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The American Journal of Cardiology Feb 2018A 37-year-old man came to the emergency department because of several days of intermittent chest pain. An electrocardiogram (ECG) showed sinus rhythm, left atrial and...
A 37-year-old man came to the emergency department because of several days of intermittent chest pain. An electrocardiogram (ECG) showed sinus rhythm, left atrial and left ventricular enlargement, and an early repolarization pattern. A second ECG recorded 10 minutes later was strikingly different, with ST-segment elevation and large upright T waves in the anterior precordial leads, interpreted as evidence of an ST-segment elevation myocardial infarction, and the cardiac catheterization team was activated. Closer inspection of the ECG, however, disclosed that the changes were because of intermittent ventricular pre-excitation of the Wolff-Parkinson-White type, and no electrocardiographic, echocardiographic, or serum markers of myocardial infarction were found.
Topics: Adult; Biomarkers; Chest Pain; Diagnosis, Differential; Echocardiography; Electrocardiography; Humans; Male; Pre-Excitation Syndromes
PubMed: 29191564
DOI: 10.1016/j.amjcard.2017.09.036 -
Herzschrittmachertherapie &... Jun 2023The term "asymptomatic WPW" (Wolff-Parkinson-White) is often used as a synonym for ventricular pre-excitation of the WPW type due to an accessory pathway characterized... (Review)
Review
The term "asymptomatic WPW" (Wolff-Parkinson-White) is often used as a synonym for ventricular pre-excitation of the WPW type due to an accessory pathway characterized by a short PR interval and a delta wave on the electrocardiogram (ECG) without the clinical occurrence of paroxysmal tachycardia. Asymptomatic WPW is often diagnosed in young and otherwise healthy people. There is a small associated risk of sudden cardiac death due to rapid antegrade conduction over the accessory pathway during atrial fibrillation. This paper highlights aspects of noninvasive and invasive risk stratification, therapy by catheter ablation, and the ongoing risk-benefit discussion in asymptomatic WPW.
Topics: Humans; Wolff-Parkinson-White Syndrome; Atrial Fibrillation; Electrocardiography; Death, Sudden, Cardiac; Risk Assessment; Catheter Ablation
PubMed: 36939928
DOI: 10.1007/s00399-023-00930-x -
The Journal of Medical Investigation :... 2018Familial Wolff-Parkinson-White (WPW) syndrome is an autosomal dominant inherited disease and consists of a small percentage of WPW syndrome which exhibits ventricular... (Review)
Review
Familial Wolff-Parkinson-White (WPW) syndrome is an autosomal dominant inherited disease and consists of a small percentage of WPW syndrome which exhibits ventricular pre-excitation by development of accessory atrioventricular pathway. A series of mutations in PRKAG2 gene encoding gamma2 subunit of 5'AMP-activated protein kinase (AMPK) has been identified as the cause of familial WPW syndrome. AMPK is one of the most important metabolic regulators of carbohydrates and lipids in many types of tissues including cardiac and skeletal muscles. Patients and animals with the mutation in PRKAG2 gene exhibit aberrant atrioventricular conduction associated with cardiac glycogen overload. Recent studies have revealed "novel" significance of canonical pathways leading to glycogen synthesis and provided us profound insights into molecular mechanism of the regulation of glycogen metabolism by AMPK. This review focuses on the molecular basis of the pathogenesis of cardiac abnormality due to PRKAG2 mutation and will provide current overviews of the mechanism of glycogen regulation by AMPK. J. Med. Invest. 65:1-8, February, 2018.
Topics: AMP-Activated Protein Kinases; Animals; Glycogen; Humans; Mutation; Myocardium; Wolff-Parkinson-White Syndrome
PubMed: 29593177
DOI: 10.2152/jmi.65.1 -
La Tunisie MedicaleEbstein's disease (ED) is a rare and heterogeneous congenital heart disease affecting the tricuspid valve and the right ventricle. Few studies have analyzed the...
INTRODUCTION
Ebstein's disease (ED) is a rare and heterogeneous congenital heart disease affecting the tricuspid valve and the right ventricle. Few studies have analyzed the electrocardiographic features of this disease.
AIM
To describe the electrocardiographic features observed in Ebstein's disease.
METHODS
We conducted a retrospective descriptive study that enrolled 26 patients followed for ED.
RESULTS
The mean age of discovery of the ME was 103.5±99 months [0-31 years]. The diagnosis of ME is most often made between 5 and 10 years. We noted right atrial hypertrophy in 11 patients (42%), right ventricular hypertrophy in half of the patients. Right axial deviation was noted in 11 patients (42%). Eight patients (30%) had wide QRS≥ 120 ms. Seven of these 8 patients (27%) had a fragmented QRS appearance. A right bandle block was noted in 22 patients (84%), it was a complete block in 7 cases (27%). A preexcitation was found in 6 patients (23%). The localization of accessory pathway was right postero-septal in all cases. Rhythmic disorders were noted in 9 patients (34%). It was a junctional tachycardia in 3 patients (11%), atrial flutter in 4 patients (15%) and atrial fibrillation in 2 patients (7%). A second degree atriventricular block was observed in one patient, it was Mobitz I type. Two cases of postoperative rhythm disturbances were recorded: paroxysmal atrial fibrillation and junctional tachycardia related to Wolf Parkinson White (WPW) syndrome.
CONCLUSION
Surface ECG in the ED is often pathological with prevalence of rhythm disturbances related to WPW syndrome.
Topics: Atrial Fibrillation; Ebstein Anomaly; Humans; Retrospective Studies; Tricuspid Valve; Wolff-Parkinson-White Syndrome
PubMed: 35244913
DOI: No ID Found -
Heart Rhythm Jan 2020
Review
Topics: Accessory Atrioventricular Bundle; Electrocardiography; Heart Rate; Humans; Pre-Excitation, Mahaim-Type
PubMed: 31351139
DOI: 10.1016/j.hrthm.2019.07.025 -
Advances in Neonatal Care : Official... Jun 2021Wolff-Parkinson-White (WPW) is a congenital defect of the cardiac conduction system (CCS), with proliferation of extra embryologic conduction pathways and rapid... (Review)
Review
BACKGROUND
Wolff-Parkinson-White (WPW) is a congenital defect of the cardiac conduction system (CCS), with proliferation of extra embryologic conduction pathways and rapid conduction of electrical impulses. The estimated neonatal incidence of 0.1% to 0.2% may be misrepresented secondary to missed or misdiagnosis. Undiagnosed WPW can result in sudden cardiac death.
PURPOSE
To discuss the pathogenesis, manifestations, diagnosis, management, and lifespan implications of WPW in the prenatal and postnatal periods.
METHODS/SEARCH STRATEGY
A literature review was conducted using PubMed, CINAHL, and Google Scholar (2013-2019). Search terms included (newborn OR infant), wolff parkinson white, pathogenesis, management, and ventricular preexcitation. After removal of duplicates, 267 references were identified, abstracts reviewed, and 30 publications fully evaluated.
FINDINGS/RESULTS
Separation of the heart chambers begins around 7 weeks' gestation with formation of the annulus fibrosis complete after term. The unknown external environmental influence on the development of the preterm infant's CCS places neonates at risk for persistent atrioventricular reentrant tachycardia with WPW development. Ensuring an appropriate diagnosis is crucial, as an incorrect diagnosis could mean death.
IMPLICATIONS FOR PRACTICE
Due to the rarity of WPW, any fetal or neonatal supraventricular tachycardia requires further evaluation with an electrocardiogram and involvement of an experienced cardiologist for diagnosis. One episode of supraventricular tachycardia warrants evaluation for WPW, as recurring episodes may result in irreversible damage.
IMPLICATIONS FOR RESEARCH
The recommendations for treatment of WPW in the prenatal and immediate postnatal periods are based heavily on standards of care for the adult population. A paucity of evidenced-based literature exists and future research is crucial to understand the true incidence, physiologic effects, and lifespan implications of WPW on neonates.
Topics: Adult; Electrocardiography; Heart Conduction System; Heart Rate; Humans; Infant; Infant, Newborn; Infant, Premature; Wolff-Parkinson-White Syndrome
PubMed: 32826411
DOI: 10.1097/ANC.0000000000000785 -
Heart Rhythm May 2021
Review
Topics: Accessory Atrioventricular Bundle; Catheter Ablation; Electrocardiography; Humans; Wolff-Parkinson-White Syndrome
PubMed: 33934815
DOI: 10.1016/j.hrthm.2021.01.008 -
Zhonghua Xin Xue Guan Bing Za Zhi Jan 2023To review the clinical data of 7 patients with Danon disease and analyze their clinical characteristics. The medical records of 7 patients with Danon disease, who were...
To review the clinical data of 7 patients with Danon disease and analyze their clinical characteristics. The medical records of 7 patients with Danon disease, who were hospitalized in Peking Union Medical College Hospital of Chinese Academy of Medical Sciences from April 2008 to July 2021, were reviewed and summarized, of which 6 cases were diagnosed as Danon disease by lysosomal-associated membrane protein-2 (LAMP-2) gene mutation detection and 1 case was diagnosed by clinicopathological features. Clinical manifestations, biochemical indexes, electrocardiogram, echocardiography, skeletal muscle and myocardial biopsy and gene detection results were analyzed, and patients received clinical follow-up after discharge. Six patients were male and average age was (15.4±3.5) years and the average follow-up time was (27.7±17.0) months. The main clinical manifestations were myocardial hypertrophy (6/7), decreased myodynamia (2/7) and poor academic performance (3/7). Electrocardiogram features included pre-excitation syndrome (6/7) and left ventricular hypertrophy (7/7). Echocardiography examination evidenced myocardial hypertrophy (6/7), and left ventricular dilatation and systolic dysfunction during the disease course (1/7). The results of skeletal muscle biopsy in 6 patients were consistent with autophagy vacuolar myopathy. Subendocardial myocardial biopsy was performed in 3 patients, and a large amount of glycogen deposition with autophagosome formation was found in cardiomyocytes. LAMP-2 gene was detected in 6 patients, and missense mutations were found in all these patients. During the follow-up period, implantable cardioverter defibrillator implantation was performed in 1 patient because of high atrioventricular block 4 years after diagnosis, and there was no death or hospitalization for cardiovascular events in the other patients. The main clinical manifestations of Danon disease are cardiomyopathy, myopathy and mental retardation. Pre-excitation syndrome is a common electrocardiographic manifestation. Autophagy vacuoles can be seen in skeletal muscle and myocardial pathological biopsies. LAMP-2 gene mutation analysis is helpful in the diagnose of this disease.
Topics: Adolescent; Child; Female; Humans; Male; Cardiomyopathies; Glycogen Storage Disease Type IIb; Hypertrophy, Left Ventricular; Lysosomal-Associated Membrane Protein 2; Pre-Excitation Syndromes
PubMed: 36655242
DOI: 10.3760/cma.j.cn112148-20221108-00876 -
Cardiology in the Young Jan 2017The Wolff-Parkinson-White pattern refers to the electrocardiographic appearance in sinus rhythm, wherein an accessory atrioventricular pathway abbreviates the P-R... (Review)
Review
The Wolff-Parkinson-White pattern refers to the electrocardiographic appearance in sinus rhythm, wherein an accessory atrioventricular pathway abbreviates the P-R interval and causes a slurring of the QRS upslope - the "delta wave". It may be asymptomatic or it may be associated with orthodromic reciprocating tachycardia; however, rarely, even in children, it is associated with sudden death due to ventricular fibrillation resulting from a rapid response by the accessory pathway to atrial fibrillation, which itself seems to result from orthodromic reciprocating tachycardia. Historically, patients at risk for sudden death were characterised by the presence of symptoms and a shortest pre- excited R-R interval during induced atrial fibrillation <250 ms. Owing to the relatively high prevalence of asymptomatic Wolff-Parkinson-White pattern and availability of catheter ablation, there has been a need to identify risk among asymptomatic patients. Recent guidelines recommend invasive evaluation for such patients where pre-excitation clearly does not disappear during exercise testing. This strategy has a high negative predictive value only. The accuracy of this approach is under continued investigation, especially in light of other considerations: Patients having intermittent pre-excitation, once thought to be at minimal risk may not be, and the role of isoproterenol in risk assessment.
Topics: Accessory Atrioventricular Bundle; Atrial Fibrillation; Catheter Ablation; Death, Sudden, Cardiac; Electrocardiography; Exercise Test; History, 20th Century; Humans; Isoproterenol; Practice Guidelines as Topic; Risk Assessment; Tachycardia, Reciprocating; Wolff-Parkinson-White Syndrome
PubMed: 28084962
DOI: 10.1017/S1047951116002250 -
Journal of Cardiovascular Medicine... Sep 2023Overt or concealed accessory pathways are the anatomic substrates of ventricular preexcitation (VP), Wolff-Parkinson-White syndrome (WPW) and paroxysmal supraventricular... (Review)
Review
Overt or concealed accessory pathways are the anatomic substrates of ventricular preexcitation (VP), Wolff-Parkinson-White syndrome (WPW) and paroxysmal supraventricular tachycardia (PSVT). These arrhythmias are commonly observed in pediatric age. PSVT may occur at any age, from fetus to adulthood, and its symptoms range from none to syncope or heart failure. VP too can range from no symptoms to sudden cardiac death. Therefore, these arrhythmias frequently need risk stratification, electrophysiologic study, drug or ablation treatment. In this review of the literature, recommendations are given for diagnosis and treatment of fetal and pediatric age (≤12 years) WPW, VP, PSVT, and criteria for sport participation.
Topics: Humans; Child; Infant, Newborn; Infant; Child, Preschool; Wolff-Parkinson-White Syndrome; Accessory Atrioventricular Bundle; Electrocardiography; Tachycardia, Paroxysmal; Tachycardia, Ventricular; Fetus
PubMed: 37409656
DOI: 10.2459/JCM.0000000000001484