Susan P Etheridge1, Carolina A Escudero2, Andrew D Blaufox3, Ian H Law4, Brynn E Dechert-Crooks5, Elizabeth A Stephenson6, Anne M Dubin7, Scott R Ceresnak7, Kara S Motonaga7, Jonathan R Skinner8, Luciana D Marcondes8, James C Perry9, Kathryn K Collins10, Stephen P Seslar11, Michel Cabrera12, Orhan Uzun13, Bryan C Cannon14, Peter F Aziz15, Peter Kubuš16, Ronn E Tanel17, Santiago O Valdes18, Sara Sami18, Naomi J Kertesz19, Jennifer Maldonado4, Christopher Erickson20, Jeremy P Moore21, Hiroko Asakai6, LuAnn Mill20, Mark Abcede9, Zebulon Z Spector11, Shaji Menon22, Mark Shwayder22, David J Bradley5, Mitchell I Cohen23, Shubhayan Sanatani24. 1. Division of Cardiology, Department of Pediatrics, Primary Children's Hospital, University of Utah, Salt Lake City, Utah. Electronic address: susan.etheridge@hsc.utah.edu. 2. Division of Cardiology, Department of Pediatrics, Stollery Children's Hospital, University of Alberta, Edmonton, Alberta, Canada. 3. Division of Pediatric Cardiology, Department of Pediatrics, Cohen Children's Medical Center of New York, Hofstra-Northwell School of Medicine, New Hyde Park, New York. 4. Department of Pediatrics, Division of Cardiology, Stead Family Children's Hospital, University of Iowa, Iowa City, Iowa. 5. Division of Cardiology, Department of Pediatrics, University of Michigan Children's Hospital, University of Michigan, Ann Arbor, Michigan. 6. Labatt Family Heart Centre, Hospital for Sick Children, Toronto, Ontario, Canada. 7. Division of Pediatric Cardiology, Department of Pediatrics, Lucile Packard Children's Hospital, Stanford University, Palo Alto, California. 8. Greenlane Paediatric and Congenital Cardiac Service, Starship Children's Hospital, University of Auckland, Auckland, New Zealand. 9. Cardiology Division, Department of Pediatrics, Rady Children's Hospital, University of California San Diego, San Diego, California. 10. Division of Cardiology, Children's Hospital Colorado, University of Colorado, Aurora, Colorado. 11. Division of Pediatric Cardiology, Department of Pediatrics, Seattle Children's Hospital, Seattle, Washington. 12. Cardiocentro Pediatrico William Soler, Havana, Cuba. 13. Department of Paediatric Cardiology, University Hospital of Wales, Cardiff, Wales, United Kingdom. 14. Department of Pediatrics, Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota. 15. Division of Pediatric Cardiology, Cleveland Clinic Foundation, Cleveland, Ohio. 16. Children's Heart Centre, Charles University and Motol University Hospital, Prague, Czech Republic. 17. Department of Pediatrics, Benioff Children's Hospital, University of California San Francisco, San Francisco, California. 18. Division of Pediatric Cardiology, Texas Children's Hospital, Baylor College of Medicine, Houston Texas. 19. Nationwide Children's Hospital, Columbus, Ohio. 20. Division of Pediatric Cardiology in the Department of Pediatrics, Children's Hospital and Medical Center, Omaha, Nebraska. 21. Department of Pediatrics, Division of Pediatric Cardiology, UCLA Health System, University of California Los Angeles, Los Angeles, California. 22. Division of Cardiology, Department of Pediatrics, Primary Children's Hospital, University of Utah, Salt Lake City, Utah. 23. Phoenix Children's Hospital, University of Arizona College of Medicine, Phoenix, Arizona. 24. Division of Cardiology, Department of Pediatrics, British Columbia Children's Hospital, Vancouver, British Columbia, Canada.
Abstract
OBJECTIVES: This study sought to characterize risk in children with Wolff-Parkinson-White (WPW) syndrome by comparing those who had experienced a life-threatening event (LTE) with a control population. BACKGROUND: Children with WPW syndrome are at risk of sudden death. METHODS: This retrospective multicenter pediatric study identified 912 subjects ≤21 years of age with WPW syndrome, using electrophysiology (EPS) studies. Case subjects had a history of LTE: sudden death, aborted sudden death, or atrial fibrillation (shortest pre-excited RR interval in atrial fibrillation [SPERRI] of ≤250 ms or with hemodynamic compromise); whereas subjects did not. We compared clinical and EPS data between cases and subjects. RESULTS: Case subjects (n = 96) were older and less likely than subjects (n = 816) to have symptoms or documented tachycardia. Mean age at LTE was 14.1 ± 3.9 years of age. The LTE was the sentinel symptom in 65%, consisting of rapidly conducted pre-excited atrial fibrillation (49%), aborted sudden death (45%), and sudden death (6%). Three risk components were considered at EPS: SPERRI, accessory pathway effective refractory period (APERP), and shortest paced cycle length with pre-excitation during atrial pacing (SPPCL), and all were shorter in cases than in control subjects. In multivariate analysis, risk factors for LTE included male sex, Ebstein malformation, rapid anterograde conduction (APERP, SPERRI, or SPPCL ≤250 ms), multiple pathways, and inducible atrial fibrillation. Of case subjects, 60 of 86 (69%) had ≥2 EPS risk stratification components performed; 22 of 60 (37%) did not have EPS-determined high-risk characteristics, and 15 of 60 (25%) had neither concerning pathway characteristics nor inducible atrioventricular reciprocating tachycardia. CONCLUSIONS: Young patients may experience LTE from WPW syndrome without prior symptoms or markers of high-risk on EPS.
OBJECTIVES: This study sought to characterize risk in children with Wolff-Parkinson-White (WPW) syndrome by comparing those who had experienced a life-threatening event (LTE) with a control population. BACKGROUND:Children with WPW syndrome are at risk of sudden death. METHODS: This retrospective multicenter pediatric study identified 912 subjects ≤21 years of age with WPW syndrome, using electrophysiology (EPS) studies. Case subjects had a history of LTE: sudden death, aborted sudden death, or atrial fibrillation (shortest pre-excited RR interval in atrial fibrillation [SPERRI] of ≤250 ms or with hemodynamic compromise); whereas subjects did not. We compared clinical and EPS data between cases and subjects. RESULTS: Case subjects (n = 96) were older and less likely than subjects (n = 816) to have symptoms or documented tachycardia. Mean age at LTE was 14.1 ± 3.9 years of age. The LTE was the sentinel symptom in 65%, consisting of rapidly conducted pre-excited atrial fibrillation (49%), aborted sudden death (45%), and sudden death (6%). Three risk components were considered at EPS: SPERRI, accessory pathway effective refractory period (APERP), and shortest paced cycle length with pre-excitation during atrial pacing (SPPCL), and all were shorter in cases than in control subjects. In multivariate analysis, risk factors for LTE included male sex, Ebstein malformation, rapid anterograde conduction (APERP, SPERRI, or SPPCL ≤250 ms), multiple pathways, and inducible atrial fibrillation. Of case subjects, 60 of 86 (69%) had ≥2 EPS risk stratification components performed; 22 of 60 (37%) did not have EPS-determined high-risk characteristics, and 15 of 60 (25%) had neither concerning pathway characteristics nor inducible atrioventricular reciprocating tachycardia. CONCLUSIONS: Young patients may experience LTE from WPW syndrome without prior symptoms or markers of high-risk on EPS.
Authors: Sushma Reddy; Bradley S Marino; Carissa M Baker-Smith; Andrea Beaton; Catherine D Krawczeski; Christina Y Miyake; James F Cnota; Andrew C Glatz; Brian Feingold; Jennifer C Romano; Antonio G Cabrera; Anitha S John; Meryl S Cohen Journal: J Am Heart Assoc Date: 2018-11-06 Impact factor: 5.501