Wolff-Parkinson-White Apresentado como QRS Alternante e Outros Diagnósticos Diferenciais em uma Grande Coorte de Triagem de ECG Pré-Participação / Wolff-Parkinson-White Presenting as QRS Alternans and Other Differential Diagnoses in a Large Pre-Participation ECG Screening Cohort

Resumo Fundamento A síndrome de Wolff-Parkinson-White (WPW) é uma condição pró-arrítmica que pode exigir restrição de atividades extenuantes e é caracterizada por sinais de ECG, incluindo ondas delta. Observamos casos de padrões intermitentes de WPW apresentando-se como QRS alternante ('WPW alternante') em uma grande coorte de triagem de ECG pré-participação de homens jovens que se candidataram ao recrutamento militar. Objetivos Nosso objetivo foi determinar o padrão de WPW alternante, as características do caso e a prevalência de outros diagnósticos diferenciais relevantes apresentando-se como alternância de QRS em um ambiente de pré-participação. Métodos Cento e vinte e cinco mil cento e cinquenta e oito recrutas militares do sexo masculino prospectivos foram revisados de janeiro de 2016 a dezembro de 2019. Uma revisão de prontuários médicos eletrônicos identificou casos de WPW alternante e padrões ou síndrome de WPW. A revisão de prontuários médicos eletrônicos identificou casos de diagnósticos diferenciais relevantes que podem causar alternância de QRS. Resultados Quatro indivíduos (2,2%) apresentaram WPW alternante em 184 indivíduos com diagnóstico final de padrão ou síndrome de WPW. Dois desses indivíduos manifestaram sintomas ou achados eletrocardiográficos compatíveis com taquicardia supraventricular. A prevalência geral de WPW alternante foi de 0,003%, e a prevalência de WPW foi de 0,147%. As WPW alternantes representaram 8,7% dos indivíduos com QRS alternantes, e QRS alternantes tiveram prevalência de 0,037% em toda a população. Conclusões A WPW alternante é uma variante da WPW intermitente, que compreendeu 2,2% dos casos de WPW em nossa coorte de triagem pré-participação. Não indica necessariamente um baixo risco de taquicardia supraventricular. Deve ser reconhecido na triagem de ECG e distinguido de outras patologias que também apresentam QRS alternantes.

Abstract Background Wolff-Parkinson-White (WPW) syndrome is a proarrhythmic condition that may require restriction from strenuous activities and is characterized by ECG signs, including delta waves. We observed cases of intermittent WPW patterns presenting as QRS alternans ('WPW alternans') in a large pre-participation ECG screening cohort of young men reporting for military conscription. Objectives We aimed to determine the WPW alternans pattern, case characteristics, and the prevalence of other relevant differential diagnoses presenting as QRS alternans in a pre-participation setting. Methods One hundred twenty-five thousand one hundred fifty-eight prospective male military recruits were reviewed from January 2016 to December 2019. A review of electronic medical records identified cases of WPW alternans and WPW patterns or syndrome. Reviewing electronic medical records identified cases of relevant differential diagnoses that might cause QRS alternans. Results Four individuals (2.2%) had WPW alternans out of 184 individuals with a final diagnosis of WPW pattern or syndrome. Two of these individuals manifested symptoms or ECG findings consistent with supraventricular tachycardia. The overall prevalence of WPW alternans was 0.003%, and the prevalence of WPW was 0.147%. WPW alternans represented 8.7% of individuals presenting with QRS alternans, and QRS alternans had a prevalence of 0.037% in the entire population. Conclusions WPW alternans is a variant of intermittent WPW, which comprised 2.2% of WPW cases in our pre-participation screening cohort. It does not necessarily indicate a low risk for supraventricular tachycardia. It must be recognized at ECG screening and distinguished from other pathologies that also present with QRS alternans.

Mahaim Revisited.

The name Ivan Mahaim is well-known to electrophysiologists. However, alternative anatomical substrates can produce the abnormal rhythms initially interpreted on the basis of the pathways he first described. These facts have prompted suggestions that Mahaim should be deprived of his eponym. It is agreed that specificity is required when describing the pathways that produce the disordered cardiac conduction, and that the identified pathways should now be described in an attitudinally appropriate fashion. The authors remain to be convinced that understanding will be enhanced simply by discarding the term 'Mahaim physiology' from the lexicon. It is fascinating to look back at the history of accessory atrioventricular junctional conduction pathways outside the normal accessory atrioventricular conduction system, and their possible role in rhythm disturbances. It took both the anatomist and the clinical arrhythmologist quite some time to understand the complex anatomical architecture and the ensuing electrophysiological properties. Over the years, the name Mahaim was often mentioned in those discussions, although these pathways were not the ones that produced the eponym. The reason for this review, therefore, is to present relevant information about the person and what followed thereafter.

Ventricular pre-excitation in primary care patients: Evaluation of the risk of mortality.

BACKGROUND: Ventricular pre-excitation is characterized by the presence of atrioventricular accessory pathways, predisposing to arrhythmias. Although it is well established that risk stratification in symptomatic patients should be invasive, there is a lack of evidence of the benefit in asymptomatic. OBJECTIVE: Evaluate ventricular pre-excitation in the electrocardiogram (ECG) as a risk factor for overall mortality in patients of Telehealth Network of Minas Gerais (TNMG), Brazil. METHODS: This observational study was developed with the database of digital ECGs (2010-2017) from TNMG. The electronic cohort was obtained by linking data from ECG exams and those from the national mortality information system. Only the first ECG was considered. Clinical data were self-reported, and ECGs were interpreted manually by cardiologists and automatically by the Glasgow University Interpreter software. Hazard ratio (HR) for mortality was estimated using weighted Cox regression. RESULTS: Nearly 1 665 667 patients were included (median age: 50 [Q1: 34; Q3: 63] years; 41.4% were male). In a mean follow-up of 3.7 years, the overall mortality rate was 3.1%. The prevalence of ventricular pre-excitation was 0.07%. In multivariate analysis, adjusting for sex and age, ventricular pre-excitation was not associated with an increased risk of mortality (HR: 1.41; 95% confidence interval [CI]: 0.56-3.57; p = .47) when compared to the whole sample or to patients with normal ECG (HR: 1.41; 95% CI: 0.53-4.36; p = .43). In a subanalysis on accessory pathway location, there was no evidence of a higher risk of death related to any location. CONCLUSION: Ventricular pre-excitation was not associated with an increased risk of mortality in a primary care cohort.

Unusual variants of pre-excitation: From anatomy to ablation: Part III-Clinical presentation, electrophysiologic characteristics, when and how to ablate nodoventricular, nodofascicular, fasciculoventricular pathways, along with considerations of permanent junctional reciprocating tachycardia.

The recognition of the presence, location, and properties of unusual accessory pathways for atrioventricular conduction is an exciting, but frequently a difficult, challenge for the clinical cardiac arrhythmologist. In this third part of our series of reviews, we discuss the different steps required to come to the correct diagnosis and management decision in patients with nodofascicular, nodoventricular, and fasciculo-ventricular pathways. We also discuss the concealed accessory atrioventricular pathways with the properties of decremental retrograde conduction that are associated with the so-called permanent form of junctional reciprocating tachycardia. Careful analysis of the 12-lead electrocardiogram during sinus rhythm and tachycardias should always precede the investigation in the catheterization room. When using programmed electrical stimulation of the heart from different intracardiac locations, combined with activation mapping, it should be possible to localize both the proximal and distal ends of the accessory connections. This, in turn, should then permit the determination of their electrophysiologic properties, providing the answer to the question "are they incorporated in a tachycardia circuit?". It is this information that is essential for decision-making with regard to the need for catheter ablation, and if necessary, its appropriate site.

Part II-Clinical presentation, electrophysiologic characteristics, and when and how to ablate atriofascicular pathways and long and short decrementally conducting accessory pathways.

Recognition of the presence, location, and properties of unusual accessory pathways for atrioventricular conduction is an exciting, frequently difficult, challenge for the clinical cardiac arrhythmologist. In this second part of our series of reviews relative to this topic, we discuss the steps required to achieve the correct diagnosis and appropriate management in patients with the so-called "Mahaim" variants of pre-excitation. We indicate that, nowadays, it is recognized that these abnormal rhythms are manifest because of the presence of atriofascicular pathways. These anatomical substrates, however, need to be distinguished from the other long and short accessory pathways which produce decremental atrioventricular conduction. The atriofascicular pathways, along with the long decrementally conducting pathways, have their atrial components located within the vestibule of the tricuspid valve. The short decremental pathways, in contrast, can originate in the vestibules of either the mitral or tricuspid valves. As a starting point, careful analysis of the 12-lead electrocardiogram, taken during both sinus rhythm and tachycardias, should precede any investigation in the catheterization room. When assessing the patient in the electrophysiological laboratory, the use of programmed electrical stimulation from different intracardiac locations, combined with entrainment technique and activation mapping, should permit the establishment of the properties of the accessory pathways, and localization of its proximal and distal ends. This should provide the answer to the question "is the pathway incorporated into the circuit underlying the clinical tachycardia". That information is essential for decision-making with regard to need, and localization of the proper site, for catheter ablation.

Left ventricular noncompaction and pre-excitation: an unusual finding in adults

Left ventricular noncompaction (LVNC) is a rare form of cardiomyopathy characterized by prominent left ventricular (LV) trabeculae, deep intertrabecular recesses, and the thin compacted layer. The disease is potentially associated with sudden cardiac death due to LV dysfunction and ventricular arrhythmias. The presence of accessory pathway and Wolff-Parkinson-White syndrome is particularly rare in adults. Here we describe the rare association of LVNC and ventricular pre-excitation in an 18-year-old female with neonatal hypoxic brain injury (AU)

Anomalía de Ebstein en edad adulta / Ebstein anomaly in adulthood

La anomalía de Ebstein es una malformación congénita poco frecuente caracterizada por el adosamiento de los elementos de la válvula tricúspide en la cavidad del ventrículo derecho. Este adosamiento produce un desplazamiento del orificio fisiológico de la válvula tricúspide hacia la cavidad del ventrículo derecho. Generalmente se asocia a alteraciones propias de la válvula, como insuficiencia o estenosis tricuspídea, así con el desarrollo de insuficiencia cardiaca y la presencia de arritmias y síndrome de preexcitación. Aunque es un diagnóstico propio del neonato o del niño, se han descrito pacientes diagnosticados en la edad adulta. En estos últimos, la forma de presentación varía, y puede ir desde encontrarse asintomático hasta comenzar con arritmias. Se presenta el caso de una paciente de 70 años de edad que ingresó con el diagnóstico de flutter auricular, se decide cardioversión eléctrica retornando a ritmo sinusal. Al realizarle el ecocardiograma se diagnostica la anomalía de Ebstein. Se revisan las características clínicas, exámenes complementarios y tratamientos de esta entidad, así como la anatomía y embriología del aparato valvular tricuspídeo. El ecocardiograma continúa siendo el examen complementario de elección en esta entidad.

Ebstein anomaly is a non-frequent congenital malformation characterized by enclosing of tricuspid valve elements in right ventricle cavity. This enclosing produces a move of physiologic orifice of tricuspid valve within the right ventricle valve. Generally, it is associated with alterations typical of this valve, as insufficiency or tricuspid stenosis, as well as the development of heart insufficiency and the presence of arrhythmias and pre-excitation syndrome. Although it is a diagnosis characteristic of neonate or the children, has described patients diagnosed in adulthood. In past years, its presentation varies, and may be asymptomatic of start with arrhythmias. This is a female case presentation aged 70 admitted with a diagnosis of atrial flutter, thus the electric cardioversion to return to sinusal rhythm. In echocardiogram it is diagnosed an Ebstein anomaly. Clinical features, complementary examinations and treatment of this entity, as well as the anatomy and embryology of tricuspid valvular apparatus. Echocardiogram is the choice complementary examination in this entity.