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    Novel Variant in the ANK2 Membrane-Binding Domain Is Associated With Ankyrin-B Syndrome and Structural Heart Disease in a First Nations Population With a High Rate of Long QT Syndrome.
    Circ Cardiovasc Genet 2017 Jan;10(1)
    From the Division of Medical Sciences, University of Victoria, BC, Canada (L.A.S., L.C., X.X., L.T.A.); University of British Columbia Island Medical Program, Victoria, BC, Canada (L.A.S., L.T.A.); Department of Medical Genetics (S.A., S.M., L.T.A.), Division of Cardiology (C.K.), and Division of Cardiology, Department of Pediatrics, BC Children's Hospital (S.S., E.S.), University of British Columbia, Vancouver, BC, Canada; Division of Cardiovascular Medicine, Department of Internal Medicine, Dorothy M. Davis Heart and Lung Research Institute (N.P.M., P.J.L., C.F.K., P.J.M.) and Department of Physiology and Cell Biology (N.P.M., P.J.L., C.F.K., P.J.M.), The Ohio State University Wexner Medical Center, Columbus, OH; Division of Life Science, State Key Laboratory of Molecular Neuroscience, Hong Kong University of Science and Technology, Clear Water Bay, Kowloon, Hong Kong, China (C.W., M.Z.); and Section of Cardiac Electrophysiology, Division of Cardiology, Department of Medicine, Western University, London, ON, Canada (J.D.R.).
    Background: Long QT syndrome confers susceptibility to ventricular arrhythmia, predisposing to syncope, seizures, and sudden death. While rare globally, long QT syndrome is ≈15× more common in First Nations of Northern British Columbia largely because of a known mutation in KCNQ1. However, 2 large multigenerational families were affected, but negative for the known mutation. Read More

    Long term risk of Wolff-Parkinson-White pattern and syndrome.
    Trends Cardiovasc Med 2016 Dec 5. Epub 2016 Dec 5.
    Bluhm Cardiovascular Institute, Northwestern Memorial Hospital, 676 St. Clair, Suite 600, Chicago, IL 60611, USA; Northwestern University, Feinberg School of Medicine, 676 St. Clair, Suite 600, Chicago, IL 60611, USA. Electronic address:
    For years, conventional wisdom has held that patients with asymptomatic ventricular pre-excitation (asymptomatic WPW or WPW pattern) were at low risk for adverse outcomes. This assumption has been challenged more recently in a number of observational/natural history studies as well as in prospective trials in which patients were more aggressively studied via invasive electrophysiology study (EPS) and more aggressively treated, in some cases, with pre-emptive catheter ablation, despite the lack of symptoms. In sum, the data do not definitively support one approach (early, up-stream EPS and/or ablation) vs. Read More

    Wolff-Parkinson-White syndrome: lessons learnt and lessons remaining.
    Cardiol Young 2017 Jan;27(S1):S62-S67
    2Heart Center, Department ofPediatric Cardiology,Phoenix Children's Hospital,University of Arizona College of Medicine-Phoenix,Phoenix, Arizona,United States of America.
    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. Read More

    Asymptomatic Wolff-Parkinson-White Pattern ECG in USAF Aviators.
    Aerosp Med Hum Perform 2017 Jan;88(1):56-60
    Introduction: Wolff-Parkinson-White (WPW) pattern is occasionally found in asymptomatic aviators during routine ECGs. Aeromedical concerns regarding WPW pattern include risk of dysrhythmia or sudden cardiac death (SCD), thus affecting the safety of flight. The purpose of this study was to determine the prevalence and outcomes of aviators with asymptomatic WPW pattern and assess for risk factors that contribute to progression to dysrhythmia or symptoms. Read More

    Histiocytoid cardiomyopathy and microphthalmia with linear skin defects syndrome: phenotypes linked by truncating variants in NDUFB11.
    Cold Spring Harb Mol Case Stud 2017 Jan;3(1):a001271
    NIHR Cardiovascular Biomedical Research Unit at Royal Brompton and Harefield NHS Foundation Trust and Imperial College London, London SW3 6NP, United Kingdom;; National Heart and Lung Institute, Imperial College London, London SW3 6NP, United Kingdom;; MRC Clinical Sciences Centre, Imperial College London, London W12 0NN, United Kingdom.
    Variants in NDUFB11, which encodes a structural component of complex I of the mitochondrial respiratory chain (MRC), were recently independently reported to cause histiocytoid cardiomyopathy (histiocytoid CM) and microphthalmia with linear skin defects syndrome (MLS syndrome). Here we report an additional case of histiocytoid CM, which carries a de novo nonsense variant in NDUFB11 (ENST00000276062.8: c. Read More

    Are Olympic athletes free from cardiovascular diseases? Systematic investigation in 2352 participants from Athens 2004 to Sochi 2014.
    Br J Sports Med 2017 Feb 30;51(4):238-243. Epub 2016 Dec 30.
    Institute of Sport Medicine and Science, Italian National Olympic Committee, Rome, Italy.
    Context: Olympic athletes represent model of success in our society, by enduring strenuous conditioning programmes and achieving astonishing performances. They also raise scientific and clinical interest, with regard to medical care and prevalence of cardiovascular (CV) abnormalities.

    Objective: Our aim was to assess the prevalence and type of CV abnormalities in this selected athlete's cohort. Read More

    Integrative Analysis of PRKAG2 Cardiomyopathy iPS and Microtissue Models Identifies AMPK as a Regulator of Metabolism, Survival, and Fibrosis.
    Cell Rep 2016 Dec;17(12):3292-3304
    Department of Genetics, Harvard Medical School, Boston, MA 02115, USA; Division of Cardiovascular Medicine, Brigham and Women's Hospital, Boston, MA 02115, USA; Howard Hughes Medical Institute, Chevy Chase, MD 20815, USA. Electronic address:
    AMP-activated protein kinase (AMPK) is a metabolic enzyme that can be activated by nutrient stress or genetic mutations. Missense mutations in the regulatory subunit, PRKAG2, activate AMPK and cause left ventricular hypertrophy, glycogen accumulation, and ventricular pre-excitation. Using human iPS cell models combined with three-dimensional cardiac microtissues, we show that activating PRKAG2 mutations increase microtissue twitch force by enhancing myocyte survival. Read More

    Making the Quick Diagnosis: A Case of Neonatal Shock.
    J Emerg Med 2016 Dec 13. Epub 2016 Dec 13.
    The University of Texas at Austin, Dell Medical School, Dell Children's Medical Center of Central Texas, Austin, Texas.
    Background: The work-up and initial management of a critically ill neonate is challenging and anxiety provoking for the Emergency Physician. While sepsis and critical congenital heart disease represent a large proportion of neonates presenting to the Emergency Department (ED) in shock, there are several additional etiologies to consider. Underlying metabolic, endocrinologic, gastrointestinal, neurologic, and traumatic disorders must be considered in a critically ill infant. Read More

    Radiofrequency catheter ablation of accessory pathways in patients with Ebstein's anomaly: At 8 years of follow-up.
    Cardiol J 2016 Dec 2. Epub 2016 Dec 2.
    Institute of Cardiology, Arrhythmia Department, Warsaw, Poland.
    Background: Data regarding long-term follow-up of radiofrequency catheter ablation (RFCA) of accessory pathways (APs) in patients with Ebstein's anomaly (EA) are limited. The procedures are challenging due to multiple or wide APs.

    Methods: Analysis was performed on clinical and periprocedural data of patients with EA referred to the centre in order to perform catheter ablation of accessory pathway (AP). Read More

    [AV-reentrant tachycardia and Wolff-Parkinson-White syndrome : Diagnosis and treatment].
    Herzschrittmacherther Elektrophysiol 2016 Dec 22;27(4):381-389. Epub 2016 Nov 22.
    Innere Medizin/Rhythmologie, Klinikum Traunstein, Traunstein, Deutschland.
    The AV-reentrant tachycardia (AVRT) is a supraventricular tachycardia with an incidence of 1-3/1000. The pathophysiological basis is an accessory atrioventricular pathway (AP). Patients with AVRT typically present with palpitations, an on-off characteristic, anxiety, dyspnea, and polyuria. Read More

    Wolff-Parkinson-White syndrome: a single exercise stress test might be misleading.
    Cardiol Young 2016 Nov 21:1-4. Epub 2016 Nov 21.
    Department of Pediatrics,Division of Pediatric Cardiology,New York-Presbyterian Morgan Stanley Children's Hospital,Columbia University Medical Center, New York,New York,United States of America.
    Risk stratification of patients with Wolff-Parkinson-White syndrome for sudden death is a complex process, particularly in understanding the utility of the repeat exercise stress test. We report a case of an 18-year-old patient who was found to have a high-risk pathway by both invasive and exercise stress testing after an initial exercise stress test showing beat-to-beat loss of pre-excitation. Read More

    PRKAG3 polymorphisms associated with sporadic Wolff-Parkinson-White syndrome among a Taiwanese population.
    J Chin Med Assoc 2016 Dec 17;79(12):656-660. Epub 2016 Nov 17.
    Department of Medical Education and Research, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan, ROC; Institute of Biomedical Sciences, National Sun Yat-Sen University, Kaohsiung, Taiwan, ROC.
    Background: The aim of this study was to investigate whether mutation in AMP-activated protein kinase (AMPK) subunit genes (PRKAG3-230) is associated with sporadic, isolated Wolff-Parkinson-White (WPW) syndrome.

    Methods: This study consisted of 87 patients with symptomatic WPW syndrome and 93 healthy controls. PRKAG3-230 genotypes were determined using real-time polymerase chain reaction assay. Read More

    Patient Specific Instruments for Complex Tumor Resection-Reconstruction Surgery within the Pelvis: A Series of 4 Cases.
    Chirurgia (Bucur) 2016 Sept-Oct;111(5):439-444
    The pelvis bone resection-reconstruction surgery is one of the most challenging fields in orthopedics. Being applied for tumors, as for other complex reconstruction cases, this type of surgery needs careful planning and is time consuming, in order to obtain proper accuracy. Unfortunately not all the time the expected accuracy is met, with consequences for the patients. Read More

    Ischemic stroke due to hypoperfusion in a patient with a previously unrecognized Danon disease.
    Neuromuscul Disord 2016 Dec 5;26(12):890-894. Epub 2016 Oct 5.
    Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy. Electronic address:
    Danon disease, an X-linked multisystemic disorder, is due to deficiency of Lysosome-Associated Membrane Protein 2 (LAMP2). It is usually characterized by hypertrophic cardiomyopathy, mental retardation and skeletal myopathy, sometimes also with atypical features. A 20-year-old man with cognitive impairment was admitted to the Emergency Room because of a sudden chest pain. Read More

    Accessory pathway location affects brain natriuretic peptide level in patients with Wolff-Parkinson-White syndrome.
    J Interv Card Electrophysiol 2017 Jan 4;48(1):81-88. Epub 2016 Nov 4.
    Division of Cardiology, Gunma Prefectural Cardiovascular Center, Gunma, Japan.
    Purpose: The purpose of this study was to investigate the relationship between the accessory pathway location and brain natriuretic peptide (BNP) level in patients with Wolff-Parkinson-White (WPW) syndrome.

    Methods: We divided 102 WPW syndrome patients with normal left ventricular systolic function into four groups: those with manifest right (MR, n = 14), manifest septal (MS, n = 11), manifest left (ML, n = 30), and concealed (C, n = 47) accessory pathways. BNP level and electrophysiological properties, including difference in timing of the ventricular electrogram between the His bundle area and the distal coronary sinus area (His-CS delay), which indicate intraventricular dyssynchrony, were compared. Read More

    Dexmedetomidine use in patients undergoing electrophysiological study for supraventricular tachyarrhythmias.
    Paediatr Anaesth 2017 Jan 25;27(1):45-51. Epub 2016 Oct 25.
    Cardiac Anesthesia, The Heart Program, Nicklaus Children's Hospital, Miami, FL, USA.
    Background: Dexmedetomidine is a selective alpha-2 adrenergic agonist with sedative, analgesic, and anxiolytic properties. Dexmedetomidine has not been approved for use in pediatrics. Dexmedetomidine has been reported to depress sinus node and atrioventricular nodal function in pediatric patients; it has been suggested that the use of dexmedetomidine may not be desirable during electrophysiological studies. Read More

    Reversible transition from a hypertrophic to a dilated cardiomyopathy.
    ESC Heart Fail 2016 Jun 9;3(2):138-142. Epub 2015 Dec 9.
    Department of CardiologyCharité-University Medicine of Berlin, Campus Virchow KlinikumBerlinGermany; Berlin-Brandenburg Center for Regenerative Therapies (BCRT)Charité-University Medicine of Berlin, Campus Virchow KlinikumBerlinGermany; Deutsches Zentrum für Herz-Kreislaufforschung (DZHK)-Standort Berlin/CharitéBerlinGermany.
    We report the case of a 17-year-old female patient with known hypertrophic cardiomyopathy and a Wolff-Parkinson-White syndrome. She came to our department for further evaluation of a new diagnosed dilated cardiomyopathy characterized by an enlargement of the left ventricle and a fall in ejection fraction. Clinically, she complained about atypical chest pain, arrhythmic episodes with presyncopal events, and dyspnea (NYHA III) during the last 6 months. Read More

    Reentry Tachycardia in Children: Adenosine Can Make It Worse.
    Pediatr Emerg Care 2016 Oct 8. Epub 2016 Oct 8.
    From the *Department of Pediatrics, Heidelberg University Children's Hospital, Heidelberg, Germany; and †Department of Pediatrics, Nîmes University Hospital, Nîmes, France.
    Objectives: We report on a rare but severe complication of adenosine use in a child with reentry tachycardia.

    Methods And Results: Treatment with adenosine, which is the standard medical therapy of atrioventricular reentry tachycardia, led to the development of an irregular wide complex tachycardia, caused by rapid ventricular response to atrial fibrillation. The girl was finally stabilized with electrical cardioversion. Read More

    Cardiac arrhythmias in patients with Danon disease.
    Europace 2016 Oct 14. Epub 2016 Oct 14.
    Department of Cardiology II/Electrophysiology, Center of Cardiology, University Medical Center, Johannes Gutenberg-University Mainz, Langenbeckstr. 1, Mainz D-55131, Germany
    Aims: Different cardiac arrhythmias have been suggested to be associated with Danon disease, e.g. Wolff-Parkinson-White syndrome. Read More

    Depression masquerading as chest pain in a patient with Wolff Parkinson White syndrome.
    Korean J Pain 2016 Oct 29;29(4):262-265. Epub 2016 Sep 29.
    Department of Anaesthesiology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India.
    Wolff Parkinson White (WPW) syndrome is a condition in which there is an aberrant conduction pathway between the atria and ventricles, resulting in tachycardia. A 42-year-old patient, who was treated for WPW syndrome previously, presented with chronic somatic pain. With her cardiac condition in mind, she was thoroughly worked up for a recurrence of disease. Read More

    Accuracy of the Electrocardiogram in Localizing the Accessory Pathway in Patients with Wolff-Parkinson-White Pattern.
    Arq Bras Cardiol 2016 Oct 12;107(4):331-338. Epub 2016 Sep 12.
    Departamento de Fisiologia Clínica, ESTESC, Instituto Politécnico de Coimbra, Coimbra, Portugal.
    Background: There are currently several electrocardiographic algorithms to locate the accessory pathway (AP) in patients with Wolff-Parkinson-White (WPW) syndrome.

    Objective: To compare the ability of electrocardiographic algorithms in identifying the location of the AP in patients with WPW pattern referred for ablation.

    Methods: Observational, cross-sectional, retrospective study with 111 patients with WPW syndrome referred for AP ablation. Read More

    Physiological Expression of AMPKγ2RG Mutation Causes Wolff-Parkinson-White Syndrome and Induces Kidney Injury in Mice.
    J Biol Chem 2016 Nov 12;291(45):23428-23439. Epub 2016 Sep 12.
    From the Departments of Cardiometabolic Disease,
    Mutations of the AMP-activated kinase gamma 2 subunit (AMPKγ2), N488I (AMPKγ2(NI)) and R531G (AMPKγ2(RG)), are associated with Wolff-Parkinson-White (WPW) syndrome, a cardiac disorder characterized by ventricular pre-excitation in humans. Cardiac-specific transgenic overexpression of human AMPKγ2(NI) or AMPKγ2(RG) leads to constitutive AMPK activation and the WPW phenotype in mice. However, overexpression of these mutant proteins also caused profound, non-physiological increase in cardiac glycogen, which might abnormally alter the true phenotype. Read More

    Genome editing with CRISPR/Cas9 in postnatal mice corrects PRKAG2 cardiac syndrome.
    Cell Res 2016 Oct 30;26(10):1099-1111. Epub 2016 Aug 30.
    Shanghai Institute of Cardiovascular Diseases, Zhongshan Hospital, Fudan University, Shanghai 200032, China.
    PRKAG2 cardiac syndrome is an autosomal dominant inherited disease resulted from mutations in the PRKAG2 gene that encodes γ2 regulatory subunit of AMP-activated protein kinase. Affected patients usually develop ventricular tachyarrhythmia and experience progressive heart failure that is refractory to medical treatment and requires cardiac transplantation. In this study, we identify a H530R mutation in PRKAG2 from patients with familial Wolff-Parkinson-White syndrome. Read More

    The use of sugammadex in a pregnant patient with Wolff-Parkinson-White syndrome.
    J Clin Anesth 2016 Sep 6;33:1-4. Epub 2016 Apr 6.
    Department of Anesthesiology and Reanimation, Bilim University Medical Faculty, Istanbul, Turkey.
    Wolff-Parkinson-White (WPW) syndrome is a rare pre-excitation syndrome which develops when atrioventricular conduction occurs through a pathologic accessory pathway known as the bundle of Kent instead of atrioventricular node, hence resulting in tachycardia. Patients with WPW syndrome may experience various symptoms arising from mild-to-moderate chest disease, palpitations, hypotension, and severe cardiopulmonary dysfunction. These patients are most often symptomatic because of cardiac arrhythmias. Read More

    A novel LAMP2 mutation associated with severe cardiac hypertrophy and microvascular remodeling in a female with Danon disease: a case report and literature review.
    Cardiovasc Pathol 2016 Sep-Oct;25(5):423-31. Epub 2016 Jul 25.
    Medical Genetics Laboratory, Department of Molecular Medicine, Sapienza University, San Camillo-Forlanini Hospital, Rome, Italy.
    Background: Danon disease (DD) is a rare disorder characterized by cardiomyopathy, intellectual disability, and proximal myopathy. It is caused by mutations in the LAMP2 gene on X chromosome. Female patients most often present with late-onset cardiomyopathy and slow disease progression, but early-onset cases with unfavorable prognosis have been reported. Read More

    Paroxysmal Supraventricular Tachycardia: Pathophysiology, Diagnosis, and Management.
    Crit Care Nurs Clin North Am 2016 Sep 7;28(3):309-16. Epub 2016 Jun 7.
    Department of Acute and Tertiary Care, School of Nursing, University of Pittsburgh, 3500 Victoria Street, 336 VB, Pittsburgh, PA 15261, USA.
    Paroxysmal supraventricular tachycardia (PSVT) is a well-known and thoroughly studied clinical syndrome, characterized by regular tachycardia rhythm with sudden onset and abrupt termination. Most patients present with palpitations and dizziness, and their electrocardiogram demonstrates a narrow QRS complex and regular tachycardia with hidden or inverted P waves. PSVT is caused by re-entry due to the presence of inhomogeneous, accessory, or concealed conducting pathways. Read More

    Spontaneous Transition of Double Tachycardias with Atrial Fusion in a Patient with Wolff-Parkinson-White Syndrome.
    Korean Circ J 2016 Jul 21;46(4):574-9. Epub 2016 Jul 21.
    Division of Cardiology, Department of Internal Medicine, School of Medicine, Dankook University, Cheonan, Korea.
    Among patients with Wolff-Parkinson-White syndrome, atrioventricular reciprocating tachycardia (AVRT) and atrioventricular nodal reentrant tachycardia (AVNRT) can coexist in a single patient. Direct transition of both tachycardias is rare; however, it can occur after premature atrial or ventricular activity if the cycle lengths of the two tachycardias are similar. Furthermore, persistent atrial activation by an accessory pathway (AP) located outside of the AV node during ongoing AVNRT is also rare. Read More

    Identification of LAMP2 Mutations in Early-Onset Danon Disease With Hypertrophic Cardiomyopathy by Targeted Next-Generation Sequencing.
    Am J Cardiol 2016 Sep 27;118(6):888-94. Epub 2016 Jun 27.
    Research Division of Birth Defects, Institute of Pediatric Translational Medicine, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China. Electronic address:
    Danon disease is an X-linked disorder with the clinical triad of cardiomyopathy, skeletal myopathy, and mental retardation. Early diagnosis of this disease remains a challenge, especially in the pediatric population. In this study, we developed a targeted panel-based next generation sequencing pipeline to identify mutations by sequencing of selected candidate genes in 136 pediatric patients with either hypertrophic cardiomyopathy (HC) or idiopathic dilated cardiomyopathy (IDC). Read More

    Evolution of Clinical and Electrophysiological Data in Children with a Preexcitation Syndrome.
    Pacing Clin Electrophysiol 2016 Sep 18;39(9):951-8. Epub 2016 Aug 18.
    Adult and Paediatric Cardiology, CHU de Brabois, Vandoeuvre Les Nancy, France.
    Background: With ablation, the follow-up of preexcitation syndrome now is difficult to assess. The purpose was to collect data of children with a preexcitation syndrome studied on two separate occasions within a minimal interval of 1 year.

    Methods: This is a retrospective chart review of 47 children initially aged 12 ± 4 years, who underwent two or more invasive electrophysiological studies (EPS) within 1-25 years of one another (6. Read More

    Successful ablation of coexistent Mahaim tachycardia and right posterior accessory pathway in a patient with Ebstein's anomaly.
    Turk Kardiyol Dern Ars 2016 Jul;44(5):423-6
    Department of Pediatric Cardiology, İstanbul Medipol University Faculty of Medicine, İstanbul, Turkey.
    The atriofascicular accessory pathway (AP), known as the Mahaim pathway, is a rare form of pre-excitation, comprising less than 3% of all APs. Mahaim AP is characterized by decremental, anterograde-only conduction, and antidromic tachycardia with left bundle branch morphology. Prevalence of Mahaim AP in Ebstein's anomaly is significantly high. Read More

    Decreased false-positive adolescent pre-athletic screening with Seattle Criteria-interpreted electrocardiograms.
    Cardiol Young 2016 Jun 20:1-6. Epub 2016 Jun 20.
    2Department of Pediatric Cardiology,University of Arizona,Tucson,Arizona,United States of America.
    Sudden cardiac arrest is a rare but devastating cause of death in young adults. Electrocardiograms may detect many causes of sudden cardiac arrest, but are not routinely included in pre-athletic screening in the United States of America partly because of high rates of false-positive interpretation. To improve electrocardiogram specificity for identifying cardiac conditions associated with sudden cardiac arrest, an expert panel developed refined criteria known as the Seattle Criteria. Read More

    Noninvasive Localization of Accessory Pathways in Wolff-Parkinson-White Syndrome by Three-Dimensional Speckle Tracking Echocardiography.
    Circ Cardiovasc Imaging 2016 Jun;9(6)
    From the Department of Clinical Laboratory Medicine, Faculty of Medicine (T.I., Y.K.) and Cardiovascular Division, Faculty of Medicine (Y.Seo, M.I., Y.Sekiguchi, K.O., K.K., T.M.-O., M.Y., A.N., K.A.), University of Tsukuba, Japan.
    Background: We have developed a noninvasive isochrone activation imaging (AI) system with 3-dimensional (3D) speckle tracking echocardiography (STE), which allows visualization of the wavefront image of mechanical propagation of the accessory pathway (ACP) in Wolff-Parkinson-White syndrome.

    Methods And Results: Patients with manifest Wolff-Parkinson-White syndrome were imaged in 3D-STE AI mode, which quantified the time from QRS onset to regional endocardial deformation. In 2 patients with left- and right-side ACP, we confirmed that intraoperative contact endocardial electric mapping and the 3D-STE AI system showed comparable images pre- and postablation. Read More

    Postnatal cumulative incidence of supraventricular tachycardia in a general pediatric population: A national birth cohort database study.
    Heart Rhythm 2016 Oct 7;13(10):2070-5. Epub 2016 Jun 7.
    Department of Pediatrics, National Taiwan University Hospital and Medical College, National Taiwan University, Genomics Research Center, Academia Sinica, Taiwan Administration of National Health Insurance, Taipei, Taiwan.
    Background: Supraventricular tachycardia (SVT) is a common pediatric tachycardia, but the true incidence is unknown.

    Objective: We sought to investigate the true postnatal incidence and its medical needs.

    Methods: We derived a birth cohort comprised children born between 2000 and 2008 who had complete postnatal medical data in the Taiwan National Health Insurance Database for the period from 2000 to 2014. Read More

    Sports-Related Sudden Cardiac Injury or Death.
    Adolesc Med State Art Rev 2015 Dec;26(3):507-27
    Symptoms such as syncope and chest pain, especially if they are accompanied by palpitations or occur with exercise in any combination, require cardiac evaluation before adolescent athletes are allowed to return to the sports field. Some life-threatening conditions will likely be associated with a family history of HCM or LQTS, but the family history may not be discovered at the first medical visit. A family history of CPVT, for example, is hard to elicit unless this diagnosis has already been established in an affected family member. Read More

    Surgical aspects of atrial arrhythmia : Right atrial ablation and anti-arrhythmic surgery in congenital heart disease.
    Herzschrittmacherther Elektrophysiol 2016 Jun 25;27(2):137-42. Epub 2016 May 25.
    Consultant Cardiac Surgeon, Royal Brompton Hospital, Sydney Street, SW3 6NP, London, UK.
    Background: Atrial arrhythmias are frequently described in congenital heart disease.

    Objectives: To provide a surgical perspective of anti-arrhythmic procedures and strategic approaches.

    Methods: Discussion of the history of anti-arrhythmic treatments in congenital heart disease. Read More

    Early onset of cardiomyopathy and intellectual disability in a girl with Danon disease associated with a de novo novel mutation of the LAMP2 gene.
    Neuropathology 2016 Dec 5;36(6):561-565. Epub 2016 May 5.
    Department of Neurology, Nara Medical University School of Medicine, Kashihara, Nara, Japan.
    Danon disease, primary lysosome-associated membrane protein-2 (LAMP-2) deficiency, is characterized clinically by cardiomyopathy, myopathy and intellectual disability in boys. Because Danon disease is inherited in an X-linked dominant fashion, males are more severely affected than females, who usually have only cardiomyopathy without myopathy or intellectual disability; moreover, the onset of symptoms in females is usually in adulthood. We describe a girl with Danon disease who presented with hypertrophic cardiomyopathy and Wolff-Parkinson-White (WPW) syndrome at 12 years of age. Read More

    Wolff-Parkinson-White and left ventricular noncompaction in a Fabry patient: A case report.
    Turk Kardiyol Dern Ars 2016 Apr;44(3):248-50
    Department of Cardiology, Dr. Siyami Ersek Cardiovascular and Thoracic Training and Research Hospital, İstanbul, Turkey.
    Fabry disease is the second most common inherited (X-linked recessive) lysosomal storage disease associated with multiple organ involvement. Cardiac involvement of Fabry disease varies. Successful radiofrequency ablation of a Fabry disease patient with Wolff-Parkinson-White syndrome and left ventricular noncompaction is described in the present report. Read More

    Spinal Anaesthesia is Safe in a Patient with Wolff-Parkinson-White Syndrome Undergoing Evacuation of Molar Pregnancy.
    J Clin Diagn Res 2016 Feb 1;10(2):UD01-2. Epub 2016 Feb 1.
    Associate Professor, Department of Anesthesiology, M.S. Ramaiah Medical College & Hospitals , New BEL Road Bangalore, India .
    Wolff-Parkinson-White (WPW) syndrome is an uncommon cardiac condition where there is an abnormal band of atrial tissue connecting atria and ventricles which can electrically bypass atrioventricular node. The anaesthetic management in these patients is challenging as life threatening complications can occur perioperatively like paroxysmal supraventricular tachycardia and atrial fibrillation. Also, regional anaesthetic technique like subarachnoid block is a safe and cost effective alternative to general anaesthesia as it avoids polypharmacy. Read More

    Relationship between local production of microRNA-328 and atrial substrate remodeling in atrial fibrillation.
    J Cardiol 2016 Dec 15;68(6):472-477. Epub 2016 Mar 15.
    Department of Cardiovascular Medicine, Institute of Biomedical Sciences, Tokushima University Graduate School, Tokushima, Japan.
    Background: The underlying mechanism of atrial substrate remodeling in atrial fibrillation (AF) remains unknown. In this study, we investigated whether local and systemic levels of microRNA (miR) might be associated with the presence of AF and with left atrial (LA) substrate properties.

    Methods: Blood from the periphery, pulmonary vein (PV), and left atrial appendage (LAA) was sampled from 30 patients with AF undergoing PV isolation, and from 10 control subjects with Wolff-Parkinson-White syndrome and without AF. Read More

    Screening high school students in Italy for sudden cardiac death prevention by using a telecardiology device: a retrospective observational study.
    Cardiol Young 2016 Mar 4:1-8. Epub 2016 Mar 4.
    2Department of Cardiovascular, Respiratory, Nephrological, Anaesthesiological and Geriatric Sciences,"Sapienza" University,Viale del Policlinico,Rome,Italy.
    Background: In 2010, an Italian project was launched aimed at using a telecardiology device in order to perform early diagnosis of young students at risk of sudden cardiac death.

    Methods: Our retrospective observational study was conducted on a population of 13,016 students, aged between 16 and 19 years, in different Italian regions. It consisted of analysis of data recorded during a telecardiology pilot study. Read More

    Irregular Wide Complex Tachycardia in a Young Man.
    J Ark Med Soc 2016 Feb;112(10):182-3
    Wolf Parkinson White syndrome is a pre-excitation syndrome due to an accessory conduction pathway. Electrocardiography demonstrates a short PR interval, long QRS interval and delta waves in normal sinus rhythm. Atrial fibrillation with underlying Wolf Parkinson White syndrome presents with irregular wide complex tachycardia, and can cause sudden cardiac death by precipitating ventricular fibrillation. Read More

    Long-term use of amiodarone before heart transplantation significantly reduces early post-transplant atrial fibrillation and is not associated with increased mortality after heart transplantation.
    Drug Des Devel Ther 2016 16;10:677-86. Epub 2016 Feb 16.
    Department of Cardiology, Angiology and Pneumology, Heidelberg University Hospital, Heidelberg, Germany.
    Background: Amiodarone is a frequently used antiarrhythmic drug in patients with end-stage heart failure. Given its long half-life, pre-transplant use of amiodarone has been controversially discussed, with divergent results regarding morbidity and mortality after heart transplantation (HTX).

    Aim: The aim of this study was to investigate the effects of long-term use of amiodarone before HTX on early post-transplant atrial fibrillation (AF) and mortality after HTX. Read More

    Utility of unipolar recordings for complex Wolff-Parkinson-White ablation.
    Indian Pacing Electrophysiol J 2015 Mar-Apr;15(2):125-9. Epub 2015 Jul 29.
    Aurora Cardiovascular Services, Aurora Sinai/Aurora St. Luke's Medical Centers, Milwaukee, WI, USA.
    Radiofrequency ablation has been shown to be a safe and effective treatment strategy for the management of symptomatic patients with Wolff-Parkinson-White syndrome. It is supported by a success rate of 95% and a recurrence rate of less than 5%. However, ablation of accessory pathways can be challenging at times. Read More

    Antidromic Atrioventricular Reciprocating Tachycardia Using a Concealed Retrograde Conducting Left Lateral Accessory Pathway.
    Card Electrophysiol Clin 2016 Mar 11;8(1):37-43. Epub 2016 Jan 11.
    Cardiac Electrophysiology, Cardiology Division, University of Colorado, Denver, Anschutz Medical Campus, 12401 East 17th Avenue, B-132, Aurora, CO 80045, USA. Electronic address:
    Atrioventricular reciprocating tachycardia is a common cause of undifferentiated supraventricular tachycardia. In patients with manifest or concealed accessory pathways, it is imperative to assess for the presence of other accessory pathways. Multiple accessory pathways are present in 4% to 10% of patients and are more common in patients with structural heart disease. Read More

    The impact of B-type natriuretic peptide levels on the suppression of accompanying atrial fibrillation in Wolff-Parkinson-White syndrome patients after accessory pathway ablation.
    J Cardiol 2016 Dec 22;68(6):485-491. Epub 2016 Feb 22.
    Heart Rhythm Center, Tokyo Medical and Dental University, Tokyo, Japan.
    Background: Atrial fibrillation (AF) often coexists with Wolff-Parkinson-White (WPW) syndrome. We compared the efficacy of Kent bundle ablation alone and additional AF ablation on accompanying AF, and examined which patients would still have a risk of AF after successful Kent bundle ablation.

    Methods: This retrospective multicenter study included 96 patients (56±15 years, 72 male) with WPW syndrome and AF undergoing Kent bundle ablation. Read More

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