Search

Accelerated ventricular rhythm (AVR) was observed in two newborn infants. In the first case. arrhythmia was noted during the foetal period. Both neonates were asymptomatic and had no evidence of cardiac disease. The arrhythmia eventually disappeared when the infants were 4 mu and 24 d old, respectively. AVR in the neonatal period is reviewed in this report and recent Information regarding appropriate diagnostic evaluation, differentiation from ventricular tachycardia and treatment is outlined. Conclusion: Accelerated ventricular rhythm is a benign and self-limited arrhythmia in the neonatal period. However, it is important to differentiate it from other serious rhythm disorders, mainly ventricular tachycardia. in order to avoid unnecessary and potentially harmful treatment and to relieve parental anxiety. [ABSTRACT FROM AUTHOR]

Abstract Introduction The combination of anomalous left coronary artery origin from the pulmonary artery and an accessory pathway has not been reported previously in the medical literature. In medicine, the coexistence of two clinical causes can lead to the same clinical findings, and this can make the researcher's attempt to distinguish between the two of them and, hence, the correct diagnosis and treatment difficult. Case presentation A six-month-old boy from Pakistan was brought to our hospital with tachypnea and supraventricular tachycardia and, on the basis of echocardiography and multi-slice computed tomography, was diagnosed with an anomalous left coronary artery origin from the pulmonary artery. The presence of an anomalous left coronary artery origin from the pulmonary artery was not initially recognized, and left ventricular dysfunction was considered as a result of supraventricular tachycardia. He underwent direct re-implantation of the left coronary artery to the aorta using the trapdoor flap technique. Recurrent episodes of supraventricular tachycardia resistant to maximal pharmacological treatment occurred post-operatively. A left posterolateral accessory pathway was successfully ablated by using a trans-septal approach. Conclusions It should not be forgotten by anyone that many times in medicine what seems obvious is not correct. It can be difficult to distinguish two clinical entities, and frequently one is considered a result of the other. This is the first report of the coexistence of an anomalous left coronary artery origin from the pulmonary artery and recurrent supraventricular tachycardia due to an accessory pathway in a child that was treated successfully with combined surgical and interventional electrophysiological treatment. This case may represent a first educational step in the field of congenital heart disease, that is, that anomalies such as an anomalous left coronary artery origin from the pulmonary artery may be concealed in a child with other serious cardiac problems, in this case mitral regurgitation, dilation of the left ventricle, and recurrent episodes of tachycardia.

Objectives: Right ventricular (RV) dysfunction is a significant cause of morbidity and mortality after surgical correction of tetralogy of Fallot (TOF). Transatrial/transpulmonary repair avoids a ventriculotomy (in contrast to the transventricular approach) emphasizing maximal preservation of RV structure and function. We have adopted this technique as less traumatic for the right ventricle. This study evaluates the early surgical results of our approach. Methods: Between September 1997 and July 2001, 110 consecutive patients with TOF were referred to our unit for surgical therapy. Of these, 14 were unsuitable for repair and underwent aortopulmonary shunting±pulmonary artery patching. In the remaining 96 patients (median age 1.4 years), complete transatrial/transpulmonary repair was performed. Previously placed shunts (ten patients) were taken down and any secondary stenoses or branch pulmonary artery distortion repaired. In all cases, subpulmonary resection and ventricular septal defect (VSD) closure were accomplished transatrially. Whenever pulmonary valvotomy and valve ring widening were necessary, it was achieved through a pulmonary arteriotomy. In 84 patients the main pulmonary artery was augmented with an autologous pericardial patch, and in 23 the patch was extended to pulmonary artery branch(es). A limited (<1 cm ) or extended (>1 cm, but≤length of RV infundibulum) transannular incision was necessary in 59 and 18 patients, respectively, in order to achieve an adequate residual RV outflow tract diameter. A monocusp autologous pericardial valve was placed in 13 patients. Results: There was no death in this series. No patient required permanent pacemaker. In one case, early reoperation for residual RV outflow tract obstruction was needed. Median ICU and hospital stay were 3.5 and 10 days, respectively. At median follow up of 26 (mean 25±12) months, all patients are asymptomatic, with no significant residual lesion. Conclusions: Transatrial/transpulmonary repair of TOF is associated with remarkably low morbidity and mortality in our early experience. [Copyright &y& Elsevier]

Key Clinical Message: Systemic to pulmonary venous collaterals (SPVCs) are common findings in patients with Fontan and may be responsible for desaturation in this group of patients. In selected cases, percutaneous closure of SPVCs with coils or vascular plugs could be offered as a safe and effective treatment modality for these patients., Abstract: A 27-year-old female patient who had operated primarily with Glenn operation and she underwent later a lateral tunnel Fontan operation. After 5 years, she presented with desaturation due to significant systemic to pulmonary venous collateral development, and a decision was made to proceed with interventional occlusion. Afterward, the arterial saturations increased., (© 2024 The Author(s). Clinical Case Reports published by John Wiley & Sons Ltd.)

Objective: Implantable loop recorders (ILRs) are increasingly being used for long-term cardiac monitoring in different clinical settings. The aim of this study was to investigate the real-world performance of ILRs-including the time to diagnosis-in unselected patients with different ILR indications., Methods and Results: In this multicenter, observational study, 871 patients with an indication of pre-syncope/syncope (61.9%), unexplained palpitations (10.4%), and atrial fibrillation (AF) detection with a history of cryptogenic stroke (CS) (27.7%) underwent ILR implantation. The median follow-up was 28.8 ± 12.9 months. In the presyncope/syncope group, 167 (31%) received a diagnosis established by the device. Kaplan-Meier estimates indicated that 16.9% of patients had a diagnosis at 6 months, and the proportion increased to 22.5% at 1 year. Of 91 patients with palpitations, 20 (22%) received a diagnosis based on the device. The diagnosis was established in 12.2% of patients at 6 months, and the proportion increased to 13.3% at 1 year. Among 241 patients with CS, 47 (19.5%) were diagnosed with AF. The diagnostic yield of the device was 10.4% at 6 months and 12.4% at 1 year. In all cases, oral anticoagulation was initiated. Overall, ILR diagnosis altered the therapeutic strategy in 26.1% of the presyncope/syncope group, 2.2% of the palpitations group, and 3.7% of the CS group in addition to oral anticoagulation initiation., Conclusion: In this real-world patient population, ILR determines diagnosis and initiates new therapeutic management for nearly one-fourth of patients. ILR implantation is valuable in the evaluation of patients with unexplained presyncope/syncope, CS, and palpitations., Competing Interests: Conflicts of interest None., (Copyright © 2024 Hellenic Society of Cardiology. Published by Elsevier Inc. All rights reserved.)

Key Clinical Message: Descending aorta to right atrial (RA) fistula is a rare and distinct clinical entity mimicking patent ductus arteriosus (PDA) and it may lead to rapid development of pulmonary vascular disease. Correct diagnosis and treatment, especially in the presence of other congenital heart defects, is very important. Interventional management is the treatment of choice., Abstract: We present a case report of a trisomy 21 infant with atrial and ventricular septal defects and small patent ductus arteriosus (PDA) complicated by the presence of descending aorta to right atrial (RA) fistula with large left to right shunt leading to rapid increase in pulmonary vascular resistance. Transcatheter occlusion of the fistula followed by closure of the PDA with Nit-Occlud coil systems led to decreased pulmonary pressure and resistance permitting successful surgical repair of the patient's intracardiac defects with good outcome over 3 years of follow-up. Descending aorta to RA fistula is a rare and distinct clinical entity mimicking PDA and its correct diagnosis and treatment, especially in the presence of other congenital heart defects, is very important as it may lead to rapid development of pulmonary vascular disease., (© 2024 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd.)

Aims: Calmodulinopathy due to mutations in any of the three CALM genes (CALM1-3) causes life-threatening arrhythmia syndromes, especially in young individuals. The International Calmodulinopathy Registry (ICalmR) aims to define and link the increasing complexity of the clinical presentation to the underlying molecular mechanisms., Methods and Results: The ICalmR is an international, collaborative, observational study, assembling and analysing clinical and genetic data on CALM-positive patients. The ICalmR has enrolled 140 subjects (median age 10.8 years [interquartile range 5-19]), 97 index cases and 43 family members. CALM-LQTS and CALM-CPVT are the prevalent phenotypes. Primary neurological manifestations, unrelated to post-anoxic sequelae, manifested in 20 patients. Calmodulinopathy remains associated with a high arrhythmic event rate (symptomatic patients, n = 103, 74%). However, compared with the original 2019 cohort, there was a reduced frequency and severity of all cardiac events (61% vs. 85%; P = .001) and sudden death (9% vs. 27%; P = .008). Data on therapy do not allow definitive recommendations. Cardiac structural abnormalities, either cardiomyopathy or congenital heart defects, are present in 30% of patients, mainly CALM-LQTS, and lethal cases of heart failure have occurred. The number of familial cases and of families with strikingly different phenotypes is increasing., Conclusion: Calmodulinopathy has pleiotropic presentations, from channelopathy to syndromic forms. Clinical severity ranges from the early onset of life-threatening arrhythmias to the absence of symptoms, and the percentage of milder and familial forms is increasing. There are no hard data to guide therapy, and current management includes pharmacological and surgical antiadrenergic interventions with sodium channel blockers often accompanied by an implantable cardioverter-defibrillator., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Background: Electroanatomic mapping systems are increasingly used during ablations to decrease the need for fluoroscopy and therefore radiation exposure. For left-sided arrhythmias, transseptal puncture is a common procedure performed to gain access to the left side of the heart. We aimed to demonstrate the radiation exposure associated with transseptal puncture., Methods: Data were retrospectively collected from the Catheter Ablation with Reduction or Elimination of Fluoroscopy registry. Patients with left-sided accessory pathway-mediated tachycardia, with a structurally normal heart, who had a transseptal puncture, and were under 22 years of age were included. Those with previous ablations, concurrent diagnostic or interventional catheterisation, and missing data for fluoroscopy use or procedural outcomes were excluded. Patients with a patent foramen ovale who did not have a transseptal puncture were selected as the control group using the same criteria. Procedural outcomes were compared between the two groups., Results: There were 284 patients in the transseptal puncture group and 70 in the patent foramen ovale group. The transseptal puncture group had a significantly higher mean procedure time (158.8 versus 131.4 minutes, p = 0.002), rate of fluoroscopy use (38% versus 7%, p < 0.001), and mean fluoroscopy time (2.4 versus 0.6 minutes, p < 0.001). The acute success and complication rates were similar., Conclusions: Performing transseptal puncture remains a common reason to utilise fluoroscopy in the era of non-fluoroscopic ablation. Better tools are needed to make non-fluoroscopic transseptal puncture more feasible.

Acute Kidney Injury (AKI) commonly complicates cardiac surgery in children with congenital heart disease (CHD). In this study we assessed incidence, risk factors, and outcomes of postoperative AKI, while testing the hypothesis that, depending on the underlying diagnosis, there would be significant differences in AKI incidence among different diagnostic groups. We conducted an observational cohort study of children with CHD undergoing cardiac surgery in a single tertiary center between January 2019 and August 2021 (n = 362). Kidney Disease Improving Global Outcome (KDIGO) criteria were used to determine the incidence of postoperative AKI. Diagnosis was incorporated into multivariate models using an anatomic-based CHD classification system. Overall survival was estimated using Kaplan−Meier curves. Log-rank test and adjusted Cox proportional hazard modelling were used to test for differences in survival distributions and determine AKI effect on survival function, respectively. AKI occurred in 70 (19.3%), with 21.4% in-hospital mortality for AKI group. Younger age, lower weight, longer cardiopulmonary bypass time, preoperative mechanical ventilation and diagnostic category were associated with postoperative AKI. Resolution rate was 92.7% prior to hospital discharge for survivors. AKI was associated with longer duration of mechanical ventilation, ICU and hospital length of stay. AKI patients had significantly higher probability of all-cause mortality postoperatively when compared to the non-AKI group (log-rank test, p < 0.001). Adjusted hazard ratio for AKI versus non-AKI group was 11.08 (95% CI 2.45−50.01; p = 0.002). Diagnostic category was associated with cardiac surgery-related AKI in children with CHD, a finding supporting the development of lesion specific models for risk stratification. Postoperative AKI had detrimental impact on clinical outcomes and was associated with decreased survival to hospital discharge.

Background: Patients with CHD can be exposed to high levels of cumulative ionising radiation. Utilisation of electroanatomic mapping during catheter ablation leads to reduced radiation exposure in the general population but has not been well studied in patients with CHD. This study evaluated the radiation sparing benefit of using three-dimensional mapping in patients with CHD., Methods: Data were retrospectively collected from the Catheter Ablation with Reduction or Elimination of Fluoroscopy multi-institutional registry. Patients with CHD were selected. Those with previous ablations, concurrent diagnostic or interventional catheterisation and unknown arrhythmogenic foci were excluded. The control cohort was matched for operating physician, arrhythmia mechanism, arrhythmia location, weight and age. The procedure time, rate of fluoroscopy use, fluoroscopy time, procedural success, complications, and distribution of procedures per year were compared between the two groups., Results: Fifty-six patients with congenital heart disease and 56 matched patients without CHD were included. The mean total procedure time was significantly higher in patients with CHD (212.6 versus 169.5 minutes, p = 0.003). Their median total fluoroscopy time was 4.4 minutes (compared to 1.8 minutes), and their rate of fluoroscopy use was 23% (compared to 13%). The acute success and minor complication rates were similar and no major complications occurred., Conclusions: With the use of electroanatomic mapping during catheter ablation, fluoroscopy use can be reduced in patients with CHD. The majority of patients with CHD received zero fluoroscopy.

Background: Catheter ablation is a safe and effective therapy for the treatment of supraventricular tachycardia in children. Current improvements in technology have allowed progressive reduction in radiation exposure associated with the procedure. To assess the impact of three-dimensional mapping, we compared acute procedural results collected from the Catheter Ablation with Reduction or Elimination of Fluoroscopy registry to published results from the Prospective Assessment after Pediatric Cardiac Ablation study., Methods: Inclusion and exclusion criteria from the Prospective Assessment after Pediatric Cardiac Ablation study were used as guidelines to select patient data from the Catheter Ablation with Reduction or Elimination of Fluoroscopy registry to compare acute procedural outcomes between cohorts. Outcomes assessed include procedural and fluoroscopy exposure times, success rates of procedure, and complications., Results: In 786 ablation procedures, targeting 498 accessory pathways and 288 atrioventricular nodal reentrant tachycardia substrates, average procedural time (156.5 versus 206.7 minutes, p < 0.01), and fluoroscopy time (1.2 versus 38.3 minutes, p < 0.01) were significantly shorter in the study group. Success rates for the various substrates were similar except for manifest accessory pathways which had a significantly higher success rate in the study group (96.4% versus 93.0%, p < 0.01). Major complication rates were significantly lower in the study group (0.3% versus 1.6%, p < 0.01)., Conclusions: In a large, multicentre study, three-dimensional systems show favourable improvements in clinical outcomes in children undergoing catheter ablation of supraventricular tachycardia compared to the traditional fluoroscopic approach. Further improvements are anticipated as technology advances.

Background: Arrhythmias are common in the post-operative course of patients with hypoplastic left heart syndrome. We sought to determine the types, incidence, risk factors, and impact of arrhythmias in patients with HLHS and anatomic variants., Methods: We performed a retrospective chart review of 120 consecutive patients with HLHS and anatomical variants, who had single-ventricle palliation at our institution from January, 2006 to December, 2016., Results: A total of thirty-one patients (26%) had 37 episodes of arrhythmias over a median follow-up period of 3.5 years. Of the 37 episodes, 12 (32.4%) were ectopic atrial tachycardia, 9 (24.3%) were paroxysmal supraventricular tachycardia, 4 (10.8%) were junctional ectopic tachycardia, 5 (13.6%) were sinus node dysfunction, 3 (8.1%) were heart block, 2 (5.4%) were atrial flutter, and 2 (5.4%) were ventricular tachycardia. Twenty-four (65%) of the arrhythmias occurred at post-stage 1 surgery. Most (64.8%) of the arrhythmias were resolved. Arrhythmias that occurred at post-stage 1 surgery were more likely to resolve compared to post-stages 2 or 3 (p = 0.006). No anatomical, surgical, or clinical variables were associated with arrhythmia except for age (OR per unit decrease in age at stage 1 palliation: 1.12 (95% CI 1.003, 1.250); p = 0.0439). Arrhythmias were not associated with length of hospital stay or mortality., Conclusion: Arrhythmias are common in patients with HLHS and anatomic variants, with EAT and PSVT being the most common types. Arrhythmias were associated with younger age at surgery, but did not affect mortality or length of hospital stay.

Objectives: The primary goal of this study was to evaluate the implant experience and midterm results of subcutaneous implantable cardioverter-defibrillators (S-ICDs) in pediatric patients and those with congenital heart disease., Background: The S-ICD was developed to avoid the lead-related complications associated with transvenous systems. The absence of intravascular or intracardiac components offers potential advantages to pediatric patients and those with congenital heart disease., Methods: This international, multicenter, retrospective, standard-of-care study was conducted through the Pediatric & Congenital Electrophysiology Society. Complications at 30 and 360 days, inappropriate shocks, and delivery of appropriate therapy were assessed., Results: The study included 115 patients with a median follow-up of 32 (19 to 52) months. Median age was 16.7 years (14.8 to 19.3 years), 29% were female, and 55% had a primary prevention indication. Underlying disease substrate was cardiomyopathy (40%), structural heart disease (32%), idiopathic ventricular fibrillation (16%), and channelopathy (13%). The complication rate was 7.8% at 30 days and 14.7% at 360 days. Overall, inappropriate shocks occurred in 15.6% of patients, with no single clinical characteristic reaching statistical significance. At implant, 97.9% of patients had successful first shock conversion with 96% requiring ≤65 J. Appropriate therapy was delivered to 11.2% of patients with an annual incidence of 3.9% and an acute first shock conversion success rate of 92.5%., Conclusions: This study found that in a heterogeneous population of pediatric patients and those with congenital heart disease, the S-ICD had comparable rates of complications, inappropriate shocks, and conversion efficacy compared with previously published studies on transvenous systems in similar populations., Competing Interests: Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2020 American College of Cardiology Foundation. All rights reserved.)

Purpose of Review: Tetralogy of Fallot (TOF) carries a long-term risk of arrhythmias and sudden death after surgical repair. Risk stratification is still less accurate than desired., Recent Findings: Several factors have been studied as risk predictors for ventricular arrhythmias and sudden death. Clinical parameters include age at surgery, time since repair, types of previous surgeries, and symptoms such as syncope and palpitations. Electrocardiographic parameters that have been studied include QRS interval, ventricular arrhythmias assessed with Holter monitors, signal averaged ECG, QRS fragmentation, QRS vector magnitude, and microvolt T-wave alternans. Exercising testing has been shown to have prognostic significance. Ventricular function assessment with imaging studies including echocardiography and magnetic resonance imaging (MRI) plays a significant role. Invasive hemodynamic and electrophysiologic studies, in addition to assessment for inducible tachycardia, can provide information regarding the electroanatomic substrate of VT., Summary: Risk stratification for TOF has improved over the last years with several clinical, electrocardiographic, imaging, and invasive electrophysiologic findings showing promise, but there still a lack of uniformity in approach between various investigators and reproducibility of findings is difficult in larger populations. With use of a combination of factors, a more informed decision can be made.

Aims: Calmodulinopathies are rare life-threatening arrhythmia syndromes which affect mostly young individuals and are, caused by mutations in any of the three genes (CALM 1-3) that encode identical calmodulin proteins. We established the International Calmodulinopathy Registry (ICalmR) to understand the natural history, clinical features, and response to therapy of patients with a CALM-mediated arrhythmia syndrome., Methods and Results: A dedicated Case Report File was created to collect demographic, clinical, and genetic information. ICalmR has enrolled 74 subjects, with a variant in the CALM1 (n = 36), CALM2 (n = 23), or CALM3 (n = 15) genes. Sixty-four (86.5%) were symptomatic and the 10-year cumulative mortality was 27%. The two prevalent phenotypes are long QT syndrome (LQTS; CALM-LQTS, n = 36, 49%) and catecholaminergic polymorphic ventricular tachycardia (CPVT; CALM-CPVT, n = 21, 28%). CALM-LQTS patients have extremely prolonged QTc intervals (594 ± 73 ms), high prevalence (78%) of life-threatening arrhythmias with median age at onset of 1.5 years [interquartile range (IQR) 0.1-5.5 years] and poor response to therapies. Most electrocardiograms (ECGs) show late onset peaked T waves. All CALM-CPVT patients were symptomatic with median age of onset of 6.0 years (IQR 3.0-8.5 years). Basal ECG frequently shows prominent U waves. Other CALM-related phenotypes are idiopathic ventricular fibrillation (IVF, n = 7), sudden unexplained death (SUD, n = 4), overlapping features of CPVT/LQTS (n = 3), and predominant neurological phenotype (n = 1). Cardiac structural abnormalities and neurological features were present in 18 and 13 patients, respectively., Conclusion: Calmodulinopathies are largely characterized by adrenergically-induced life-threatening arrhythmias. Available therapies are disquietingly insufficient, especially in CALM-LQTS. Combination therapy with drugs, sympathectomy, and devices should be considered., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2019. For permissions, please email: journals.permissions@oup.com.)

Background: Gain-of-function variants in the CACNA1C-encoded L-type calcium channel (LTCC, Ca v 1.2) cause type 8 long QT syndrome (LQT8). The pore region contains highly conserved glutamic acid (E) residues that collectively form the LTCC's selectivity filter. Here, we identified and characterized a pore-localizing missense variant, E1115K, that yielded a novel perturbation in the LTCC., Objective: The purpose of this study was to determine whether CACNA1C-E1115K alters the LTCC's selectivity and is the substrate for the patient's LQTS., Methods: The proband was a 14-year-old male with idiopathic QT prolongation and bradycardia. Genetic testing revealed a missense variant, CACNA1C-E1115K. The whole-cell patch clamp technique was used to measure CACNA1C-WT and -E1115K currents when heterologously expressed in TSA201 cells., Results: The CACNA1C-E1115K channel exhibited no inward calcium current. Instead, robust cardiac transient outward potassium current (I to )-like outward currents that were blocked significantly by nifedipine were measured when 2 mM/0.1 mM extracellular/intracellular CaCl2 or 4 mM/141 mM extracellular/intracellular KCl was applied. Furthermore, when 140 mM extracellular NaCl was applied, the CACNA1C-E1115K channel revealed both robust inward persistent Na + currents with slower inactivation and outward currents, which were also nifedipine sensitive. In contrast, CACNA1C-WT revealed only a small inward persistent Na + current without a robust outward current., Conclusion: This CACNA1C-E1115K variant destroyed the LTCC's calcium selectivity and instead converted the mutant channel into a channel with a marked increase in sodium-mediated inward currents and potassium-mediated outward currents. Despite the anticipated 50% reduction in LTCC, the creation of a new population of channels with accentuated inward and outward currents represents the likely pathogenic substrates for the patient's LQTS and arrhythmia phenotype., (Copyright © 2018. Published by Elsevier Inc.)

Background: The relationship of atrioventricular nodal reentrant tachycardia to congenital heart disease (CHD) and the outcome of catheter ablation in this population have not been studied adequately., Methods and Results: A multicenter retrospective study was performed on patients with CHD who had atrioventricular nodal reentrant tachycardia and were treated with catheter ablation. There were 109 patients (61 women), aged 22.1±13.4 years. The majority, 86 of 109 (79%), had CHD resulting in right heart pressure or volume overload. Patients were divided into 2 groups: group A (n=51) with complex CHD and group B (n=58) with simple CHD. There were no significant differences between groups in patients' growth parameters, use of 3-dimensional imaging, and type of ablation (radiofrequency versus cryoablation). Procedure times (251±117 versus 174±94 minutes; P =0.0006) and fluoroscopy times (median 20.8 versus 16.6 minutes; P =0.037) were longer in group A versus group B. There were significant differences between groups in the acute success of ablation (82% versus 97%; P =0.04), risk of atrioventricular block (14 versus 0%; P =0.004), and need for chronic pacing (10% versus 0%; P =0.008). There was no permanent atrioventricular block in patients who underwent cryoablation. After 3.2±2.7 years of follow-up, long-term success was 86% in group A and 100% in group B ( P =0.004)., Conclusions: Atrioventricular nodal reentrant tachycardia can complicate the course of patients with CHD. This study demonstrates that the outcome of catheter ablation is favorable in patients with simple CHD. Patients with complex CHD have increased risk of procedural failure and atrioventricular block., (© 2017 American Heart Association, Inc.)

Sudden death from aortic dissection of an ascending aortic aneurysm is an uncommon but important finding in all series of sudden death in young, apparently healthy athletes. Individuals at risk include those having any of a variety of conditions in which structural weakness of the ascending aorta predisposes to pathological dilation under prolonged periods of increased wall stress. These conditions include Marfan syndrome, Loeys-Dietz syndrome, bicuspid aortic valve, and the vascular form of Ehlers-Danlos syndrome. Diagnostic criteria, surveillance strategies, medical management, and surgical indications are discussed. Finally, the current recommendations for sports participation are provided.