Automatic Cardioverter Defibrillator Research Articles

СИНДРОМ БРУГАДА В КЛИНИЧЕСКОЙ ПРАКТИКЕ

Brugada syndrome is a congenital channelopathy with an autosomal dominant type of inheritance and low penetrance, characterized by the presence of a typical clinical and electrocardiographic symptom complex, including a special form of blockade of the right bundle branch block with ST segment elevation in one or more right chest leads on the electrocardiogram, the absence of organic pathology of the heart and various life-threatening ventricular arrhythmias, which can lead to the development of sudden cardiac death. The article defines the concept of "Brugada's syndrome", the geography and frequency of occurrence of the disease. It is indicated that more than 450 pathogenic variants in 24 genes are involved in the development of Brugada syndrome. The article presents two main hypotheses of pathogenesis, describes the electrocardiographic picture, three types of ECG changes, clinical manifestations and classification of the Brugada syndrome. Listed diseases with which it is necessary to carry out differential diagnosis of Brugada syndrome. Various aspects of the treatment of Brugada syndrome are presented: non-drug methods, pharmacotherapy, installation of an implantable automatic cardioverter-defibrillator, epicardial catheter ablation of abnormal areas of the anterior part of the outflow tract of the right ventricle.

Cardiocutaneous Syndrome: Naxos Disease

Naxos disease is a recessively inherited condition, caused by mutation in genes encoding desmosomal proteins Plakoglobin. It is characterized by peculiar woolly hair, palmoplantar keratoderma with arrhythmogenic Right Ventricular dysplasia /cardiomyopathy. A syndrome (a variant of Naxos) with same cutaneous phenotype and predominant Left Ventricular involvement is CARVAJAL syndrome. Patients are at risk of sudden cardiac death due to cardiomyopathy. We herein report a rare case of Naxos disease in a 16 years old male born of a 3º consanguineous marriage, presented with features suggestive of congestive cardiac failure with clubbing (grade 1), woolly hair, and focal keratoderma over palms and soles. ECG demonstrated – inverted T waves (V1-V3), QRS prolongation and complete RBBB. Echocardiography revealed-severe biventricular dysfunction with EF-15-20%, severe MR, severe TR and PAH. Patient was treated with diuretics, ß blockers and ACE inhibitors. These children may need implantation of automatic cardioverter defibrillator to prevent arrhythmogenic attacks and sudden cardiac death. CONCLUSION: For child with woolly hair and palmoplantar keratoderma, the pediatrician should provide a cardiac assessment, considering Naxos/Carvajal disease associated cardiomyopathy. When an early diagnosis is made, life expectancy can be increased by treatment of Heart failure and arrhythmias. Keywords: Naxos disease, Palmoplantar keratoderma, Cardiomyopathy, Wolly hair

P1475Impact of automatic screening and parasternal rights positions in the eligibility of patients with hypertrophic cardiomyopathy for subcutaneous automatic cardioverter defibrillator implant

Abstract Background A high percentage of failures in the detection of QRS and T wave in patients (pts) with hypertrophic cardiomyopathy (HCM) have been reported. This finding would prevent them from being eligible for an implantable subcutaneous automatic defibrillator (S-ICD). However, recently 2 changes in the detection have been proposed, automatic screening and the use of right parasternal position of the lead. The aim of our work was to study if the elegibility proportion of patients was increased with both 2 advacements. Methods We included 31 patients (18 male)with a diagnosis of HCM and at least 1 risk factor for sudden death, in follow-up at the outdoor clinic of of 2 cardiology centers. We performed elegibility screening test in supine position and standing using both the automatic screening (AS) obtained by Boston Scientific Zoom Latitude programmer) and the manual (MS), to simulate the detection of the 3 vectors utilized in S-ICD detection. And both screens were registered with the surface electrodes in parasternal left and right position. A pte was considered eligible if at least one vector was correct in supine position and in standing position, well in parasternal left or right position. Results Using MS with left parasternal position, 22 patients (71%) were eligible. Adding the right parasternal lead, the eligibility increased by 10%, reaching 81%. In addition, in automatic screening, eligibility in right shifts (81%) it is 7% more than in the left and, with the addition of the rights to the left, the eligibility reaches up to 84%. Figure shows the three-lead ECG factors influencing screening pass vs failure. Conclusion AS, right parasternal lead position and the combination of right and left parasternal lead position, increase the eligibility of sICD candidates with HCM. Abstract Figure. ECG factors influencing screening pass

Peri-procedural routines, implantation techniques, and procedure-related complications in patients undergoing implantation of subcutaneous or transvenous automatic cardioverter-defibrillators: results of the European Snapshot Survey on S-ICD Implantation (ESSS-SICDI).

The aim of this European Heart Rhythm Association (EHRA) prospective snapshot survey is to assess peri-procedural practices, implantation techniques, and short-term procedure-related complications associated with implantation of subcutaneous implantable cardioverter-defibrillator (S-ICD) or transvenous implantable cardioverter-defibrillator (TV-ICD), across tertiary European electrophysiology centres. An internet-based electronic questionnaire concerning implantation settings, peri-procedural routines, techniques, personnel, complications, and patient outcomes was sent to the centres routinely implanting both TV-ICDs and S-ICDs. The centres were requested to prospectively include consecutive patients implanted with either TV-ICD or S-ICD during the 8-week enrolment period. Overall, 20 centres from 6 countries enrolled 429 consecutive patients. Subcutaneous implantable cardioverter-defibrillators (20%) compared with TV-ICD were implanted mainly under general anaesthesia (72% vs. 14%), in the surgical operation room settings (69% vs. 43%), with more frequent prophylactic antibiotic administration (82% vs. 91%), and post-implant defibrillation testing (85% vs. 7%, all P < 0.05). Feasibility (implantation duration of 45 min) and short-term complication rates (4%) were comparable for S-ICDs and TV-ICDs, but the spectrum of complications varied, despite different baseline characteristics of patients undergoing the S-ICD vs. TV-ICD implantation. This EHRA snapshot survey provides important insights into the implantable cardioverter-defibrillator implantation routines and patient outcomes. Our study showed differences between the S-ICD and TV-ICD implantation routines with respect to implantation settings, peri-procedural management, and pre-defined procedural endpoints. However, the comparable duration of S-ICD or TV-ICD implantation and similar rates of peri-procedural complications indicate that both devices can be routinely used in clinical practice.

Video-assisted thoracoscopic cardiac sympathetic denervation for prolonged QT syndrome—a case report

Congenital long QT syndrome (LQTS) is a rare genetic disorder associated with multiple mutations causing life-threatening cardiac arrhythmias and sudden death. The treatment of choice for LQTS is therapy with beta blockers. Individuals for whom beta blockers are unsuccessful may be treated by implantable automatic cardioverter defibrillator or left cardiac sympathetic denervation. Video-assisted thoracoscopic left cardiac sympathetic denervation is a simple and minimally invasive technique that results in good long-term benefits in patients with congenital long QT syndromes. Although there is substantial literature about this therapy in adults, few reports detail the outcomes in children who undergo left thoracoscopic sympathectomy to treat LQTS.

Predicting adherence to use of remote health monitoring systems in a cohort of patients with chronic heart failure.

It is unclear whether subgroups of patients may benefit from remote monitoring systems (RMS) and what user characteristics and contextual factors determine effective use of RMS in patients with heart failure (HF). The study was conducted to determine whether certain user characteristics (i.e. personal and clinical variables) predict use of RMS using advanced machine learning software algorithms in patients with HF. This pilot study was a single-arm experimental study with a pre- (baseline) and post- (3 months) design; data from the baseline measures were used for the current data analyses. Sixteen patients provided consent; only 7 patients (mean age 65.8 ± 6.1, range 58-83) accessed the RMS and transmitted daily data (e.g. weight, blood pressure) as instructed during the 12 week study duration. Baseline demographic and clinical characteristics of users and non-users were comparable for a majority of factors. However, users were more likely to have no HF specialty based care or an automatic internal cardioverter defibrillator. The precision accuracy of decision tree, multilayer perceptron (MLP) and k-Nearest Neighbor (k-NN) classifiers for predicting access to RMS was 87.5%, 90.3%, and 94.5% respectively. Our preliminary data show that a small set of baseline attributes is sufficient to predict subgroups of patients who had a higher likelihood of using RMS. While our findings shed light on potential end-users more likely to benefit from RMS-based interventions, additional research in a larger sample is warranted to explicate the impact of user characteristics on actual use of these technologies.

Brugada syndrome: A major cause of sudden cardiac death

In 1992 a new syndrome was described consisting of syncopal episodes or sudden death in patients with a structurally normal heart and an electrocardiogram characteristic of right bundle branch block with ST segment elevation in leads V1 to V3. Brugada syndrome is an autosomal dominant disorder. It has been shown to be associated with mutations in the gene (SCN5A) that encodes for the sodiumion channel in cardiac myocyte. Over 160 mutations of gene SCN5A have been identified. The incidence of the disease is difficult to estimate, but it causes sudden deaths of 5 per 10,000 inhabitants per year and involved much more frequently in people of Asian ancestry. Diagnosis can be easily made by means of genetic analysis and ECG. Recent data suggest that loss of the action potential dome in the right ventricular epicardium underlies ST segment elevation seen in this syndrome. Right ventricular epicardium is preferentially affected because of the predominance of transient outward current in this tissue. Antiarrhythmic drugs like amiodarone and beta-blockers do not prevent death in symptomatic or asymptomatic individuals. Though Implantation of an automatic cardioverter–defibrillator is the only recently proven effective therapy; Quinidine has been found to decrease Ventricular fibrillation and could prove to be a secured option of implantable cardioverter–defibrillator. However, researcher set focus on gene therapy that may offer an enduring cure in future years. The purpose of this brief review is to record the past highlights that have brought us to our present understanding of Brugada syndrome.

Go with the flow: progress in mechanical circulatory support.

Go with the flow: progress in mechanical circulatory support.

Miocardiopatía arritmogénica del ventrículo derecho. Breve revisión de la literatura a propósito de un caso

A 51-year-old man was admitted to this hospital because of palpitations and a feeling of dizziness for a period of 2h. The electrocardiogram revealed a regular wide-QRS complex tachycardia at a rate of 250 beats per minute, with superior axis and left bundle branch block morphology without hemodynamically decompensation, the patient was cardioverted to sinus rhythm after the administration of a loading and maintenance dose of amiodarone. The elechtrophysiological study showed the ventricular origin of the arrhythmia. In order to diagnose the etiology of the ventricular tachycardia we performed a coronary arteriography that showed normal epicardial vessels, thus ruling out coronary disease. Doppler echocardiography revealed systolic and diastolic functions of both left and right ventricles within normal parameters, and normal diameters as well. A cardiac magnetic resonance with late enhancement was done, showing structural abnormalities of the right ventricle wall with moderate impairment of the ejection fraction, and a mild dysfunction of the left ventricle. The diagnosis of arrhythmogenic right ventricular cardiomyopathy was performed as 2 major Task Force criteria were met. We implanted an automatic cardioverter defibrillator as a prophylactic measure. The patient was discharged without complications.

Acute Myocarditis Presenting with Ventricular Arrhythmias: The Role of CMR in the Differential Diagnosis of ARVD

We herein present the findings of the case of a 23-year-old man who was hospitalized for ventricular tachycardia (VT) with no previous history of cardiac disease or any family history of sudden death. Based on the clinical features as well as the echographic and MRI results, the patient was diagnosed with both acute viral myocarditis and arythmogenic right ventricular dysplasia (ARVD). The patient underwent implantation of an automatic cardioverter defibrillator. There was no recurrent VT during the 24 month follow-up. This case demonstrates the link between ARVD and myocarditis, and highlights the importance of conducting an RV assessment through a cardiac magnetic resonance (CMR) study in the context of arrhythmia and myocarditis.

Left Thoracoscopic Sympathectomy Used as Primary Therapy for a Young Child With Intractable Long QT Syndrome

A 3-year-old boy with familial long QT syndrome type 2 presented with recurrent syncope despite adequate beta-blocker therapy. Two family members had experienced sudden cardiac arrest, and one other relative had experienced sudden cardiac death. Given the high risk for ventricular arrhythmia/syncope, the decision was made to perform primary cardiac denervation therapy through a minimally invasive approach without concomitant automatic cardioverter-defibrillator implantation. Using video-assisted thoracoscopic surgery, the left-sided sympathetic ganglia from T2-T5 were identified, and dissection along the sympathetic chain with transection of the corresponding rami along T2-T5 in addition to the lower half of the stellate ganglion was performed. The chest tube was removed on day 1 after surgery, and the patient was discharged on postoperative day 4. During 14 months of follow-up evaluation, no intervening episodes of ventricular arrhythmia or syncope and no symptoms of Horner's syndrome were noted.

Left thorascopic sympathectomy for refractory long QT syndrome in children

Congenital long QT syndrome (LQTS) is a rare genetic ion transmembrane disorder that has been associated with multiple various genetic mutations including life-threatening cardiac arrhythmias and sudden death. Left thorascopic sympathectomy is an effective treatment for patients who are refractory to medical therapy or who need frequent epicardial internal cardiodefibrillator intervention. Although there is substantial literature about this therapy in adults, few reports detail the outcomes in children who undergo left thorascopic sympathectomies to treat LQTS. The authors report the successful use of a left thoracic sympathectomy for the treatment of an 11-year-old girl who had persistently symptomatic LQTS, even after implantation of an automatic cardioverter-defibrillator. The patient remained clinically stable without arrhythmias through 6 months' of follow-up. The authors also reviewed the relevant literature and found that it suggests that 77% of patients will have immediate resolution of their symptoms/arrhythmias after the procedure. When the outcome definition was broadened to include patients who had only 1 or 2 cardiac episodes in the follow-up period, 88% of cases were considered successful. The results of this case study and literature review suggest that left thorascopic sympathectomy is a safe and effective approach for treating pediatric patients with LQTS.

Are Ultrafine Particles a Risk Factor for Cardiovascular Diseases?

1885-5857/$ – see front matter 2011 Sociedad Esp doi:10.1016/j.rec.2011.05.006 Numerous epidemiological studies support the association of exposure to air pollution with adverse health effects leading to enhanced morbidity and mortality of considerable significance. In fact, the World Health Organization ranks it as the 13th leading cause of worldwide mortality. Cumulative evidence over the last decade suggests that the largest portion of air pollution-related mortality is due to cardiovascular diseases, on the basis of which it has been proposed to be a ‘‘modifiable’’ novel cardiovascular risk factor of great importance. While air pollution is a complex mixture of compounds in the gaseous and particle phases, more evidence implicates the particulate matter (PM) components as responsible for a major portion of the cardiovascular effects. The PM components are classified according to their aerodynamic diameter into size fractions such as PM10 (‘‘thoracic’’ particles, < 10 mm), PM2.5-10 (‘‘coarse’’ particles, 2.5 to 10 mm), PM2.5 (‘‘fine’’ particles, < 2.5mm) and UFP (‘‘ultrafine’’ particles, < 0.1 mm). These PM of different sizes appear to carry different abilities to cause harmful effects and there is increasing debate about the notion that systemic cardiovascular effects could be favored by a smaller particle size. Thus, exposure to ambient PM leads to enhanced cardiovascularmorbidity andmortality due to amyriad of acute and chronic effects. Acute exposure to PM has been associated with the triggering of acute myocardial infarctions, discharge of implanted automatic cardioverter defibrillators, hospitalizations for ischemic strokes, and decompensated congestive heart failure. Therefore, the article by Dominguez-Rodriguez et al. published in Revista Espanola de Cardiologia is very important as it approached the question whether air pollution could preferentially associate with hospital admissions due to heart failure (HF) vs acute coronary syndromes (ACS), in a terciary university hospital in Tenerife, Spain. While exposure to ambient particulate could enhance the incidence of both HF and ACS, leading to increased hospital admissions due to both causes, the study design evaluated whether there were differences between the two types of admissions. There was a small variation in environmental exposure parameters which impeded the authors from conducting a time series analysis.

Strategies for the prevention and treatment of sudden cardiac death

Cardiovascular diseases account for 40% of all deaths in the West. Sudden cardiac death (SCD) is a major health problem affecting over 300,000 patients annually in the United States alone. Presence of coronary artery disease (CAD), usually in the setting of diminished left ventricular ejection fraction, is still the single major risk factor for SCD. Additionally, acute myocardial ischemia, structural cardiac defects, anomalous coronary arteries, cardiomyopathies, genetic mutations, and ventricular arrhythmias are all attributed to SCD, demonstrating the perplexity of this condition. With the recent advancements in cardiovascular medicine, the incidence of SCD is expected to increase steeply as the prevalence of CAD and heart failure is uprising in general population. Considering SCD, the major challenge confronting contemporary cardiology, multiple strategies for prevention against SCD have been developed. β-blockers have been shown to reduce the risk of SCD, whereas implantable cardioverter–defibrillator devices are found to be effective at terminating the malignant arrhythmias. In recent years, multiple clinical trials were carried out to identify patients who may benefit from preventive intervention, including medical therapy and automatic cardioverter–defibrillator implantations. This review article provides insight into the advanced strategies for the prevention and treatment of SCD based on the data available in medical literature to date.

Serotonin and catecholaminergic polymorphic ventricular tachycardia : a possible therapeutic role for SSRIs?

SummaryCatecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare malignant arrhythmia, usually diagnosed in the adolescent years. The diagnosis can typically be made by one or more of the following: a positive family history, exercise electrocardiography, ambulatory ECG monitoring and/ or an intra-cardiac, electrophysiological examination. This is a case report of a patient with CPVT that was refractory to treatment with beta-blockade and an implanted automatic cardioverter defibrillator. However, after a selective serotonin re-uptake inhibitor (SSRI) was added to the therapeutic regimen, no further episodes of ventricular tachycardia occurred during the following two years.

Utility and Safety of Axillo-subclavian Venous Imaging with Carbon Dioxide (CO2) Prior to Chronic Lead System Revisions

Prior to attempting placement of one or more electrodes to revise existing rhythm control devices, patency of the central veins should be documented, in view of a high incidence of significant chronic occlusions. Since iodinated contrast venography may be contraindicated in select situations, imaging of the axillo-subclavian venous system with gaseous carbon dioxide (CO(2)) was evaluated prospectively in 23 consecutive individuals who were considered for revision of previously implanted pacemaker or automatic cardioverter defibrillator lead systems. Approximately 20 mL of CO(2) were manually infused via CO(2) primed injection tubing into a vein at or above the level of the antecubital fossa ipsilateral to the side of prior lead placements. Digital subtraction imaging over the axillo-subclavian region, lower neck, and mediastinum was performed. Formal interpretation was obtained from one of three interventional radiologists and at least one electrophysiologist. Significant venous occlusions were identified in five (22%) patients. Vascular access utilized for the subsequent 18 revisions performed included the imaged patent ipsilateral vein in 14 patients and the contralateral, right-sided subclavian venous system in three patients. One patient required epicardial left ventricular lead placement. There were no complications from venography. Axillo-subclavian venography with gaseous CO(2) in patients undergoing pacemaker or implantable cardioverter defibrillator lead revisions is feasible and safe when use of iodinated dye is contraindicated. This technique should be employed in patients with azotemia, dye contrast allergies, or significant inflammation in the vicinity of the intravenous line insertion.

Brugada-Syndrom

In 1992, a new syndrome (Brugada syndrome, BS) was described consisting of syncopal episodes and/or sudden death in patients with a structurally normal heart and an electrocardiogram characteristic of right bundle branch block with ST segment elevation in leads V 1 -V 3 . The disease is genetically determined with an autosomal dominant pattern of transmission, and different mutations that affect the structure and function of the cardiac sodium channel gene SCN5A have been identified. BS can be diagnosed by ECG if the manifest form is present; at the present time, three different ECG types of BS are known. In patients with transient forms, the ECG can be modulated by the administration of antiarrhythmic drugs. Application of ajmaline (1 mg/kg i.v.), procainamide (10 mg/kg i.v.) or flecainide (2 mg/kg i.v.) accentuate ST segment elevation or are capable of unmasking concealed or intermittent forms of the disease. BS has been described as being associated with life-threatening ventricular tachyarrhythmias and a high incidence of sudden death. Antiarrhythmic drugs such as amiodarone or beta-blocking agents do not prevent sudden death in symptomatic or asymptomatic individuals. Implantation of an automatic cardioverter defibrillator is the only form of treatment currently proven to be effective. Gene therapy may offer a cure in the future.

Cardiac Device Implantation in the United States from 1997 through 2004: A Population-based Analysis

OBJECTIVEUse of cardiac devices has been increasing rapidly along with concerns over their safety and effectiveness. This study used hospital administrative data to assess cardiac device implantations in the United States, selected perioperative outcomes, and associated patient and hospital characteristics.METHODSWe screened hospital discharge abstracts from the 1997–2004 Healthcare Cost and Utilization Project Nationwide Inpatient Samples. Patients who underwent implantation of pacemaker (PM), automatic cardioverter/defibrillator (AICD), or cardiac resynchronization therapy pacemaker (CRT-P) or defibrillator (CRT-D) were identified using ICD-9-CM procedure codes. Outcomes ascertainable from these data and associated hospital and patient characteristics were analyzed.MEASUREMENTS AND MAIN RESULTSApproximately 67,000 AICDs and 178,000 PMs were implanted in 2004 in the United States, increasing 60% and 19%, respectively, since 1997. After FDA approval in 2001, CRT-D and CRT-P reached 33,000 and 7,000 units per year in the United States in 2004. About 70% of the patients were aged 65 years or older, and more than 75% of the patients had 1 or more comorbid diseases. There were substantial decreases in length of stay, but marked increases in charges, for example, the length of stay of AICD implantations halved (from 9.9 days in 1997 to 5.2 days in 2004), whereas charges nearly doubled (from $66,000 in 1997 to $117,000 in 2004). Rates of in-hospital mortality and complications fluctuated slightly during the period. Overall, adverse outcomes were associated with advanced age, comorbid conditions, and emergency admissions, and there was no consistent volume–outcome relationship across different outcome measures and patient groups.CONCLUSIONSThe numbers of cardiac device implantations in the United States steadily increased from 1997 to 2004, with substantial reductions in length of stay and increases in charges. Rates of in-hospital mortality and complications changed slightly over the years and were associated primarily with patient frailty.

Brugada Syndrome in Pure Black Africans

Although all races are concerned with the Brugada syndrome, no case has ever been reported among black Africans. We describe five different cases in this specific group of populations. In all patients, Brugada syndrome was identified after detailed noninvasive and invasive evaluations. Sex ratio was four males for one female. Convulsive syncope was noticed in 1 patient with a family history of sudden death. Diagnostic coved-type pattern was observed spontaneously in the normal position of right precordial leads in 3 patients and in a higher position of leads in 3 patients. Sixty percent had first-degree atrioventricular block. An ajmaline test was performed in 4 patients and was positive either in normal position of leads or in superior position in all of them. Sustained ventricular tachycardia (VT) or ventricular fibrillation (VF) was inducible during programmed ventricular stimulation in 3 patients. Right ventricular cineangiography found localized apical hypokinesia with preserved systolic function in 1 patient. Automatic cardioverter defibrillator was implanted in 2 patients. SCN5A was not found in any of the patients. These observations demonstrate that Brugada syndrome is also present in black African populations, and increasingly reported cases of apparent sudden death in the sub-Saharan part of the world need to rule out cardiac electrical disturbance such as Brugada syndrome.

Malignant Arrhythmias Secondary to a Cardiac Fibroma Requiring Transplantation in a Teenager

We report a case of a 13-year old girl with a diagnosis of cardiac fibroma who was followed for a decade. Although she was predominantly asymptomatic over this time period, she eventually developed significant ventricular arrhythmias. The arrhythmias were originally treated with anti-arrhythmic medications along with an automatic internal cardioverter-defibrillator. However, a nearly fatal event prompted a cardiac transplantation. This represents a unique case of a patient with cardiac fibroma who was bridged to transplantation with an internal cardioverter-defibrillator.