A rare complication after radiofrequency catheter ablation in an adolescent case: skin burn.

Radiofrequency catheter ablation is a preferred treatment method for cardiac arrhythmias in children due to its high success rate and low complication risk. We present an adolescent patient who underwent radiofrequency catheter ablation for Wolff-Parkinson-White syndrome and developed a skin burn at the site of the electrode patch. Skin burns can catastrophic consequences, especially in patients with life-threatening arrhythmias; therefore, clinicians should be aware of this complication.

Cryoablation of para-Hisian and mid-septal accessory pathways: long-term outcomes of a specific stepwise cryoablation protocol.

BACKGROUND: Although cryoablation (CA) of septally located accessory pathways (APs) is an established treatment for Wolff-Parkinson-White Syndrome, its major limitation is the lack of data regarding long-term follow-up (FU). The present study sought to investigate long-term outcomes of a specific CA protocol targeting para-Hisian (P-H) and mid-septal (M-S) APs. METHODS: Twenty-six patients who previously underwent CA of PH or MS APs from 2004 to 2014, were prospectively considered to receive a FU during 2021. All subjects received an outpatient control visit, performing an exercise stress test and a 24-h ECG Holter monitoring. RESULTS: Acute success was achieved in 22 patients (85%). One case of recurrence was reported at short-term FU. Long-term FU, performed after a mean time of 150±37 months, did not show ventricular preexcitation recurrences, with a success rate of 81%, and without late adverse events. Symptoms reduction (12% vs. 96%, P<.001) and lower rates of antiarrhythmic drug use (12% vs. 62%, P<.001) were observed at long term-FU with respect to baseline. This clinical outcome was detected also among patients who underwent unsuccessful CA at baseline. CONCLUSIONS: Our CA protocol confirmed remarkable safety and efficacy throughout a long-term FU. Significant clinical improvement in terms of antiarrhythmic therapy discontinuation and symptoms reduction was also shown among patients who experienced acute failure of CA.

Wolff-Parkinson-White Syndrome after Fontan-Bjork operation and its Successful Ablation from Coronary Sinus.

Wolff-Parkinson-White (WPW) syndrome causes paroxysmal supraventricular tachycardia in which short PR intervals and delta waves are seen in electrocardiography, which may cause sudden cardiac death. A 19-year female presented with increasing episodes of wide and narrow QRS complex tachycardia for the past 5 years. She had tricuspid atresia and Fontan Bjork operation in her past history. She was then diagnosed with narrow QRS complex tachycardia; and WPW syndrome was discovered when she returned to sinus rhythm. Ablation was performed from the coronary sinus ostium region via the left subclavian vein. Fontan Bjork procedure leading to accessory connections stemming from the surgery in the atrio-infundibular anastomosis may be one reason for WPW syndrome. In this case, since the ablation area was close to the Fontan anastomotic line, it could not be determined clearly whether WPW syndrome was secondary to Fontan anastomosis or it was congenital occult WPW syndrome, which became overt following Fontan surgery.

Clinical Findings and Prognosis of Danon Disease. An Analysis of the Spanish Multicenter Danon Registry.

INTRODUCTION AND OBJECTIVES: Danon disease (DD) is caused by mutations in the LAMP2 gene. It is considered a multisystemic disease characterized by hypertrophic cardiomyopathy with pre-excitation and extreme hypertrophy, intellectual disability, myopathy, childhood presentation, and worse prognosis in men. There are scarce data on the clinical characteristics and prognosis of DD. METHODS: We analyzed the clinical records of patients with DD from 10 Spanish hospitals. RESULTS: Twenty-seven patients were included (mean age, 31 ± 19 years; 78% women). Male patients showed a high prevalence of extracardiac manifestations: myopathy (80%), learning disorders (83%), and visual alterations (60%), which were uncommon findings in women (5%, 0%, and 27%, respectively). Although hypertrophic cardiomyopathy was the most common form of heart disease (61%), the mean maximum wall thickness was 15 ± 7 mm and dilated cardiomyopathy was present in 12 patients (10 women). Pre-excitation was found in only 11 patients (49%). Age at presentation was older than 20 years in 16 patients (65%). After a median follow-up of 4 years (interquartile range, 2-9), 4 men (67%) and 9 women (43%) died or required a transplant. Cardiac disease and adverse events occurred later in women (37 ± 9 vs 23 ± 16 and 36 ± 20 vs 20 ± 11 years, respectively). CONCLUSIONS: The clinical characteristics of DD differ substantially from traditional descriptions: age at presentation of DD is older, the disease is not multisystemic in women, and pre-excitation is infrequent.

Drug-Induced Loss of Preexcitation in Pediatric Patients with WPW Pattern During Electrophysiologic Study.

Ablation of accessory pathways (AP) is one of the most often performed procedures in pediatric electrophysiology. In pediatric patients these procedures are mostly performed in anaesthesia or sedation. In some of these patients who are referred for electrophysiologic (EP) study, we could observe disappearance of the preexcitation, i.e. antegrade conduction of an AP during introduction of sedation. As a suppression of AP conduction capacities has been reported as negative side effect of propofol and other anaesthetics, the aim of this study was to evaluate risk factors for drug-induced suppression of AP conduction properties. Consecutive, pediatric patients with Wolff-Parkinson-White (WPW) pattern referred for EP study in the period of 2016-2017 were reviewed in retrospect. Patients with complex congenital heart disease were excluded. An entire chart review including ECG, bicycle stress testing, and periprocedural data was performed. In 4 of 37 patients included into the study, loss of preexcitation could be observed during sedation. Data analysis showed weaker conduction capacities of the AP as a risk factor (p = 0.009). Interestingly, absolute (p = 0.11) or adjusted to body weight (p = 0.92) drug doses were not a relevant risk factor. Patients with WPW and weaker conduction capacities of the AP, as implied by an early disappearance of preexcitation during exercise stress testing, seem to be more prone to drug-induced suppression of an AP.

Wolff-Parkinson-White syndrome presenting with steroid-induced bradycardia in a patient with acute rheumatic fever.

Steroids are used in the treatment of acute rheumatic fever with moderate-to-severe carditis. Corticosteroids have several cardiovascular side affects that are more common in adults than in children. Corticosteroid-related bradycardia is a rarely seen side effect. Children with bradycardia following oral corticosteroid use are rarely reported previously. We present a child who developed bradycardia after oral corticosteroid treatment and concurrent Wolff-Parkinson-White pattern.

Molecular Pathogenesis of Familial Wolff-Parkinson-White Syndrome.

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.

Long term risk of Wolff-Parkinson-White pattern and syndrome.

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. the other (watchful waiting with close monitoring). The most recent pediatric and adult guidelines reflect this ambiguity with a broad spectrum of approaches endorsed.

Exome analysis of a family with Wolff-Parkinson-White syndrome identifies a novel disease locus.

Wolff-Parkinson-White (WPW) syndrome is a common cause of supraventricular tachycardia that carries a risk of sudden cardiac death. To date, mutations in only one gene, PRKAG2, which encodes the 5'-AMP-activated protein kinase subunit γ-2, have been identified as causative for WPW. DNA samples from five members of a family with WPW were analyzed by exome sequencing. We applied recently designed prioritization strategies (VAAST/pedigree VAAST) coupled with an ontology-based algorithm (Phevor) that reduced the number of potentially damaging variants to 10: a variant in KCNE2 previously associated with Long QT syndrome was also identified. Of these 11 variants, only MYH6 p.E1885K segregated with the WPW phenotype in all affected individuals and was absent in 10 unaffected family members. This variant was predicted to be damaging by in silico methods and is not present in the 1,000 genome and NHLBI exome sequencing project databases. Screening of a replication cohort of 47 unrelated WPW patients did not identify other likely causative variants in PRKAG2 or MYH6. MYH6 variants have been identified in patients with atrial septal defects, cardiomyopathies, and sick sinus syndrome. Our data highlight the pleiotropic nature of phenotypes associated with defects in this gene.

Two-dimensional speckle tracking imaging as a novel strategy for accurately identifying the accessory pathway.

A 14-year-old patient was diagnosed with hypertrophic cardiomyopathy associated with Wolff-Parkinson-White syndrome. The two-dimensional speckle tracking strain method showed normal left ventricular local contraction, but the peak systolic longitudinal strain of the right ventricular (RV) anterior wall was earlier than that of the septal wall. As expected, the location of the accessory pathway was at the RV anterior wall. The patient's RV local contraction was normalized by successful radiofrequency application.

Electroconvulsive therapy-induced Wolff-Parkinson-White syndrome: a case report.

OBJECTIVE: Wolff-Parkinson-White (WPW) syndrome is characterized by premature ventricular excitation due to the presence of an abnormal accessory pathway. Electrocardiography (ECG) of patients with WPW syndrome portrays a short PR interval and a wide QRS interval with a delta wave. METHODS: Herein, we report the case of a patient with schizophrenia who developed a wide QRS interval with a delta wave immediately following electroconvulsive therapy (ECT). RESULTS: Initially, the delta wave disappeared within 2 days after ECT. However, the duration of the delta wave increased exponentially to 4 months when ECT was repeated. CONCLUSION: Although the patient's cardiocirculatory dynamics remained normal, we continued to monitor her ECG until the delta wave disappeared because WPW syndrome can lead to serious arrhythmia.