Author + information
- Received August 16, 2017
- Revision received October 3, 2017
- Accepted October 12, 2017
- Published online April 16, 2018.
- Susan P. Etheridge, MDa,∗ (, )
- Carolina A. Escudero, MDb,
- Andrew D. Blaufox, MDc,
- Ian H. Law, MDd,
- Brynn E. Dechert-Crooks, RN, MSNe,
- Elizabeth A. Stephenson, MDf,
- Anne M. Dubin, MDg,
- Scott R. Ceresnak, MDg,
- Kara S. Motonaga, MDg,
- Jonathan R. Skinner, MBChB, MDh,
- Luciana D. Marcondes, MDh,
- James C. Perry, MDi,
- Kathryn K. Collins, MDj,
- Stephen P. Seslar, MDk,
- Michel Cabrera, MDl,
- Orhan Uzun, MDm,
- Bryan C. Cannon, MDn,
- Peter F. Aziz, MDo,
- Peter Kubuš, MDp,
- Ronn E. Tanel, MDq,
- Santiago O. Valdes, MDr,
- Sara Sami, MDr,
- Naomi J. Kertesz, MDs,
- Jennifer Maldonado, MBA, CCRPd,
- Christopher Erickson, MDt,
- Jeremy P. Moore, MDu,
- Hiroko Asakai, MDf,
- LuAnn Mill, RN, BSNt,
- Mark Abcede, MBA, CCRPi,
- Zebulon Z. Spector, MDk,
- Shaji Menon, MDa,
- Mark Shwayder, MDa,
- David J. Bradley, MDe,
- Mitchell I. Cohen, MDv and
- Shubhayan Sanatani, MDw
- aDivision of Cardiology, Department of Pediatrics, Primary Children’s Hospital, University of Utah, Salt Lake City, Utah
- bDivision of Cardiology, Department of Pediatrics, Stollery Children’s Hospital, University of Alberta, Edmonton, Alberta, Canada
- cDivision of Pediatric Cardiology, Department of Pediatrics, Cohen Children’s Medical Center of New York, Hofstra-Northwell School of Medicine, New Hyde Park, New York
- dDepartment of Pediatrics, Division of Cardiology, Stead Family Children’s Hospital, University of Iowa, Iowa City, Iowa
- eDivision of Cardiology, Department of Pediatrics, University of Michigan Children’s Hospital, University of Michigan, Ann Arbor, Michigan
- fLabatt Family Heart Centre, Hospital for Sick Children, Toronto, Ontario, Canada
- gDivision of Pediatric Cardiology, Department of Pediatrics, Lucile Packard Children’s Hospital, Stanford University, Palo Alto, California
- hGreenlane Paediatric and Congenital Cardiac Service, Starship Children’s Hospital, University of Auckland, Auckland, New Zealand
- iCardiology Division, Department of Pediatrics, Rady Children’s Hospital, University of California San Diego, San Diego, California
- jDivision of Cardiology, Children’s Hospital Colorado, University of Colorado, Aurora, Colorado
- kDivision of Pediatric Cardiology, Department of Pediatrics, Seattle Children’s Hospital, Seattle, Washington
- lCardiocentro Pediatrico William Soler, Havana, Cuba
- mDepartment of Paediatric Cardiology, University Hospital of Wales, Cardiff, Wales, United Kingdom
- nDepartment of Pediatrics, Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota
- oDivision of Pediatric Cardiology, Cleveland Clinic Foundation, Cleveland, Ohio
- pChildren's Heart Centre, Charles University and Motol University Hospital, Prague, Czech Republic
- qDepartment of Pediatrics, Benioff Children’s Hospital, University of California San Francisco, San Francisco, California
- rDivision of Pediatric Cardiology, Texas Children’s Hospital, Baylor College of Medicine, Houston Texas
- sNationwide Children’s Hospital, Columbus, Ohio
- tDivision of Pediatric Cardiology in the Department of Pediatrics, Children’s Hospital and Medical Center, Omaha, Nebraska
- uDepartment of Pediatrics, Division of Pediatric Cardiology, UCLA Health System, University of California Los Angeles, Los Angeles, California
- vPhoenix Children’s Hospital, University of Arizona College of Medicine, Phoenix, Arizona
- wDivision of Cardiology, Department of Pediatrics, British Columbia Children’s Hospital, Vancouver, British Columbia, Canada
- ↵∗Address for correspondence:
Dr. Susan P. Etheridge, University of Utah and Primary Children’s Hospital, Pediatrics, 81 North Mario Capecchi Drive, Salt Lake City, Utah 84113.
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.
Dr. Kubuš is supported by the Ministry of Health, Czech Republic (MHCZ-DRO), University Hospital Motol, Prague, Czech Republic 00064203. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.
All authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the JACC: Clinical Electrophysiology author instructions page.
- Received August 16, 2017.
- Revision received October 3, 2017.
- Accepted October 12, 2017.
- 2018 American College of Cardiology Foundation