Jeremy P Moore1, Roberto G Gallotti2, Kevin M Shannon2, J Martijn Bos3, Elham Sadeghi4, Janette F Strasburger4, Ronald T Wakai5, Hitoshi Horigome6, Sally-Ann Clur7, Allison C Hill8, Maully J Shah9, Shashank Behere9, Georgia Sarquella-Brugada10, Richard Czosek11, Susan P Etheridge12, Peter Fischbach13, Prince J Kannankeril14, Kara Motonaga15, Andrew P Landstrom16, Matthew Williams17, Akash Patel18, Federica Dagradi19, Reina B Tan20, Elizabeth Stephenson21, Mani Ram Krishna22, Christina Y Miyake23, Michelle E Lee23, Shubhayan Sanatani24, Seshadri Balaji25, Ming-Lon Young26, Saad Siddiqui27, Peter J Schwartz28, Kalyanam Shivkumar2, Michael J Ackerman3. 1. Division of Pediatric Cardiology, University of California Los Angeles (UCLA) Medical Center, Los Angeles, California, USA; UCLA Cardiac Arrhythmia Center and Ahmanson Adult Congenital Heart Disease Center, UCLA Health System, Los Angeles, California, USA. Electronic address: jpmoore@mednet.ucla.edu. 2. Division of Pediatric Cardiology, University of California Los Angeles (UCLA) Medical Center, Los Angeles, California, USA; UCLA Cardiac Arrhythmia Center and Ahmanson Adult Congenital Heart Disease Center, UCLA Health System, Los Angeles, California, USA. 3. Department of Cardiovascular Medicine (Division of Heart Rhythm Services), Mayo Clinic, Rochester, Minnesota, USA; Division of Pediatric Cardiology, Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota, USA; Department of Molecular Pharmacology and Experimental Therapeutics, Windland Smith Rice Sudden Death Genomics Laboratory, Mayo Clinic, Rochester, Minnesota, USA. 4. Department of Pediatrics, Medical College of Wisconsin, Herma Heart Institute, Milwaukee, Wisconsin, USA. 5. Biomagnetism Laboratory, Department of Medical Physics, University of Wisconsin, Madison, Wisconsin, USA. 6. Department of Pediatrics, Tsukuba University, Ibaraki, Japan. 7. Department of Pediatric Cardiology, Emma Children's Hospital, Amsterdam University Medical Center, Amsterdam, the Netherlands. 8. Division of Cardiology, Children's Hospital Los Angeles, Los Angeles, California, USA. 9. Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA. 10. Arrhythmia, Inherited Cardiac Diseases Unit, Hospital Sant Joan de Déu, Barcelona, Spain; Medical Sciences Department, School of Medicine, University of Girona, Girona, Spain. 11. The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA. 12. Primary Children's Hospital, University of Utah School of Medicine, Salt Lake City, Utah, USA. 13. Division of Pediatric Cardiology, Children's Healthcare of Atlanta, Emory University, Atlanta, Georgia, USA. 14. Monroe Carrell Children's Hospital, Vanderbilt University School of Medicine, Nashville, Tennessee, USA. 15. Division of Pediatric Cardiology, Stanford University, Palo Alto, California, USA. 16. Department of Pediatrics, Division of Cardiology, Duke University School of Medicine, Durham, North Carolina, USA; Department of Cell Biology, Duke University School of Medicine, Durham, North Carolina, USA. 17. Division of Cardiology, Rady Children's Hospital, University of California San Diego, San Diego, California, USA. 18. Division of Pediatric Cardiology, University of California San Francisco Benioff Children's Hospital, University of California, San Francisco, California, USA. 19. Center for Cardiac Arrhythmias of Genetic Origin, Istituto di Ricovero e Cura a Carattere Scientifico, Istituto Auxologico Italiano, Milan, Italy. 20. Division of Pediatric Cardiology, New York University Langone School of Medicine, New York, New York, USA. 21. Labbatt Family Heart Centre, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. 22. Amrita Institute of Medical Science, Kochi, India. 23. Department of Pediatrics, Baylor College of Medicine, Houston, Texas, USA; Department of Molecular Physiology and Biophysics, Baylor College of Medicine, Houston, Texas, USA. 24. Division of Cardiology, British Columbia Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada. 25. Division of Pediatric Cardiology, Oregon Health and Science University, Portland, Oregon, USA. 26. Joe DiMaggio Children's Hospital Heart Institute, Memorial Healthcare System, Hollywood, Florida, USA. 27. The Heart Institute for Children, Advocate Children's Hospital, Oak Lawn, Illinois, USA. 28. Center for Cardiac Arrhythmias of Genetic Origin, Istituto di Ricovero e Cura a Carattere Scientifico, Istituto Auxologico Italiano, Milan, Italy; Department of Cardiology, Fondazione IRCCS Policlinico S. Matteo, Pavia, Italy; Molecular Cardiology Laboratory, Fondazione IRCCS Policlinico S. Matteo, Pavia, Italy.
Abstract
OBJECTIVES: This study sought to determine the relationship between long QT syndrome (LQTS) subtype (LTQ1, LTQ2, LTQ3) and postnatal cardiac events (CEs). BACKGROUND: LQTS presenting with 2:1 atrioventricular block or torsades de pointes in the fetus and/or neonate has been associated with risk for major CEs, but overall outcomes and predictors remain unknown. METHODS: A retrospective study involving 25 international centers evaluated the course of fetuses/newborns diagnosed with congenital LQTS and either 2:1 atrioventricular block or torsades de pointes. The primary outcomes were age at first CE after dismissal from the newborn hospitalization and death and/or cardiac transplantation during follow-up. CE was defined as aborted cardiac arrest, appropriate shock from implantable cardioverter-defibrillator, or sudden cardiac death. RESULTS: A total of 84 fetuses and/or neonates were identified with LQTS (12 as LQT1, 35 as LQT2, 37 as LQT3). Median gestational age at delivery was 37 weeks (interquartile range: 35 to 39 weeks) and age at hospital discharge was 3 weeks (interquartile range: 2 to 5 weeks). Fetal demise occurred in 2 and pre-discharge death in 1. Over a median of 5.2 years, there were 1 LQT1, 3 LQT2, and 23 LQT3 CEs (13 aborted cardiac arrests, 5 sudden cardiac deaths, and 9 appropriate shocks). One patient with LQT1 and 11 patients with LQT3 died or received cardiac transplant during follow-up. The only multivariate predictor of post-discharge CEs was LQT3 status (LQT3 vs. LQT2: hazard ratio: 8.4; 95% confidence interval: 2.6 to 38.9; p < 0.001), and LQT3, relative to LQT2, genotype predicted death and/or cardiac transplant (p < 0.001). CONCLUSIONS: In this large multicenter study, fetuses and/or neonates with LQT3 but not those with LQT1 or LQT2 presenting with severe arrhythmias were at high risk of not only frequent, but lethal CEs. Published by Elsevier Inc.
OBJECTIVES: This study sought to determine the relationship between long QT syndrome (LQTS) subtype (LTQ1, LTQ2, LTQ3) and postnatal cardiac events (CEs). BACKGROUND: LQTS presenting with 2:1 atrioventricular block or torsades de pointes in the fetus and/or neonate has been associated with risk for major CEs, but overall outcomes and predictors remain unknown. METHODS: A retrospective study involving 25 international centers evaluated the course of fetuses/newborns diagnosed with congenital LQTS and either 2:1 atrioventricular block or torsades de pointes. The primary outcomes were age at first CE after dismissal from the newborn hospitalization and death and/or cardiac transplantation during follow-up. CE was defined as aborted cardiac arrest, appropriate shock from implantable cardioverter-defibrillator, or sudden cardiac death. RESULTS: A total of 84 fetuses and/or neonates were identified with LQTS (12 as LQT1, 35 as LQT2, 37 as LQT3). Median gestational age at delivery was 37 weeks (interquartile range: 35 to 39 weeks) and age at hospital discharge was 3 weeks (interquartile range: 2 to 5 weeks). Fetal demise occurred in 2 and pre-discharge death in 1. Over a median of 5.2 years, there were 1 LQT1, 3 LQT2, and 23 LQT3 CEs (13 aborted cardiac arrests, 5 sudden cardiac deaths, and 9 appropriate shocks). One patient with LQT1 and 11 patients with LQT3 died or received cardiac transplant during follow-up. The only multivariate predictor of post-discharge CEs was LQT3 status (LQT3 vs. LQT2: hazard ratio: 8.4; 95% confidence interval: 2.6 to 38.9; p < 0.001), and LQT3, relative to LQT2, genotype predicted death and/or cardiac transplant (p < 0.001). CONCLUSIONS: In this large multicenter study, fetuses and/or neonates with LQT3 but not those with LQT1 or LQT2 presenting with severe arrhythmias were at high risk of not only frequent, but lethal CEs. Published by Elsevier Inc.
Authors: Arnold L Fenrich; Daniel P Shmorhun; Gregory C Martin; Jill A Young; Mitchell I Cohen; Amy S Kelleher; Martin A Anyebuno; Evelyn D Rider; Cheryl L Motta; Reese H Clark Journal: Pediatr Cardiol Date: 2022-06-03 Impact factor: 1.838
Authors: Estefanía Martínez-Barrios; Sergi Cesar; José Cruzalegui; Clara Hernandez; Elena Arbelo; Victoria Fiol; Josep Brugada; Ramon Brugada; Oscar Campuzano; Georgia Sarquella-Brugada Journal: Biomedicines Date: 2022-01-05
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