[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.

[History of surgical treatment of cardiac arrhythmias in Germany : Surgical treatment of ventricular tachycardia and supraventricular tachycardia, especially pre-excitation syndromes (WPW)]. / Geschichte der chirurgischen Behandlung von Herzrhythmusstörungen in Deutschland : Chirurgische Therapie ventrikulärer Tachykardien sowie supraventrikulärer Tachykardien, insbesondere Präexzitationssyndromen (WPW).

The history of surgical treatment of ventricular tachycardias (VT) is short, lasting from 1978 until 1993. "Indirect procedures" with infarct scar resection were performed without electrophysiologic studies, whereas "direct procedures" consisted of either complete endocardial incisions ("encircling endocardial ventriculotomy") or large endocardial resections ("endocardial peel-off" technique) after precise epicardial and endocardial mapping procedures. In Germany, the first to report on intra-operative electrophysiologic mapping for VT treatment were Ostermeyer, Breithardt and Seipel in 1979. In 1981, the Hannover group (Frank, Klein) published their first results of surgical treatment of VT. In 1984, Ostermeyer et al. demonstrated that a partial endocardial incision resulted in more beneficial results with less myocardial damage (8% versus 46%) than applying a complete encircling incision. In 1987, the Düsseldorf group reported treatment results of 93 patients. After 5 years, 77% had no VT recurrence, while total mortality after 1 year was 11% and after 5 years 30%. In 1992, the Hannover group reported results of 147 patients after endocardial resection for VT. Total mortality after 3 years was 27%; recurrence of VT events occurred in 18% of the surviving cohort.The history of surgical procedures for supraventricular tachycardia (SVT), in particular Wolff-Parkinson-White (WPW) syndrome, is even shorter than that of surgery for VT. As early as 1969, Sealy, Gallagher and Cox reported the first cases of surgical intervention for WPW syndrome via endocardial access in cardioplegic arrest. In 1984, Guiraudon and Klein reported on a new procedure with epicardial access to the accessory bundle without cardioplegia in laterally localised conduction pathways. In Germany, too, the groups in Düsseldorf (Ostermeyer, Seipel, Breithardt, Borggrefe) from 1980 and the Hannover group (Frank, Klein and Kallfelz) from 1981 performed surgical procedures for WPW syndrome. In 1987, Borggrefe reported on 18 patients with WPW syndrome and atrial fibrillation who had undergone surgery. After 2 years, 14 of 18 patients had no recurrences of tachycardia; in 1989, Frank, Klein and Kallfelz (Hannover) reported on 10 children (2-14 years) operated on using the cryoablation technique. Between 1984 and 1992, a total of 120 patients with SVT, mostly WPW syndrome, were operated on in Hannover; after 42 months, 12 patients had a recurrence of SVT. Two patients died during the reoperation.

The appearance of new accessory pathway after the Cone procedure.

INTRODUCTION: Acquired Wolff-Parkinson-White (WPW) syndrome can occur after congenital heart disease (CHD) surgery. METHODS AND RESULTS: A 27-year-old male with Ebstein's anomaly and manifest WPW syndrome received catheter ablation twice. The first electrophysiology study (EPS) induced orthodromic atrioventricular reentrant tachycardia and successfully eliminated the posteroseptal accessory pathway (AP). Six months after the Cone procedure, the patient suffered from palpitation. The second EPS was performed and abolished the right lateral AP. CONCLUSION: The appearance of a new AP after the reconstruction of CHD is a rare finding and should raise suspicion of an acquired AP connection.

Hypertrophic Cardiomyopathy and Ventricular Preexcitation in the Young: Cause and Accessory Pathway Characteristics.

BACKGROUND: The cause of hypertrophic cardiomyopathy (HCM) in the young is highly varied. Ventricular preexcitation (preexcitation) is well recognized, yet little is known about the specificity for any cause and the characteristics of the responsible accessory pathways (AP). METHODS: Retrospective cohort study of patients <21 years of age with HCM/preexcitation from 2000 to 2022. The cause of HCM was defined as isolated HCM, storage disorder, metabolic disease, or genetic syndrome. Atrioventricular AP (true AP) were distinguished from fasciculoventricular fibers (FVF) using standard invasive electrophysiology study criteria. AP were defined as high risk if any of the following were <250 ms: shortest preexcited RR interval in atrial fibrillation, shortest paced preexcited cycle length, or anterograde AP effective refractory period. RESULTS: We identified 345 patients with HCM and 28 (8%) had preexcitation (isolated HCM, 10/220; storage disorder, 8/17; metabolic disease, 5/19; and genetic syndrome, 5/89). Six (21%) patients had clinical atrial fibrillation (1 with shortest preexcited RR interval <250 ms). Twenty-two patients underwent electrophysiology study which identified 23 true AP and 16 FVF. Preexcitation was exclusively FVF mediated in 8 (36%) patients. Five (23%) patients had AP with high-risk conduction properties (including ≥1 patient in each etiologic group). Multiple AP were seen in 8 (36%) and AP plus FVF in 10 (45%) patients. Ablation was acutely successful in 13 of 14 patients with recurrence in 3. One procedure was complicated by complete heart block after ablation of a high-risk midseptal AP. There were significant differences in QRS amplitude and delta wave amplitude between groups. There were no surface ECG features that differentiated AP from FVF. CONCLUSIONS: Young patients with HCM and preexcitation have a high likelihood of underlying storage disease or metabolic disease. Nonisolated HCM should be suspected in young patients with large QRS and delta wave amplitudes. Surface ECG is not adequate to discriminate preexcitation from a benign FVF from that secondary to potentially life-threatening AP.

Coronary sinus ablations in pediatric patients with supraventricular arrhythmias.

BACKGROUND: Intracoronary sinus ablations have been performed for various arrhythmical substrates. The aim of this study is to report our experience on pediatric patients of the safety and efficacy of ablations in the coronary sinus. METHODS: This is a retrospective study of all patients who underwent ablations in the coronary sinus from October 2013 to October 2021 at a single center. Clinical presentation, type of arrhythmia causing tachycardia, ablation procedure, and outcome were recorded. RESULTS: A total of 27 patients were included in the study. Nineteen (69%) of those followed up received a diagnosis of Wolff-Parkinson-White syndrome (WPW), 4 (15%) were cases of supraventricular tachycardia with concealed accessory pathway (AP), 2 (8%) were cases of focal atrial tachycardia, and 2 (8%) were cases of permanent junctional reciprocating tachycardia. Negative delta wave was noteworthy especially in lead II in 11/19 (58%) cases and coronary sinus diverticulum was detected in the WPW cases. Of those with manifest AP (19 cases), 15 (79%) had a high-risk AP and the AP in all WPW cases was adenosine unresponsive. Radiofrequency (RF) catheter ablation was performed in 25/27 (93%) cases during the procedure, and 16/25 (64%) of these were irrigated RF catheters. No complications were observed in the follow-up, including coronary artery injury. CONCLUSIONS: Catheter ablation of supraventricular tachyarrhythmias can be accomplished effectively and potentially safely within the coronary sinus. Coronary sinus diverticula should be suspected in patients with manifest posteroseptal APs who have a previous failed ablation and typical electrocardiographic signs.

Antidromic and orthodromic reciprocating tachycardias over a novel left-sided accessory pathway involving the vein of Marshall and coronary sinus musculature.

INTRODUCTION: Herein, we present a rare case of the successful ablation of an accessory pathway (AP) involving the Marshall Bundle (MB) and coronary sinus musculature (CSM) in a 40-year-old man with Wolff-Parkinson-White syndrome. METHODS AND RESULTS: An orthodromic reciprocating tachycardia (ORT) was inducible with the earliest atrial activation site located at the posterolateral mitral annulus. The local conduction and the cycle length of ORT was prolonged by peri-mitral ablation; however, it failed to block the AP. The atrial insertion of the AP was identified by remapping during ORT at the left atrial ridge, which was away from the mitral annulus, where ablation was successful. Together with the electrophysiological findings in CSM potentials, we conclude that the epicardial MB-CSM connection functioned as the AP in this patient. CONCLUSION: The novel variant form of AP comprised of MB and CSM should be noted. The atrial insertion of the MB may be the target of catheter ablation.

Two-dimensional speckle tracking imaging to assess the hazards of left ventricular function and ventricular wall motion disorders in children with pre-excitation syndrome and the efficacy of radiofrequency ablation treatment.

OBJECTIVE: Our study aimed at analyzing the echocardiographic multi-indicator evaluation of the risk of Wolff-Parkinson-White syndrome (WPW) on the left ventricular function and ventricular wall motion disorders, as well as the effect of radiofrequency ablation treatment. PATIENTS AND METHODS: The clinical data of 55 WPW patients treated with radiofrequency (RF) ablation at the Children's Hospital of Nanjing Medical University between January 2018 and December 2022 were retrospectively analyzed and included in the observation group, while other 50 healthy children were included in the control group during the same time. We analyzed the echocardiographic indices of the patients, assessed the effects of the disease on left ventricular myocardial function and ventricular wall motion disorders, and evaluated the effects of radiofrequency ablation treatment on the myocardium of the left ventricle. The echocardiographic parameters were analyzed to assess the effect of the disease on left ventricular myocardial function and ventricular wall dyskinesia. RESULTS: Of the 55 patients with pre-excited syndrome, 20 had type A bypass and 35 had type B bypass. Ten patients had pre-excited dilated cardiomyopathy with significant enlargement of the left ventricular cavity, reduced left ventricular systolic function, and a significant impairment of ventricular wall motion; the other 5 patients had basal segmental septal motion incoordination. Compared to the control group, patients with left ventricular end-diastolic diameter (LVEDD) (42.9±5.0 mm vs. 39.2±3.0 mm), peak strain dispersion (PSD) (38.8±15.3 ms vs. 21.7±2.2 ms), maximum peak time difference (MPTD) (200.2±92.8 ms vs. 89.5±9.8 ms) and interventricular mechanical delay (IVMD) (36.2±13.7 ms vs. 21.2±2.1 ms) before RF ablation were increased. Left ventricular ejection fraction (LVEF) (57.1±9.1% vs. 65.9±2.6%), E/A (1.1±0.2 vs. 1.8±0.2) and global longitudinal strain (GLS) (-18.7±2.2% vs. -22.4±0.5%) decreased, with statistically significant differences (p<0.05). All 55 patients had a successful procedure, and all postoperative echocardiographic parameters were found to be improved, compared to the preoperative period. The results of the postoperative review after 3 months showed differences in E/A, PSD, MPTD, and IVMD compared to the healthy group, suggesting that left ventricular diastolic function and synchrony had not fully returned to normal. CONCLUSIONS: Echocardiography can better evaluate myocardial motion and function in patients with Wolff-Parkinson-White syndrome and monitor the effect and progress of disease treatment, and has high clinical application value.

Diagnosis and treatment of fetal and pediatric age patients (0-12 years) with Wolff-Parkinson-White syndrome and atrioventricular accessory pathways.

Overt or concealed accessory pathways are the anatomic substrates of ventricular preexcitation (VP), Wolff-Parkinson-White syndrome (WPW) and paroxysmal supraventricular tachycardia (PSVT). These arrhythmias are commonly observed in pediatric age. PSVT may occur at any age, from fetus to adulthood, and its symptoms range from none to syncope or heart failure. VP too can range from no symptoms to sudden cardiac death. Therefore, these arrhythmias frequently need risk stratification, electrophysiologic study, drug or ablation treatment. In this review of the literature, recommendations are given for diagnosis and treatment of fetal and pediatric age (≤12 years) WPW, VP, PSVT, and criteria for sport participation.

Utility of open-window mapping for catheter ablation of an accessory pathway in patients with Wolff-Parkinson-White syndrome.

BACKGROUND: Open-window mapping (OWM) is a novel automated mapping method for catheter ablation of an accessory pathway (AP), in which the local signal is annotated with window-of-interest parameters to analyze both atrial and ventricular signals. This study aimed to determine the utility of OWM in visualizing the location and width of APs in patients with Wolff-Parkinson-White syndrome. METHODS: This two-center study enrolled 30 patients (20 males; mean age: 56 years, interquartile range [IQR]: 22-69 years) who underwent high-density OWM with the extended early-meets-late (EEML) algorithm using a 20-electrode, 5-spline catheter (PENTARAY, Biosense Webster). The lower threshold of the EEML was set to adjust the EEML gap to match the propagation mapping, and broad APs were defined as an EEML gap > 1 cm. RESULTS: The median mapping points, mapping time, and lower threshold of the EEML were 2482 (IQR: 1755-4000) points, 23 (IQR: 15-30) min, and 23 (IQR: 18-25), respectively. All 30 APs (24 in the mitral annulus and 6 in the tricuspid annulus) were successfully eliminated. Of these APs, 21 (70%) were eliminated by the first radio frequency (RF) application. OWM revealed broad APs in 11 patients (37%), in four of whom (36%) the first RF application achieved a loss of AP conduction (vs. 90% of patients without broad APs; p = .004). CONCLUSION: OWM facilitates the visualization of the location and width of APs, which may be particularly useful for predicting whether multiple RF applications are required for broad APs.

Myocardial work in children with Wolff-Parkinson-White syndrome.

Wolff-Parkinson-White Syndrome (WPW) has been associated with reduced local myocardial deformation, and when left ventricular dysfunction is present, catheter ablation of the accessory pathway may be required, even in asymptomatic patients. We aimed to evaluate the diagnostic value of non-invasive myocardial work in predicting subtle abnormalities in myocardial performance in children with WPW.Seventy-five paediatric patients (age 8.7 ± 3.5 years) were retrospectively recruited for the study: 25 cases with manifest WPW and 50 age- and sex- matched controls (CTR). Global myocardial work index (MWI) was measured as the area of the left ventricle (LV) pressure-strain loops. From MWI, global Myocardial Constructive Work (MCW), Wasted Work (MWW), and Work Efficiency (MWE) were estimated. In addition, standard echocardiographic parameters of LV function were evaluated. Despite normal LV ejection fraction (EF) and global longitudinal strain (GLS), children with WPW had worse MWI, MCW, MWW, and MWE. At multivariate analysis, MWI and MCW were associated with GLS and systolic blood pressure, and QRS was the best independent predictor of low MWE and MWW. In particular, a QRS > 110 ms showed good sensitivity and specificity for worse MWE and MWW values. In children with WPW, myocardial work indices were found significantly reduced, even in the presence of normal LV EF and GLS. This study supports the systematic use of myocardial work during the follow-up of paediatric patients with WPW. Myocardial work analysis may represent a sensitive measure of LV performance and aid in decision-making.

Successful catheter ablation of the accessory pathway in an unusual location in a 13-year-old girl with a coronary sinus diverticulum and Wolff-Parkinson-White syndrome.

Wolff-Parkinson-White syndrome is a congenital cardiac pre-excitation syndrome that is effectively treated by ablating the accessory pathway. However, accessory pathways located in the posteroseptal region can sometimes be challenging. In this paper, we present the successful ablation of the epicardial posteroseptal accessory pathway through the middle cardiac vein in a 13-year-old girl with a coronary sinus diverticulum and Wolff-Parkinson-White syndrome, after unsuccessful ablation attempts at different locations. If the ablation procedure fails, the possibility of the posteroseptal pathway should be kept in mind, and coronary sinus angiography should be performed. In cases with a coronary sinus diverticulum where ablation is not successful, other coronary sinus structures, for example, the middle cardiac vein should be considered as potential accessory pathways.

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.

Successful ablation of a right epicardial accessory pathway via the right ventricular diverticulum in a patient with Wolff-Parkinson-White syndrome.

INTRODUCTION: We describe one rare case of successful ablation of a right epicardial accessory pathway (AP) via the right ventricular diverticulum in a patient with Wolff-Parkinson-White syndrome. METHODS: A 42-year-old woman was referred to the hospital for a catheter ablation of Wolf-Parkinson-White syndrome. The earliest activation was shown to be present in the region of the tricuspid annulus. However, ablation had no effect on the AP. RESULTS: We decided to do a selected angiography, in which a big diverticulum near the right tricuspid annulus was shown to be present. Ablation in this region successfully repressed the AP without any recurrences within a follow-up period of 12 months. CONCLUSION: The ventricular diverticulum-mediated AP is a novel variant of pre-excitation. It can serve as an anatomical substrate of supraventricular tachycardia, and can be ablated endocardially using an irrigation tip catheter within the diverticulum.

Validation of qrs-polarity algorithm with special emphasis in parahisian pathways.

BACKGROUND: In 1996 Iturralde et al. published an algorithm based on the QRS polarity to determine the location of the accessory pathways (AP), this algorithm was developed before the massive practice of invasive electrophysiology. PURPOSE: To validate the QRS-Polarity algorithm in a modern cohort of subjects submitted to radiofrequency catheter ablation (RFCA). Our objective was to determinate its global accuracy and its accuracy for parahisian AP. METHODS: We conducted a retrospective analysis of patients with Wolff-Parkinson-White (WPW) syndrome who underwent an electrophysiological study (EPS) and RFCA. We employed the QRS-Polarity algorithm to predict the AP anatomical location and we compared this result with the real anatomic location determined in the EPS. To determine accuracy, the Cohen's kappa coefficient (k) and the Pearson correlation coefficient were used. RESULTS: A total of 364 patients were included (mean age 30 years, 57% male). The global k score was 0.78 and the Pearson's coefficient was 0.90. The accuracy for each zone was also evaluated, the best correlation was for the left lateral AP (k of 0.97). There were 26 patients with a parahisian AP, who showed a great variability in the ECG features. Employing the QRS-Polarity algorithm, 34.6% patients had a correct anatomical location, 42.3% had an adjacent location and only 23% an incorrect location. CONCLUSION: The QRS-Polarity algorithm has a good global accuracy; its precision is high, especially for left lateral AP. This algorithm is also useful for the parahisian AP.

ANTECEDENTES: En 1996 Iturralde y colaboradores publicaron un algoritmo basado en la polaridad del QRS para determinar la ubicación de las vías accesorias (VA), este algoritmo fue desarrollado antes de la práctica masiva de la electrofisiología invasiva. OBJETIVO: Validar el algoritmo de la polaridad del QRS en una cohorte moderna de sujetos sometidos a ablación con catéter por radiofrecuencia (ACRF). Nuestro objetivo fue determinar su precisión global y su precisión para las VA parahisianas. MÉTODOS: Realizamos un análisis retrospectivo de pacientes con síndrome de Wolff-Parkinson-White (WPW) a los que se les realizó estudio electrofisiológico (EEF) y ACRF. Empleamos el algoritmo de la polaridad del QRS para predecir la ubicación anatómica de la VA y comparamos este resultado con la ubicación anatómica real determinada en el EEF. Para determinar la precisión se utilizaron el coeficiente kappa de Cohen (k) y el coeficiente de correlación de Pearson. RESULTADOS: Se incluyeron un total de 364 pacientes (edad media 30 años, 57 % varones). La puntuación k global fue de 0,78 y el coeficiente de Pearson de 0,90. También se evaluó la precisión para cada zona, la mejor correlación fue para las VA laterales izquierdas (k de 0.97). Hubo 26 pacientes con VA parahisianas, que mostraron una gran variabilidad en las características del ECG. Empleando el algoritmo de la polaridad del QRS, el 34,6 % de los pacientes tenía una ubicación anatómica correcta, el 42,3 % tenía una ubicación adyacente y solo el 23 % una ubicación incorrecta. CONCLUSIÓN: El algoritmo de la polaridad del QRS tiene una buena precisión global; su precisión es alta, especialmente para VA lateral izquierdo. Este algoritmo también es útil para la VA parahisiana.

Multiple accessory pathways coexisting with a persistent left superior vena cava: a case report.

BACKGROUND: Wolff-Parkinson-White syndrome is characterized by a short PR interval (delta-wave), long QRS complex, and the appearance of paroxysmal supraventricular tachycardia. Patients with Wolff-Parkinson-White syndrome usually have one accessory pathway, whereas cases with multiple accessory pathways are rare. Persistent left superior vena cava is a vascular anomaly in which the vein drains into the right atrium through the coronary sinus at the junction of the left internal jugular and subclavian veins due to abnormal development of the left cardinal vein. The simultaneous presence of multiple accessory pathways and persistent left superior vena cava has not been reported before. CASE PRESENTATION: A 56-year-old Japanese man with a 5-year history of palpitations was referred for radiofrequency catheter ablation due to increased frequency of tachycardia episodes in the previous 2 months. Persistent left superior vena cava was confirmed by transthoracic echocardiography and computed tomography. An electrophysiological study revealed that the accessory pathways were located in the left lateral wall, anterolateral wall, and posteroseptal region. They were completely ablated with radiofrequency energy application. CONCLUSIONS: We reported an extremely rare case of a patient with multiple accessory pathways and persistent left superior vena cava. Our case may suggest a potential embryological relationship between the multiple accessory pathways and persistent left superior vena cava.

Open-window mapping for ablation of Wolff-Parkinson-White syndrome related hemodynamically unstable tachycardia.

During catheter ablation (CA) of tachycardia, conventional point-by-point mapping may be hindered due to hemodynamic instability. We performed open-window mapping (CARTO, Biosense Webster, USA) in a patient who developed hemodynamic instability during orthodromic atrioventricular reciprocating tachycardia (AVRT) and unwarranted induction of AVRT during attempts to map accessory pathway (AP) with ventricular pacing. With over 11,000 points acquired rapidly, the system accurately identified AP at the mitral valve (MV) annulus where the application of single radiofrequency (RF) lesion promptly eliminated AP's conduction. Our case illustrates the utility of open-window mapping for the ablation of AVRT.

Cardiac arrest in previously asymptomatic child with Wolff-Parkinson-White syndrome.

In this case report, a previously asymptomatic 11-year-old boy presented with sudden palpitations and syncope. He eventually went into cardiac arrest and was successfully resuscitated. The ECG showed pre-excited atrial fibrillation degenerating into pulseless ventricular tachycardia. The patient was found to have Wolff-Parkinson-White syndrome (WPW) with an accessory pathway between right atrium and ventricle which was successfully ablated. Sudden cardiac death (SCD) is rare in WPW, however, early diagnosis is essential for eliminating the risk of SCD.