Pre-excitation cardiac problems in children: recognition and treatment.

The prevalence of ventricular pre-excitation is 0.07-0.2% in the pediatric population. Kent bundle is the most common atrioventricular accessory pathway and Mahaim fiber is relatively rare. Approximately, 30-60% of children with ventricular pre-excitation have onset of atrioventricular reentrant tachycardia. Persistent atrioventricular reentrant tachycardia can lead to tachycardiomyopathy. The anterograde conduction of right accessory pathway might lead to ventricular systolic dyssynchrony which might result in cardiac dysfunction even in patients with no tachycardia onset. This type of dilated cardiomyopathy was named as accessory pathway-induced dilated cardiomyopathy. Antiarrhythmic drugs can be used to acutely terminate tachycardia or taken orally to decrease tachycardia recurrence in the long term. However, antiarrhythmic drugs that can be chosen for children are quite limited. Sotalol has become a new choice. With the maturation of radiofrequency catheter ablation technique, progress in three-dimensional electro-anatomic mapping, use of cryoablation, and accumulation of experience in children with small age and weight, catheter ablation has become the first choice for children with pre-excitation syndrome.Conclusion: For ventricular pre-excitation co-exists with dilated cardiomyopathy, differential diagnosis of tachycardiomyopathy or accessory pathway-induced dilated cardiomyopathy should be considered. Catheter ablation (radiofrequency and cryoablation) is a relatively safe and effective treatment option and has become the first choice to treat children with ventricular pre-excitation. What is Known: • Persistent atrioventricular reentrant tachycardia in children can lead to tachycardiomyopathy; • Antiarrhythmic drugs that can be chosen for children are quite limited. What is New: • The anterograde conduction of right accessory pathway (not related to supraventricular tachycardia) might lead to accessory pathway-induced dilated cardiomyopathy. • Catheter ablation (including radiofrequency and cryoablation) has become the first choice for children with pre-excitation syndrome.

Pulseless Electric Activity with Pre-Excitation.

A 41-year-old man developed cardiac arrest. A resting 12-lead electrocardiogram showed a delta wave, suggestive of preexcitation syndrome. An electrophysiological test revealed the existence of inducible atrial fibrillation and a fasciculoventricular accessory pathway (FVAP). After these examinations, idiopathic ventricular arrhythmia was suspected. For evaluating concealed Brugada syndrome, pilsicainide was administered, which diminished the delta wave and no Brugada-like electrocardiogram was observed. Ventricular double extra-stimulation from the RV apex easily induced VF, which could not be defibrillated by an external defibrillator, and later stopped spontaneously. These results established the diagnosis of FVAP and idiopathic VF, and not pre-excited atrial fibrillation or Brugada syndrome.

[Preexcitation Syndromes in Children and Adolescents].

Preexcitation syndromes are becoming a more often detected disease in the pediatric population. Their essential feature is the presence of additional paths / pathways in the heart, through which the impulse is conducted faster than physiologically. This leads to faster ventricular excitation and may lead to creating dangerous arrhythmias. The most common preexcitation syndrome is Wolff - Parkinson-White syndrome, which affects 2/ 1000 people. The presence of an accessory pathway could result in serious consequences, ranging from supraventricular tachycardia to sudden cardiac death. There are both invasive and non-invasive methods of diagnosing preexcitation syndromes. Therapeutic treatment includes pharmacotherapy and ablation, which makes it possible to permanently remove the cause of the arrhythmia.

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 on Clinical Practice Guidelines and the Heart Rhythm Society.

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=1594). 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.

Application of the 2015 ACC/AHA/HRS guidelines for risk stratification for sudden death in adult patients with asymptomatic pre-excitation.

The management of the asymptomatic pre-excited patient largely hinges on risk stratification and individual patient considerations and choice. A high threshold to treat patients may lead to a small overall risk of death while a low threshold clearly leads to increased invasive testing and ablation with associated cost and procedural risk. A firm recommendation to uniformly assess all by electrophysiology study or, alternatively, reassure all is inappropriate and unjustified by data as reflected in the recent guideline recommendations. The use of noninvasive and invasive parameters to identify the potentially at-risk individual with surveillance for symptoms in those comfortable with this approach or ablation for those choosing this alternative for individual reasons remains the cornerstone of best practice.

[Historical developments in the diagnosis and treatment of pre-excitation syndromes (WPW)]. / Historische Entwicklung in Diagnostik und Therapie bei Präexzitationssyndromen (WPW).

In 1930, Wolff, Parkinson and White described the syndrome that bears their names. The mechanisms of supraventricular tachycardias were analyzed by brilliant electrocardiography interpretation by Pick and Langendorf. Wellens and Durrer using electrophysiologic studies analyzed the tachycardia mechanism invasively. In Germany the group by Seipel and Breithardt as well as Neuss and Schlepper studied the tachycardia mechanisms and response to antiarrhythmic drugs invasively by electrophysiological studies. Following the first successful interruption of an accessory pathway by Sealy in 1967, surgeons and electrophysiologists cooperated in Germany. Two centers, Hannover and Düsseldorf were established. Direct current (DC) ablation of accessory pathways was introduced by Morady and Scheinman. Because of side effects induced by barotrauma of DC, alternative strategies were studied. In 1987, radiofrequency ablation was introduced and thereafter established as curative therapy of accessory pathways in all locations.

Radiofrequency ablation for pre-excitation syndromes and AV nodal re-entrant tachycardia.

Radiofrequency catheter ablation for supraventricular tachycardia was introduced in 1990. Since then it has become the standard for definitive treatment of pre-excitation syndromes and atrioventricular re-entrant tachycardia. In general, catheter ablation of supraventricular tachycardia results in improved outcomes compared to pharmacologic treatment. Over 95% of patients will be successfully treated with catheter ablation with less than a 5% chance of recurrence and <1% risk of major complications.

[Asymptomatic ventricular pre-excitation: what management?] / Pre-eccitazione ventricolare in assenza di sintomi: che strada percorrere?

The management of asymptomatic patients with ventricular pre-excitation diagnosed occasionally is controversial. In fact, the lack of clinical arrhythmias does not necessarily define a benign condition: it could be possibly due to poor conduction over the accessory pathway or, conversely, to peculiar and individual conditions, which, even if the accessory pathway is capable of fast conduction, can prevent the onset of arrhythmias. These can occur unexpectedly during follow-up and may include malignant ventricular arrhythmias, although sudden death is very rare in this clinical scenario. An aggressive strategy aiming at extensive ablation in all cases with asymptomatic ventricular pre-excitation is not justified, as well as the "wait-and-see" approach. Clinically, it is important to accurately define the individual risk of any arrhythmia related to the accessory pathway, which may require treatment. For decades, the management of asymptomatic ventricular pre-excitation has been quite inhomogeneous among centers and in some cases it is still very different. Recently, a consensus document proposed the combined use of non-invasive and invasive diagnostic tools for accurate screening of these patients. If non-invasive methodologies are unable to demonstrate poor conduction over the accessory pathway, then an invasive approach is justified for arrhythmia risk definition and, if necessary, adequate therapy.

Sports Eligibility After Risk Assessment and Treatment in Children with Asymptomatic Ventricular Pre-excitation.

BACKGROUND AND OBJECTIVE: Many studies concern the management of young patients with symptomatic Wolff-Parkinson-White (WPW) syndrome, but little information exists on the significance and prognosis of ventricular pre-excitation (VPE) in asymptomatic children. The aim of the study was to evaluate the risk of sudden death in young athletes with asymptomatic VPE by transesophageal electrophysiological study (TEEPS) and their sports eligibility after the risk assessment and/or ablative treatment. METHODS: Ninety-one asymptomatic children and adolescents underwent TEEPS both at rest and during adrenergic stress (exercise testing or isoproterenol infusion). After electrophysiological testing, patients were assessed in the 36 months of follow-up. RESULTS: Thirty-three patients (36.3 %) had a benign form of VPE and were allowed to participate in competitions. Ten patients (11 %) were at borderline risk; thus, sport eligibility was evaluated individually. Forty-eight patients (52.7 %) showed inducible sustained atrioventricular reentrant tachycardia and/or atrial fibrillation (AF), 11 of whom (12.1 % of total population) had a potential risk of sudden cardiac death due to AF inducibility during physical stress. Forty-five young athletes underwent transcatheter ablation (TCA). TCA was interrupted in 12 patients (26.7 %) because of the high procedural risk linked to septal accessory pathway (AP) location. There were no TCA-related complications, and all patients remained asymptomatic during follow-up. CONCLUSION: Most of the young athletes with asymptomatic VPE may be allowed to participate in competitive sports after an adequate risk assessment and/or ablative treatment. However, in our opinion, special care should be taken to avoid procedural complications, which are unacceptable in asymptomatic patients.

Influence of advancing age on clinical presentation, treatment efficacy and safety, and long-term outcome of pre-excitation syndromes: a retrospective cohort study of 961 patients included over a 25-year period.

OBJECTIVES: There are very little data on pre-excitation syndrome (PS) in the elderly. We investigated the influence of advancing age on clinical presentation, treatment and long-term outcome of PS. SETTING: Single-centre retrospective study of patient files. PARTICIPANTS: In all, 961 patients (72 patients ≥60 years (mean 68.5±6), 889 patients <60 years (mean 30.5±14)) referred for overt pre-excitation and indication for electrophysiological study (EPS) were followed for 5.3±5 years. Usual care included 24 h Holter monitoring, echocardiography and EPS. Patients underwent accessory pathway (AP) ablation if necessary. PRIMARY AND SECONDARY OUTCOME MEASURES: Occurrence of atrial fibrillation (AF) or procedure-induced adverse event. RESULTS: Electrophysiological data and recourse to AP ablation (43% vs 48.5%, p=0.375) did not significantly differ between the groups. Older patients more often had symptomatic forms (81% vs 63%, p=0.003), history of spontaneous AF (8% vs 3%, p=0.01) or adverse presentation (poorly tolerated arrhythmias: 18% vs 7%, p=0.0009). In multivariable analysis, patients ≥60 years had a significantly higher risk of history of AF (OR=4.2, 2.1 to 8.3, p=0.001) and poorly tolerated arrhythmias (OR=3.8, 1.8 to 8.1, p=0.001). Age ≥60 years was associated with an increased major AP ablation complication risk (10% vs 1.9%, p=0.006). During follow-up, occurrence of AF (13.9% vs 3.6%, p<0.001) and incidence of poorly tolerated tachycardia (4.2% vs 0.6%, p=0.001) were more frequent in patients ≥60 years, although frequency of ablation failure or recurrence was similar (20% vs 15.5%, p=0.52). In multivariable analysis, patients ≥60 years had a significantly higher risk of AF (OR=2.9, 1.2 to 6.8, p≤0.01). CONCLUSIONS: In this retrospective monocentre study, patients ≥60 years referred for PS work up appeared at higher risk of AF and adverse presentation, both prior and after the work up. These results suggest that, in elderly patients, the decision for EPS and AP ablation should be discussed in light of their suspected higher risk of events and ablation complications. However, these findings should be further validated in future prospective multicentre studies.

A functional approach to the preexcitation syndromes.

Electrophysiologic studies have been used to elicit the mechanisms of the preexcitation syndromes and have become a therapeutic tool over the past decade. A thorough understanding of the physiology and anatomy of accessory pathways that are responsible for preexcitation and the associated arrhythmias is necessary before considering the various forms of intervention. The approach to patients with preexcitation syndromes is discussed, with an emphasis on the functional properties of accessory pathways and the associated arrhythmias.

Left atrial-coronary sinus dissociation following an attempt at radiofrequency ablation for a left lateral accessory pathway.

Coronary sinus electrograms generally represent the sequence of left atrial activation, and are very helpful in localizing and differentiating left lateral accessory pathway-mediated tachycardia from other supraventricular tachycardias. The activation of the coronary sinus from the left atrium occurs through muscle bridges, which may be discrete or form an intermingled continuum. These muscle bridges, if disconnected, may dissociate the coronary sinus from the left atrium, in which case the coronary sinus electrograms do not represent left atrial activation, and do not help to understand, or may cause misinterpretation of, the mechanism of supraventricular tachycardia. We report one such case of orthodromic supraventricular tachycardia mediated through the left lateral accessory pathway in which the coronary sinus got dissociated from the left atrium during radiofrequency ablation.