Wolff-Parkinson-White Syndrome Publications (5968)
Wolff-Parkinson-White Syndrome Publications
the other (watchful waiting with close monitoring). The most recent pediatric and adult guidelines reflect this ambiguity with a broad spectrum of approaches endorsed.
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.
262C > T; p.[Arg88*]) identified using whole-exome sequencing (WES) of a family trio. An identical variant has been previously reported in association with MLS syndrome. The case we describe here lacked the diagnostic features of MLS syndrome, but a detailed clinical comparison of the two cases revealed significant phenotypic overlap. Heterozygous variants in HCCS (which encodes an important mitochondrially targeted protein) and COX7B, which, like NDUFB11, encodes a protein of the MRC, have also previously been identified in MLS syndrome including a case with features of both MLS syndrome and histiocytoid CM. However, a systematic review of WES data from previously published histiocytoid CM cases, alongside four additional cases presented here for the first time, did not identify any variants in these genes. We conclude that NDUFB11 variants play a role in the pathogenesis of both histiocytoid CM and MLS and that these disorders are allelic (genetically related).
2352 Olympic athletes, mean age 25±6, 64% men, competing in 31 summer or 15 winter sports, were examined with history, physical examination, 12-lead and exercise ECG and echocardiography. Additional testing (cardiac MRI, CT scan) or electrophysiological assessments were selectively performed when indicated.
Prevalence and type of CV findings, abnormalities and diseases found in Olympic athletes over 10 years.
A subset of 92 athletes (3.9%) showed abnormal CV findings. Structural abnormalities included inherited cardiomyopathies (n=4), coronary artery disease (n=1), perimyocarditis (n=4), myocardial bridges (n=2), valvular and congenital diseases (n=45) and systemic hypertension (n=10). Primary electrical diseases included atrial fibrillation (n=2), supraventricular reciprocating tachycardia (n=14), complex ventricular tachyarrhythmias (non-sustained ventricular tachycardia, n=7; bidirectional ventricular tachycardia, n=1) or major conduction disorders (Wolff-Parkinson-White (WPW), n=1; Long QT syndrome (LQTS), n=2).
Our study revealed an unexpected prevalence of CV abnormalities among Olympic athletes, including a small, but not negligible proportion of pathological conditions at risk. This observation suggests that Olympic athletes, despite the absence of symptoms or astonishing performances, are not immune from CV disorders and might be exposed to unforeseen high-risk during sport activity.
Integrating RNA sequencing with metabolomics, PRKAG2 mutations that activate AMPK remodeled global metabolism by regulating RNA transcripts to favor glycogen storage and oxidative metabolism instead of glycolysis. As in patients with PRKAG2 cardiomyopathy, iPS cell and mouse models are protected from cardiac fibrosis, and we define a crosstalk between AMPK and post-transcriptional regulation of TGFβ isoform signaling that has implications in fibrotic forms of cardiomyopathy. Our results establish critical connections among metabolic sensing, myocyte survival, and TGFβ signaling.
Several potential etiologies will present with nonspecific and overlapping signs and symptoms, and the diagnosis often is not evident at the time of ED assessment.
We present the case of a neonate in shock, with a variety of nonspecific signs and symptoms who was ultimately diagnosed with tachycardia-induced cardiomyopathy secondary to a resolved dysrhythmia. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: This case highlights the diagnostic and therapeutic approach to the critically ill neonate in the ED, and expands the differential diagnosis beyond sepsis and critical congenital heart disease. Knowledge of the potential life-threatening etiologies of shock in this population allows the Emergency Physician to appropriately test for, and empirically treat, several potential etiologies simultaneously. Additionally, we discuss the diagnosis and management of supraventricular tachycardia and Wolff-Parkinson-White syndrome in the neonatal and pediatric population, which is essential knowledge for an Emergency Physician.
The group consisted of 22 patients (female 40.9%, mean age 33.6 ± 19.1 years). The follow-up utilized electrocardiogram and Holter monitoring.
Twenty-two patients had 33 accessory pathways (8 patients had multiple APs, 11 patients broad AP). Twenty-nine different arrhythmias were ablated: 20 orthodromic atrioventricular reciprocating tachycardia (O-AVRT), 5 antidromic atrioventricular reciprocating tachycardia (A-AVRT), 3 slow/fast atrioventricular nodal reentry tachycardia (s/f AVNRT) and 1 cavotricuspid-isthmus-dependent atrial flutter (CTI-AFL). In 3 patients (13.6%) multiple ablation targets for RFCA ablation were observed. The acute procedural success rate after the first RFCA performed was: 100% for AVNRT, 77.3% for APs and 50.0% for CTI-AFL ablation. Follow-up (mean 95.7 ± 49.8 months) was completed in 86.4% of patients. One patient had paroxysmal atrial fibrillation not targeted during ablation. One patient died due to heart failure 12 years after RFCA. Three patients who underwent RFCA of accessory pathways in the mid-1990s were lost in follow-up.
Radiofrequency ablation in patients with EA is challenging but safe and have a high short-term as well as long-term success rate.
This type of tachycardia may often be terminated by vagal maneuvers. Although the clinical presentation of AVRT is quite similar to AV-nodal reentrant tachycardias, the correct diagnosis is often facilitated by analyzing a standard 12-lead ECG at normal heart rate showing ventricular preexcitation. Curative catheter ablation of the AP represents the therapy of choice in symptomatic patients. This article is the fourth part of a series written to improve the professional education of young electrophysiologists. It explains pathophysiology, symptoms, and electrophysiological findings of an invasive EP study. It focusses on mapping and ablation of accessory pathways.