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Journal of Cardiovascular... Aug 2022The notion that medically-refractory arrhythmias might one day be amenable to interventional therapy slowly began to appear in the early 1960's. At that time, there were... (Review)
Review
INTRODUCTION
The notion that medically-refractory arrhythmias might one day be amenable to interventional therapy slowly began to appear in the early 1960's. At that time, there were no "interventional electrophysiologists" or "arrhythmia surgeons" and there was little appreciation of the relationship between anatomy and electrophysiology outside the heart's specialized conduction system.
METHODS
In this review, we describe the evolution of collaboration between electrophysiologists and surgeons.
RESULTS
Although accessory atrio-ventricular (AV) connections were first identified in 1893 and the Wolff-Parkinson-White (WPW) syndrome was described 37 years later (1930), it was another 37 years (1967) before those anatomic AV connections were proven to be responsible for the clinical syndrome. The success of the subsequent surgical procedures for the WPW syndrome, AV node reentry tachycardia, automatic atrial tachycardias, ischemic and non-ischemic ventricular tachycardias and atrial fibrillation over the next two decades depended on a close, sometimes daily, collaboration between electrophysiologists and surgeons. In the past two decades, that tight collaboration was largely abandoned until the recent introduction of "hybrid procedures" for the treatment of atrial fibrillation.
CONCLUSIONS
A retrospective assessment of the 50 years of interventional therapy for arrhythmias clearly demonstrates the clinical benefits of a close collaboration between electrophysiologists and arrhythmia surgeons, regardless of which one is actually performing the intervention.
Topics: Atrial Fibrillation; Humans; Retrospective Studies; Surgeons; Tachycardia, Atrioventricular Nodal Reentry; Wolff-Parkinson-White Syndrome
PubMed: 35695795
DOI: 10.1111/jce.15598 -
Clinical Cardiology Aug 2020Accessory pathways are present in 1 in 300 young individuals. They are often asymptomatic and potentially lethal arrhythmias may be the first presentation. During... (Review)
Review
Accessory pathways are present in 1 in 300 young individuals. They are often asymptomatic and potentially lethal arrhythmias may be the first presentation. During long-term follow-up, up to 20% of asymptomatic individuals with pre-excitation go on to develop an arrhythmia and the absence of traditional clinical and electrophysiological high-risk markers does not guarantee the "safe" nature of an accessory pathway. The widespread availability of permanent cure for the condition at low risk by catheter ablation, creates an incentive to screen for accessory pathways with a 12-lead ECG, particularly in individuals who are perceived to be at increased risk, such as athletes and high-risk professions. We review the existing literature on the assessment and management of accessory pathways (Wolff-Parkinson-White [WPW] syndrome) and discuss its implications for the young athletic population.
Topics: Athletes; Catheter Ablation; Electrocardiography; Heart Conduction System; Heart Rate; Humans; Wolff-Parkinson-White Syndrome
PubMed: 32592213
DOI: 10.1002/clc.23399 -
JACC. Clinical Electrophysiology Mar 2023
Topics: Humans; Atrial Fibrillation; Takotsubo Cardiomyopathy; Wolff-Parkinson-White Syndrome
PubMed: 36990599
DOI: 10.1016/j.jacep.2023.01.036 -
Arquivos Brasileiros de Cardiologia Sep 2022PRKAG2 syndrome is a rare autosomal dominant disease, a phenocopy of hypertrophic cardiomyopathy characterized by intracellular glycogen accumulation. Clinical...
BACKGROUND
PRKAG2 syndrome is a rare autosomal dominant disease, a phenocopy of hypertrophic cardiomyopathy characterized by intracellular glycogen accumulation. Clinical manifestations include ventricular preexcitation, cardiac conduction disorder, ventricular hypertrophy, and atrial arrhythmias.
OBJECTIVE
To compare the clinical and electrophysiological characteristics observed in patients with atrial flutter, with and without PRKAG2 syndrome.
METHODS
An observational study comparing patients with atrial flutter: group A consisted of five patients with PRKAG2 syndrome from a family, and group B consisted of 25 patients without phenotype of PRKAG2 syndrome. The level of significance was 5%.
RESULTS
All patients in group A had ventricular preexcitation and right branch block, and four had pacemakers (80%). Patients in group A were younger (39±5.4 vs 58.6±17.6 years, p=0.021), had greater interventricular septum (median=18 vs 10 mm; p<0.001) and posterior wall thickness (median=14 vs 10 mm; p=0.001). In group A, four patients were submitted to an electrophysiological study, showing a fasciculoventricular pathway, and atrial flutter ablation was performed in tree. All patients in group B were submitted to ablation of atrial flutter, with no evidence of accessory pathway. Group B had a higher prevalence of hypertension, diabetes mellitus, coronary artery disease and sleep apnea, with no statistically significant difference.
CONCLUSION
Patients with PRKAG2 syndrome presented atrial flutter at an earlier age and had fewer comorbidities when compared to patients with atrial flutter without mutation phenotype. The occurrence of atrial flutter in young individuals, especially in the presence of ventricular preexcitation and familial ventricular hypertrophy, should raise the suspicion of PRKAG2 syndrome.
PubMed: 36102422
DOI: 10.36660/abc.20210792 -
Annals of Noninvasive Electrocardiology... Jul 2014Sudden cardiac death (SCD) remains a major public health problem. Current established criteria identifying those at risk of sudden arrhythmic death, and likely to... (Review)
Review
BACKGROUND
Sudden cardiac death (SCD) remains a major public health problem. Current established criteria identifying those at risk of sudden arrhythmic death, and likely to benefit from implantable cardioverter defibrillators (ICDs), are neither sensitive nor specific. Exercise electrocardiogram (ECG) testing was traditionally used for information concerning patients' symptoms, exercise capacity, cardiovascular function, myocardial ischemia detection, and hemodynamic responses during activity in patients with hypertrophic cardiomyopathy.
METHODS
We conducted a systematic review of MEDLINE on the utility of exercise ECG testing in SCD risk stratification.
RESULTS
Exercise testing can unmask suspected primary electrical diseases in certain patients (catecholaminergic polymorphic ventricular tachycardia or concealed long QT syndrome) and can be effectively utilized to risk stratify patients at an increased (such as early repolarization syndrome and Brugada syndrome) or decreased risk of SCD, such as the loss of preexcitation on exercise testing in asymptomatic Wolff-Parkinson-White syndrome.
CONCLUSIONS
Exercise ECG testing helps in SCD risk stratification in patients with and without arrhythmogenic hereditary syndromes.
Topics: Death, Sudden, Cardiac; Electrocardiography; Exercise Test; Humans; Risk Assessment
PubMed: 25040480
DOI: 10.1111/anec.12191 -
Revista Portuguesa de Cardiologia :... Sep 2022Wolff-Parkinson-White (WPW) syndrome is the most common manifestation of ventricular pre-excitation syndrome and is mostly found in individuals with no structural heart...
Wolff-Parkinson-White (WPW) syndrome is the most common manifestation of ventricular pre-excitation syndrome and is mostly found in individuals with no structural heart disease. Although the risk of malignant arrhythmias is low, sudden cardiac death (SCD) as the first clinical manifestation of WPW syndrome is well documented, and atrial fibrillation (AF) with a rapid ventricular response is the main mechanism involved. Unfortunately, the signs of pre-excitation and arrhythmias are sometimes under-diagnosed and under-treated. We describe the case of a 31-year-old man who was admitted with an irregular wide complex tachycardia consistent with pre-excited AF, which was not promptly diagnosed, and who developed ventricular fibrillation (VF) after administration of atrioventricular (AV) nodal blockers, as a primary manifestation of WPW syndrome. Blocking the AV node in patients with pre-excited AF may increase the ventricular rate and potentially result in hemodynamic instability. Among patients with WPW syndrome who survive an episode of SCD, catheter ablation of the accessory pathway is the treatment of choice.
PubMed: 36114111
DOI: 10.1016/j.repc.2019.06.008 -
Circulation. Arrhythmia and... Jan 2016
Review
Topics: AMP-Activated Protein Kinases; DNA; Heart Conduction System; Humans; Mutation; Wolff-Parkinson-White Syndrome
PubMed: 26729852
DOI: 10.1161/CIRCEP.115.003121 -
Cardiology Journal 2022In contrast to adults, in whom cardiac rhythm disorders are mainly conditioned by coronary artery disease, in children, arrhythmias are most often associated with...
BACKGROUND
In contrast to adults, in whom cardiac rhythm disorders are mainly conditioned by coronary artery disease, in children, arrhythmias are most often associated with inherited heart disorders. Catheter ablation (CA) has an important role in the management of cardiac arrhythmias, in adults and children. The aim of the study was to assess and compare the efficacy and safety of CA in children and adults with preexcitation syndrome.
METHODS
The study population comprised 43 adults and 43 children diagnosed with a Wolff-Parkinson-White syndrome (WPW). The mean age of the study population was 41 ± 15 years for adults and 14 ± 2.5 years for children. In all patients, an electrophysiological study and CA were performed. Analysis with respect to the procedure duration, fluoroscopy exposure time, location of accessory pathways (AP), immediate success rate and complications were performed.
RESULTS
Electrophysiological study revealed the most frequent presence of left-sided AP (56% in children and 70% in adults). The mean procedure duration was 96 ± 36 min and 106 ± 51 min in children and adults, respectively (p = NS). The mean fluoroscopy duration was 8.5 ± 4.3 min and 5.9 ± 5.8 min in children and adults, respectively p < 0.05. The CA procedure was successful in 40 out of 43 (93%) adults and in 36 out of 43 (83.7%) children (p = NS). In 2 (4%) children minor complications occurred.
CONCLUSIONS
Ablation in children and adults are equally effective with respect to short-term clinical observation.
Topics: Accessory Atrioventricular Bundle; Adult; Arrhythmias, Cardiac; Catheter Ablation; Child; Humans; Middle Aged; Pre-Excitation Syndromes; Treatment Outcome; Wolff-Parkinson-White Syndrome
PubMed: 32207846
DOI: 10.5603/CJ.a2020.0030 -
Arquivos Brasileiros de Cardiologia Dec 2022
Topics: Humans; Heart Ventricles; Wolff-Parkinson-White Syndrome; Mutation; AMP-Activated Protein Kinases
PubMed: 36541985
DOI: 10.36660/abc.20220795 -
BMC Cardiovascular Disorders Aug 2023Danon disease (DD) is an exceptionally uncommon X-linked dominant lysosomal glycogen storage disorder characterized by pronounced ventricular hypertrophy and cardiac...
BACKGROUND
Danon disease (DD) is an exceptionally uncommon X-linked dominant lysosomal glycogen storage disorder characterized by pronounced ventricular hypertrophy and cardiac insufficiency. The timely identification of cardiac impairment in individuals with DD holds significant clinical importance.
CASE PRESENTATION
We present a case of Danon Disease in a three-generation pedigree from Anhui Province, China. Clinical features and laboratory data were collected and analyzed for a 16-year-old male proband (III-1) and two affected female family members (II-2 and II-3). The proband exhibited Wolf-Parkinson-White syndrome, hypertrophic cardiomyopathy, abnormal cognitive function, and muscle weakness. Gene sequencing confirmed a mutation (c.963G > A) in the LAMP-2 gene.
CONCLUSION
Patients with DD may present both dilated and hypertrophic cardiomyopathy. Comprehensive myocardial tissue characterization by MRI plays a key role in the diagnosis of the disease.
Topics: Male; Female; Humans; Glycogen Storage Disease Type IIb; Cardiomyopathy, Hypertrophic; Mutation; Wolff-Parkinson-White Syndrome; Magnetic Resonance Imaging
PubMed: 37568080
DOI: 10.1186/s12872-023-03356-y