Figure 4 - available via license: Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International
Content may be subject to copyright.
Source publication
A 49-year-old woman, with no relevant family history, was admitted in 1996 for arrhythmic storm with polymorphic ventricular tachycardia (torsade de pointes) which degenerated into ventricular fibrillation. Iatrogenic causes were excluded, the electrocardiogram (ECG) was normal and there was no structural heart disease. She refused cardioverter-def...
Similar publications
Ventricular Arrhythmias (VAs) may present with a wide spectrum of clinical manifestations ranging from mildly symptomatic frequent premature ventricular contractions to life-threatening events such as sustained ventricular tachycardia, ventricular fibrillation and sudden cardiac death. Myocardial scar plays a central role in the genesis and mainten...
Ventricular tachycardia is a potentially life-threatening arrhythmia associated with an overall high morbi-mortality, particularly in patients with structural heart disease. Despite their pivotal role in preventing sudden cardiac death, implantable cardioverter-defibrillators, although a guideline-based class I recommendation, are unable to prevent...
Sudden cardiac death (SCD) occurs unexpectedly and is usually a result of ventricular arrhythmia in patients with structural heart disease. The implantable cardioverter-defibrillator (ICD), with or without biventricular pacing, has been proven to be protective for heart failure patients with reduced ejection fraction of <35 % (HFrEF). This device t...
Coronavirus disease 2019 (COVID-19) has affected medical practice. More than 7,000,000 patients died worldwide after being infected with COVID-19; however, no specific laboratory markers have yet been established to predict death related to this disease. In contrast, electrocardiographic changes due to COVID-19 include QT prolongation and ST-T chan...
A 49-year-old woman, with no relevant family history, was admitted in 1996 for arrhythmic storm with polymorphic ventricular tachycardia (torsade de pointes) which degenerated into ventricular fibrillation. Iatrogenic causes were excluded, the electrocardiogram (ECG) was normal and there was no structural heart disease. She refused cardioverter-def...
Citations
Short-coupled idiopathic ventricular fibrillation (IVF) is a subtype of IVF in which episodes of polymorphic ventricular tachycardia or ventricular fibrillation are initiated by short-coupled premature ventricular contractions (PVCs). Our understanding of the pathophysiology is evolving, with evidence suggesting that these malignant PVCs originate from the Purkinje system. In most cases, the genetic underpinning has not been identified. Whereas the implantation of an implantable cardioverter-defibrillator is uncontroversial, the choice of pharmacological treatment is the subject of discussion. In this review, we summarize the available knowledge on pharmacological therapy in short-coupled IVF and provide our recommendations for management of patients with this syndrome.
Background:
The short-coupled variant of torsade de pointes (scTdP) is characterized by a particular electrocardiogram (ECG) pattern that shows a short-coupling interval of the initial Tdp beat and that can degenerate into ventricular fibrillation without the presence of structural heart disease. However, its etiology, epidemiology, clinical characteristics, underlying mechanism, treatment, and prognosis remain unclear. This study aimed to systematically review case reports and series of scTdP to synthesize existing data on the demography, clinical characteristics, ECG features, management, and outcomes.
Methods:
A literature search was conducted for eligible published articles using the Medline, Embase, and PubMed databases. All eligible case reports and case series were included without any language restrictions. SPSS 24 was used for statistical analysis.
Results:
A total of 22 case reports and 103 case series of patients with scTdP were identified and included in the analysis. All selected cases had acceptable quality of evidence. Most young patients without sex differences had no trigger or a negative programmed simulation. The ECGs of all selected patients showed a short first-coupling interval (302 ± 62 ms) and a long QRS duration of ventricular extrasystole (VE) (135 ± 17 ms). The first coupling interval levels and QRS duration levels of VE were significantly longer and wider in patients with scTdP originating from the right ventricular outflow tract (RVOT) than in those with scTdP originating from the Purkinje fibers (380 ± 70 vs. 274 ± 28 ms, P < 0.001; 147 ± 8 vs. 131 ± 17 ms, P < 0.001), respectively. The receiver operating characteristic curve showed that the optimal cutoff values of the first coupling interval triggering TdP and QRS duration of VE were more than 319 ms and 141 ms (92% sensitivity, 95.7% specificity; 82.6% sensitivity, 77.8% specificity) for predicting the RVOT origin, respectively. The Kaplan-Meier survival curve revealed increased survival in patients with implantable cardioverter defibrillator (ICD) implantation than in patients without ICD implantation (log-rank =10.127, P = 0.001).
Conclusion:
Some agreements were confirmed in selected case reports regarding the clinical features, diagnosis, and management of scTdPs. Further large-scale and long-term follow-up studies are required to clarify the existing arrhythmogenic entities.
Idiopathic ventricular fibrillation is responsible for approximately 10% of cases of aborted cardiac arrest. Recent studies have shown that short-coupled ventricular premature complexes are present at the onset of idiopathic ventricular fibrillation in 6.6 to 17% of patients. The present review provided information on 86 patients with short-coupled malignant ventricular arrhythmias which were reported as case reports or small patient series during the last 70 years. In 75% of the 81 patients published during the last 40 years, extended information and follow-up (from 2.63+4.5 to 10.67+7.8 years, p<0.001, between the original publication to the latest update) could be obtained from the authors. The review shows that short-coupled malignant ventricular arrhythmias occurred almost equally in males and females, at the mean age of 40 years. A tendency for later occurrence of the arrhythmia by 4 years was observed in females. A prior history of syncope was noted in 45.3% of the patients while arrhythmic storm occurred in 42% at presentation. The most common mode of revelation of short-coupled malignant ventricular arrhythmias was syncope (53.5%), followed by aborted cardiac arrest (26.7%) and recurrent arrhythmic event after prior ICD implantation for idiopathic ventricular fibrillation (17.4%). For the first time, short-coupled malignant arrhythmias exhibiting “not-so-short” coupling intervals (>350ms) were found in a significant proportion of patients (17.4%). During long-term follow-up, quinidine yielded a slightly higher success rate in arrhythmia control than ablation. Larger studies are necessary to assess the best strategy for the management of this potentially lethal arrhythmia.
Introduction: Ventricular arrhythmias are often seen in association with structural heart disease. However, approximately a tenth of affected patients have apparently normal hearts, where such arrhythmias typically occur in young patients, are sometimes inherited and can occasionally lead to sudden cardiac death (SCD). Over the past two decades, increased understanding of the underlying pathophysiology resulted in improved targeted pharmacological therapy.
Areas covered: This article reviews current knowledge regarding drug therapy for inherited arrhythmia syndromes (Brugada, early repolarization, long QT and short QT syndromes, and catecholaminergic polymorphic ventricular tachycardia), and acquired arrhythmias (idiopathic ventricular fibrillation, short-coupled torsade de pointes, outflow tract ventricular tachycardia, idiopathic left, papillary muscle and annular ventricular tachycardias).
Expert opinion: In inherited arrhythmia syndromes, appropriate clinical and genetic diagnoses followed by proper selection and dosing of antiarrhythmic drugs are of utmost importance to prevent SCD, most often without the need of implantable cardioverter-defibrillators. In acquired arrhythmias, appropriate pharmacotherapy in selected patients can also provide symptomatic relief and avoid the need for invasive therapy. Further research is needed to develop novel antiarrhythmic drugs or targeted therapy to increase efficacy and limit side effects.
After the most common causes of sudden cardiac death including ischemic and structural heart disease have been ruled out, clinicians on the front lines of emergent medical care can be faced with unexplained and recurrent life-threatening arrhythmia episodes in children and adults. In these cases, an inherited arrhythmia syndrome should be suspected, and a departure from conventional advanced cardiac life support algorithms may be required. This review focuses on the electrocardiographic clues of an inherited arrhythmia syndrome that can be uncovered through a careful analysis of the baseline electrocardiogram (ECG) and classification of the presenting ventricular arrhythmia and its mode of onset. After presenting an informed working diagnosis and an explanation of the implied electrophysiologic mechanisms, discussion provides a protocol approach to acute and subacute management decisions. Careful attention to a patient's response to treatment and its ECG surrogates have the potential to facilitate tailored therapy based on the underlying arrhythmogenic substrate and pathophysiology.
