Implantation Of A Cardioverter Defibrillator Research Articles

Permanent pacemaker at the time of coronary artery bypass grafting and long-term outcome: a nationwide study from the SWEDEHEART registry

Abstract Introduction A permanent pacemaker is not rare in patients who undergo coronary artery bypass grafting (CABG). It is unknown if the presence of a pacemaker influences the long-term prognosis after surgery. Purpose To investigate if patients with permanent pacemaker have a higher risk for severe complications after CABG. Methods All patients who underwent CABG in Sweden 2006-2020 were included in a nationwide observational study based on prospectively collected data. Exclusion criteria were implantation of a cardioverter defibrillator at any time (prior to or after surgery) and death within 30 days. Individual patient data was merged from SWEDEHEART, The Swedish ICD- and pacemaker registry, the Cause of Death Register and the National Patient Register. Multivariable Cox regressions models were used to obtain hazard ratios (HR) with 95% confidence intervals (95%CI). The model was adjusted for age at operation, sex, left ventricular ejection fraction, heart failure, previous myocardial infarction, diabetes, peripheral artery disease, chronic respiratory disease, hypertension and atrial fibrillation. Results In total 41,722 patients underwent CABG during the study period, 555 (1.3%) of these had a previously implanted permanent pacemaker. Patients with a previous pacemaker were older, more often male, and had more often reduced left ventricular ejection fraction heart failure, atrial fibrillation, diabetes, hypertension and peripheral artery disease. Permanent pacemaker was not associated with higher risk for all-cause mortality (adjusted HR (aHR) 1.09 (95%CI=0.96-1.26), p=0.30, Figure 1), myocardial infarction (aHR: 1.01 (95%CI=0.73-1.41), p=0.94), or cardiovascular mortality (HR: 1.19 (95%CI=0.98-1.44), p=0.082). However, permanent pacemaker implantation was associated with increased risk for heart failure hospitalization (HR: 2.16 (95%CI=1.84-2.53), p<0.001). Conclusions A permanent pacemaker before CABG is not associated with increased mortality, cardiovascular mortality nor new myocardial infarction, but an increased risk for heart failure hospitalization after CABG. These patients could benefit from increased surveillance for heart failure symptoms after surgery to prevent hospitalizations.

Prognostic Value of [99mTc]Tc-DPD Quantitative SPECT/CT in Patients with Suspected and Confirmed Amyloid Transthyretin-Related Cardiomyopathy and Preserved Left Ventricular Function.

Quantitative 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid ([99mTc]Tc-DPD) SPECT may be used for risk-stratifying patients with amyloid transthyretin-related cardiomyopathy (ATTR-CM). We aimed to analyze the predictive value of quantitative [99mTc]Tc-DPD SPECT/CT in suspected and confirmed ATTR-CM according to different disease stages. Methods: The study enrolled consecutive patients with suspected ATTR-CM who were referred to a single tertiary center and underwent quantitative [99mTc]Tc-DPD SPECT/CT allowing SUVmax and SUVpeak analysis. Patients were divided into 2 groups according to left ventricular ejection fraction (LVEF) at baseline (i.e., ≥50% and <50%). Clinical, laboratory, and echocardiographic parameters and major adverse cardiac events (i.e., all-cause death, sustained ventricular tachyarrhythmia, hospitalization for heart failure, implantation of a cardioverter defibrillator) were investigated for any correlation with quantitative uptake values. Results: In total, 144 patients with suspected ATTR-CM were included in the study (98 with LVEF ≥ 50% and 46 with LVEF < 50%), of whom 99 were diagnosed with ATTR-CM (68.8%; 69 with LVEF ≥ 50% and 30 with LVEF < 50%). A myocardial SUVmax of at least 7 was predictive of major adverse cardiac events at 21.9 ± 13.0 mo of follow-up (hazard ratio, 2.875; 95% CI, 1.23-6.71; P = 0.015) in patients with suspected or confirmed ATTR-CM (global χ2 = 6.892, P = 0.02) and an LVEF of at least 50%. SUVmax was not predictive in patients with an LVEF of less than 50% and suspected or confirmed ATTR-CM. Conclusion: In patients with suspected or confirmed ATTR-CM and preserved LVEF, representing an early disease stage, quantitative [99mTc]Tc-DPD SPECT should be considered to improve early-stage risk stratification.

Європейські рекомендації 2023 року щодо лікування кардіоміопатій та впровадження їх у практику в Україні

In this article we analyzed the main positions of the recommendations of the European Society of Cardiology regarding the treatment of cardiomyopathies and presented the actual issues of implementing recommendations into clinical practice in Ukraine. The diagnostic flowchat of a patient with suspected cardiomyopathy is presented. Five phenotypes of cardiomyopathies are presented with current definition. New fenotype of non-dilated left ventricular cardiomyopathy is presented. The role of various imaging methods in diagnosis of cardiomyopathy phenotype is considered. The role of methods of genetic testing and magnetic resonance imaging of the heart with contrast is determined. The attention is focused on the need to differentiate the etiology of phenotypes and phenocopies of cardiomyopathies for pathogenetic treatment. Methods of assessing the risk of sudden cardiac death in cardiomyopathies and methods of secondary and primary prevention are discussed. Calculators for the risk of sudden death in cardiomyopathies and indications for implantation of a cardioverter-defibrillator are given. The management of patients with atrial fibrillation in various cardiomyopathies is determined. The issue of treatment of heart failure in patients with different phenotypes and ethiology of cardiomyopathies is discussed. Attention is focused on the role of «cardiomyopathy teams» in cardiological centers. An experience of echocardiographic diagnostics of cardiomyopathies, genetic testing, usage of magnetic resonance imaging in cardiomyopathies is presented.

Syncope and loss of consciousness after implantation of a cardioverter-defibrillator in patients with Brugada syndrome: Prevalence and characteristics in long-term follow-up.

Syncope is a significant prognostic factor in patients with Brugada syndrome (BrS). However, the risk of ventricular arrhythmia in patients with nonarrhythmic loss of consciousness (LOC) is similar to that in asymptomatic patients. LOC events after implantable cardioverter-defibrillator (ICD) implantation may provide insights into underlying causes of the initial LOC episode. The purpose of this study was to examine LOC characteristics following ICD implantation. We retrospectively analyzed 112 patients with BrS (mean age 47 years; 111 men) who were treated with an ICD. The patients were classified into 3 groups based on symptoms at implantation: asymptomatic (35 patients); LOC (46 patients); and ventricular tachyarrhythmia (VTA) (31 patients). We evaluated the incidence and cause of LOC during long-term follow-up after ICD implantation. During mean follow-up of 12.2 years, 41 patients (37%) experienced LOC after ICD implantation. Arrhythmic LOC occurred in 5 asymptomatic patients, 14 LOC patients, and 16 patients with VTA. Nonarrhythmic LOC, similar to the initial episode, occurred after ICD implantation in 6 patients with prior LOC (2 with neurally mediated syncope and 4 with epilepsy). Most epileptic patients experienced LOC during rest or sleeping, and did not show an abnormal encephalogram during initial evaluation of the LOC episodes. After ICD implantation, 13% of patients had nonarrhythmic LOC similar to the initial episode. Accurate classification of LOC based on a detailed medical history is important for risk stratification, although distinguishing arrhythmic LOC from epilepsy-related LOC episodes can be challenging depending on the circumstances and characteristics of the LOC event.

New old life of patients after implantation of implantable cardioverter-defibrillator

The aim of this article is to provide a detailed description of patients' lives after implantation of an implantable cardioverter defibrillator (ICD). First, the basic function and mechanism of the device as well as the types of experienced shocks, including phantom shocks, are described. Then, the three most important dilemmas faced by patients are discussed: ICD implantation, battery replacement and device deactivation. Subsequently, the scientific literature on psychopathological symptoms in patients is reviewed. The latest results of research on anxiety and depression in patients with ICD are presented, and the relationship between anxiety and depression and experienced shocks is discussed in detail. The results of a few studies on the occurrence of stress and post-traumatic stress disorder in patients are also presented. In addition, the focus was on the perception of the implantation of a cardioverter defibrillator as a traumatic event. The aim of this article was also to discuss the quality of life of patients with a cardioverter defibrillator. The results of studies that indicate a deterioration, improvement or maintenance of the level of quality of life of patients comparable to those of healthy people are presented. In addition, a specific, important from the perspective of people with ICD aspect of the quality of life, which is the health-related quality of life, was discussed. The results of research in this area are also not unambiguous. In addition, predictors of health-related quality of life were indicated. The article also addresses the issue of changing the identity of patients after implantation of a cardioverter defibrillator. The results of research conducted in this field, which were most often of a qualitative nature, were discussed. In the discussion on the identity of people with ICD, changes in self-image were emphasized, but this issue was not a frequent subject of psychological research. Finally, the challenges faced by partners of patients with an implanted cardioverter defibrillator were characterized. Also in this area, most of the research was qualitative, and the data come from interviews with relatives of cardiac patients.

Brugada syndrome is still a current clinical problem. What do we know today - a literature review

Brugada syndrome (BrS) is a rare congenital arrhythmia syndrome. BrS is characterized by a tendency to syncope, ventricular arrhythmias and sudden cardiac death. These symptoms occur mainly at night and can occur spontaneously or as a result of provocation by, for example, fever, stress or medication. Based on the differences in electrocardiography, three types were distinguished. Genetic studies are being conducted, which have shown a significant defect of the SCN5A gene, which in turn leads to disturbances in the functioning of the sodium channel NaV1.5 has a negative impact on the course of depolarization of myocardial cells. Patients with symptoms of the disease are subjected to implantation of a cardioverter-defibrillator, which protects them against sudden death. Data was obtained from the PubMed and Google Scholar platforms.

Cardiovascular system damage in the late-onset Pompe disease

The article presents a clinical case of metabolic hypertrophic cardiomyopathy against the background of a late-onset form of Pompe disease, illustrating the difficulties of differential diagnostic search for the cause of the disease. The clinical, laboratory and genetic aspects of the diagnosis of Pompe disease are highlighted. The features of laboratory diagnostics, the difficult path to the correct diagnosis and the appointment of enzyme replacement therapy are discussed. Much attention is paid to the clinical symptoms of the disease — the most significant damage to the cardiovascular system, there is no damage to the musculoskeletal sphere. Clinical picture of late Pompe disease is presented: cardiac rhythm and conduction disorders (ventricular preexcitation syndrome — multiple additional atrioventricular fenestrations), unstable ventricular tachycardia, supraventricular tachycardia, sinus node weakness syndrome. Considered approaches to the prevention of sudden cardiac death the patient underwent surgical treatment: radiofrequency ablation, endocardial implantation of a cardioverter defibrillator. Pathogenetic therapy for Pompe disease has been started.

Features of Diagnostics and Course of Hypertrophic Cardiomyopathy in Real Clinical Practice

Introduction and purpose. Hypertrophic cardiomyopathy (HCM) is characterized by left ventricular (LV) wall thickening not associated with increased afterload (hypertension and aortic stenosis), is usually caused by mutations in sarcomeric protein genes, and is inherited in an autosomal dominant manner. Unlike HCM, myocardial hypertrophy in its phenocopies is associated with the accumulation of substances such as amyloid, glycogen, etc. in the myocardium. The aim of our work was to analyze the features of the diagnosis and course of HCM in real clinical practice. Material and methods. A retrospective analysis of medical records of 80 patients (56.3 % of men) discharged with a diagnosis of HCM from a multidisciplinary hospital in Moscow in the period from 2007 to 2021 was carried out. The diagnosis of HCM in all patients was established on the basis of echocardiography data. The median age (25th and 75th percentiles are indicated in brackets) was 57 (48.5; 63) years. The duration of hospitalization was 8 (6; 12.5) days. Results. The reason for hospitalization was angina syndrome in 35 %, suspicion of acute coronary syndrome in 16.3 %, paroxysmal atrial fibrillation (AF) in 11.3 %, decompensation of chronic heart failure in 11.3 %, syncope in 7.5 % %, hypertensive crisis in 3.8 %, coronary angiography in 3.8 %, pacemaker implantation in 2.5 %, consultation with an arrhythmologist in 2.5 %, implantation of a cardioverter-defibrillator in 1.2 %, medical examination to resolve the issue of fitness for military service in 1.2 %, acute cerebrovascular accident in 1.2 %, hypotension in 1.2 %, drug bradycardia in 1.2 % of patients. Before hospitalization, a history of myocardial infarction was diagnosed in 15 %, arterial hypertension — in 53.8 %, chronic heart failure — in 77.6 %, chronic kidney disease — in 21.3 % of patients. Prior to the analyzed hospitalization, a history of myocardial infarction was diagnosed in 15 %, arterial hypertension in 53.8 %, chronic heart failure in 77.6 %, chronic kidney disease in 21.3 % of patients. LV wall thickness ≥1.5 cm was detected in 91.2 %, symmetrical form of hypertrophy — 22.1 %, apical — 5.2 %, papillary muscle hypertrophy — 1.3 %, interventricular septum — 71.4 % of patients. Permanent obstruction of the LV outflow tract (LVOTO) was detected in 62.8 % (9.0 % of patients had a history of septal reduction), transient LVOTO — in 1.3 %, non-obstructive HCM — in 35.9 %. The ejection fraction (EF) of the LV (according to Simpson) was 63 (55-70) %, CHF with reduced LV EF &lt;40 % was detected in 3.8 %, with a moderately reduced LV EF (40-49 %) — in 5 %, with preserved LV EF — in 68.8 % of patients. Anterior systolic movement of the anterior leaflet of the mitral valve occurred in 47.5 %, prolapse of the anterior leaflet of the mitral valve was described in 7.14 %. Mitral regurgitation was registered in 75 % of patients. 45 % of patients with HCM suffered from AF: permanent 15 %, paroxysmal 23.8 %, persistent 6.2 % of patients. During hospitalization, ventricular tachycardia was registered in 7.5 %, supraventricular tachycardia — 3.8 %, conduction disturbances were noted in 36.3 % of patients, of which atrioventricular block in 6.3 %, blockade of the right bundle branch block in 21.3 %, left bundle branch block in 15 %, and Wolff-Parkinson-White syndrome in 1.3 % . Implantation of a pacemaker in history was in 5 %, including in connection with MorgagniAdams-Stokes attacks — in 3.8 % of patients. During a median follow-up of 87 (interquartile range 45–131.5) months, 13.8 % of patients with HCM died. In deceased patients, LVOTO was significantly more common (in the dead 100 %, in the living 58.2 %, p = 0.006) and AF (in the dead 72.7 %, in the living 40.6 %, p = 0.047). Genetic testing and exclusion of HCM phenocopies was not performed during hospitalization and was not recommended for any patient. Conclusion. In real clinical practice, in most cases, only phenotypic diagnosis of HCM is carried out according to echocardiography, and screening for genetic mutations and HCM phenocopies is not performed. It is necessary to widely introduce genetic testing and screening for HCM phenocopies for the timely diagnosis of pathology that requires the appointment of specific pathogenetic therapy to improve the prognosis of patients

Cardiovascular rehabilitation with a WCD

Abstract Funding Acknowledgements Type of funding sources: Private company. Main funding source(s): ZOLL Background The wearable cardioverter-defibrillator (WCD) is a temporary treatment option for patients at high risk for sudden cardiac death (SCD) in whom implantation of a cardioverter defibrillator (ICD) is temporarily not possible. Exercise training and rehabilitation has an IA recommendation in these patients. There are no data on safety and outcomes of patients with a WCD undergoing cardiac rehabilitation. Purpose The aim of this study is to evaluate the safety and feasibility of wearing a WCD while undergoing a cardiac rehabilitation program. Methods We performed a retrospective analysis of all patients with a successfully completed stationary rehabilitation program. Results 55/896 patients (6.1%) registered in the Austrian WCD registry completed a stationary rehab program (60±11 years, 1% female). Underlying cardiac disease was ischaemic cardiomyopathy (CMP) in 27/55 (49%), dilatative CMP in 15/55 (27%), inflammatory CMP in 10/55 (18%) patients. One patient had valvular CMP, one patient had tako tsubo CMP, one patient had a primary electrical disease (each 1,8%). The baseline LVEF at rehab start was 36% [12;80%], duration of rehab was 28 days [18;42] compared to a total prescription duration of 94 days [26;294]. 75.6% (41/55) patients completed a regular rehab duration with a WCD, 3.6% (2/55) got their WCD stopped during rehab due to recovery of the LV function, 21,8% (12/55) got a WCD prescribed during rehab. The daily wearing duration was 23.4h [12.6;23.9]. 2848 (mean 52±117/patient) automatic alarms and 340 (mean 6±9/patient) manual alarms were generated. Within this cohort 3,6% (2/55) patients had shocks for VT/VF during prescription: one patient for a VF event that was terminated with the first shock, another patient had three VT events that were also terminate with the first WCD shock. All events happened before rehabilitation, so no patient had a WCD treatment during rehab, neither appropriate, nor inappropriate. Two patient had recurrent stable VTs that were controlled with antiarrhythmic drugs during prescription, only one of them during rehab. 3 patients had a reported syncope during rehab, all of them associated with hypotension, one patient had an ambulatory check-up at the CIED clinic because of false automatic alarms due to artefacts. Following a relevant weight loss through rehab measurements, the fabric garment of the WCD had a poor fit and was adapted. The LVEF after rehab was 42% [23;73%]. At the end of WCD prescription 33/55 (60%) had a restitution of their cardiac function with no need for an ICD, 19/55 (35%) patients were implanted with an ICD. One patient (1,8%) had a heart transplantation, one patient (1,8%) had the desire to stop WCD prescription and one patient (1,8%) had a terminal non-cardiac disease. Conclusion Completing a cardiovascular rehabilitation program is feasible and safe for patients prescribed with a WCD and may contribute positively to the restitution of the cardiac function.

Hypertrophic cardiomyopathy in an athlete, occurring under the mask of sports remodeling. Secondary prevention of sudden cardiac death. Analysis of a clinical case

HCMP is a severe heart disease characterized by high rates of sudden mortality, especially in young people. Optimization of the tactics of treatment of children with HCMP in order to improve the prognosis remains one of the urgent tasks of cardiology. A clinical case of a 15-yearold boy with moderate myocardial hypertrophy and an asymptomatic course of HCMP is presented, in connection with which the disease was not diagnosed for a long time. The boy was engaged in professional sports, during the competition he suddenly lost consciousness, clinical death occurred. Only timely resuscitation measures allowed to save the patient’s life. Implantation of a cardioverter defibrillator was performed as a secondary prevention of SCD (class 1 indications). The importance of timely examination of asymptomatic athletes with myocardial hypertrophy for differential diagnosis between HCMP and myocardial remodeling against the background of intense physical exertion (“athlete’s heart”) is emphasized. The importance of solving the issue of the inadmissibility of intense physical exertion, even with an asymptomatic course of HCMP, is shown. It should be remembered that it has been established that intense sports loads act as a trigger for cardiac arrest in patients with HCMP. Retrospective calculation of SCD risk based on HCM risc-SDD calculator/The BCC risk calculator at GCMP showed 3.19 %, which corresponded to a low risk. The possibility of SCD in patients with HCMP is emphasized, even with an estimated low risk, which requires further research to find predictors of an unfavorable outcome.

Hypertrophic cardiomyopathy in a girl with Danon disease

A prospective observation for 4 years (from 14 to 17 years) is presented for a girl with Danone’s disease. The early onset of the disease was noted, the clinical picture was dominated by syncopal and syncopal conditions. According to the clinical picture, presyncopal conditions were of a vasovagal nature, which was verified by the results of the Tilt test when the vasodepressor variant was detected. The disease was characterized by isolated cardiac disorders in the form of concentric hypertrophic cardiomyopathy, widespread fibrous changes in the myocardium. According to the standard ECG and daily ECG monitoring, ventricular preexitation syndrome was detected. Electrophysiological examination established the nodo-ventricular tract. Myopathy, cognitive impairment and visual changes were absent. A mutation in the heterozygous state was detected in exon 5 of 9 exons of the LMP2 gene, leading to the acquisition of a premature stop codon, was identified. X:g.119581719G&gt;A ENST00000434600.2: c.718C&gt;T. The mutation was verified by Sanger. The case in the pedigree is sporadic. As a prevention of sudden cardiac death, implantation of a cardioverter-defibrillator was performed. Effective relief of induced stable ventricular fibrillation (Burst stimulation protocol) was achieved only with the use of a ventricular electrode with two shock coils.

Analysis of the left ventricular involvement in patients with a biventricular phenotype of arrhythmogenic ardiomyopathy: a case series description

Aim. To assess the prevalence of the left ventricle involvement and the features of the biventricular phenotype of arrhythmogenic cardiomyopathy in patients with pathogenic mutations in the PKP2 and DSP genes.Material and methods. Three unrelated probands underwent a comprehensive molecular-genetic, clinical and instrumental examination, which included a 12-lead ECG, 24-hour ECG monitoring, transthoracic echocardiography, and cardiac magnetic resonance imaging with late gadolinium enhancement.Results. The results of our clinical observations showed that in three studied patients with arrhythmogenic cardiomyopathy left ventricle involvement of various degree was found. The left ventricle damage was characterized by fibrous or fibro-fatty infiltration of the myocardium, as well as regional or global systolic dysfunction of different severity. The patients had pathogenic mutations c.1912C &gt; T (p.Gln638*, rs397517012, rs397517012); c.1237C &gt; T (p.Arg413*, rs372827156) in the PKP2 gene and a new probably pathogenic variant in the form of a c.3494delA deletion in the DSP gene. It was found that the mutation in the DSP gene was associated with a more pronounced systolic dysfunction and a greater percentage of fibrous replacement of the left ventricular myocardium compared with carriers of mutations in the PKP2 gene. All patients had life-threatening ventricular arrhythmias with the need for implantation of a cardioverter-defibrillator.Conclusion. Our clinical observations have shown that in patients with biventricular arrhythmogenic cardiomyopathy, the detection of a mutation in the DSP gene is associated with a more pronounced systolic dysfunction and a higher percentage of fibrous replacement of the left ventricle myocardium compared with carriers of mutations in the PKP2 gene.

Depressive disorders in a group of patients after implantation of a cardioverter-defibrillator

Depressive disorders in a group of patients after implantation of a cardioverter-defibrillator

Predictive factors of left ventricular ejection fraction improvement after myocardial infarction treated invasively.

One indication for the implantation of a cardioverter-defibrillator is a reduction in the left ventricular ejection fraction (LVEF) ≤ 35%. However, in certain patients following an acute myocardial infarction (AMI) a gradual improvement in LVEF has been observed. The factors determining this increase in LVEF have not been conclusively determined. To ascertain the independent predictors associated with the improvement of LVEF in patients following AMI who underwent invasive treatment, while observing their progress over a 6-month follow-up period. Among 665 patients with AMI, a population with LVEF ≤ 35% was selected. After 6 months, a follow-up echocardiogram was performed. Further analysis compared patients with at least 5% improvement in LVEF (Group I) with those without an increase (Group II). Group I consisted of 34 individuals out of 80 patients (43%) with LVEF ≤ 35%. The factors linked to a reduced probability of LVEF improvement were: higher levels of cardiac troponin T (cTnT) (OR 0.841 for 1 ng/ml increase in cTnT, CI 0.715-0.989; p = 0.037), presence of diabetes mellitus (OR = 0.217, 95% CI: 0.058-0.813, p = 0.023) and moderate or severe mitral regurgitation (OR = 0.178, 95% CI: 0.053-0.597; p = 0.005). The study findings indicate that the presence of severe or moderate mitral regurgitation is the most significant factor contributing to the lack of LVEF improvement following AMI. Moreover, the extent of myocardial damage, as indicated by elevated cTnT values, along with compromised adaptation to hypoxia in patients with diabetes, are identified as independent factors associated with reduced chances of an increase in LVEF.

240 A PEDIATRIC CASE OF BIVENTRICULAR END-STAGE ARRHYTHMOGENIC CARDIOMYOPATHY

Abstract Background Arrhythmogenic cardiomyopathy (ACM) is a rare heart muscle disease characterized by cardiomyocyte necrosis and fibro-adipose substitution which contribute both to the genesis of malignant ventricular arrhythmias and the morpho-functional alterations of the ventricles. ACM has always been considered a right ventricular disease with secondary involvement of the left ventricle, but recently a left dominant phenotype has been also described. Case report A 14-year-old patient was admitted to our hospital after out-of-hospital cardiac arrest due to ventricular fibrillation, which occurred when she was at rest. A family history of sudden death and dilated cardiomyopathy was retrieved (her brother died at the age of 18 with a dilated heart), but other family members had never been screened to date. Cardiac magnetic resonance imaging (cMRI) appearance was consistent with biventricular dilatation along with severe systolic dysfunction (left ventricular ejection fraction 20%; right ventricular ejection fraction 25%). It showed also thinning and bulging of the basal free wall of the right ventricle proximal to the outflow tract (up to 4 mm) and multiple foci of subepicardial, mid-wall and transmural late gadolinium enhancement (LGE) in both ventricles. Signs of acute decompensated heart failure and a high arrhythmic burden were present. After cardiopulmonary stabilization, she underwent the implantation of a cardioverter-defibrillator (ICD). Conclusion We herein report an uncommon case of biventricular AC (‘definite diagnosis’ according to both Task force criteria 2010 and Padua criteria) with advanced morpho-functional dysfunction occurring already in pediatric age, without any alerting symptom until the cardiac arrest (the typical onset of the ACM in children). It shows how severe biventricular involvement may affect young patients with a rapid progression of the disease. It also stresses the need for a strict follow-up of the family members of a proband since his first diagnosis, highlighting the pivotal role of cMRI in the diagnosis of pediatric disease. This has not been thoroughly studied and lacks sensitive and specific diagnostic criteria.

Complications after implantation of a cardioverter defibrillator – direction of patient education

Complications after implantation of a cardioverter defibrillator – direction of patient education

A Case of Mitral Annular Disjunction Combined with Ventricular Arrhythmias

The article presents a clinical case of a combination of mitral valve prolapse (MVP), mitral annular disjunction (MAD), and ventricular arrhythmia. The presence of MAD worsens the prognosis in MVP and predisposes to life-threatening ventricular arrhythmias. In a 42-year-old patient, MAD was detected during echocardiography to determine the indications for surgical correction of mitral insufficiency in MVP. Severe myxomatous degeneration of the mitral valve leaflets, polysegmental prolapse, and typical auscultatory pattern (systolic click followed by systolic murmur in the second half of systole) were the indications for the targeted search for MAD. Multi-day (ECG) monitoring recorded nonsustained ventricular tachycardias and premature ventricular complexes (PVCs). Cardiac magnetic resonance imaging was performed for confirmation the diagnosis and searched for left ventricular myocardial fibrosis accompanying MAD. Finally, MAD was confirmed, but myocardial fibrotic changes were not detected. Owing to the absence of myocardial fibrosis, the patient was treated conservatively with a beta-adrenoblocker (25 mg/day slow-release metoprolol succinate) in combination with 25 mg/day allaforte. Repeated 24-h ECG monitoring did not detect ventricular tachycardias and nonsustained registered a significant decrease of number of PVCs. The patient is followed up prospectively due to high risk factors for fibrosis and worsening prognosis, which may require surgical correction of the existing disturbances and/or implantation of a cardioverter-defibrillator.

Elevated α-hydroxybutyrate dehydrogenase is associated with in-hospital mortality in non-ischemic dilated cardiomyopathy.

BackgroundPrevious Study Found That Implantation of a Cardioverter-Defibrillator Likely Caused a Worse Prognosis in Older Patients With non-Ischemic Systolic Heart Failure. This Suggests That More Precise Risk Stratification Is Needed in Elderly Patients. We Conducted a Retrospective Study to Evaluate the Association of α-Hydroxybutyrate Dehydrogenase (α-HBDH) With Mortality During Hospitalization in Elderly Patients With non-Ischemic Dilated Cardiomyopathy (NIDCM).Methods1,019 Elderly Patients (age ≥60 Years) Diagnosed With NIDCM Were Retrospectively Enrolled From January 2010 to December 2019. Univariate and Multivariate Analyses Were Showed to Explore the Relationship Between α-HBDH and in- Hospital Death.ResultsPatients in elevated α-HBDH group (>182 U/L) had a longer hospital stays and higher in-hospital mortality. Univariate logistics regression analysis showed that elevated α-HBDH was significantly related to mortality (OR: 7.004, 95% CI: 3.583–13.693, p < 0.001). Receiver operator characteristic (ROC) curve analysis reflected that α-HBDH levels had excellent predictive power for in-hospital death (AUC = 0.810, 95% CI: 0.745–0.876, p < 0.001). After adjustment of age, serum creatine, albumin and LVEF, multivariate regression analysis validated the association of elevated α-HBDH with increased risk of in-hospital death (p < 0.05).ConclusionsElevated α-HBDH level is significantly related to in-hospital mortality in older patients with NIDCM.

Radiofrequency Ablation in Brugada Syndrome

In 1992, Brugada brothers first described the clinical-electrocardiographic syndrome which was characterized by changes in the electrocardiogram (ECG), family history of syncopal states and sudden cardiac death (SCD) in the absence of structural heart disease. The standard therapy recommended by the European Society of Cardiology for the prevention of SCD in type I Brugada syndrome (BS) is implantation of a cardioverter-defibrillator. Radiofrequency ablation (RFA) of BS has been performed since the 2000s as an alternative therapy for BS with recurrent ventricular arrhythmias. To date, more than 300 such interventions have been performed by various centers around the world, and multicenter randomized trials are underway to study the long-term results of catheter destruction. We reviewed our experience of catheter ablation in a patient with BS. The patient suffered from ventricular arrhythmias and short-term loss of consciousness. ECG and 24-hour monitoring data recorded ventricular arrhythmias of more than 34% per day and type II BS. Electroanatomical mapping and RFA of the areas with low amplitude graphics were performed by endocardial access. This zone coincided with the earliest activation of the myocardium during ventricular arrhythmias. Extrasystoles were eliminated. In the remote period (10-12 weeks) the patient had no ventricular arrhythmias and there are also no ECG signs of BS.&#x0D; Most major arrhythmological centers perform RFA with epicardial access. Our patient chose the endocardial approach as safer. In her specific case, localization of arimogenic substrate allowed to limit this access, and RFA was successful.

Clinical characteristics of patients with various genetic types of long QT syndrome

The aim of the study is to evaluate clinical characteristics, including adverse events and outcomes, in patients with various genetic types of long QT syndrome (LQTS).Material and methods. We examined 24 patients with a clinical diagnosis of LQTS, observed in the for 5 years. The clinical and instrumental study included registration of electrocardiography (ECG), Holter monitoring, collection of a genealogical history with an ECG assessment of all family members and identification of cases of sudden cardiac death (SCD) in the family or the presence of a family form of the disease, echocardiography and cardiac magnetic resonance imaging to exclude structural changes in the myocardium. The search for mutations in the coding sequences of genes associated with the development of channelopathy and other hereditary heart rhythm disorders was carried out by next generation sequencing (NGS).Results. Mutations in 4 genes associated with LQTS (KCNQ1, KCNH2, CACNA1C, ANK2) were detected in 18 out of 24 (75.0%) patients. Mutations in the KCNQ1, KCNH2 and CACNA1C genes were detected in 14 (58.0%) patients. In 4 out of 24 (17%) patients, two or more variants of clinical significance (VUS) were detected in the genes associated with LQTS and hereditary arrhythmias, 6 patients had no genetic changes. The most severe form of the disease with pronounced clinical manifestations and episodes of clinical death with subsequent resuscitation measures, as well as a significant increase in the QTc interval exceeding 500 ms, was observed in patients with LQT2 and multiple mutations. Implantation of a cardioverter-defibrillator (CD) was required in 14 (58.3%) patients, including 11 (78.56%) - for secondary prevention of SCD and 3 (21.4%) - for primary prevention.Conclusion. A comparative analysis between different genetic types of LQTS (LQT1; LQT2; patients with multiple VUS) showed that in patients with LQT1 syndrome, despite the early manifestation of the disease and the presence of syncopal conditions, life-threatening arrhythmias, SCD and the frequency of CD implantation were significantly less often recorded than in other LQTS. The most severe form of the disease with pronounced clinical manifestations, episodes of clinical death with subsequent resuscitation and CD implantation was observed both in the group of probands with LQT2 and in patients with several nucleotide variants (VUS), one of which was in the CACNA1C or ANK2 genes.