A Phenomenal Binomaly : 2 Rare Diseases Presenting With Electrical Storm
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ABSTRACT:
SUBMITTER: Buckley A
PROVIDER: S-EPMC10240273 | biostudies-literature | 2023 May
REPOSITORIES: biostudies-literature
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Project description:Electrical storm (ES) is a life threatening clinical situation. Though a few clinical pointers exist, the occurrence of ES in a patient with remote myocardial infarction (MI) is generally unpredictable. Genetic markers for this entity have not been studied. In the present study, we carried out genetic screening in patients with remote myocardial infarction presenting with ES by next generation sequencing and identified 25 rare variants in 19 genes predominantly in RYR2, SCN5A, KCNJ11, KCNE1 and KCNH2, CACNA1B, CACNA1C, CACNA1D and desmosomal genes - DSP and DSG2 that could potentially be implicated in electrical storm. These genes have been previously reported to be associated with inherited syndromes of Sudden Cardiac Death. The present study suggests that the genetic architecture in patients with remote MI and ES of unstable ventricular tachycardia may be similar to that of Ion channelopathies. Identification of these variants may identify post MI patients who are predisposed to develop electrical storm and help in risk stratification.
| S-EPMC5986548 | biostudies-literature
Project description:BackgroundThe study sought to assess the prognostic impact of chronic kidney disease (CKD) in patients with electrical storm (ES). ES represents a life-threatening heart rhythm disorder. In particular, CKD patients are at risk of suffering from ES. However, data regarding the prognostic impact of CKD on long-term mortality in ES patients is limited.MethodsAll consecutive ES patients with an implantable cardioverter-defibrillator (ICD) were included retrospectively from 2002 to 2016. Patients with CKD (MDRD-GFR < 60 ml/min/1.73 m2) were compared to patients without CKD. The primary endpoint was all-cause mortality at 3 years. Secondary endpoints were in-hospital mortality, cardiac rehospitalization, recurrences of electrical storm (ES-R), and major adverse cardiac events (MACE) at 3 years.ResultsA total of 70 consecutive ES patients were included. CKD was present in 43% of ES patients with a median glomerular filtration rate (GFR) of 43.3 ml/min/1.73 m2. CKD was associated with increased all-cause mortality at 3 years (63% vs. 20%; p = 0.001; HR = 4.293; 95% CI 1.874-9.836; p = 0.001) and MACE (57% vs. 30%; p = 0.025; HR = 3.597; 95% CI 1.679-7.708; p = 0.001). In contrast, first cardiac rehospitalization (43% vs. 45%; log-rank p = 0.889) and ES-R (30% vs. 20%; log-rank p = 0.334) were not affected by CKD. Even after multivariable adjustment, CKD was still associated with increased long-term mortality (HR = 2.397; 95% CI 1.012-5.697; p = 0.047), as well as with the secondary endpoint MACE (HR = 2.520; 95% CI 1.109-5.727; p = 0.027).ConclusionsIn patients with ES, the presence of CKD was associated with increased long-term mortality and MACE.
| S-EPMC8755690 | biostudies-literature
Project description:BackgroundIn cases of electrical storm, identifying the etiology is essential, as patients with reversible causes do not benefit from implantable cardioverter defibrillator (ICD). Given the diversity of pharmacologic and nonpharmacologic management tools available for hemodynamically unstable patients in electrical storm, all must be considered and tailored to each individual patient.Case descriptionThis report describes a 36-year-old female without prior cardiac history who presented in ventricular fibrillation (VF) electrical storm. While she lacked significant electrolyte abnormalities or ischemia to explain etiology of electrical storm, she incidentally had variant coronary anatomy noted on angiography. After thorough consideration of possible etiologies of storm, selective serotonin reuptake inhibitor (SSRI) intoxication was the most highly suspected etiology. Regarding management of her hemodynamically unstable electrical storm, she was treated with lidocaine, amiodarone, as well as mechanical circulatory support devices including extracorporeal membrane oxygenation (ECMO) and intra-aortic balloon pump (IABP). The patient ultimately was decannulated from ECMO, had IABP removal, and achieved recovery of ejection fraction (EF) to baseline. She was not offered ICD as the etiology of her cardiac arrest was reversable. She was discharged with recommendation to discontinue SSRI and follow up with psychiatry regarding SSRI overdose.ConclusionsIdentification of electrical storm etiology is crucial as reversible causes do not warrant ICD placement. Selection of appropriate pharmacologic and nonpharmacologic management in the hemodynamically unstable electrical storm patient is important given the wide range of available options.
| S-EPMC11760934 | biostudies-literature
Project description: Not available
| S-EPMC6404163 | biostudies-literature
Project description:BackgroundPatients with congenital long QT syndrome (LQTS) are at high risk for sudden cardiac death (SCD). Although several triggers can provoke ventricular fibrillation (VF) in patients suffering from LQTS acquired heart disease in addition to LQTS should not be overlooked.Case summaryWe present a case of a 47-year-old female patient who was diagnosed with congenital LQTS Type 2 at the age of 23 after surviving SCD. At that time, she underwent implantable cardioverter-defibrillator (ICD) implantation and was free of events for 24 years. Recently, the patient was referred to our institution after suffering from an ICD shock during sleep. Upon arrival she developed electrical storm and received overall six ICD shocks. The initial electrocardiogram (ECG) showed atrially triggered ventricular pacing. However, distinct ST segment elevations in the inferior leads could be observed. Thus, coronary angiography was immediately performed and showed subtotal occlusion of the right coronary artery, which was treated by drug-eluting stent implantation. Atrioventricular conduction immediately resumed after revascularization and the following non-paced ECG revealed a prolonged QT interval. Laboratory measurements confirmed acute myocardial infarction with elevated cardiac enzymes. The patient was put on betablockers, dual antiplatelet therapy, and statins and discharged in good condition.DiscussionThis case report highlights that diagnostic work-up in patients with LQTS presenting with VF should always include the search for additional acquired heart disease such as myocardial infarction, as a potential trigger for electrical storm. Moreover, signs of ischaemia can be discerned even in a paced ECG which should lead to immediate cardiac catheterization.
| S-EPMC9536292 | biostudies-literature
Project description:BackgroundDespite substantial technical improvements in long-term mechanical circulatory support, ventricular tachycardia (VT) remains a major challenge in left ventricular assist device (LVAD) patients. Recurrent VTs in LVAD patients are not only associated with limited quality of life, but also increased mortality. Although LVAD therapy improves VT tolerance of the left ventricle, haemodynamical deterioration of the right ventricle is the limiting factor in these patients.Case summaryWe present a case report of a hybrid epicardial VT ablation of incessant VTs in a 53-year-old man with advanced heart failure and St.p. LVAD implantation. With this unique clinical case report, we describe an epicardial VT ablation using secondary surgical open-heart access in a patient with therapy-refractory VTs combing left-sided minithoracotomy with high-density (HD) mapping and catheter ablation.DiscussionTo the best of our knowledge, the presented approach is the first interdisciplinary case of epicardial VT ablation using secondary surgical open-heart access in an LVAD patient with therapy-refractory VTs. By combing left-sided minithoracotomy with HD mapping and catheter ablation, we could to demonstrate that even in these complex patients epicardial VT ablations can be performed safely and improve quality of life of LVAD patients with recurrent implantable cardioverter-defibrillator shock therapies.
| S-EPMC8233488 | biostudies-literature
Project description:Sudden cardiac death, arising from abnormal electrical conduction, occurs frequently in patients with coronary heart disease. Myocardial ischemia simultaneously induces arrhythmia and massive myocardial leukocyte changes. In this study, we optimized a mouse model in which hypokalemia combined with myocardial infarction triggered spontaneous ventricular tachycardia in ambulatory mice, and we showed that major leukocyte subsets have opposing effects on cardiac conduction. Neutrophils increased ventricular tachycardia via lipocalin-2 in mice, whereas neutrophilia associated with ventricular tachycardia in patients. In contrast, macrophages protected against arrhythmia. Depleting recruited macrophages in Ccr2 -/- mice or all macrophage subsets with Csf1 receptor inhibition increased both ventricular tachycardia and fibrillation. Higher arrhythmia burden and mortality in Cd36 -/- and Mertk -/- mice, viewed together with reduced mitochondrial integrity and accelerated cardiomyocyte death in the absence of macrophages, indicated that receptor-mediated phagocytosis protects against lethal electrical storm. Thus, modulation of leukocyte function provides a potential therapeutic pathway for reducing the risk of sudden cardiac death.
| S-EPMC9410341 | biostudies-literature
Project description:Congenital long QT syndrome (LQTS) is a genetic heart disorder, which may lead to life-threatening arrhythmias, especially in children. Here, we reported two children who were initially misdiagnosed with epilepsy and experienced Torsades de Pointes (TdP) cardiac electrical storm (ES). Through whole exome sequencing (WES), we identified two Potassium voltage-gated channel subfamily H member 2 (KCHN2) mutations (c.1841 C > T and c.1838 C > T) respectively in a 6-year-old boy and a 13-year-old girl. Clinical data indicated that the QT interval was significantly prolonged, the T-wave pattern of chest V5-V6 leads and limb leads were inverted. Our study suggests that patients with epilepsy, especially those refractory epilepsy with atypical features, need comprehensive evaluation of cardiovascular function. KCNH2 mutation in pore region, QT interval prolongation and T wave inversion are high risk factors for ES. For LQT2 patients with ES, Nadolol and left cardiac sympathetic denervation are indicated, sometimes with an ICD.
| S-EPMC11468024 | biostudies-literature
Project description: Not available
| S-EPMC8717761 | biostudies-literature
Project description:Electrical storm during the acute inflammatory phase caused by myocarditis may be resistant to antiarrhythmic therapy. Cardiac imaging including magnetic resonance tomography, positron emission tomography, and endomyocardial biopsy are crucial to guide potential therapeutic options. Optimal management involves a multidisciplinary approach, including expertise beyond cardiology.
| S-EPMC5538064 | biostudies-other