Wide complex tachycardia in a patient with non-ischemic cardiomyopathy: What is the diagnosis?

A 55-year-old male presented with acute heart failure and incessant wide complex tachycardia resembling an outflow tract ventricular tachycardia. Meticulous analysis of the electrocardiograms established the diagnosis of pre-excitation with prolonged atrio-ventricular (A-V) conduction over a decrementally conducting accessory A-V pathway. "Linking" between the accessory A-V pathway and normal A-V conduction system resulted in sustained maximal pre-excitation as well as periodic transition to normal A-V conduction without appreciable change in heart rate. Successful radiofrequency ablation of this unusual accessory A-V pathway was performed at the aortic-mitral junction. This ameliorated the mechanical dysynchrony, allowed discontinuation of hemodynamic/inotropic support, and resulted in sustained symptomatic improvement.

Comparison of Outcomes of Catheter Ablation in Asymptomatic Versus Symptomatic Preexcitation to Guidelines and Beyond.

Management of asymptomatic subjects with preexcitation remains controversial. Our objective was to analyze the reasons an electrophysiological study (EPS) was performed in an asymptomatic population referred for the procedure, and compare the results of catheter ablation between asymptomatic and symptomatic patients. Patients ≥18 years of age with preexcitation referred for an EPS and ablation were grouped as either symptomatic or asymptomatic. We analyzed in both subsets for (1) reasons for the procedure, (2) EPS results (anterograde effective refractory period of the accessory pathway, tachycardia/atrial fibrillation inducibility, anatomical localization), (3) success of the procedure, and (4) incidence of complications. We included 175 patients, 121 of which were symptomatic (39 ± 16 years) and 54 were asymptomatic (35 ± 14 years, p = NS not significant). The most frequent symptoms were palpitations (87%) and syncope (7%). EPS was performed in 44 of 54 asymptomatic patients mainly because of involvement in sports (60%) or high-risk employment (14%). Anterograde effective refractory period was significantly longer in asymptomatic patients (314 ± 55 milliseconds) than in symptomatic patients (278 ± 46 milliseconds; p <0.001). Orthodromic tachycardia inducibility was significantly higher in symptomatic than in asymptomatic patients (69% and 27%, respectively; p <0.001). A total of 170 accessory pathways (49% left free wall, 12% right free wall, 39% septal) were observed without significant differences in the anatomical location between groups. Catheter ablation was attempted in all patients, succeeding in 98% of symptomatic and 95% of asymptomatic patients, without major complications in either group. In conclusion, the reasons for invasive evaluation of asymptomatic patients with preexcitation may be outside the scope of current guidelines. Catheter ablation produces excellent results without major complications.

Conduction Properties and Ablation of Adenosine Sensitive Accessory Pathways in Children.

Block in accessory pathway (AP) conduction with adenosine has been previously described. However, conduction characteristics of these APs has not been well defined to date. All patients with APs

Fasciculoventricular and atrioventricular accessory pathways in patients with Danon disease and preexcitation: A multicenter experience.

BACKGROUND: Studies have suggested that a fasciculoventricular pathway (FVP) may be the cause of preexcitation in patients with Danon disease, a rare X-linked dominant genetic disorder of hypertrophic cardiomyopathy. OBJECTIVE: The purpose of this study was to describe the prevalence of ventricular preexcitation on resting 12-lead electrocardiogram (ECG) in patients with Danon disease and the electrophysiological study (EPS) results of those with preexcitation. METHODS: Patients with confirmed Danon disease diagnosed with preexcitation (PR ≤120 ms, delta wave, QRS >110 ms) on ECG were included from a multicenter registry. The incidence of arrhythmias, implantable cardioverter-defibrillator (ICD) procedures, ICD shocks, and EPS results were collected. RESULTS: Thirteen of 40 patients (32.5%) with Danon disease were found to have preexcitation (mean age 17.3 years; 38% women). EPS performed in 9 of 13 patients (69%) demonstrated FVP only in 2 (22.2%), extranodal pathway without exclusion of FVP in 2 (22.2%), and both FVP and extranodal pathway in 5 (55.6%). Two patients had malignant accessory pathway (AP) properties. Over median follow-up of 842 days (interquartile range 138-1678), 11 patients (85%) had ICD placement, and 6 (46.1%) underwent heart transplantation. No patients required therapy for ventricular tachycardia, and 2 patients (15%) had paroxysmal atrial fibrillation. CONCLUSION: In a large multicenter cohort of patients with Danon disease, there was a high prevalence of FVP and extranodal pathways diagnosed on EPS in those with preexcitation. These findings suggest patients with preexcitation and Danon disease should undergo EPS to assess for FVP and potentially malignant extranodal AP.

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.

Sudden Cardiac Death in Patients with Ventricular Preexcitation.

Patients with the Wolff-Parkinson-White syndrome may experience benign and malignant arrhythmias, the most common being atrioventricular reentrant tachycardias. This arrhythmia may degenerate into atrial fibrillation, which can be conducted over an accessory pathway capable of exceptionally fast conduction to the ventricles and degenerate into ventricular fibrillation, leading to sudden cardiac death. These life-threatening events generally affect symptomatic patients in their third or fourth decade. Although rare, ventricular fibrillation may be the first clinical manifestation in subjects who are asymptomatic or unaware of their conditions. Electrophysiologic study may be useful to identify subjects at high risk of sudden cardiac death.

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.

Variable Presentations and Ablation Sites for Manifest Nodoventricular/Nodofascicular Fibers.

BACKGROUND: Nodofascicular and nodoventricular (NFV) accessory pathways connect the atrioventricular node and the Purkinje system or ventricular myocardium, respectively. Concealed NFV pathways participate as the retrograde limb of supraventricular tachycardia (SVT). Manifest NFV pathways can comprise the anterograde limb of wide-complex SVT but are quite rare. The purpose of this report is to highlight the electrophysiological properties and sites of ablation for manifest NFV pathways. METHODS: Eight patients underwent electrophysiology studies for wide-complex tachycardia (3), for narrow-complex tachycardia (1), and preexcitation (4). RESULTS: NFV was an integral part of the SVT circuit in 3 patients. Cases 1 to 2 were wide-complex tachycardia because of manifest NFV SVT. Case 3 was a bidirectional NFV that conducted retrograde during concealed NFV SVT and anterograde causing preexcitation during atrial pacing. NFV was a bystander during atrioventricular node re-entrant tachycardia, atrial fibrillation, atrial flutter, and orthodromic atrioventricular re-entrant tachycardia in 4 cases and caused only preexcitation in 1. Successful NFV ablation was achieved empirically in the slow pathway region in 1 case. In 5 cases, the ventricular insertion was mapped to the slow pathway region (2 cases) or septal right ventricle (3 cases). The NFV was not mapped in cases 5 and 7 because of its bystander role. QRS morphology of preexcitation predicted the right ventricle insertion sites in 4 of the 5 cases in which it was mapped. During follow-up, 1 patient noted recurrent palpitations but no documented SVT. CONCLUSIONS: Manifest NFV may be critical for wide-complex tachycardia/manifest NFV SVT, act as the retrograde limb for narrow-complex tachycardia/concealed NFV SVT, or cause bystander preexcitation. Ablation should initially target the slow pathway region, with mapping of the right ventricle insertion site if slow pathway ablation is not successful. The QRS morphology of maximal preexcitation may be helpful in predicting successful right ventricle ablation site.

Unusual variants of pre-excitation: From anatomy to ablation: Part I-Understanding the anatomy of the variants of ventricular pre-excitation.

The famous quotation of Winston Churchill, made in his radio broadcast of 1939 regarding Russia's next move, specifically "A riddle wrapped up in a mystery, inside an enigma," perfectly fits the current understanding of unusual accessory atrioventricular pathways, including the variants producing ventricular pre-excitation. It was many decades after their original descriptions that we came better to begin to understand most of their structure-function relationships. Their mysterious pathophysiology was sometimes unveiled after invasive treatments, such as surgical ablation of the atrioventricular conduction axis instead of the accessory pathway itself. Speculations made on this basis have largely been validated by subsequent clinical experience. Most of the names suggested for description of the pathways have stood well the test of time. For some of them, however, this is not the case, with the initial names becoming confusing. In a series of reviews, we re-visit those accessory pathways producing ventricular pre-excitation other than classical Wolff-Parkinson-White syndrome. To set the scene, in this initial review, we describe the development and anatomy of the normal atrioventricular conduction axis, along with the insulating tissues of the atrioventricular junctions. We have sought to illustrate our explanations by using virtual dissection of computerized tomographic datasets, since they retain the intact heart within the setting of the body. These images illustrate well the value of attitudinally appropriate terminology. Thereafter, we discuss the electrophysiological manifestations of the abnormal anatomical pathways which provide the potential for both accessory atrioventricular and intraventricular conduction.

Perioperative management of patients with genetic multisystem diseases associated with pre­excitation.

Pre-excitation is associated with life-threatening arrhythmias. Apart from the well-known Wolff-Parkinson-White syndrome, a number of rare diseases are associated with pre-excitation due to the existence of accessory pathways. The present review aims to focus on anaesthesia and perioperative care of patients with rare genetic diseases associated with pre-excitation due to the existence of a bundle of Kent or other accessory pathways. The Danon disease, Fabry disease and Pompe disease, tuberous sclerosis, Leber hereditary optic neuropathy (LHON), and mitochondrial encephalopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome are genetic multisystem disorders which may involve pre-excitation, usually combined with cardiomyopathy. The anaesthetic management of the above syndromes may become quite challenging. We conducted a PubMed and manual literature search for all types of relevant publications; we identified 58 articles suitable to be included in the present review. According to the literature, a high index of suspicion for the possibility of pre-excitation is required, and anaesthetic drugs and adjuvants should be chosen carefully, in order to prevent or at least not facilitate arrhythmias associated with accessory pathways. The perioperative management should be further tailored to the specific abnormalities of each condition. Multidisciplinary consultation and care, according to the affected organs, are mandatory for a safe outcome. The anaesthetic plan should be focused on preoperative clinical optimization and on case-specific management, tailored to the various systems involved.

Comparison of Electrophysiologic Profiles in Pediatric Patients with Incidentally Identified Pre-Excitation Compared with Wolff-Parkinson-White Syndrome.

The rising utilization of screening electrocardiograms has resulted in increased incidental identification of ventricular pre-excitation in pediatric patients. We compared accessory pathways of incidentally identified pre-excitation to Wolff-Parkinson-White Syndrome (WPW) with the aim to identify factors important in preprocedural counseling and planning. This single-center, retrospective study of patients ≤18 years without congenital heart disease identified 227 patients diagnosed with pre-excitation and referred for invasive electrophysiology study between 2008 and 2017. WPW Syndrome was diagnosed in 178 patients, while 49 patients had incidental identification of pre-excitation. Anterograde conduction of incidentally identified accessory pathways was not clinically different between the two cohorts at baseline or upon isoproterenol infusion. However, the proportion of accessory pathways meeting high-risk criteria was significantly lower than in patients diagnosed with WPW, 12% versus 28% (p < 0.05). Retrograde conduction at baseline of incidentally diagnosed accessory pathways was slower with a median block cycle length 365 milliseconds (IQR 260 to 450) versus 290 milliseconds (IQR 260 to 330, p < 0.01). In the incidentally identified cohort, right-sided, paraHisian, and fascicular pathways were more common with fewer attempted ablations (71% vs 94%, p < 0.001) and lower success rate (91% vs 97%, p < 0.001). A binomial logistic regression analysis further indicated patients incidentally identified with pre-excitation were associated with having lower rates of inducible supraventricular tachycardia, atrial fibrillation, and ablations performed, in addition, to having right-sided pathways. In conclusion, as patients with incidentally identified pre-excitation present more frequently for consideration of invasive electrophysiology study, these results impact procedural approaches, technical considerations, patient counseling, and outcome expectations.

Can noninvasive testing identify benign patterns of suggested pre-excitation on electrocardiogram?

BACKGROUND: The presence of anterograde conduction through an accessory pathway (AP) has been linked to sudden cardiac death. Unfortunately, pre-excitation associated with classic pathways can be difficult to differentiate from benign APs such as nodofascicular fibers. OBJECTIVE: Identifying characteristics on electrocardiogram (ECG) and exercise that differentiate classic and benign AP connections in suggested pre-excitation patterns. METHODS: Retrospective review of patients presenting between 1995 and 2017 with ventricular pre-excitation on ECG, determined to have either typical left-lateral AP during electrophysiology study (EPS), or benign, or no AP determined by either transesophageal electrophysiology study (TEP), or EPS. RESULTS: A total of 96 patients were included, 14.2 years (4-24), 45% female, 90% Caucasian. Of these, 60 (63%) had a classic APs identified on EPS and 58 (97%) underwent successful ablation. Conversely, 36 (37%) had benign pathways identified. ECG findings differed between the groups: PR-interval 102 versus 120 ms (P < .0001), QRS-duration 110 versus 102 ms (P < .0001), QRS-axis 74 versus 59 degrees (P = .0005), and QRS onset to peak R/S in limb leads 64 versus 42 ms (P < .0001), and precordial leads 66 versus 46 ms (P < .0001). Change in QRS duration during exercise differed between the groups: 25 versus 2 ms (P < .0001) and ECG characteristics identified the presence of an AP with 97% sensitivity and 94% negative predictive value. CONCLUSION: Classic and benign APs exhibit different ECG characteristics, though clinical overlap does not allow for absolute differentiation. These data may help with risk stratification decision making though does not obviate the need for additional invasive testing.

Classical response of antidromic atriofascicular tachycardia to premature atrial extrastimulus delivered during septal refractoriness.

A 23-year-old gentleman presented with a history of palpitations. The 12-lead electrocardiogram showed no manifest ventricular pre-excitation. Echocardiogram was within normal limits. A retrograde study showed concentric activation of the atrium with decremental conduction. Atrial pacing from right atrial free wall showed progressive pre-excitation. No anterograde nodal duality was documented.