Modern physiological approach to inappropriate ICD shocks due to atrial fibrillation with very fast ventricular response. A case report.

BACKGROUND: Fast-conducting atrial fibrillation misinterpreted as ventricular tachycardia is the leading cause for inappropriate shocks in patients with implantable cardiac defibrillators (ICD). These inappropriate shocks are associated with significant morbidity and mortality and cause great discomfort and stress. CASE PRESENTATION: We report the case of a patient with ischemic cardiomyopathy, permanent atrial fibrillation, and a single-chamber DF-1 ICD implanted for the primary prevention of sudden cardiac death, who presented for multiple inappropriate internal shocks due to very fast-conducting atrial fibrillation, which was mislabeled as ventricular fibrillation by the ICD. Since the patient was under maximal atrioventricular nodal blocking medical therapy (beta-blockers and digitalis) and we didn`t find any reversible causes for the heart rate acceleration, we opted for rate control with atrioventricular node ablation. To counteract the risk of pacing-induced cardiomyopathy in this patient who would become totally pacemaker-dependent, we successfully performed left bundle branch area pacing. Because the patient`s ICD had a DF-1 connection and the battery had a long life remaining, we connected the physiological pacing lead to the IS-1 sense-pace port of the ICD. The 6-month follow-up showed an improvement in left ventricular function with no more inappropriate shocks. CONCLUSIONS: Left bundle branch area pacing and atrioventricular node ablation in patients with an implantable single-chamber DF-1 defibrillator and fast-conducting permanent atrial fibrillation is a cost-efficient and very effective method to prevent and treat inappropriate shocks, avoiding the use of an additional dual-chamber or CRT-D device.

Parenteral Anticoagulation at First Medical Contact Improves Infarct Related Artery Patency in STEMI.

(1) Background: Acute ST-segment elevation myocardial infarction (STEMI) remains one of the main morbidity and mortality contributors worldwide. Its main treatment, primary percutaneous coronary intervention (pPCI), can only be performed with a high anticoagulation regimen, usually with heparin. There is still not enough evidence regarding the timing of heparin administration. (2) Methods: We conducted a multicenter observational study of 614 consecutive STEMI patients treated between 2017 and 2019. We split the population in two groups: one that received heparin at the first medical contact, as early as possible, and the second group that received heparin at the PCI capable center or in the cath lab. (3) Results: There was a significantly higher rate of infarct-related artery (IRA) patency at the time of the coronary angiogram in the pre-transfer heparin group than in the on-site heparin group, 44.7% vs. 37.3%, p = 0.042. Also, the early heparin group received shorter and wider stents. There was no difference in bleeding rates or in the in-hospital and two-year mortality rates. (4) Conclusions: Early administration of heparin leads to a higher rate of reperfusion in the IRA, before pPCI, with significant related benefits, such as better stent implantation parameters, without increased bleeding rates.

MSA-VT Score for Assessment of Long-Term Prognosis after Electrical Storm Ablation.

INTRODUCTION: Prognosis after electrical storm (ES) ablation remains severe, especially in patients with recurrent sustained monomorphic ventricular tachycardia (SMVT) or progressive heart failure (HF). However, single-factor-based prediction is suboptimal and may be refined by more complex algorithms. We sought to evaluate if a novel score MSA-VT (M = moderate/severe mitral regurgitation, S = severe HF at admission, A = atrial fibrillation at admission, VT = inducible SMVT after ablation) may improve prediction of death and recurrences compared to single factors and previous scores (PAINESD, RIVA and I-VT). METHODS: A total of 101 consecutive ES ablation patients were retrospectively analyzed over a 32.8-month (IQR 10-68) interval. The MSA-VT score was calculated as the sum of the previously mentioned factors' coefficients based on hazard ratio values in Cox regression analysis. The AUC for death prediction by MSA-VT was 0.84 (p < 0.001), superior to PAINESD (AUC 0.63, p = 0.03), RIVA (AUC 0.69, p = 0.02) and I-VT (0.56, p = 0.3). MSA-VT ≥ 3 was associated with significantly higher mortality during follow-up (52.7%, p < 0.001). CONCLUSIONS: Prediction by single factors and previously published scores after ES ablation may be improved by the novel MSA-VT score; however, this requires further external validation in larger samples.

Ventricular Tachycardia Catheter Ablation: Retrospective Analysis and Prospective Outlooks-A Comprehensive Review.

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 arrhythmic episodes and significantly alter the quality of life by delivering recurrent therapies. From open-heart surgical ablation to the currently widely used percutaneous approach, catheter ablation is a safe and effective procedure able to target the responsible re-entry myocardial circuit from both the endocardium and the epicardium. There are four main mapping strategies, activation, entrainment, pace, and substrate mapping, each of them with their own advantages and limitations. The contemporary guideline-based recommendations for VT ablation primarily apply to patients experiencing antiarrhythmic drug ineffectiveness or those intolerant to the pharmacological treatment. Although highly effective in most cases of scar-related VTs, the traditional approach may sometimes be insufficient, especially in patients with nonischemic cardiomyopathies, where circuits may be unmappable using the classic techniques. Alternative methods have been proposed, such as stereotactic arrhythmia radioablation or radiotherapy ablation, surgical ablation, needle ablation, transarterial coronary ethanol ablation, and retrograde coronary venous ethanol ablation, with promising results. Further studies are needed in order to prove the overall efficacy of these methods in comparison to standard radiofrequency delivery. Nevertheless, as the field of cardiac electrophysiology continues to evolve, it is important to acknowledge the role of artificial intelligence in both the pre-procedural planning and the intervention itself.

Left Ventricular-Arterial Coupling as an Independent Predictor of Adverse Events in Young Patients with ST Elevation Myocardial Infarction-A 3D Echocardiographic Study.

Left ventricular-arterial coupling (VAC) is a key determinant of global cardiovascular performance, calculated as the ratio between arterial elastance (EA) and left ventricular end-systolic elastance (EES). Over the years, acute myocardial infarction (STEMI) has remained an important cause of morbidity and mortality worldwide. Although, until recently, it was considered a disease occurring mostly in older patients, its prevalence in the young population is continuously rising. In this study, we aimed to investigate the role of 3D VAC and its derived indices in predicting adverse outcomes in young patients with STEMI. We prospectively enrolled 84 young patients (18-51 years) with STEMI who underwent primary PCI and 28 healthy age and sex-matched controls. A 3D echocardiography was used for non-invasive measurements of end-systolic elastance (EES), arterial elastance (EA), and VAC (EA/EES). The occurrence of major adverse cardiac events (MACE) was assessed one year after the index STEMI. Out of 84 patients, 15.4% had adverse events at 12 months follow-up. Patients were divided into two groups according to the presence or absence of MACE. There were no significant differences in arterial elastance between the two groups. EA was higher in the MACE group but without statistical significance (2.65 vs. 2.33; p = 0.09). EES was significantly lower in the MACE group (1.25 ± 0.34 vs. 1.91 ± 0.56. p < 0.0001) and VAC was higher (2.2 ± 0.62 vs. 1.24 ± 0.29, p < 0.0001). ROC analysis showed that VAC has a better predictive value for MACE (AUC 0.927) compared with EA or EEA but also compared with a classical determinant of LV function (LVEF and LVGLS). A VAC value over 1.71 predicts unfavourable outcome with 83.3% sensitivity and 97.1% specificity. In both univariate and multivariate COX regression analysis, VAC remained an independent predictor for MACE and demonstrated incremental prognostic value over LVEF and LVGLS in the proposed statistical models. In conclusion, 3D VAC is an independent predictor of adverse events in young patients with STEMI at a 12 month follow-ups and could be used for a more accurate risk stratification in the acute phase.

Nanotechnology Innovations in Pediatric Cardiology and Cardiovascular Medicine: A Comprehensive Review.

(1) Background: Nanomedicine, incorporating various nanoparticles and nanomaterials, offers significant potential in medical practice. Its clinical adoption, however, faces challenges like safety concerns, regulatory hurdles, and biocompatibility issues. Despite these, recent advancements have led to the approval of many nanotechnology-based products, including those for pediatric use. (2) Methods: Our approach included reviewing clinical, preclinical, and animal studies, as well as literature reviews from the past two decades and ongoing trials. (3) Results: Nanotechnology has introduced innovative solutions in cardiovascular care, particularly in managing myocardial ischemia. Key developments include drug-eluting stents, nitric oxide-releasing coatings, and the use of magnetic nanoparticles in cardiomyocyte transplantation. These advancements are pivotal for early detection and treatment. In cardiovascular imaging, nanotechnology enables noninvasive assessments. In pediatric cardiology, it holds promise in assisting the development of biological conduits, synthetic valves, and bioartificial grafts for congenital heart defects, and offers new treatments for conditions like dilated cardiomyopathy and pulmonary hypertension. (4) Conclusions: Nanomedicine presents groundbreaking solutions for cardiovascular diseases in both adults and children. It has the potential to transform cardiac care, from enhancing myocardial ischemia treatment and imaging techniques to addressing congenital heart issues. Further research and guideline development are crucial for optimizing its clinical application and revolutionizing patient care.

Statins-Beyond Their Use in Hypercholesterolemia: Focus on the Pediatric Population.

Statins are a class of medications primarily used in adults to lower cholesterol levels and reduce the risk of cardiovascular events. However, the use of statins in children is generally limited and carefully considered despite the well-documented anti-inflammatory, anti-angiogenic, and pro-apoptotic effects, as well as their effect on cell signaling pathways. These multifaceted effects, known as pleiotropic effects, encompass enhancements in endothelial function, a significant reduction in oxidative stress, the stabilization of atherosclerotic plaques, immunomodulation, the inhibition of vascular smooth muscle proliferation, an influence on bone metabolism, anti-inflammatory properties, antithrombotic effects, and a diminished risk of dementia. In children, recent research revealed promising perspectives on the use of statins in various conditions including neurological, cardiovascular, and oncologic diseases, as well as special situations, such as transplanted children. The long-term safety and efficacy of statins in children are still subjects of ongoing research, and healthcare providers carefully assess the individual risk factors and benefits before prescribing these medications to pediatric patients. The use of statins in children is generally less common than in adults, and it requires close monitoring and supervision by healthcare professionals. Further research is needed to fully assess the pleiotropic effects of statins in the pediatric population.

Feasibility of Conduction System Pacing in Patients with Baseline Bundle Branch Block-A Single-Center Mid-Term Follow-Up Study.

BACKGROUND: The primary prerequisite for a successful conduction system pacing (CSP) procedure is the integrity of the conduction system, which may be impaired if a baseline bundle branch block (BBB) is present. This study aimed to evaluate the feasibility and mid-term performance of permanent CSP in patients with baseline BBB and to compare the results between left bundle branch block (LBBB) and right bundle branch block (RBBB) patterns. MATERIAL AND METHODS: A total of 101 patients with typical BBB and an attempt at CSP were retrospectively reviewed. Procedural characteristics, pacing, sensing parameters, and complications at baseline and after a mid-term follow-up were analyzed. RESULTS: The global procedural success for CSP was 93%. His bundle pacing (HBP) had a significantly lower success rate than left bundle branch area pacing (LBBAP) (50.5% vs. 86%). The paced QRS duration was significantly narrower with HBP. The pacing and sensing thresholds were significantly better with LBBAP. Procedural complications occurred only in the LBBAP group (two acute perforations in the LV cavity and one acute chest pain during lead fixation) without long-term sequelae. The HBP and the LBBAP procedural success rates were higher in the RBBB versus the LBBB group (62.5% vs. 44.9% and 100% vs. 81.5%, respectively). Baseline QRS duration, atrial volumes, and right ventricular diameters were significantly associated with HBP procedural failure. The follow-up pacing and sensing thresholds were similar to the baseline values for all pacing methods and BBB morphology. Only one device-related complication leading to pacing interruption was recorded. CONCLUSION: In patients with bundle branch blocks, CSP is a feasible procedure associated with a high success rate, stable pacing and sensing parameters, and low complication rates over a mid-term follow-up.

Can Artificial Intelligence Revolutionize the Diagnosis and Management of the Atrial Septal Defect in Children?

Atrial septal defects (ASDs) present a significant healthcare challenge, demanding accurate and timely diagnosis and precise management to ensure optimal patient outcomes. Artificial intelligence (AI) applications in healthcare are rapidly evolving, offering promise for enhanced medical decision-making and patient care. In the context of cardiology, the integration of AI promises to provide more efficient and accurate diagnosis and personalized treatment strategies for ASD patients. In interventional cardiology, sometimes the lack of precise measurement of the cardiac rims evaluated by transthoracic echocardiography combined with the floppy aspect of the rims can mislead and result in complications. AI software can be created to generate responses for difficult tasks, like which device is the most suitable for different shapes and dimensions to prevent embolization or erosion. This paper reviews the current state of AI in healthcare and its applications in cardiology, emphasizing the specific opportunities and challenges in applying AI to ASD diagnosis and management. By exploring the capabilities and limitations of AI in ASD diagnosis and management. This paper highlights the evolution of medical practice towards a more AI-augmented future, demonstrating the capacity of AI to unlock new possibilities for healthcare professionals and patients alike.

Arrhythmogenic Right Ventricular Cardiomyopathy in Children: A Systematic Review.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited disease characterized by the progressive replacement of the normal myocardium by fibroadipocytic tissue. The importance of an early diagnosis is supported by a higher risk of sudden cardiac death in the pediatric population. We reviewed the literature on diagnosis, risk stratification, and prognosis in the pediatric population with ARVC. In case reports which analyzed children with ARVC, the most common sign was ventricular tachycardia, frequently presenting as dizziness, syncope, or even cardiac arrest. Currently, there is no gold standard for diagnosing ARVC in children. Nevertheless, genetic analysis may provide a proper diagnosis tool for asymptomatic cases. Although risk stratification is recommended in patients with ARVC, a validated prediction model for risk stratification in children is still lacking; thus, it is a matter of further research. In consequence, even though ARVC is a relatively rare condition in children, it negatively impacts the survival and clinical outcomes of the patients. Therefore, appropriate and validated diagnostic and risk stratification tools are crucial for the early detection of children with ARVC, ensuring a prompt therapeutic intervention.

Assessment of Sudden Cardiac Death Risk in Pediatric Primary Electrical Disorders: A Comprehensive Overview.

Sudden cardiac death (SCD) in children is a devastating event, often linked to primary electrical diseases (PED) of the heart. PEDs, often referred to as channelopathies, are a group of genetic disorders that disrupt the normal ion channel function in cardiac cells, leading to arrhythmias and sudden cardiac death. This paper investigates the unique challenges of risk assessment and stratification for channelopathy-related SCD in pediatric patients-Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, idiopathic ventricular fibrillation, long QT syndrome, Anderson-Tawil syndrome, short QT syndrome, and early repolarization syndrome. We explore the intricate interplay of genetic, clinical, and electrophysiological factors that contribute to the complex nature of these conditions. Recognizing the significance of early identification and tailored management, this paper underscores the need for a comprehensive risk stratification approach specifically designed for pediatric populations. By integrating genetic testing, family history, and advanced electrophysiological evaluation, clinicians can enhance their ability to identify children at the highest risk for SCD, ultimately paving the way for more effective preventive strategies and improved outcomes in this vulnerable patient group.

Left Atrial Low-Voltage Areas Predict the Risk of Atrial Fibrillation Recurrence after Radiofrequency Ablation.

Atrial fibrillation (AF), the most frequently encountered arrhythmia worldwide, is associated with increased cardiovascular morbidity and mortality. Left atrial (LA) and antral region of the pulmonary veins (PVs) remodeling are risk factors for AF perpetuation. Among the methods of LA fibrosis quantification, bipolar voltage mapping during three-dimensional electro-anatomical mapping is less studied. The main aim of this study was to analyze the relationship between the degree of LA fibrosis quantified in low-voltage areas and the efficacy of AF radiofrequency catheter ablation. All consecutive patients with AF ablation were included, and the degree of LA fibrosis was measured based on the low-voltage areas in the LA and the antral region of PVs (<0.5 mV for patients in sinus rhythm and <0.25 mV for patients in AF at the time of the ablation procedure). The efficacy of AF ablation was determined by the rate of recurrence after a blanking period of three months. A total of 106 patients were included; from these, 38 (35.8%) had AF recurrence after RF ablation, while 68 (64.2%) were free of events. The area and percentage of LA fibrosis were significantly higher in the patients with AF recurrence (p = 0.018 and p = 0.019, respectively). However, no significant differences were found between the patients with and without AF recurrence in terms of the area and percentage of PVs fibrosis (p = 0.896 and p = 0.888, respectively). Moreover, LA fibrosis parameters proved to be excellent predictors for AF recurrence (areas under the curve of 0.834 and 0.832, respectively, p < 0.001) even after adjustment for LA indexed volume and CHA2DS2-VASc score. In conclusion, LA fibrosis measured on bipolar voltage maps increases the risk of AF recurrence after the RF catheter ablation procedure.

Autoinflammatory Recurrent Pericarditis Associated with a New NLRP12 Mutation in a Male Adolescent.

Idiopathic recurrent pericarditis (IRP) can be the hallmark of an autoinflammatory syndrome with recurrent attacks of chest pain and symptom-free intervals following an acute episode. The recurrence rate may be 35% in the pediatric population, frequently with less severe manifestations than at the first episode. Pericarditis can be the sole clinical manifestation or may be part of a systemic autoinflammatory disease (SAID), especially in the case of a recurrence. Familial Mediterranean Fever (FMF), Tumor Necrosis Factor Receptor-Associated Periodic Syndrome (TRAPS), Mevalonate-Kinase Deficiency (MKD), nucleotide-binding oligomerization domain 2 (NOD2)-associated autoinflammatory syndrome, and others are closely related to IRP based on similar clinical manifestations and treatment responses to anti-interleukin 1 (IL-1) agents, such as anakinra, and should therefore be excluded in patients with IRP. A newly described SAID, an autosomal dominant disorder known as NLRP12-AID (nucleotide-binding leucine-rich repeat-containing receptor 12-related autoinflammatory disease) is caused by heterozygous mutations in the NLRP12 gene and most commonly affects children. Fewer than 40 pediatric patients with NLRP12-AID have been described in the medical literature, with none presenting with RP. We report a case of relapsing pericarditis responsive to anti-IL-1 therapy in a male adolescent who carried a missense mutation in the NLRP12 gene potentially causative of the excessive activation of inflammatory pathways. This is a unique case in the medical literature that associates recurrent pericarditis in an adolescent presumed to be related to the missense mutation in the NLRP12 gene. The role of the NLRP12 inflammasome in generating and maintaining recurrent pericardial inflammation should be considered.

Transvenous Lead Extraction of Cardiac Implantable Electronic Devices: Reimplant Strategy and Outcomes in a Single-Center Experience.

Cardiac implantable electronic devices are now widely used worldwide and the numbers are increasing exponentially. Subsequently, long-term complications have increased. Transvenous lead-extraction (TLE) is the gold standard for removing infected devices, treating systemic device-related infections including endocarditis, and removing devices for other non-infectious complications. Most patients still require device therapy after TLE for several indications, including lifesaving defibrillation or pacing in pacemaker-dependent patients. The decision to reimplant is challenging, particularly when the primary cause for device removal includes device-related infections and patients frequently are pacemaker dependent. We aim to present our strategy for reimplanting after performing TLE in 88 consecutive patients. We performed transvenous removal of 150 pacemaker and defibrillator leads, of which 74% for local or systemic infection. We report a 99.3% clinical success after TLE. Out of 88 patients who had undergone TLE and after reanalysing the device indication, 67 patients (76%) still had indication for device removal, but three of them refused to undergo reimplant and 58 were re-implanted. Of the re-implanted patients, 58.6% were implanted on the contra-lateral side, 38% on the ipsilateral side and two patients who had been previously implanted with ICD were reimplanted with a subcutaneous implantable cardioverter defibrillator (S-ICD). Up to 34.4% of patients were re-implanted during the same TLE procedure, which was performed for device up-grade in patients with venous occlusion, 24% were implanted during the same hospital admission for TLE but not during TLE, and 41.3% were discharged and reimplanted afterwards. With this strategy for reimplantation, we report no re-infections and no device-removal-related arrhythmic major events or deaths during one-year follow-up. In conclusion, most patients still require device therapy after TLE. In patients with previous infection of the device, the reimplantation strategy should be carefully analyzed to prevent infection relapse and limit the consequences of the absence of the device.

Optimal Cardiac Resynchronization Therapy with Conduction System Pacing Guided by Electro-Anatomical Mapping: A Case Report.

INTRODUCTION: Biventricular pacing has been the gold standard for cardiac resynchronization therapy in patients with left bundle branch block and severely reduced left ventricular ejection fraction for decades. However, in the past few years, this role has been challenged by the promising results of conduction system pacing in these patients, which has proven non-inferior and, at times, superior to biventricular pacing regarding left ventricular function outcomes. One of the most important limitations of both procedures is the long fluoroscopy times. CASE DESCRIPTION: We present the case of a 60-year-old patient with non-ischemic dilated cardiomyopathy and left bundle branch block in whom conduction system pacing was chosen as the first option for resynchronization therapy. A 3D electro-anatomical mapping system was used to guide the lead to the His bundle region, where correction was observed at high amplitudes, and afterward to the optimal septal penetration site. After reaching the left endocardium, left bundle branch pacing achieved a narrow, paced QRS complex with low fluoroscopy exposure. The three-month follow-up showed a significant improvement in clinical status and left ventricular function. CONCLUSION: Since conduction system pacing requires a great deal of precision, targeting specific, narrow structures inside the heart, 3D mapping is a valuable tool that increases the chances of success, especially in patients with complex anatomies, such as those with indications for cardiac resynchronization therapy.

Non-revascularized chronic total occlusions impact on substrate and post-ablation results in drug-refractory electrical storm.

Background and aims: There is limited data concerning the effect of non-revascularized chronic total occlusions (NR-CTOs) after VT ablation. This study sought to evaluate the impact of NR-CTOs after ablation for electrical storm (ES). Methods: Post-hoc retrospective analysis of data regarding 64 consecutive post-myocardial infarction patients (out of which 12 patients with NR-CTOs and 52 without NR-CTOs) undergoing substrate ablation for ES with an available median follow-up of 37.53 (7.25-64.65) months. Ablation result was assessed by inducibility of sustained monomorphic VT (SMVT) during final programmed ventricular stimulation (PVS). The primary endpoints were all-cause mortality and VT/VF recurrences after ablation, respectively, stratified by the presence of NR-CTOs. The secondary endpoint was to assess the predictive effect of NR-CTOs on all-cause mortality and VT/VF recurrences in relation to other relevant prognostic factors. Results: At baseline, the presence of NR-CTOs was associated with higher bipolar BZ-to-total scar ratio (72.4% ± 17.9% vs. 52% ± 37.7%, p = 0.022) and more failure to eliminate the clinical VT (25% (3) vs. 0% (0), p < 0.001). During follow-up, overall all-cause mortality and recurrences were more frequent in the NR-CTO subgroup (75% (9) vs. 19.2% (10), log rank p = 0.003 and 58.3% vs. 23.1% (12), log rank p = 0.042 respectively). After adjusting for end-procedural residual SMVT inducibility, NR-CTOs predicted death during follow-up (HR 3.380, p = 0.009) however not recurrence (HR 1.986, p = 0.154). Conclusions: NR-CTO patients treated by RFCA for drug-refractory ES demonstrated a higher ratio of BZ-to-total-scar area. In this analysis, NR-CTO was associated with worse acute procedural results and may as well impact long-term outcomes which should be further assessed in larger patient populations.

Infective Endocarditis among Pediatric Patients with Prosthetic Valves and Cardiac Devices: A Review and Update of Recent Emerging Diagnostic and Management Strategies.

Infective endocarditis (IE) is a disease of the endocardium, which leads to the appearance of vegetation on the valves, cardiac structures, or, potentially, vascular endothelium of the heart. The risk of IE can be increased more than 140 times by congenital heart disease (50-59% of all IE), particularly if cyanotic. An increase in mortality may result from IE in patients with a complex cardiac pathology or patients with an implanted prosthetic material, most frequently conduits in a pulmonary position. Cardiac implantable electronic devices (CIED) infective endocarditis is a life-threatening complication representing 10% of all cases of endocarditis. Common signs of presentation are often fever and chills; redness and swelling at the pocket of the pacemaker, including the erosion and exteriorization of the device; and life-threatening sepsis. The use of intracardiac echocardiography for the diagnosis of IE is an innovative method. This may be needed, especially in older children undergoing complex cardiac surgery, when transthoracic echocardiography (TTE) and transesophageal echocardiography (TOE) failed to provide a reliable diagnosis. The 2018 European Heart Rhythm Association (EHRA) experts' consensus statement on transvenous lead extraction recommends complete device removal and antimicrobial therapy for any device-related infection, including CIED-IE. The most detected microorganism was Staphylococcus Aureus. In addition, cardiac surgery and interventional cardiology associated with the placement of prostheses or conduits may increase the risk of IE up to 1.6% for Melody valve implantation. Our manuscript presents a comprehensive review of infective endocarditis associated with cardiac devices and prostheses in the pediatric population, including recent advances in diagnosis and management.

Arrhythmic Risk and Treatment after Transcatheter Atrial Septal Defect Closure.

Atrial septal defect (ASD) represents the most common congenital heart defect identified in adulthood. Atrial and ventricular geometric remodeling due to intracardiac shunt increase the risk of arrhythmias, especially atrial fibrillation (AF). Clinical, echocardiography, electrocardiogram, and device-related predictors may be used to assess the risk of atrial arrhythmias after ASD closure. The underlying mechanisms in these patients are complex and at least in part independent of the structural remodeling secondary to hemodynamic overload. Device closure of the ASD itself and its timing impact future arrhythmia risk, as well as posing a challenge for when transseptal puncture is required. Sudden cardiac death (SCD) risk is higher than in the general population and an implantable cardioverter-defibrillator (ICD) may be indicated in selected cases.

Intrafamilial Phenotypical Variability Linked to PRKAG2 Mutation-Family Case Report and Review of the Literature.

PRKAG2 syndrome (PS) is a rare, early-onset autosomal dominant phenocopy of sarcomeric hypertrophic cardiomyopathy (HCM), that mainly presents with ventricular pre-excitation, cardiac hypertrophy and progressive conduction system degeneration. Its natural course, treatment and prognosis are significantly different from sarcomeric HCM. The clinical phenotypes of PRKAG2 syndrome often overlap with HCM due to sarcomere protein mutations, causing this condition to be frequently misdiagnosed. The syndrome is caused by mutations in the gene encoding for the γ2 regulatory subunit (PRKAG2) of 5' Adenosine Monophosphate-Activated Protein Kinase (AMPK), an enzyme that modulates glucose uptake and glycolysis. PRKAG2 mutations (OMIM#602743) are responsible for structural changes of AMPK, leading to an impaired myocyte glucidic uptake, and finally causing storage cardiomyopathy. We describe the clinical and investigative findings in a family with several affected members (NM_016203.4:c.905G>A or p.(Arg302Gln), heterozygous), highlighting the various phenotypes even in the same family, and the utility of genetic testing in diagnosing PS. The particularity of this family case is represented by the fact that the index patient was diagnosed at age 16 with cardiac hypertrophy and ventricular pre-excitation while his mother, by age 42, only had Wolff−Parkinson−White syndrome, without left ventricle hypertrophy. Both the grandmother and the great-grandmother underwent pacemaker implantation at a young age because of conduction abnormalities. Making the distinction between PS and sarcomeric HCM is actionable, given the early-onset of the disease, the numerous life-threatening consequences and the high rate of conduction disorders. In patients who exhibit cardiac hypertrophy coexisting with ventricular pre-excitation, genetic screening for PRKAG2 mutations should be considered.

Nonischemic Super-Responders in Fusion CRT Pacing with Normal Atrioventricular Conduction.

Background: Fusion CRT pacing (FCRT) is noninferior to biventricular pacing, according to the current data. The aim of this study is to assess the response to FCRT and to identify predictors of super-responders (SRs) in a nonischemic population with normal AV conduction. Methods: LV-only CRT patients (pts) with a right atrium/left ventricle pacing system implanted in two CRT centers in Romania were included. Device interrogation, exercise tests, echocardiography, and individualized drug optimization were performed every 6 months during close follow-up. SRs pts were defined as those with left ventricular end-systolic volume (LVESV) improvement ≥30% and stable ejection fraction (LVEF) ≥45%. Results: A total of 25 out of 83 pts (31%) were SRs, with nonischemic LBBB low EF cardiomyopathy (50 male, 62 ± 9 y.o.) initially included. Mean follow-up was 5 years ± 27 months. Patients were divided in two groups: SRs and non-SRs (52 responders/6 hypo-responders). Two predictors were found in the SRs group: a higher baseline LVEF (SRs 29 ± 5% vs. non-SRs 26 ± 5%, p = 0.02) and a lower pulmonary arterial systolic pressure (SRs 38 ± 11 mm Hg vs. non-SRs 50 ± 15 mmHg, p = 0.003). Baseline severe mitral regurgitation was found in 11% of SRs vs. 64% in the non-SRs group. Conclusions: SRs in the selected NICM-FCRT group are significative high. Higher baseline LVEF and mild pulmonary arterial hypertension were independently associated with super-response.