Wolff–Parkinson–White Syndrome in a Term Infant Presenting With Cardiopulmonary Arrest

Hoeffler, Christina D.; Krenek, Michele; Brand, M Colleen

doi:10.1097/anc.0000000000000246

Cited by 7 publications

(55 citation statements)

References 15 publications

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“…Particular care must be taken with infants, in whom it is challenging to determine the absence of symptoms and therefore, to determine the real risk by the clinical history alone remains a dilemma. Remarkably, the incidence of SCD seems to be higher in pediatric than adult patients (1.93 versus 0.86 per 1,000 person-years) 11 , and SCD may be the initial symptom in up to 53% of cases [5][6][7][8]14,15 . Also, it is essential to bear in mind that the unavailability of ECG during resuscitation could underestimate the low incidence of infants presenting with SCD.…”

Section: Real Risk Of Sudden Cardiac Death In Asymptomatic Patientsmentioning

confidence: 98%

“…A randomised clinical trial that evaluated the results of prophylactic catheter ablation in children (aged 5-12 years) with AWPWS showed that the absence of prophylactic ablation was an independent predictor of arrhythmic events 12 . However, EPS is an invasive procedure with a risk of complications (5%-15%), with major ones reported in 0.9%-4.2% (death 0.12%), being higher in infants less than 15kg of weight or 18 months of age [5][6][7][8][16][17][18][19] . Also, prolonged exposure to radiation and high recurrence rates of arrhythmia after successful procedures (7%-17%) are of particular concern [5][6][7][8][16][17][18][19] .…”

Section: Invasive Risk-stratificationmentioning

confidence: 99%

“…However, EPS is an invasive procedure with a risk of complications (5%-15%), with major ones reported in 0.9%-4.2% (death 0.12%), being higher in infants less than 15kg of weight or 18 months of age [5][6][7][8][16][17][18][19] . Also, prolonged exposure to radiation and high recurrence rates of arrhythmia after successful procedures (7%-17%) are of particular concern [5][6][7][8][16][17][18][19] . So, uniform referral of every infant for an EPS or RFA could result in severe and potentially life-threatening complications, that possibly surpass the number of deaths caused by untreated disease, and usually AWPWS infants (less than 15 Kg) are not considered to be an indication for invasive risk stratification or RFA 21 .…”

Section: Invasive Risk-stratificationmentioning

confidence: 99%

“…Remarkably, sudden cardiac death (SCD) may occur and may be the initial symptom in these patients [5][6][7][8] . The assumed mechanism is ventricular fibrillation (VF) secondary to rapid stimulation of the ventricles due to atrial fibrillation (AF) rapidly conducted through the AP.…”

Section: Introductionmentioning

confidence: 99%

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Sudden Cardiac Death as the First Clinical Manifestation in Infants With Asymptomatic Ventricular Pre-excitation: Two Case Reports and Review of the Literature

2019

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Introduction: The real risk of sudden cardiac death (SCD) in asymptomatic Wolff-Parkinson-White syndrome (AWPWS) is still not well known, and controversial literature is found about the best management strategy. Most worrisome is that SCD has been reported as the first event in asymptomatic or undiagnosed AWPWS infants. So adequate risk stratification to prevent the occurrence of life-threatening arrhythmias is warranted in these patients, but none of the available tests for this is a good option. Case report: We report 2 cases of AWPW infants that experienced SCD as the first clinical manifestation. Conclussion: AWPWS in infants is a non-rare and challenging condition that implies a very low but real risk of SCD, which is very difficult to determine accurately with diagnostic methods currently available. In this article we review the literature about the subject andand discuss about the adequate management of these patients.

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Section: Real Risk Of Sudden Cardiac Death In Asymptomatic Patientsmentioning

confidence: 98%

Section: Invasive Risk-stratificationmentioning

confidence: 99%

Section: Invasive Risk-stratificationmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Sudden Cardiac Death as the First Clinical Manifestation in Infants With Asymptomatic Ventricular Pre-excitation: Two Case Reports and Review of the Literature

2019

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“…Pulmonary valve or supravalvular pulmonary stenosis have been well described with RASopathies, such as Noonan and Casitas B‐cell lymphoma (CBL) syndrome, which are among the more common genetic syndromes underlying NIHF . Cardiac arrhythmias may also lead to NIHF, with genetic etiologies including Costello, Barth, long QT, and Wolff‐Parkinson White syndromes . More rarely reported vascular disorders associated with NIHF include Klippel‐Trenaunay‐Weber syndrome, RASA1 ‐related disorders, and PIK3CA ‐associated segmental overgrowth syndromes .…”

Section: Genetic Causes Of Nihf By Organ Systemmentioning

confidence: 99%

A system‐based approach to the genetic etiologies of non‐immune hydrops fetalis

et al. 2019

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A wide spectrum of genetic causes may lead to nonimmune hydrops fetalis (NIHF), and a thorough phenotypic and genetic evaluation are essential to determine the underlying etiology, optimally manage these pregnancies, and inform discussions about anticipated prognosis. In this review, we outline the known genetic etiologies of NIHF by fetal organ system affected, and provide a systematic approach to the evaluation of NIHF. Some of the underlying genetic disorders are associated with characteristic phenotypic features that may be seen on prenatal ultrasound, such as hepatomegaly with lysosomal storage disorders, hyperechoic kidneys with congenital nephrosis, or pulmonary valve stenosis with RASopathies. However, this is not always the case, and the approach to evaluation must include prenatal ultrasound findings as well as genetic testing and many other factors. Genetic testing that has been utilized for NIHF ranges from standard chromosomal microarray or karyotype to gene panels and broad approaches such as whole exome sequencing. Family and obstetric history, as well as pathology examination, can yield additional clues that are helpful in establishing a diagnosis. A systematic approach to evaluation can guide a more targeted approach to genetic evaluation, diagnosis, and management of NIHF.

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

Chambers

Newberry

2020

Advances in Neonatal Care

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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.

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Wolff–Parkinson–White Syndrome in a Term Infant Presenting With Cardiopulmonary Arrest

Cited by 7 publications

References 15 publications

Sudden Cardiac Death as the First Clinical Manifestation in Infants With Asymptomatic Ventricular Pre-excitation: Two Case Reports and Review of the Literature

Sudden Cardiac Death as the First Clinical Manifestation in Infants With Asymptomatic Ventricular Pre-excitation: Two Case Reports and Review of the Literature

A system‐based approach to the genetic etiologies of non‐immune hydrops fetalis

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

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