Asymptomatic Wolff-Parkinson-White Syndrome: An Ounce of Prevention Is Worth the Risk of Cure.

PURPOSE OF REVIEW: With increased electrocardiogram screening, asymptomatic preexcitation has become more prevalent. Historically, the asymptomatic-symptomatic dichotomy has directed management. This approach warrants scrutiny, as asymptomatic Wolff-Parkinson-White (WPW) syndrome is not without risk. Children may be unreliable symptom reporters, have atypical arrhythmia symptoms, yet have years to become symptomatic. RECENT FINDINGS: In a large WPW study, symptomatic patients were more likely to undergo ablation than asymptomatic patients, yet, except for symptoms, there were no differences in clinical or electrophysiology study (EPS) characteristics. Present data confirm real risk in asymptomatic WPW-sudden death can be the first symptom. Although malignant arrhythmias correlate better with EPS risk stratification than with symptoms, EPS data are imperfect predictors. Unlike adults with WPW, children have yet to prove survivorship. Asymptomatic children must be treated differently than adults. Sudden death risk is low but front-loaded in the young. An aggressive approach to asymptomatic WPW is warranted in this era of highly successful, low-risk catheter ablations.

The Pathophysiology, Diagnosis, and Management of Wolff-Parkinson-White Syndrome in the Neonate.

BACKGROUND: Wolff-Parkinson-White (WPW) is a congenital defect of the cardiac conduction system (CCS), with proliferation of extra embryologic conduction pathways and rapid conduction of electrical impulses. The estimated neonatal incidence of 0.1% to 0.2% may be misrepresented secondary to missed or misdiagnosis. Undiagnosed WPW can result in sudden cardiac death. PURPOSE: To discuss the pathogenesis, manifestations, diagnosis, management, and lifespan implications of WPW in the prenatal and postnatal periods. METHODS/SEARCH STRATEGY: A literature review was conducted using PubMed, CINAHL, and Google Scholar (2013-2019). Search terms included (newborn OR infant), wolff parkinson white, pathogenesis, management, and ventricular preexcitation. After removal of duplicates, 267 references were identified, abstracts reviewed, and 30 publications fully evaluated. FINDINGS/RESULTS: Separation of the heart chambers begins around 7 weeks' gestation with formation of the annulus fibrosis complete after term. The unknown external environmental influence on the development of the preterm infant's CCS places neonates at risk for persistent atrioventricular reentrant tachycardia with WPW development. Ensuring an appropriate diagnosis is crucial, as an incorrect diagnosis could mean death. IMPLICATIONS FOR PRACTICE: Due to the rarity of WPW, any fetal or neonatal supraventricular tachycardia requires further evaluation with an electrocardiogram and involvement of an experienced cardiologist for diagnosis. One episode of supraventricular tachycardia warrants evaluation for WPW, as recurring episodes may result in irreversible damage. IMPLICATIONS FOR RESEARCH: The recommendations for treatment of WPW in the prenatal and immediate postnatal periods are based heavily on standards of care for the adult population. A paucity of evidenced-based literature exists and future research is crucial to understand the true incidence, physiologic effects, and lifespan implications of WPW on neonates.

A case report of delayed diagnosis of danon disease: Caused by a newly recognized mutation in the lysosome-associated membrane protein-2 gene.

INTRODUCTION: Danon disease is a rare X-linked dominant genetic disorder caused by defects in the lysosome-associated membrane protein 2 (LAMP2) gene. Unless treated, cardiogenic death is the main cause of mortality. This case report describes a 19-year-old man who was diagnosed with Danon disease and survived for 3 years from symptom onset to death. The mutation in his LAMP2 gene (p.Gly221Ilefs*19) had not been previously reported. PATIENT CONCERNS: A 19-year-old man patient was hospitalized for intermittent palpitations. He had no family history of cardiomyopathy, arrhythmia, or sudden cardiac death, but his sister had died of cirrhosis at age 12 years, but the exact cause of cirrhosis was unknown. DIAGNOSIS: Exome sequencing and Sanger sequencing identified a novel missense mutation (p.Gly221Ilefs*19) in the LAMP2 gene of the proband. This mutation was also detected in his mother, confirming the diagnosis of Danon disease. INTERVENTIONS: The patient experienced various types of arrhythmia throughout the clinical process, including Wolff-Parkinson-White syndrome, non-sustained atrial tachycardia, atrial flutter, and third-degree atrioventricular block. He was therefore treated with cardiac ablation procedures and cardiac resynchronization therapy. OUTCOMES: The period from the onset of symptoms to the onset of heart failure was 2 years. The patient died of cardiogenic death during the third year, at age 22 years. LESSONS: Danon disease is a rare disease that is difficult to recognize because of its hidden early manifestations. Early identification of its clinical symptoms can lead to early diagnosis and treatment.

¿Vía accesoria aturdida o conducción intermitente? / Stunned accessory pathway or intermittent conduction?

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Concealed Wolff-Parkinson-White Syndrome revealed by acute coronary syndrome.

Wolff-Parkinson-White (WPW) syndrome is a conduction disturbance in which atrial impulses are transmitted to the ventricles by an accessory pathway instead of the normal atrioventricular conduction. The WPW syndrome may either simulate myocardial infarction or mask the electrocardiographic abnormalities of an acute MI. However, concealed WPW revealed after an acute coronary syndrome is rare with few cases reported in the literature. This article reports a case of coronary artery disease with ST-segment elevation in a 57-year-old man, previously asymptomatic, with an initial electrocardiogram showing no conduction abnormalities that subsequently presented with an ECG compatible with WPW.

Management of Asymptomatic Wolff-Parkinson-White Pattern by Pediatric Electrophysiologists.

OBJECTIVE: To determine the present-day approach of pediatric cardiac electrophysiologists to asymptomatic Wolff-Parkinson-White (WPW) pattern and to contrast to both published consensus statements and a similar survey. STUDY DESIGN: A questionnaire was sent to 266 Pediatric and Congenital Electrophysiology Society physician members in 25 countries; 21 questions from the 2003 survey were repeated, with new questions added regarding risk stratification and decision making. RESULTS: We received 113 responses from 13 countries, with responders having extensive electrophysiology experience (median 15 years [IQR 8.5-25 years]). Only 12 (11%) believed that intermittent pre-excitation and 37 (33%) that sudden loss of pre-excitation on exercise test were sufficient evidence of accessory pathway safety to avoid an invasive electrophysiology study. Optimal weight for electrophysiology study was 20 kg (IQR 18-22.5 kg), and 61% and 58% would then ablate all right-sided or left-sided accessory pathways, respectively, regardless of electrophysiological properties, whereas only 23% would ablate all septal accessory pathways (P < .001). Compared with 2003, respondents were more likely to consider inducible arrhythmia (77% vs 26%, P < .001) as sufficient indication alone for ablation. CONCLUSIONS: In the context of recent literature regarding the reliability of risk-stratification tools, most operators are now performing electrophysiology study for asymptomatic Wolff-Parkinson-White regardless of noninvasive findings. Many will then proceed to default ablation of all accessory pathways distant from critical conduction structures.

Management of Asymptomatic Wolff-Parkinson-White Pattern in Young Patients: Has Anything Changed?

The approach to pediatric asymptomatic Wolff-Parkinson-White (WPW) patients is controversial. The objective of this review is to update the last consensus of specialists of the Pediatric and Congenital Electrophysiology Society/Heart Rhythm Society on this subject in order to summarize the most recent evidence on the management of young patients with asymptomatic WPW pattern. A systematic review of the literature published between 2008 and 2018 was performed taking into account the protocol of the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) in PubMed (including Cochrane), Embase, and Web of Science. Observational, experimental, and multicentric studies were included. Out of a total of 37 articles selected, 4 were considered eligible. Most studies considered a cutoff age of 8 or greater as recommended in the 2012 consensus. The identification of a shortest pre-excitatory RR interval (SPERRI) ≤ 250 ms seems to be the best predictor for risk stratification. The importance of routine isoprenaline use to improve the sensitivity of the electrophysiological study to identify patients at high risk of sudden death was consensual. Prophylactic ablative therapy has been indicated in asymptomatic children with an accessory pathway (AP) who have a low SPERRI and/or a low effective anterograde period of the AP and/or multiple APs. Despite the evidence found in the most recent studies, more studies are warranted in this setting.

Cardiac electrophysiologic procedures - A ten years' experience at National Institute of Cardiovascular Diseases, Karachi.

OBJECTIVE: To review 10 years of clinical practice of cardiac electrophysiology study and radiofrequency catheter ablation in the treatment of supraventricular tachycardia. METHODS: The retrospective chart review was conducted at the National Institute of Cardiovascular Diseases, Karachi, and comprised records of all patients who underwent electrophysiological study and / or radiofrequency catheter ablation from January2007 to December 2016. SPSS 21 was used for data analysis. RESULTS: Of the 627 patients, 335(53.4%) were females. The overall mean age was 40.99}13.59 years. The major indication for procedure was supraventricular tachycardia 376(59.97%). Final electrophysiological study diagnosis was typical slow fast atrioventricular nodal re-entrant tachycardia in 303(48.3%) patients. The overall success rate was 472(75.3%). Procedure-related complications were reported in 25(4%) patients, and there was 1(0.15%) mortality. CONCLUSIONS: Cardiac electrophysiology studies and radiofrequency catheter ablation were found to be an effective and safe method for diagnosis and treatment of supraventricular tachycardia.

Pre-excitation induced ventricular dysfunction and successful berlin heart explantation after accessory pathway ablation.

A 13 kg, 20 month-old, Caucasian girl, presented with cardiomyopathy, biventricular dysfunction and pre-excitation on electrocardiogram. She had normal intracardiac anatomy with severely dilated left ventricle and severely diminished biventricular function (Fig. 1). She was treated with milrinone and epinephrine infusions, mechanical ventilation and listed for heart transplant. She underwent Berlin Heart EXCOR biventricular assist device (BiVAD) placement (30 ml LVAD and 25 ml RVAD pumps). No supraventricular tachycardia (SVT) was inducible or noted during her hospitalization. First ablation attempt without BiVAD support was unsuccessful; however, 18 days post BiVAD implantation, another electrophysiology study and successful radiofrequency ablation of a right anterolateral accessory pathway was performed on BiVAD support. After successful ablation and loss of pre-excitation, the cardiac dysfunction rapidly improved with initial improvement noted as early as 48 h after the successful ablation. Due to recovery of cardiac function, a BiVAD wean protocol was initiated and BiVAD explantation was performed 48 days after the implant (30 days after the successful ablation). To the best of our knowledge, this is the first report of successful BiVAD explantation.

Coexistence of Brugada and Wolff Parkinson White syndromes: A case report and review of the literature.

A 31-year-old male patient presented with complaints of palpitations, dizziness, and recurrent episodes of syncope. A 12-lead electrocardiogram (ECG) revealed manifest ventricular preexcitation, which suggested Wolff Parkinson White syndrome. In addition, an incomplete right bundle branch block and a 3-mm ST segment elevation ending with inverted T-waves in V2 were consistent with coved-type (type 1) Brugada pattern. An electrophysiological study was performed, and during the mapping, the earliest ventricular activation with the shortest A-V interval was found on the mitral annulus posterolateral site. After successful radiofrequency catheter ablation of the accessory pathway, the Brugada pattern on the ECG changed, which prompted an ajmaline provocation test. A type 1 Brugada ECG pattern occurred following the administration of ajmaline. Considering the probable symptom combinations of these 2 coexisting syndromes and the presence of recurrent episodes of syncope, programmed ventricular stimulation was performed and subsequently, ventricular fibrillation was induced. An implantable cardioverter-defibrillator was implanted soon after.

Prognostic Significance and Risk of Atrial Fibrillation of Wolff-Parkinson-White Syndrome in Patients With Hypertrophic Cardiomyopathy.

To assess the mid-term mortality and risk of atrial fibrillation (AF) in patients with hypertrophic cardiomyopathy (HC) and Wolff-Parkinson-White (WPW) syndrome, 40 patients with HC and WPW were enrolled in our center between 2010 and 2017. An age- and gender-matched comparison cohort of patients with HC without WPW (n = 160) was generated from the same center. The clinical profile and outcomes were assessed. Of 40 patients with WPW, 28 underwent accessory pathway (AP) elimination. Two patients (7%) had failed in AP elimination. During mid-term follow-up, 1 patient had an implantable cardioverter-defibrillator intervention. Fourteen patients had AF. A previous history of AF (hazard ratio [HR]: 4.69; 95% confidence interval [CI] 1.51 to 14.63) and left atrial dimension (HR: 1.12; 95% CI 1.03 to 1.23) at baseline were risk factors for AF occurrence during follow-up. The AP elimination significantly reduced risk for the incidence of AF (HR: 0.22; 95% CI 0.06 to 0.83). Compared with the control group, the prevalence of syncope and AF were significantly higher in the WPW group. During follow-up, no difference was identified in outcome measures consisting of all-cause death, cardiac transplantation, and implantable cardioverter-defibrillator intervention. A previous history of AF (HR: 5.20; 95% CI 2.63 to 10.30, p <0.001) and persistent existing WPW (HR: 3.64; 95% CI 1.63 to 8.11, p = 0.002) were independent risk factors for AF occurrence during follow-up in the entire cohort. In conclusion, although WPW was uncommon and might not be correlated with mid-term mortality in HC patients, WPW might increase the risk of AF occurrence. Additionally, AP elimination may reduce the risk of AF occurrence.

[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.

When a Pseudo-Infarct Electrocardiogram (ECG) Pattern in a Posterior Accessory (Wolff-Parkinson-White) Pathway Masks a True Inferior Infarct.

BACKGROUND Wolff-Parkinson-White (WPW) pattern is due to a pre-excitation leading to characteristic ECG changes in sinus rhythm as short PR interval, the presence of delta waves, wide QRS complexes, and potentially Q wave-T wave vector discordance (pseudo-infarct pattern). These later changes can mask the underlying ECG depolarizing solely through the His-Purkinje system. Our case highlights how the ECG of a WPW pattern with a pseudo-infarct pattern can in fact mask a true infarct on the underlying ECG without pre-excitation. CASE REPORT A 61-year-old diabetic man with a recent history of supra-ventricular tachycardia (SVT) presented with the ECG characteristic of a Wolff-Parkinson-White pattern i-e short PR interval of 0.10 s (<0.12 s) and the presence of delta waves in sinus rhythm. In addition, there was a wide significant Q wave in the inferior leads meeting the criteria for significant and pathologic Q waves, related to the pre-excitation and known as a pseudo-infarct pattern. The patient underwent successful ablation of his left inferoseptal accessory pathway. The pre-excitation pattern (short PR and delta wave) disappeared after successful ablation revealed a narrower Q wave in inferior leads, likely from unexpected true old inferior infarction, which was later confirmed by 2D echocardiogram and nuclear stress test (fixed inferior defect). CONCLUSIONS The presence of pseudo-infarct pattern due to a WPW does not always preclude the presence of underlying true infarct pattern, especially in the presence of coronary artery disease risk factors.

Reentry Tachycardia in Children: Adenosine Can Make It Worse.

OBJECTIVES: We report on a rare but severe complication of adenosine use in a child with reentry tachycardia. METHODS AND RESULTS: Treatment with adenosine, which is the standard medical therapy of atrioventricular reentry tachycardia, led to the development of an irregular wide complex tachycardia, caused by rapid ventricular response to atrial fibrillation. The girl was finally stabilized with electrical cardioversion. We analyze the pathomechanism and discuss possible treatment options. CONCLUSIONS: Atrial fibrillation, as well as its conduction to the ventricles, can be caused by adenosine. Rapid ventricular response in children with Wolff-Parkinson-White syndrome is more frequent than previously believed. A patient history of atrial fibrillation is a contraindication for cardioversion with adenosine and needs to be assessed in children with reentry tachycardia. High-risk patients may potentially profit from prophylactic comedication with antiarrhythmic agents, such as flecainide, ibutilide, or vernakalant, before adenosine administration.

Papillary Muscle Rupture in an Adolescent with No Coronary Lesions.

A 21-year-old man with Wolff-Parkinson-White syndrome presented to the authors' hospital with ventricular fibrillation. Coronary angiography failed to demonstrate coronary stenosis, but temporary mechanical circulatory support resolved the ventricular fibrillation and the patient was extubated eight days later. On the next day, however, he had to be re-intubated with symptoms of congestive heart failure. Echocardiography revealed new severe mitral regurgitation and a mobile mass, while emergency surgery revealed a posteromedial papillary muscle rupture (PMR). The mitral regurgitation was repaired with ruptured papillary muscle relocation, artificial chordae implantation, and ring annuloplasty. Postoperative examinations suggested that an arrhythmia-induced coronary circulation hypoperfusion and septic embolization had caused the PMR.

Long term risk of Wolff-Parkinson-White pattern and syndrome.

For years, conventional wisdom has held that patients with asymptomatic ventricular pre-excitation (asymptomatic WPW or WPW pattern) were at low risk for adverse outcomes. This assumption has been challenged more recently in a number of observational/natural history studies as well as in prospective trials in which patients were more aggressively studied via invasive electrophysiology study (EPS) and more aggressively treated, in some cases, with pre-emptive catheter ablation, despite the lack of symptoms. In sum, the data do not definitively support one approach (early, up-stream EPS and/or ablation) vs. the other (watchful waiting with close monitoring). The most recent pediatric and adult guidelines reflect this ambiguity with a broad spectrum of approaches endorsed.