrent polymorphic ventricular tachycardia due to coronary vasospasm: Lessons from an interesting case

Coronary artery vasospasm can cause recurrent anginal episodes with ST-segment elevation. Vasospasm induced myocardial ischemia can lead to arrhythmias including life threatening ventricular tachycardia (VT). Percutaneous coronary intervention (PCI), although not routinely recommended for treating vasospastic angina, can be considered for discrete coronary spasm that is not amenable to vasodilator therapy. We present a challenging case of a 41-year-old lady with recurrent episodes of vasospastic angina and VT refractory to medical therapy, which was successfully treated with PCI and an implantable cardioverter defibrillator.

Acquired Complete Heart Block with Long QT Interval and Recurrent Polymorphic Ventricular Tachycardia: A Case Report

We are reporting a case of acquired complete heart block and long QT interval (a dispersion of repolarization that leads to polymorphic ventricular tachycardia) that has presented with loss of conscious and proved to be due to torsade de pointes. The patient responded well to cardiac pacing and beta blocker therapy. The association of complete acquired heart block and long QT interval is quite rare.

Licorice‐Induced Severe Hypokalemia with Recurrent Torsade de Pointes

A 38-year-old obese woman presented with recurrent polymorphic ventricular tachycardia secondary to persistent hypokalemia necessitating more than 40 DC shocks. All endocrine investigations for hypokalemia were negative with impression of "mysterious hypokalemia." On repeated inquiry, a hidden history of licorice use was elicited causing persistent hypokalemia. The case highlights a life threatening complication of licorice use. In addition, it reiterates the importance of repeated history taking in a patient with undiagnosed hypokalemia and torsade de pointes which avoided a device therapy.

Atrial standstill in a patient with progressive severe heart failure

Atrial standstill (AS) is a very rare condition characterized by complete loss of electrical and mechanical activity and excitability of the atria. Absence of P waves, bradycardia and junctional escape rhythm may suggest AS on surface ECG. In nearly half of the reported cases, the condition manifested with syncope [1, 2]. AS must be differentiated from common sinus node disease with sinus arrest or highgrade sinoatrial block that may have similar symptoms and surface ECG presentation [3]. AS has been rarely described to be primary with familial clustering and/or associated cardiac ion channel mutations [2, 4]. More commonly, it has been linked to heart disorders such as myocarditis, ischemic heart disease, or dilated cardiomyopathy [5–7]. AS occurring in the context of structural myocardial disease may indicate a poor prognosis [1, 8]. A 48-year-old female patient was referred to our emergency department due to fatigue, progressive dyspnoea on exertion and recurring syncope since a few months. Three years earlier she had presented to our outpatient clinic for recurrent pulmonary infections, where a normal ECG had been registered (Fig. 1a). Four months before she had been hospitalized at an external institution for ascites and pleural effusion of unknown origin and atrial fibrillation was diagnosed on surface ECG. A diuretic as well as oral anticoagulation were initiated. At presentation, the patient reported no family history of syncope or sudden death, and physical examination revealed cachexia (BMI 18.6 kg/m), but no obvious cardiopulmonary pathologies. Shortly after admission, the patient exhibited rapid polymorphic ventricular tachycardia (VT), requiring CPR and defibrillation. Twelve-lead surface ECG, recorded prior the arrhythmic event, showed absence of P waves with an escape rhythm of 48/min, a QRS duration of 70 ms, constant R–R intervals, and a prolonged QT interval of 600 ms (QTc 536 ms) (Fig. 1b). Laboratory findings revealed mild hypokalemia (3.2 mmol/ L), slightly elevated liver enzymes, an INR value of 2.1, N-terminal pro-BNP of 1,465 pg/mL (7.39 of normal value) and an ANP of 2.89 nmol/L (1.59 of normal value), whereas normal values were found for calcium, blood cell count, CRP, renal and thyroid functional parameters and CK. Chest X-ray revealed marked cardiomegaly, and the echocardiogram showed a dilated left ventricle with a severely reduced left ventricular ejection fraction (LVEF) of 20 %. Of note, LVEF had been reported to be 45 and 60 %, 2 and 3 months earlier, respectively. Furthermore, echocardiography showed dilated right and left atria along with moderate to severe tricuspid and moderate mitral regurgitation. A transesophageal echocardiogram revealed a thrombus in the left atrial appendage, where no significant flow was identifiable with Doppler imaging (Fig. 1c, d). Few hours after admission, the patient again developed recurrent polymorphic VT with syncope and need for defibrillation despite normalization of potassium levels (Fig. 1e). Coronary artery disease was ruled out angiographically and a temporary transvenous pacemaker was inserted. Myocardial biopsies were taken from the right ventricle, but provided no evidence for acute myocarditis [9], arrhythmogenic right ventricular dysplasia [10], amyloidosis or hemosiderosis. This was in line with findings from cardiac MRI, so that idiopathic dilated cardiomyopathy was diagnosed. With ventricular overdrive pacing at 80 bpm and initiation of beta-blocker medication VT did M. R. Schroeter (&) G. Hasenfus M. Zabel D. Vollmann Abt. Kardiologie und Pneumologie, Universitatsmedizin Gottingen, Herzzentrum, Robert-Koch-Str. 40, 37099 Gottingen, Germany e-mail: mschroeter@med.uni-goettingen.de

Successful Use of Quinine in the Treatment of Electrical Storm in a Child with Brugada Syndrome

A 10-year-old girl developed life-threatening recurrent polymorphic ventricular tachycardia following surgical closure of a simple secundum atrial septal defect. Post hoc analysis of a Holter recording suggested Brugada syndrome. After managing the acute phase, a dual chamber defibrillator was implanted. One week later she experienced VF storm, needing 96 appropriate shocks within a few hours. Quinidine, by virtue of its I(to) blocking property, is the only drug reported to be useful in managing VF storm in Brugada syndrome. Nonavailability of quinidine led us to try its diastereomer, intravenous quinine, which succeeded in controlling the ventricular tachycardia. Arrhythmia storm in the setting of ion channelopathy can be difficult to manage, and sometimes requires innovative therapies.

Löffler Endocarditis Presenting With Recurrent Polymorphic Ventricular Tachycardia Diagnosed by Cardiac Magnetic Resonance Imaging

A 56-year-old white man was admitted with palpitations, chest pressure, and dyspnea on exertion. He had an embolic stroke 2 months before admission without a defined embolic source. He had history of well-controlled hypertension and allergic asthma treated with theophylline. On admission, the resting ECG revealed sinus rhythm, right axis deviation, anterolateral ST-segment depression, and a normal QT interval (Figure 1). His cardiac biomarkers were elevated to indicate myocardial injury (troponin I, 2.66 ng/mL; creatine kinase-MB, 5.8 ng/mL). While in hospital, he developed episodes of polymorphic ventricular tachycardia (Figure 2) requiring multiple cardioversions and eventually temporary pacing. The initial peripheral blood count revealed a marked leukocytosis (22 000/μL) with a severe hypereosinophilia (47%, 10 000/μL). A transthoracic echocardiogram showed a normal-size left ventricle (LV) with normal global systolic function. The right ventricle (RV) was mildly enlarged with a mildly decreased systolic function. The LV and RV apexes appeared thickened and hypokinetic (Movie I in the online-only Data Supplement). Subsequent left and right cardiac catheterization demonstrated normal coronary arteries and elevated right and left heart filling pressures (right atrial pressure, 20 mm Hg; capillary wedge pressure, 23 mm Hg) with signs of restrictive physiology (Figure 3). An extensive workup for conditions associated with hypereosinophilia was negative. The patient had no rash or other evidence of vasculitis. Parasitosis was ruled out with serial …

Frequent Recurrent Polymorphic Ventricular Tachycardia during Sleep Due to Managed Ventricular Pacing

We report a case of a patient with an implantable cardioverter defibrillator and no prior history of heart block with managed ventricular pacing (MVP) programmed who had frequent recurrent episodes of polymorphic ventricular tachycardia. All of the episodes were initiated by transient atrioventricular block which resulted in short-long-short sequences permitted by MVP. This case illustrates that MVP should be used with caution not only in patients with complete heart block, but also in patients at risk for brief heart block due to such states as hypervagatonia due to sleep apnea.

Termination of Polymorphic Ventricular Tachycardia Storm by Catheter Ablation in a Patient with Cardiomyopathy Induced by Incessant Idiopathic Left Ventricular Tachycardia

We detail findings in a patient with incessant idiopathic left ventricular tachycardia (ILVT), induced cardiomyopathy, and an "electrical storm" consisting of recurrent polymorphic ventricular tachycardia (PVT). Catheter ablation not only eradicated the ILVT, but also additionally suppressed recurrent PVT. These findings suggest that the recurrent PVT storm in this patient related to long-standing tachycardia-induced cardiac electrical remodeling that led to QT prolongation.

P3-115: Recurrent polymorphic ventricular tachycardia associated with variant angina effective treatment with implantable cardioverter defibrillator

A 66 year-old man presented with recurrent nonexertional, substernal chest pain. The baseline ECG showed sinus bradycardia. Cardiac enzymes, exercise stress test, and echocardiogram were normal. On the first night, he was awakened by chest pain followed by repetitive bursts of nonsustained ventricular tachycardia, which spontaneously resolved. Cardiac catheterization revealed minimal atherosclerotic disease. An electrophysiology study showed no inducible ventricular arrhythmias, and a beta-blocker was initiated. He awoke with substernal chest pain the next night and the ECG monitor revealed ST segment elevation followed by rapid polymorphic ventricular tachycardia (VT) at 174 BPM (Figure 1, A-D). Vasospasm-induced ischemia was diagnosed and management was changed to nifedipine extended-release (ER) at a dose of 90 mg daily. VT recurred despite medical therapy, an ICD was implanted. A day after discharge, the patient experienced chest pain followed by an ICD shock. The electrogram recorded by the ICD showed rapid ventricular tachycardia at 267 BPM (Figure 2). The patient’s nifedipine ER dose was increased to 60 mg twice daily. The patient had no chest pain or ICD events at one-month follow-up. There is a paucity of data regarding the use of ICDs in cases of vasospastic angina with ventricular arrhythmias. We implanted an ICD because of the malignant nature of the patient’s arrhythmia and the inability to test the efficacy of therapy in preventing recurrence. Our case demonstrates the effectiveness of ICD therapy in this setting. View Large Image Figure Viewer