Wolff-Parkinson-White syndrome and dilated cardiomyopathy: Not only an electrical issue?

The present case describes a dilated cardiomyopathy associated with both antidromic and orthodromic atrio-ventricular reentrant tachycardias supported by multiple right accessory pathways. Both right accessory pathways were successfully eliminated by catheter ablation and the patient progressively recovered during the follow up. The following etiologies might be involved: 1) primitive dilated cardiomyopathy (or post-inflammatory); 2) septal dyssinchrony due to ventricular pre-excitation; 3) tachycardiomyopathy.

Left ventricular dysfunction in a child with asymptomatic pre-excitation: Cure with catheter ablation.

Wolff-Parkinson-White syndrome, is known to cause left ventricular dysfunction or dilated cardiomyopathy secondary to sustained tachycardia in infants and children. However, left ventricular dysfunction secondary to pre-excitation related abnormal ventricular activation has been reported in a limited number of cases. This condition should be recognized early, as catheter ablation of the accessory pathway can permit rapid ventricular function improvement. In this paper, we present a 2.5-year-old patient diagnosed with tachycardia-free Wolff-Parkinson-White syndrome with a right free wall accessory pathway and depressed cardiac function, whose left ventricular function is completely restored after successful catheter cryoablation.

The characteristics of pre-excitation syndrome concomitant with atrial tachyarrhythmia and the effect of radiofrequency ablation.

BACKGROUND: Wolff-Parkinson-White (WPW) concomitant with atrial tachyarrhythmia (ATA) has not been systemically characterized. METHODS: Detailed electroanatomical mapping of the right atrium (RA) and/or left atrium (LA) was performed using three-dimensional mapping and the accessory pathway (AP) was mapped. RESULTS: WPW syndrome with ATA was diagnosed in 11 patients (median age 60 years). The characteristic of unidirectional anterograde conduction over the AP was displayed in nine patients, six of whom were intermittent. Sustained atrial tachycardia, that is, counterclockwise atrial flutter (AFL) with a median tachycardia cycle length (TCL) of 225 (220-275) ms, was mapped in eight patients; furthermore, "figure 8" right atrial reentry was mapped with TCL 250 ms in one patient with a surgical history of ventricular septal defect repair. The remaining two patients underwent mitral annulus-dependent AT after paroxysmal atrial fibrillation (PAF) ablation and LA micro-reentry AT, respectively. In four patients, the location of the APs was left posterior. Left-lateral APs were identified in four patients. The locations of the APs in the remaining three patients were the right posterior and middle septum. All ATAs and APs were successfully ablated. After a median follow-up of 37 (15-72) months, no anterograde conduction over the AP was recorded, new onset of PAF was recorded in three patients, and all of them underwent circumferential pulmonary vein isolation. CONCLUSIONS: WPW with concomitant ATA frequently had continuous anterograde conduction over the AP with a rapid ventricular rate. Most WPWs displayed the characteristic of unidirectional anterograde conduction.

Accessory pathway properties are similar in symptomatic and asymptomatic preexcitation.

PURPOSE: Patients with WPW syndrome have an increased mortality rate compared to the general population. Although asymptomatic preexcitation has previously been considered benign, recent studies have found that also asymptomatic patients have clinical and electrophysiological factors associated with increased risk of sudden cardiac death. This study compares the baseline electrophysiological characteristics of accessory pathways in symptomatic and asymptomatic patients with preexcitation. We hypothesized that a significant proportion of asymptomatic patients has inducible orthodromic tachycardia during programmed electrical stimulation. METHODS: This retrospective study includes 1853 patients with preexcitation who underwent invasive electrophysiological testing in two Swedish University Hospitals between 1991 and 2018. The mean age was 36 ± 17 years with a range of 3-89 years. Thirty-nine percent was women. A total of 269 patients (15%) were children younger than 18 years. Electrophysiological data included effective refractory period of the accessory pathway (APERP, in 1069 patients), tachycardia cycle length, inducibility and type of tachycardia, and AP localization. RESULTS: A total of 1703 (93%) patients reported symptoms suggesting tachyarrhythmias before the study and 128 (7%) were asymptomatic. The proportion of potentially dangerous pathways with short APERP (≤ 250 ms) were similar in symptomatic and asymptomatic patients (187/949, 20% vs. 25/108, 23%) (P = 0.40) as was the mean APERP (303 ± 68 ms vs. 307 ± 75) (P = 0.61). The proportion of patients who had inducible arrhythmia was larger in the symptomatic group (64% vs. 31%) (P < 0.001). CONCLUSION: The results of this study strengthen the present guideline recommendation (IIA) to consider invasive risk assessment in patients with asymptomatic preexcitation.

A Frequent Observation of Wolff-Parkinson-White Syndrome and Fasciculoventricular Pathways in Patients With Danon Disease.

BACKGROUND: Danon disease is typically associated with cardiomyopathy and ventricular pre-excitation. The study aimed to characterize the clinical profile of Danon disease, analyze electrocardiographic (ECG) and electrophysiologic features, and investigate their association with Wolff-Parkinson-White (WPW) syndrome and fasciculoventricular pathways (FVPs).Methods and Results:Clinical course, family history, ECG and electrophysiological data were collected from 16 patients with Danon disease. Over 0.4-8 years of follow up, 1 female patient died suddenly, and 5 male patients died of progressive heart failure by age 13-20 years. Family history analysis revealed that 3 mothers experienced hospitalization or death for heart failure at age 28-41 years. There was 100% penetrance for ECG abnormalities in 13 patients with original ECGs. Short PR intervals and delta waves were present in 9 and 8 patients, respectively. There were significant age-associated increases in the QRS complex width (r=0.556, P=0.048) and the number of leads with notched QRS (r=0.575, P=0.04). Four patients who underwent electrophysiological studies all had FVPs, and 2 of them still had left-side atrioventricular pathways. CONCLUSIONS: Danon disease causes a malignant clinical course characterized by early death caused by heart failure in both genders and progressive ECG changes as patients age. The pre-excited ECG pattern is related to FVPs and WPW, which is suggestive of extensive cardiac involvement.

Success rate and safety of catheter ablation in preexcitation syndrome: A comparison between adult and pediatric patients.

BACKGROUND: In contrast to adults, in whom cardiac rhythm disorders are mainly conditioned by coronary artery disease, in children, arrhythmias are most often associated with inherited heart disorders. Catheter ablation (CA) has an important role in the management of cardiac arrhythmias, in adults and children. The aim of the study was to assess and compare the efficacy and safety of CA in children and adults with preexcitation syndrome. METHODS: The study population comprised 43 adults and 43 children diagnosed with a Wolff-Parkinson-White syndrome (WPW). The mean age of the study population was 41 ± 15 years for adults and 14 ± 2.5 years for children. In all patients, an electrophysiological study and CA were performed. Analysis with respect to the procedure duration, fluoroscopy exposure time, location of accessory pathways (AP), immediate success rate and complications were performed. RESULTS: Electrophysiological study revealed the most frequent presence of left-sided AP (56% in children and 70% in adults). The mean procedure duration was 96 ± 36 min and 106 ± 51 min in children and adults, respectively (p = NS). The mean fluoroscopy duration was 8.5 ± 4.3 min and 5.9 ± 5.8 min in children and adults, respectively p < 0.05. The CA procedure was successful in 40 out of 43 (93%) adults and in 36 out of 43 (83.7%) children (p = NS). In 2 (4%) children minor complications occurred. CONCLUSIONS: Ablation in children and adults are equally effective with respect to short-term clinical observation.

Echocardiographic characteristics of pediatric preexcitation cardiomyopathy.

Preexcitation cardiomyopathy is a rare cardiac anomaly but it can be life threatening. It may occur in type B preexcitation syndrome. A patients with this condition typically has an enlarged left ventricle and reduced systolic function without tachycardia-associated cardiomyopathy. Echocardiography plays an important role in diagnosis in identifying this condition by revealing the "rebounce" movement of the inter-ventricular septum (IVS). We report echocardiographic findings of five pediatric patients with preexcitation cardiomyopathy. All of them had a radiofrequency catheter ablation (RFCA) and recovered during the follow-up.

Successful risk stratification of a patient with ventricular preexcitation by improved transesophageal electrophysiological study.

The patient is a 19 years-old man who often wakes up in dreams with palpitations and fatigue. The ECG shows: 1. Sinus rhythm; 2. Preexcitation syndrome. Transesophageal electrophysiological study (TEEPS) diagnosis:High-risk accessory pathway. During radiofrequency catheter ablation, the patient suddenly developed atrial fibrillation and quickly converted to ventricular fibrillation. After defibrillation, ventricular fibrillation is transformed into sinus rhythm. Subsequently, the patient's high-risk accessory pathway was successfully ablated. Studies have shown that about 25% of patients with WPW syndrome have a refractory period of less than 250 ms, which is one of the risk factors for the conversion of atrial fibrillation to ventricular fibrillation. Therefore, risk stratification is recommended for these symptomatic patients. From 1980 to 1990, there were literature reports on risk stratification of patients with preexcitation syndrome by TEEPS. But it has not become a routine examination of risk stratification in patients with preexcitation syndrome.The reason may be related to the hardware conditions and risk stratification methods used at that time. The TEEPS equipment currently used in our hospital can control the pacing voltage at about 12 mv on average. The voltage in this case report is 9 mv only. In addition, we successfully stratified the risk of patient with preexcitation syndrome without inducing atrial fibrillation. All the electrophysiological records of the patient during the examination were recorded simultaneously with the 12-lead ECG and the esophageal lead ECG. These improvements makes TEEPS a simple, safe and reliable non-invasive cardiac electrophysiological detection technology, which is worth popularizing in hospitals.

Situs inversus totalis asociado a síndrome de preexcitación ventricular: reporte de un caso / Situs inversus totalis and pre excitation syndrome

Resumen: El situs inversus totalis es la inversión congénita completa de órganos torácicos y abdominales. Se presenta el caso de una paciente de 3 años sin antecedentes médicos previos, a quien en atención primaria, y por un cuadro respiratorio agudo, se evidencia el hallazgo de dextrocardia y burbuja gástrica a derecha en la radiografía de tórax, sospechándose situs inversus totalis. Fue derivada a cardiología infantil donde se confirmó el diagnóstico con un ecocardiograma transtorácico, asociado a un hallazgo de preexcitación ventricular en el electrocardiograma. Una vez resuelto el cuadro respiratorio agudo, la paciente se mantiene controlada de manera periódica en atención primaria y por especialista de manera semestral.

Abstract: Situs inversus totalis is the complete congenital inversion of thoracic and abdominal organs. We present the case of a 3-year-old girl with no previous medical history. When seen with an acute respiratory syndrome, dextrocardia and gastric bubble on the right side led to the diagnosis of Situs inversus. She was referred to infant cardiology where the diagnosis was confirmed with a transthoracic echocardiogram. In addition, the electrocardiogram identified the presence of ventricular preexitation. Once the acute respiratory symptoms subsided, the patient remains controlled periodically in primary care and by a specialist every six months. No episodes of tachycardia have been detected.

Evolution of acute ST-segment elevation myocardial infarction in a patient with ventricular preexcitation.

Wolff-Parkinson-White pattern is a relatively common electrocardiographic phenomenon caused by accessory atrioventricular (AV) conduction, which can make the diagnosis of acute myocardial infarction challenging. There is little existing literature regarding the interpretation of electrocardiograms in patients with accessory AV conduction pathways who present with acute myocardial infarction. We describe a case of electrocardiographic evolution of acute ST-segment elevation myocardial infarction in a patient with Wolff-Parkinson-White pattern, review pseudo-infarction patterns, and discuss proposed mechanisms for these repolarization abnormalities.

Características clínicas y pronóstico de la enfermedad de Danon. Análisis del registro multicéntrico español / Clinical findings and prognosis of danon disease. An analysis of the Spanish multicenter danon registry

Introducción y objetivos: La enfermedad de Danon (ED) es una enfermedad producida por mutaciones en el gen LAMP2. Se la considera una enfermedad multisistémica caracterizada por miocardiopatía hipertrófica con preexcitación e hipertrofia extrema, discapacidad intelectual, miopatía, presentación infantil y peor pronóstico en varones. Hay pocas series que permitan conocer las características clínicas y el pronóstico de la ED. Métodos: Se analizaron los registros clínicos de los pacientes con ED de 10 hospitales españoles. Resultados: Se incluyó a 27 pacientes (edad, 31 +/- 19 años; el 78% mujeres). Los varones mostraron una elevada prevalencia de manifestaciones extracardiacas -miopatía (80%), trastornos del aprendizaje (83%) y alteraciones visuales (60%)- que eran infrecuentes en las mujeres (el 5, el 0 y el 27% respectivamente). Aunque la miocardiopatía hipertrófica era la cardiopatía más habitual (61%), el grosor ventricular máximo fue 15 +/- 7 mm y 12 pacientes (10 mujeres) presentaron miocardiopatía dilatada. Solo 11 pacientes (49%) mostraron preexcitación y en 16 (65%) la enfermedad se inició después de los 20 años. Tras una mediana de seguimiento de 4 años [intervalo intercuartílico, 2-9], 4 varones (67%) y 9 mujeres (43%) fallecieron o se sometieron a trasplante. El daño cardiaco y los eventos adversos ocurrieron más tardíamente en las mujeres (37 +/- 9 frente a 23 +/- 16 años y 36 +/- 20 frente a 20 +/- 11 años). Conclusiones: Las características clínicas de la ED difieren sustancialmente de lo considerado tradicionalmente. La edad de presentación de la ED es más tardía, no se expresa como una enfermedad multisistémica en las mujeres y la preexcitación es poco frecuente

Introduction and objectives: Danon disease (DD) is caused by mutations in the LAMP2 gene. It is considered a multisystemic disease characterized by hypertrophic cardiomyopathy with pre-excitation and extreme hypertrophy, intellectual disability, myopathy, childhood presentation, and worse prognosis in men. There are scarce data on the clinical characteristics and prognosis of DD. Methods: We analyzed the clinical records of patients with DD from 10 Spanish hospitals. Results: Twenty-seven patients were included (mean age, 31 +/- 19 years; 78% women). Male patients showed a high prevalence of extracardiac manifestations: myopathy (80%), learning disorders (83%), and visual alterations (60%), which were uncommon findings in women (5%, 0%, and 27%, respectively). Although hypertrophic cardiomyopathy was the most common form of heart disease (61%), the mean maximum wall thickness was 15 +/- 7 mm and dilated cardiomyopathy was present in 12 patients (10 women). Pre-excitation was found in only 11 patients (49%). Age at presentation was older than 20 years in 16 patients (65%). After a median follow-up of 4 years (interquartile range, 2-9), 4 men (67%) and 9 women (43%) died or required a transplant. Cardiac disease and adverse events occurred later in women (37 +/- 9 vs 23 +/- 16 and 36 +/- 20 vs 20 +/- 11 years, respectively). Conclusions: The clinical characteristics of DD differ substantially from traditional descriptions: age at presentation of DD is older, the disease is not multisystemic in women, and pre-excitation is infrequent

Does the age of evaluation change the long-term follow-up of untreated pre-excitation syndrome?

BACKGROUND: Due to high rates of ablation at the time of diagnostic EP study, follow-up of the natural history of untreated pre-excitation syndrome has become difficult. We present patients in which such data is available and study the effect of initial age on the evolution. METHODS: In this retrospective review, 126 patients, 47 aged ≤19 years, 79 aged more than>19 underwent 2 similar electrophysiological studies (EPS) within 1 to 25 years of one another (8.8 ±â€¯6.8) for occurrence of symptoms or new evaluation. First EPS was indicated for syncope (10), atrioventricular re-entrant tachycardias (AVRT) (58), atrial fibrillation (AF) (5), spontaneous PS-related adverse event (7) or asymptomatic PS (46). RESULTS: Clinical data remained unchanged in 76 patients (60.3%). AVRT symptom was more frequently unchanged than other symptoms. Electrophysiological data remained unchanged in 105 patients (82%), but signs of initial malignant signs were variable with a disappearance in 53.5% of patients. At EPS1, AF induction was rarer in patients ≤19 years. Syncope had a low predictive value of malignant form. AVRT induction at EPS1 was not predictive of AVRT occurrence. Maximal rate over accessory pathway increased, but unexpected changes could occur. After multivariate analysis, data of first EPS were limited for the prediction of AVRT or adverse event; effect of age was not significant. CONCLUSIONS: Clinical data remained unchanged in 60.3% of patients and electrophysiological data in 82%. Initial age of evaluation did not change the modifications. Electrophysiological signs associated with sudden death varied over time. Clinical AVRT was inconstantly related to inducible AVRT (78.5%).

Coexistence of atrioventricular accessory pathways and drug-induced type 1 Brugada pattern.

BACKGROUND: Atrial arrhythmias, particularly atrioventricular nodal reentrant tachycardia, can coexist with drug-induced type 1 Brugada electrocardiogram (ECG) pattern (DI-Type1-BrP). The present study was designed to determine the prevalence of DI-Type1-BrP in patients with atrioventricular accessory pathways (AV-APs) and to investigate the clinical, electrocardiographic, electrophysiologic, and genetic characteristics of these patients. METHODS: One-hundred twenty-four consecutive cases of AV-APs and 84 controls underwent an ajmaline challenge test to unmask DI-Type1-BrP. Genetic screening and analysis was performed in 55 of the cases (19 with and 36 without DI-Type1-BrP). RESULTS: Patients with AV-APs were significantly more likely than controls to have a Type1-BrP unmasked (16.1 vs 4.8%, P = 0.012). At baseline, patients with DI-Type1-BrP had higher prevalence of chest pain, QR/rSr' pattern in V1 and QRS notching/slurring in V2 and aVL during preexcitation, rSr' pattern in V1 -V2 , and QRS notching/slurring in aVL during orthodromic atrioventricular reentrant tachycardia (AVRT) compared to patients without DI-Type1-BrP. Abnormal QRS configuration (QRS notching/slurring and/or fragmentation) in V2 during preexcitation was present in all patients with DI-Type1 BrP. The prevalence of spontaneous preexcited atrial fibrillation (AF) and history of AF were similar (15% vs 18.3%, P = 0.726) in patients with and without DI-Type1-BrP, respectively. The prevalence of mutations in Brugada-susceptibility genes was higher (36.8% vs 8.3%, P = 0.02) in patients with DI-Type1-BrP compared to patients without DI-Type1-BrP. CONCLUSIONS: DI-Type1-BrP is relatively common in patients with AV-APs. We identify 12-lead ECG characteristics during preexcitation and orthodromic AVRT that point to an underlying type1-BrP, portending an increased probability for development of malignant arrhythmias.

Respuesta al ECG de octubre de 2017 / Response to ECG, October 2017

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Cambios en las propiedades de conducción de las vías accesorias: de preexcitación intermitente a fibrilación auricular preexcitada de riesgo / Changes in conduction properties of accessory pathways: from intermittent pre-excitation to rapid pre-excited ventricular response to atrial fibrillation

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