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

Abstract

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 Rare and Challenging Case of Refractory Fetal Supraventricular Tachycardia

“…AVRT, antidromic or orthodromic, is typically the presenting arrhythmia of WPW. Clinical manifestations of WPW can be recognized in utero, however, most cases are not diagnosed until early childhood [ 5 ]. A fetus may present with episodes of SVT which can result in hydrops fetalis depending on the duration and severity of the tachyarrhythmia.…”

“…In addition to causing supraventricular tachyarrhythmias, WPW can lead to cardiac arrest and even sudden cardiac death; therefore, early diagnosis and treatment is essential. WPW has an incidence of 0.1%-0.2% among neonates, and up to 37% of infants diagnosed with WPW also have a congenital cardiac defect [ 5 ].…”

A Neonate Diagnosed with Wolff-Parkinson-White Syndrome Presenting with Cardiogenic Shock

“…In addition, WPW syndrome is rare, with an incidence rate of 0.1% to 0.2% among neonates; however, the first manifestation may be shock, requiring immediate attention 1,2) . Furthermore, 50% of young patients with WPW syndrome with cardiac arrest had no previous cardiac symptoms 1,2) . Depending on the duration of SVT, symptoms can range from asymptomatic presentation to cardiogenic shock.…”

“…In neonates, arrhythmia is considered benign. However, if the HR is consistently >180 bpm at rest, there may be hemodynamic instability; hence, ECG should be performed immediately 1) . ECG is the gold standard for the diagnosis of WPW syndrome.…”

“…Up to 37% of infants diagnosed with WPW syndrome may have congenital heart malformations, such as Ebstein anomaly, congenitally corrected transposition of the great arteries, and ventricular septal defects. In 18%, the patients with cardiac rhabdomyomas associated with tuberous sclerosis are diagnosed as WPW syndrome 1) . Congenital heart disease can cause severe hypotension or cardiac arrest during SVT.…”