Author + information
- Jeremy P. Moore, MD, MSa,b,∗ (, )
- Roberto G. Gallotti, MDa,b,
- Kevin M. Shannon, MDa,b,
- J. Martijn Bos, MD, PhDc,d,e,
- Elham Sadeghi, BSN, PhDf,
- Janette F. Strasburger, MDf,
- Ronald T. Wakai, PhDg,
- Hitoshi Horigome, MDh,
- Sally-Ann Clur, MBBCh, MSc, PhDi,
- Allison C. Hill, MDj,
- Maully J. Shah, MBBSk,
- Shashank Behere, MDk,
- Georgia Sarquella-Brugada, MD, PhDl,m,
- Richard Czosek, MDn,
- Susan P. Etheridge, MDo,
- Peter Fischbach, MDp,
- Prince J. Kannankeril, MD, MSq,
- Kara Motonaga, MDr,
- Andrew P. Landstrom, MD, PhDs,t,
- Matthew Williams, MDu,
- Akash Patel, MDv,
- Federica Dagradi, MDw,
- Reina B. Tan, MDx,
- Elizabeth Stephenson, MD, MSy,
- Mani Ram Krishna, MBBSz,
- Christina Y. Miyake, MD, MSaa,bb,
- Michelle E. Leeaa,bb,
- Shubhayan Sanatani, MD, MScc,
- Seshadri Balaji, MBBS, PhDdd,
- Ming-Lon Young, MDee,
- Saad Siddiqui, MDff,
- Peter J. Schwartz, MDw,gg,hh,
- Kalyanam Shivkumar, MD, PhDa,b and
- Michael J. Ackerman, MD, PhDc,d,e
- aDivision of Pediatric Cardiology, University of California Los Angeles (UCLA) Medical Center, Los Angeles, California
- bUCLA Cardiac Arrhythmia Center and Ahmanson Adult Congenital Heart Disease Center, UCLA Health System, Los Angeles, California
- cDepartment of Cardiovascular Medicine (Division of Heart Rhythm Services), Mayo Clinic, Rochester, Minnesota
- dDivision of Pediatric Cardiology, Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota
- eDepartment of Molecular Pharmacology and Experimental Therapeutics, Windland Smith Rice Sudden Death Genomics Laboratory, Mayo Clinic, Rochester, Minnesota
- fDepartment of Pediatrics, Medical College of Wisconsin, Herma Heart Institute, Milwaukee, Wisconsin
- gBiomagnetism Laboratory, Department of Medical Physics, University of Wisconsin, Madison, Wisconsin
- hDepartment of Pediatrics, Tsukuba University, Ibaraki, Japan
- iDepartment of Pediatric Cardiology, Emma Children’s Hospital, Amsterdam University Medical Center, Amsterdam, the Netherlands
- jDivision of Cardiology, Children's Hospital Los Angeles, Los Angeles, California
- kDivision of Cardiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
- lArrhythmia, Inherited Cardiac Diseases Unit, Hospital Sant Joan de Déu, Barcelona, Spain
- mMedical Sciences Department, School of Medicine, University of Girona, Girona, Spain
- nThe Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio
- oPrimary Children’s Hospital, University of Utah School of Medicine, Salt Lake City, Utah
- pDivision of Pediatric Cardiology, Children's Healthcare of Atlanta, Emory University, Atlanta, Georgia
- qMonroe Carrell Children’s Hospital, Vanderbilt University School of Medicine, Nashville, Tennessee
- rDivision of Pediatric Cardiology, Stanford University, Palo Alto, California
- sDepartment of Pediatrics, Division of Cardiology, Duke University School of Medicine, Durham, North Carolina
- tDepartment of Cell Biology, Duke University School of Medicine, Durham, North Carolina
- uDivision of Cardiology, Rady Children’s Hospital, University of California San Diego, San Diego, California
- vDivision of Pediatric Cardiology, University of California San Francisco Benioff Children's Hospital, University of California, San Francisco, California
- wCenter for Cardiac Arrhythmias of Genetic Origin, Istituto di Ricovero e Cura a Carattere Scientifico, Istituto Auxologico Italiano, Milan, Italy
- xDivision of Pediatric Cardiology, New York University Langone School of Medicine, New York, New York
- yLabbatt Family Heart Centre, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
- zAmrita Institute of Medical Science, Kochi, India
- aaDepartment of Pediatrics, Baylor College of Medicine, Houston, Texas
- bbDepartment of Molecular Physiology and Biophysics, Baylor College of Medicine, Houston, Texas
- ccDivision of Cardiology, British Columbia Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
- ddDivision of Pediatric Cardiology, Oregon Health and Science University, Portland, Oregon
- eeJoe DiMaggio Children's Hospital Heart Institute, Memorial Healthcare System, Hollywood, Florida
- ffThe Heart Institute for Children, Advocate Children’s Hospital, Oak Lawn, Illinois
- ggDepartment of Cardiology, Fondazione IRCCS Policlinico S. Matteo, Pavia, Italy
- hhMolecular Cardiology Laboratory, Fondazione IRCCS Policlinico S. Matteo, Pavia, Italy
- ↵∗Address for correspondence:
Dr. Jeremy P. Moore, Division of Pediatric Cardiology, University of California Los Angeles Medical Center, 100 Medical Plaza Drive, Suite 770, Los Angeles, California 90095.
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.
- atrioventricular block
- cardiac sympathetic denervation
- fetal arrhythmia
- genetic testing
- implantable cardioverter-defibrillator
- long QT syndrome
- sudden cardiac death
- torsades de pointes
Dr. Strasburger has received support from the National Institutes of Health grant RO1HL143485. Dr. Wakai has received support from the National Institutes of Health grant RO1HL063174. Dr. Miyake has received support from the National Heart, Lung, and Blood Institute grant K23HL136932 and American Heart Association grant 17SDG33410183. Dr. Schwartz has received support from the National Institutes of Health grant HL083374 and Italian Ministry of Health grant (Sindrome della morte improvvisa del lattante: meccanismi e prevenzione). All other authors have reported that they have no relationships relevant to the contents of this paper to disclose. Samuel Viskin, MD, served as Guest Editor for this paper. Katja Zeppenfeld, MD, served as Guest Editor-in-Chief for this paper.
The 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 March 26, 2020.
- Revision received May 26, 2020.
- Accepted June 2, 2020.
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