Seshadri Balaji1, Michael P DiLorenzo2, Frank A Fish3, Susan P Etheridge4, Peter F Aziz5, Mark W Russell6, Svjetlana Tisma7, Andreas Pflaumer8, Narayanswami Sreeram9, Peter Kubus10, Ian H Law11, Michal J Kantoch12, Naomi J Kertesz13, Margaret Strieper14, Christopher C Erickson15, Jeremy P Moore16, Stephanie J Nakano17, Harinder R Singh18, Philip Chang19, Mitchell Cohen20, Anne Fournier21, Maria V Ilina22, Richard T Smith23, Frank Zimmerman24, Michaela Horndasch25, Walter Li26, Anjan Batra27, Leonardo Liberman28, Robert Hamilton29, Christopher M Janson30, Shubhayan Sanatani31, Ilana Zeltser32, George McDaniel33, Andrew D Blaufox34, Jason M Garnreiter35, Hannah Katcoff2, Maully Shah2. 1. Oregon Health & Science University, Portland, Oregon. Electronic address: balajis@ohsu.edu. 2. Children's Hospital of Philadelphia, Philadelphia, Pennsylvania. 3. Vanderbilt University, Nashville, Tennessee. 4. University of Utah, Salt Lake City, Utah. 5. Cleveland Clinic, Cleveland, Ohio. 6. C.S. Mott Children's Hospital, Ann Arbor, Michigan. 7. Mercy Children's Hospital, Kansas City, Missouri. 8. Royal Children's Hospital, Melbourne, Australia. 9. University of Cologne, Cologne, Germany. 10. Motol University, Prague, Czech Republic. 11. University of Iowa, Iowa City, Iowa. 12. University of Alberta, Edmonton, Alberta, Canada. 13. Nationwide Children's Hospital, Columbus, Ohio. 14. Sibley Heart Center, Atlanta, Georgia. 15. Children's Hospital, Omaha, Nebraska. 16. University of California, Los Angeles, Los Angeles, California. 17. University of Colorado, Denver, Colorado. 18. Children's Hospital of Michigan, Detroit, Michigan. 19. Children's Hospital of Los Angeles, Los Angeles, California. 20. Phoenix Children's Hospital, Phoenix, Arizona. 21. University of Montreal, Montreal, Quebec, Canada. 22. Royal Children's Hospital, Glasgow, Scotland, United Kingdom. 23. Carolinas Health System, Charlotte, North Carolina. 24. Advocate Hospital, Chicago, Illinois. 25. German Heart Center, Munich, Germany. 26. University of California, San Francisco, San Francisco, California. 27. University of California, Irvine, Irvine, California. 28. Columbia University, New York, New York. 29. Hospital for Sick Kids, Toronto, Ontario, Canada. 30. Montefiore Hospital, New York, New York. 31. University of British Columbia, Vancouver, British Columbia, Canada. 32. University of Texas-Southwestern, Dallas, Texas. 33. University of Virginia, Charlottesville, Virginia. 34. Zucker School of Medicine at Hofstra/Northwell, Hempstead, New York. 35. Saint Louis University, Saint Louis, Missouri.
Abstract
BACKGROUND: Predictors of risk of lethal arrhythmic events (LAE) is poorly understood and may differ from adults in children with hypertrophic cardiomyopathy (HCM). OBJECTIVE: The purpose of this study was to determine predictors of LAE in children with HCM. METHODS: A retrospective data collection was performed on 446 children and teenagers 20 years and younger (290 [65%] male; mean age 10.1 ± 5.7 years) with idiopathic HCM from 35 centers. Patients were classified as group 1 (HCM with LAE) if having a secondary prevention implantable cardioverter-defibrillator (ICD) or primary prevention ICD with appropriate interventions or group 2 (HCM without LAE) if having a primary prevention ICD without appropriate interventions. RESULTS: There were 152 children (34%) in group 1 and 294 (66%) in group 2. Risk factors for group 1 by univariate analysis were septal thickness, posterior left ventricular (LV) wall thickness, lower LV outflow gradient, and Q wave > 3 mm in inferior electrocardiographic leads. Factors not associated with LAE were family history of SCD, abnormal blood pressure response to exercise, and ventricular tachycardia on ambulatory electrocardiographic monitoring. Risk factors for SCD by multivariate analysis were age at ICD placement (hazard ratio [HR] 0.9; P = .0025), LV posterior wall thickness z score (HR 1.02; P < .005), and LV outflow gradient < 30 mm Hg (HR 2.0; P < .006). LV posterior wall thickness z score ≥ 5 was associated with LAE. CONCLUSION: Risk factors for LAE appear different in children compared to adults. Conventional adult risk factors were not significant in children. Further prospective studies are needed to improve risk stratification for LAE in children with HCM.
BACKGROUND: Predictors of risk of lethal arrhythmic events (LAE) is poorly understood and may differ from adults in children with hypertrophic cardiomyopathy (HCM). OBJECTIVE: The purpose of this study was to determine predictors of LAE in children with HCM. METHODS: A retrospective data collection was performed on 446 children and teenagers 20 years and younger (290 [65%] male; mean age 10.1 ± 5.7 years) with idiopathic HCM from 35 centers. Patients were classified as group 1 (HCM with LAE) if having a secondary prevention implantable cardioverter-defibrillator (ICD) or primary prevention ICD with appropriate interventions or group 2 (HCM without LAE) if having a primary prevention ICD without appropriate interventions. RESULTS: There were 152 children (34%) in group 1 and 294 (66%) in group 2. Risk factors for group 1 by univariate analysis were septal thickness, posterior left ventricular (LV) wall thickness, lower LV outflow gradient, and Q wave > 3 mm in inferior electrocardiographic leads. Factors not associated with LAE were family history of SCD, abnormal blood pressure response to exercise, and ventricular tachycardia on ambulatory electrocardiographic monitoring. Risk factors for SCD by multivariate analysis were age at ICD placement (hazard ratio [HR] 0.9; P = .0025), LV posterior wall thickness z score (HR 1.02; P < .005), and LV outflow gradient < 30 mm Hg (HR 2.0; P < .006). LV posterior wall thickness z score ≥ 5 was associated with LAE. CONCLUSION: Risk factors for LAE appear different in children compared to adults. Conventional adult risk factors were not significant in children. Further prospective studies are needed to improve risk stratification for LAE in children with HCM.
Authors: Gabrielle Norrish; Henry Chubb; Ella Field; Karen McLeod; Maria Ilina; Georgia Spentzou; Jan Till; Piers E F Daubeney; Alan Graham Stuart; Jane Matthews; Dominic Hares; Elspeth Brown; Katie Linter; Vinay Bhole; Krishnakumar Pillai; Michael Bowes; Caroline B Jones; Orhan Uzun; Amos Wong; Arthur Yue; Shankar Sadagopan; Tara Bharucha; Norah Yap; Eric Rosenthal; Sujeev Mathur; Satish Adwani; Zdenka Reinhardt; Jasveer Mangat; Juan Pablo Kaski Journal: Europace Date: 2021-03-08 Impact factor: 5.214
Authors: Robert D Whitehill; Seshadri Balaji; Michael Kelleman; Stephanie F Chandler; Dominic J Abrams; Chad Mao; Peter Fischbach; Robert Campbell Journal: Pediatr Cardiol Date: 2021-08-18 Impact factor: 1.655
Authors: Gabrielle Norrish; Tao Ding; Ella Field; Elena Cervi; Lidia Ziółkowska; Iacopo Olivotto; Diala Khraiche; Giuseppe Limongelli; Aris Anastasakis; Robert Weintraub; Elena Biagini; Luca Ragni; Terrence Prendiville; Sophie Duignan; Karen McLeod; Maria Ilina; Adrián Fernández; Chiara Marrone; Regina Bökenkamp; Anwar Baban; Peter Kubus; Piers E F Daubeney; Georgia Sarquella-Brugada; Sergi Cesar; Sabine Klaassen; Tiina H Ojala; Vinay Bhole; Constancio Medrano; Orhan Uzun; Elspeth Brown; Ferran Gran; Gianfranco Sinagra; Francisco J Castro; Graham Stuart; Gabriele Vignati; Hirokuni Yamazawa; Roberto Barriales-Villa; Luis Garcia-Guereta; Satish Adwani; Katie Linter; Tara Bharucha; Pablo Garcia-Pavia; Ana Siles; Torsten B Rasmussen; Margherita Calcagnino; Caroline B Jones; Hans De Wilde; Toru Kubo; Tiziana Felice; Anca Popoiu; Jens Mogensen; Sujeev Mathur; Fernando Centeno; Zdenka Reinhardt; Sylvie Schouvey; Costas O'Mahony; Rumana Z Omar; Perry M Elliott; Juan Pablo Kaski Journal: Circ Arrhythm Electrophysiol Date: 2022-05-02
Authors: Anastasia Miron; Myriam Lafreniere-Roula; Chun-Po Steve Fan; Katey R Armstrong; Andreea Dragulescu; Tanya Papaz; Cedric Manlhiot; Beth Kaufman; Ryan J Butts; Letizia Gardin; Elizabeth A Stephenson; Taylor S Howard; Pete F Aziz; Seshadri Balaji; Virginie Beauséjour Ladouceur; Lee N Benson; Steven D Colan; Justin Godown; Heather T Henderson; Jodie Ingles; Aamir Jeewa; John L Jefferies; Ashwin K Lal; Jacob Mathew; Emilie Jean-St-Michel; Michelle Michels; Stephanie J Nakano; Iacopo Olivotto; John J Parent; Alexandre C Pereira; Christopher Semsarian; Robert D Whitehill; Samuel G Wittekind; Mark W Russell; Jennifer Conway; Marc E Richmond; Chet Villa; Robert G Weintraub; Joseph W Rossano; Paul F Kantor; Carolyn Y Ho; Seema Mital Journal: Circulation Date: 2020-05-18 Impact factor: 29.690
Authors: Maully J Shah; Michael J Silka; Jennifer N Avari Silva; Seshadri Balaji; Cheyenne M Beach; Monica N Benjamin; Charles I Berul; Bryan Cannon; Frank Cecchin; Mitchell I Cohen; Aarti S Dalal; Brynn E Dechert; Anne Foster; Roman Gebauer; M Cecilia Gonzalez Corcia; Prince J Kannankeril; Peter P Karpawich; Jeffery J Kim; Mani Ram Krishna; Peter Kubuš; Martin J LaPage; Douglas Y Mah; Lindsey Malloy-Walton; Aya Miyazaki; Kara S Motonaga; Mary C Niu; Melissa Olen; Thomas Paul; Eric Rosenthal; Elizabeth V Saarel; Massimo Stefano Silvetti; Elizabeth A Stephenson; Reina B Tan; John Triedman; Nicholas H Von Bergen; Philip L Wackel Journal: Indian Pacing Electrophysiol J Date: 2021-07-29