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Texas Heart Institute Journal Jan 2021
Topics: Cardiomyopathy, Hypertrophic; Diagnosis, Differential; Echocardiography; Electrocardiography; Female; Humans; Middle Aged; Outpatients; Wolff-Parkinson-White Syndrome
PubMed: 33946107
DOI: 10.14503/THIJ-20-7263 -
Annals of Noninvasive Electrocardiology... Jul 2016Intravenous adenosine is a short-acting blocker of the atrioventricular node that has been used to unmask subtle or latent preexcitation, and also to enable catheter...
Intravenous adenosine is a short-acting blocker of the atrioventricular node that has been used to unmask subtle or latent preexcitation, and also to enable catheter ablation in selected patients with absent or intermittent preexcitation. Depending on the accessory pathway characteristics, intravenous adenosine may produce specific electrocardiographic changes highly suggestive of the preexcitation variant. Herein, we view different ECG responses to this pharmacological test in various preexcitation patterns that were confirmed by electrophysiological studies. Careful analysis of electrocardiographic changes during adenosine test, with emphasis on P-delta interval, preexcitation degree, and atrioventricular block, can be helpful to diagnose the preexcitation variant/pattern.
Topics: Adenosine; Adolescent; Adult; Anti-Arrhythmia Agents; Catheter Ablation; Electrocardiography; Exercise Test; Female; Humans; Male; Pre-Excitation Syndromes; Wolff-Parkinson-White Syndrome
PubMed: 26969821
DOI: 10.1111/anec.12348 -
Journal of the American College of... Jan 1985The records of 90 patients with Wolff-Parkinson-White syndrome who presented with supraventricular tachycardia in the first 4 months of life were reviewed. Among these,...
The records of 90 patients with Wolff-Parkinson-White syndrome who presented with supraventricular tachycardia in the first 4 months of life were reviewed. Among these, 63% were male. Structural heart disease was present in 20%, most commonly Ebstein's anomaly. All patients presented with a regular narrow QRS tachycardia, and pre-excitation became evident only when normal sinus rhythm was established. Only one infant had atrial flutter and none had atrial fibrillation. Type A Wolff-Parkinson-White syndrome was most common (49%), with heart disease occurring in only 5% of these patients. In contrast, heart disease was identified in 45% of those with type B syndrome. Initially, normal sinus rhythm was achieved in 88% of the 66 infants treated with digoxin with no deaths. Normal sinus rhythm resumed after electrical countershock in 87% of the 15 infants so treated. Maintenance digoxin therapy was used in 85 patients. The Wolff-Parkinson-White pattern disappeared in 36% of the patients. Four infants died of cardiac causes during the mean follow-up period of 6.5 years. Two of these four infants had congenital heart disease; the third, with a normal heart initially, developed ventricular fibrillation and died from a cardiomyopathy considered related to resuscitation. The remaining infant, with a normal heart, died suddenly at 1 month of age. All were receiving digoxin. A wide QRS tachycardia later appeared in three patients, all with heart disease, one of whom died.(ABSTRACT TRUNCATED AT 250 WORDS)
Topics: Combined Modality Therapy; Digoxin; Ebstein Anomaly; Electric Countershock; Electrophysiology; Female; Follow-Up Studies; Heart Conduction System; Humans; Infant; Infant, Newborn; Male; Recurrence; Sinoatrial Node; Tachycardia; Wolff-Parkinson-White Syndrome
PubMed: 3964800
DOI: 10.1016/s0735-1097(85)80095-4 -
Emergency Medicine Journal : EMJ Sep 2003Wolff-Parkinson-White syndrome is not uncommon in the emergency department. Its early recognition and initial treatment allows rapid restoration to sinus rhythm. Prompt... (Review)
Review
Wolff-Parkinson-White syndrome is not uncommon in the emergency department. Its early recognition and initial treatment allows rapid restoration to sinus rhythm. Prompt referral to cardiology is essential for risk stratification through electrophysiological studies.
Topics: Adolescent; Electrocardiography; Female; Humans; Male; Middle Aged; Wolff-Parkinson-White Syndrome
PubMed: 12954704
DOI: 10.1136/emj.20.5.491 -
Korean Circulation Journal Sep 2014Pre-excitation-syndrome has not been reported as a phenotypic feature of facio-scapulo-humeral muscular dystrophy (FSH-MD). In a 39-year-old male with FSH-MD due to a...
Pre-excitation-syndrome has not been reported as a phenotypic feature of facio-scapulo-humeral muscular dystrophy (FSH-MD). In a 39-year-old male with FSH-MD due to a reduced tandem repeat size in the D4Z4-locus on chromosome 4q35, cardiac involvement, manifesting as an incomplete right bundle-branch-block, tall T-waves in V 3-5, ST-elevation in V 2-4, and mild thickening of the left ventricular myocardium, was first recognised 10 years earlier. Follow-up at age 39 years revealed mild myocardial thickening, two intra-ventricular aberrant bands, and, surprisingly, intermittent pre-excitation on a routine electrocardiography. Cardiac involvement in FSH-MD may manifest as hypertrophic cardiomyopathy or various arrhythmias, of which one may be pre-excitation-syndrome.
PubMed: 25278989
DOI: 10.4070/kcj.2014.44.5.348 -
American Journal of Physiology. Heart... Jan 2007Some mutations of the sodium channel gene Na(V1.5) are multifunctional, causing combinations of LQTS, Brugada syndrome and progressive cardiac conduction system disease...
Some mutations of the sodium channel gene Na(V1.5) are multifunctional, causing combinations of LQTS, Brugada syndrome and progressive cardiac conduction system disease (PCCD). The combination of Brugada syndrome and PCCD is uncommon, although they both result from a reduction in the sodium current. We hypothesize that slow conduction is sufficient to cause S-T segment elevation and undertook a combined experimental and theoretical study to determine whether conduction slowing alone can produce the Brugada phenotype. Deletion of lysine 1479 in one of two positively charged clusters in the III/IV inter-domain linker causes both syndromes. We have examined the functional effects of this mutation using heterologous expression of the wild-type and mutant sodium channel in HEK-293-EBNA cells. We show that DeltaK1479 shifts the potential of half-activation, V(1/2m), to more positive potentials (V(1/2m) = -36.8 +/- 0.8 and -24.5 +/- 1.3 mV for the wild-type and DeltaK1479 mutant respectively, n = 11, 10). The depolarizing shift increases the extent of depolarization required for activation. The potential of half-inactivation, V(1/2h), is also shifted to more positive potentials (V(1/2h) = -85 +/- 1.1 and -79.4 +/- 1.2 mV for wild-type and DeltaK1479 mutant respectively), increasing the fraction of channels available for activation. These shifts are quantitatively the same as a mutation that produces PCCD only, G514C. We incorporated experimentally derived parameters into a model of the cardiac action potential and its propagation in a one dimensional cable (simulating endo-, mid-myocardial and epicardial regions). The simulations show that action potential and ECG changes consistent with Brugada syndrome may result from conduction slowing alone; marked repolarization heterogeneity is not required. The findings also suggest how Brugada syndrome and PCCD which both result from loss of sodium channel function are sometimes present alone and at other times in combination.
Topics: Action Potentials; Brugada Syndrome; Cell Line; Computer Simulation; Heart Conduction System; Humans; Ion Channel Gating; Kidney; Kinetics; Models, Cardiovascular; Muscle Proteins; Mutation; NAV1.5 Voltage-Gated Sodium Channel; Pre-Excitation Syndromes; Sodium Channels; Structure-Activity Relationship
PubMed: 16877553
DOI: 10.1152/ajpheart.01025.2005 -
Circulation Jan 2005We identified a gene (PRKAG2) that encodes the gamma-2 regulatory subunit of AMP-activated protein kinase (AMPK) with a mutation (Arg302Gln) responsible for familial...
Transgenic mouse model of ventricular preexcitation and atrioventricular reentrant tachycardia induced by an AMP-activated protein kinase loss-of-function mutation responsible for Wolff-Parkinson-White syndrome.
BACKGROUND
We identified a gene (PRKAG2) that encodes the gamma-2 regulatory subunit of AMP-activated protein kinase (AMPK) with a mutation (Arg302Gln) responsible for familial Wolff-Parkinson-White (WPW) syndrome. The human phenotype consists of ventricular preexcitation, conduction abnormalities, and cardiac hypertrophy.
METHODS AND RESULTS
To elucidate the molecular basis for the phenotype, transgenic mice were generated by cardiac-restricted expression of the wild-type (TG(WT)) and mutant(TG(R302Q)) PRKAG2 gene with the cardiac-specific promoter alpha-myosin heavy chain. ECG recordings and intracardiac electrophysiology studies demonstrated the TG(R302Q) mice to have ventricular preexcitation (PR interval 10+/-2 versus 33+/-5 ms in TG(WT), P<0.05) and a prolonged QRS (20+/-5 versus 10+/-1 ms in TG(WT), P<0.05). A distinct AV accessory pathway was confirmed by electrical and pharmacological stimulation and substantiated by induction of orthodromic AV reentrant tachycardia. Enzymatic activity of AMPK in the mutant heart was significantly reduced (0.009+/-0.003 versus 0.025+/-0.001 nmol x min(-1) x g(-1) in nontransgenic mice), presumably owing to the mutation disrupting the AMP binding site. Excessive cardiac glycogen was observed. Hypertrophy was confirmed by increases in heart weight (296 versus 140 mg in TG(WT)) and ventricular wall thickness.
CONCLUSIONS
We have developed a genetic animal model of WPW that expresses a mutation responsible for a familial form of WPW syndrome with a phenotype identical to that of the human, including induction of supraventricular arrhythmia. The defect is due to loss of function of AMPK. Elucidation of the molecular basis should provide insight into development of the cardiac conduction system and accessory pathways.
Topics: AMP-Activated Protein Kinases; Adenosine Monophosphate; Amino Acid Substitution; Animals; Binding Sites; Cardiac Pacing, Artificial; Cardiomegaly; Disease Models, Animal; Heart Conduction System; Heart Ventricles; Humans; Mice; Multienzyme Complexes; Mutagenesis, Site-Directed; Mutation, Missense; Myocardium; Myosin Heavy Chains; Organ Specificity; Phenotype; Point Mutation; Promoter Regions, Genetic; Protein Serine-Threonine Kinases; Tachycardia, Atrioventricular Nodal Reentry; Wolff-Parkinson-White Syndrome
PubMed: 15611370
DOI: 10.1161/01.CIR.0000151291.32974.D5 -
The Western Journal of Emergency... Jul 2016
Topics: Adult; Catheter Ablation; Electrocardiography; Female; Heart Atria; Humans; Tachycardia; Treatment Outcome; Wolff-Parkinson-White Syndrome
PubMed: 27429700
DOI: 10.5811/westjem.2016.4.30323 -
Journal of the American College of... Apr 2016To review the literature systematically to determine whether noninvasive or invasive risk stratification, such as with an electrophysiological study of patients with... (Review)
Review
Risk Stratification for Arrhythmic Events in Patients With Asymptomatic Pre-Excitation: A Systematic Review for the 2015 ACC/AHA/HRS Guideline for the Management of Adult Patients With Supraventricular Tachycardia: A Report of the American College of Cardiology/American Heart Association Task Force...
OBJECTIVE
To review the literature systematically to determine whether noninvasive or invasive risk stratification, such as with an electrophysiological study of patients with asymptomatic pre-excitation, reduces the risk of arrhythmic events and improves patient outcomes.
METHODS
PubMed, EMBASE, and the Cochrane Central Register of Controlled Trials (all January 1, 1970, through August 31, 2014) were searched for randomized controlled trials and cohort studies examining noninvasive or invasive risk stratification in patients with asymptomatic pre-excitation. Studies were rejected for low-quality design or the lack of an outcome, population, intervention, or comparator of interest or if they were written in a language other than English.
RESULTS
Of 778 citations found, 9 studies met all the eligibility criteria and were included in this paper. Of the 9 studies, 1 had a dual design-a randomized controlled trial of ablation versus no ablation in 76 patients and an uncontrolled prospective cohort of 148 additional patients-and 8 were uncontrolled prospective cohort studies (n=1,594). In studies reporting a mean age, the range was 32 to 50 years, and in studies reporting a median age, the range was 19 to 36 years. The majority of patients were male (range, 50% to 74%), and <10% had structural heart disease. In the randomized controlled trial component of the dual-design study, the 5-year Kaplan-Meier estimates of the incidence of arrhythmic events were 7% among patients who underwent ablation and 77% among patients who did not undergo ablation (relative risk reduction: 0.08; 95% confidence interval: 0.02 to 0.33; p<0.001). In the observational cohorts of asymptomatic patients who did not undergo catheter ablation (n=883, with follow-up ranging from 8 to 96 months), regular supraventricular tachycardia or benign atrial fibrillation (shortest RR interval >250 ms) developed in 0% to 16%, malignant atrial fibrillation (shortest RR interval ≤250 ms) in 0% to 9%, and ventricular fibrillation in 0% to 2%, most of whom were children in the last case.
CONCLUSIONS
The existing evidence suggests risk stratification with an electrophysiological study of patients with asymptomatic pre-excitation may be beneficial, along with consideration of accessory-pathway ablation in those deemed to be at high risk of future arrhythmias. Given the limitations of the existing data, well-designed and well-conducted studies are needed.
Topics: Adult; American Heart Association; Arrhythmias, Cardiac; Asymptomatic Diseases; Catheter Ablation; Humans; Observational Studies as Topic; Practice Guidelines as Topic; Pre-Excitation Syndromes; Randomized Controlled Trials as Topic; Risk Assessment; Societies, Medical; United States
PubMed: 26409260
DOI: 10.1016/j.jacc.2015.09.018 -
Kardiologia Polska Jun 2020Several electrocardiographic (ECG) algorithms have been developed for predicting accessory pathway (AP) location in Wolff-Parkinson-White syndrome. However, their...
BACKGROUND
Several electrocardiographic (ECG) algorithms have been developed for predicting accessory pathway (AP) location in Wolff-Parkinson-White syndrome. However, their accuracy may be related to the manifested degree of preexcitation on ECG.
AIMS
Our goal was to assess the effect of the degree of preexcitation on the accuracy of 4 traditional AP localization algorithms and to compare them with the algorithm specifically designed for ECGs with maximal preexcitation (Pambrun) Methods: The study included 300 patients who underwent successful ablation of an overt atrioventricular AP. Resting and maximally preexcited ECGs obtained during incremental atrial pacing were assessed using 4 traditional AP localization algorithms: Xie, d'Avila, Iturralde, and Taguchi. Maximally preexcited ECGs were additionally assessed with the Pambrun algorithm. We compared the precision of the algorithms to predict accurate or anatomically adjacent AP location.
RESULTS
Theoverall accuracy of traditional AP localization algorithms using resting ECG ranged between 26% and 53.7% and improved to a range of 47.3% to 69.7% when adjacent locations were accepted. When used with maximal preexcitation, all algorithms had significantly higher accuracy, with a mean improvement of 14.3 and 15.6 percentage points for precise and adjacent sites, respectively. The Pambrun algorithm for maximally preexcited ECGs had the highest precision for both accurate and adjacent locations of the APs (89.7% and 97%, respectively).
CONCLUSIONS
Greater preexcitation on ECG improved the accuracy of the traditional AP localization algorithms. The algorithm designed to use maximally preexcited ECGs has the best accuracy. Maximally preexcited ECG recordings should preferably be used in clinical practice to facilitate the ablation procedure.
Topics: Accessory Atrioventricular Bundle; Algorithms; Catheter Ablation; Electrocardiography; Humans; Wolff-Parkinson-White Syndrome
PubMed: 32438794
DOI: 10.33963/KP.15378