Ventricular Pre-excitation Pattern Research Articles

Shortened PQ interval in the differential diagnosis of Anderson-Fabry disease: a case report

In this article, we present a case of a patient with a late diagnosis of Fabry disease caused by a pathogenic variant in the GLA gene (p.1287_1288dup), who repeatedly attempted interventional treatment of Wolff-Parkinson-White Syndrome due to characteristic electrocardiographic pattern of ventricular preexcitation and paroxysmal arrhythmias. The proposed pathognomonic signs of the disease will ensure timely diagnosis and the appointment of specific treatment.

Charge Syndrome Hallmarked with Wolff-Parkinson-White Syndrome and Patent Ductus Arteriosus; 20 Years Post-Repairing; An Extreme Combination

Rationale: CHARGE syndrome or Hall-Hittner syndrome is a pleiotropic disorder, in which the name is derived from the abbreviation epitomizing its six clinical criteria: ocular coloboma, cardiac defects, choanal atresia, growth or developmental retardation, genital hypoplasia, and ear anomalies or deafness. Wolff-Parkinson-White syndrome is the most frequent pattern of ventricular pre-excitation. Patent ductus arteriosus is one of the most frequent congenital heart diseases due to failure of closure of the ductus arteriosus within 72 hours of birth. CHARGE syndrome, Wolff-Parkinson-White syndrome, and patent ductus arteriosus are so difficult to be present in a single entity. Patient concerns: A young female girl patient presented to the physician’s outpatient clinic with acute confusion status with a past repaired patent ductus arteriosus. Diagnosis: CHARGE syndrome hallmarked with Wolff-Parkinson-White syndrome and patent ductus arteriosus; 20 years post-repairing. Interventions: Plain chest x-ray, electrocardiography, oxygenation, and echocardiography. Outcomes: A dramatic clinical improvement post-oxygenation had happened. Lessons: CHARGE syndrome with Wolff-Parkinson-White syndrome and repaired patent ductus arteriosus is an extreme combination. The existence of infantile electrocardiographic Tee-Pee sign of hypocalcemia and adult low ionized calcium with CHARGE syndrome is highly suggestive of associated DiGeorge phenotype syndrome. An absence of tachycardia post- repairing of patent ductus arteriosus from 11 mo until the 20th-year-old is a good prognostic sign. The presence of an infantile T-wave alternance will strengthen both the risk of serious arrhythmia and the efficacy of patent ductus arteriosus repairing.

Ventricular Preexcitation: An Anomalous Wave Interfering with the Ordered Ventricular Activation.

Ventricular preexcitation is a depolarization of the ventricles that occurs before the conventional sequence, and the electrocardiogram is the specific test for diagnosis. A Kent bundle is the paradigm of ventricular preexcitation, and it is associated with short PR, wide QRS and delta wave. This finding is not always very evident, as it can have different degrees of pre-eccitazione; therefore great diagnostic care must be taken in this field. If not properly identified, the pattern of ventricular preexcitation may lead to an incorrect diagnosis. The methodology of precision electrocardiology is able to confront all these aspects.

P1443Accuracy of three algorithms in predicting accessory pathway localization in a pediatric population with ventricular pre-excitation

Abstract BACKGROUND Prediction of accessory pathway location is extremely important before scheduling ablation, even more in paediatric patients. Given the absence in the literature of conclusive and recent studies that established the best algorithm to predict location in these patients, especially after the introduction of 3D mapping systems, we designed a study to compare three previously published algorithms. AIM The aim of this study is to assess and compare the accuracy in predicting accessory pathway location of the algorithm by Arruda, Boersma and Chiang. METHODS This is a multicenter, observational, retrospective clinical study based on the analysis of the resting 12-leads ECGs of children (aged 0-18 years) with ventricular pre-excitation pattern. The study lasts from January 2013 to June 2019. We analyzed the accuracy (defined as percentage of true positive values) of predicted location by each algorithm, which could include one or more than one of 13 regions around mitral and tricuspid annuli. RESULTS Study population was composed by 120 patients, mean age was 12.7 +/- 3.6 years (height 155.6 +/-19.3 cm and weight 48.3 +/- 17.1 kg). The algorithm by Boersma has the highest percentage of predicted pathway locations that are found to be concordant with the site of successful AP ablation (see table). When we corrected for pre-test probability, the algorithm by Arruda makes the possibility of one specific location 8 times higher, the one by Boersma 6.4 times higher and the one by Chiang 6.9 times higher than by chance. CONCLUSIONS The algorithm by Boersma showed the highest accuracy in accessory pathway location, on the other hand the one by Arruda seems to have the highest corrected accuracy in accessory pathway among children. table 1 Arruda Boersma Chiang Number of locations 13 9 13 Total accuracy 0,617 0,717 0,533 Corrected accuracy per locations 8,0158 6,4494 6,929 Abstract Figure. picture 1

Electrocardiographic Diagnosis of Fasciculoventricular Accessory Pathway

A 28-year-old patient with no structural heart disease was referred for electrophysiological study and ablation to treat an electrocardiographically-documented pattern of ventricular preexcitation. The baseline electrocardiogram (Figure 1A) showed sinus rhythm with a short P-R interval, positive delta wave at leads I, II, III, and aVF, and transition at lead V3, which indicated an anteroseptal or midseptal pathway. Three catheters were positioned: for the coronary sinus (CS) (proximal [pCS] and distal [dCS]), His bundle, and right ventricle (RV). Coinciding with the initial acceleration of sinus rhythm, episodes of second-degree (Wenckebach) atrioventricular block were observed (Figure 1B; asterisks mark blocked P waves), with the peculiarity that ventricular preexcitation remained constant in all the ventricular complexes. This occurred even in the escape complexes that followed the blocked P waves, coinciding with the following sinus P wave. These spontaneous phenomena were diagnostic for the presence of a fasciculoventricular pathway. These are uncommon accessory pathways that connect the bundle of His or its branches with the ventricular septal myocardium. The diagnosis of accessory fasciculoventricular pathway usually requires intravenous adenosine administration or atrial pacing maneuvers. The baseline intracardiac recordings confirmed the existence of ventricular preexcitation, with an HV interval of 10 ms (Figure 2A). Thereafter, preexcitation remained the same during progressive prolongation of the P-R interval, and was also observed in the escape beats following the blocked P waves (Figure 2B). Once these accessory pathways have been identified, they do not need to be eliminated because they are not associated with sustained tachycardia. A I

Coexistence of Wolff–Parkinson–White and Brugada Syndrome: Mere Curiosity?

The association between Brugada syndrome (BS) and ventricular preexcitation is a rare condition, with sporadic cases already reported. We report the case of a 29-year-old man, with palpitation unrelated to physical or emotional stress. The electrocardiogram of the first visit revealed a ventricular preexcitation pattern and an end-conduction delay, with negative T wave in V1 and intraventricular conduction disturbance in V2 (atypical for BS). The typical aspect of BS occurred after introduction of propafenone for the prevention of atrioventricular tachycardia. We discuss the recognition of this rare association, the proarrhythmic effects of some drugs, treatment options, and prognosis.

Clinical Outcome and Phenotypic Expression in <emph type="ital">LAMP2</emph> Cardiomyopathy

Mutations in X-linked lysosome-associated membrane protein gene (LAMP2; Danon disease) produce a cardiomyopathy in young patients that clinically mimics severe hypertrophic cardiomyopathy (HCM) due to sarcomere protein mutations. However, the natural history and phenotypic expression of this newly recognized disease is incompletely resolved and its identification may have important clinical implications. To determine the clinical consequences, outcome, and phenotypic expression of LAMP2 cardiomyopathy associated with diagnostic and management strategies. Clinical course and outcome were assessed prospectively in 7 young patients (6 boys) with defined LAMP2 mutations from the time of diagnosis (age 7-17 years; median, 14 years) to October 2008. Phenotypic expression of this disease was assessed both clinically and at autopsy. Progressive heart failure, cardiac death, and transplant. Over a mean (SD) follow-up of 8.6 (2.6) years, and by age 14 to 24 years, the study patients developed left ventricular systolic dysfunction (mean [SD] ejection fraction, 25% [7%]) and cavity enlargement, as well as particularly adverse clinical consequences, including progressive refractory heart failure and death (n = 4), sudden death (n = 1), aborted cardiac arrest (n = 1), or heart transplantation (n = 1). Left ventricular hypertrophy was particularly marked (maximum thickness, 29-65 mm; mean [SD], 44 [15] mm), including 2 patients with massive ventricular septal thickness of 60 mm and 65 mm at ages 23 and 14 years, respectively. In 6 patients, a ventricular pre-excitation pattern at study entry was associated with markedly increased voltages of R-wave or S-wave (15-145 mm; mean [SD], 69 [39] mm), and deeply inverted T-waves. Autopsy findings included a combination of histopathologic features that were consistent with a lysosomal storage disease (ie, clusters of vacuolated myocytes) but also typical of HCM due to sarcomere protein mutations (ie, myocyte disarray, small vessel disease, myocardial scarring). LAMP2 cardiomyopathy is a profound disease process characterized by progressive clinical deterioration leading rapidly to cardiac death in young patients (<25 years). These observations underscore the importance of timely molecular diagnosis for predicting prognosis and early consideration of heart transplantation.

Diagnostic trilemma: Isorhythmic AV dissociation mimicking intermittent preexcitation and acute inferior myocardial infarction

In this case report, we present a patient with intermittent isorhythmic atrioventricular dissociation mimicking two different electrocardiographic entities: ventricular preexcitation pattern and recurrent acute inferior myocardial infarction.

Late cure after radiofrequency catheter ablation in a pediatric patient

A 12-year-old female with the Wolff-Parkinson-White syndrome underwent an electrophysiologic study followed by radiofrequency catheter ablation of the left-lateral accessory pathway. After several unsuccessful attempts, the procedure was stopped because of early recurrence of accessory pathway conduction. Twenty-four hours after the procedure, the patient was found without ventricular pre-excitation pattern on the electrocardiogram. During a 12-month follow-up, the Wolff-Parkinson-White pattern was no longer present. A transesophageal electrophysiologic study showed no further tachycardia induction and the patient is still asymptomatic. This report raises the issue that ablation-induced lesions may evolve considerably during the first few days after ablation, leading to either recurrent accessory pathway conduction or long-lasting complete cure.

Wolff-Parkinson-White syndrome. Identification and management.

Patients with Wolff-Parkinson-White (WPW) pattern of ventricular pre-excitation may develop paroxysmal re-entrant tachyarrhythmias through the Kent bundle and, less commonly, atrial fibrillation. WPW patients are at risk of sudden death when a rapid ventricular response occurs during atrial fibrillation due to conduction through the accessory pathway. Conduction properties of the accessory pathway and atrial vulnerability, which is the propensity to develop atrial fibrillation, are important parameters for evaluation in these patients. The former can be assessed by means of noninvasive tests, such as stress and pharmacological tests, and with electrophysiological study; the latter only by electrophysiological study. There is no indication for treatment of asymptomatic patients. Antiarrhythmic prophylaxis is required in patients with previous episodes of atrial fibrillation with rapid ventricular response, in patients with paroxysmal re-entrant tachycardias and rapid conduction through the accessory pathway, and in patients with frequent episodes of re-entrant tachycardias of long duration. Vaughan-Williams class IC anti-arrhythmic drugs (propafenone, flecainide) are the first choice for drugs in patients with rapid anterograde conduction through the accessory pathway due to their high efficacy and low incidence of adverse effects, while beta-blockers (atenolol, nadolol) are indicated for patients with re-entrant tachycardias and low conduction capacity through the bypass tract. When pharmacological therapy is ineffective, surgical or catheter ablation of the accessory pathway may be considered.