title: Clinical Presentation of Pediatric Patients at Risk for Sudden Cardiac Arrest.
abstract: To identify the clinical presentation of children and adolescents affected by 1 of 4 cardiac conditions predisposing to sudden cardiac arrest: hypertrophic cardiomyopathy, long QT syndrome (LQTS), catecholaminergic polymorphic ventricular tachycardia (CPVT), and anomalous origin of the left coronary artery from the right sinus of Valsalva (ALCA-R).
  
  
  
  This was a retrospective review of newly diagnosed pediatric patients with hypertrophic cardiomyopathy, LQTS, CPVT, and ALCA-R referred for cardiac evaluation at 6 US centers from 2008 to 2014.
  
  
  
  A total of 450 patients (257 male/193 female; median age 10.1 years [3.6-13.8 years, 25th-75th percentiles]) were enrolled. Patient age was ≤13 years for 70.4% of the cohort (n = 317). Sudden cardiac arrest was the initial presentation in 7%; others were referred on the basis of abnormal or suspicious family history, personal symptoms, or physical findings. Patients with LQTS and hypertrophic cardiomyopathy were referred most commonly because of family history concerns. ALCA-R was most likely to have abnormal signs or symptoms (eg, exercise chest pain, syncope, or sudden cardiac arrest). Patients with CPVT had a high incidence of syncope and the greatest incidence of sudden cardiac arrest (45%); 77% exhibited exercise syncope or sudden cardiac arrest. This study demonstrated that suspicious or known family history plays a role in identification of many patients ultimately affected by 1 of the 3 genetic disorders (hypertrophic cardiomyopathy, LQTS, CPVT).
  
  
  
  Important patient and family history and physical examination findings may allow medical providers to identify many pediatric patients affected by 4 cardiac disorders predisposing to sudden cardiac arrest.
authors: Children's Healthcare of Atlanta, Department of Pediatrics, Emory University School of Medicine, Atlanta, GA; Monroe Carell Jr. Children's Hospital at Vanderbilt, Department of Pediatrics, Vanderbilt University School of Medicine, Nashville, TN.
authors: Aarti
authors: A
authors: Dalal
authors: The Heart Institute, Cincinnati Children's Hospital Medical Center, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH.
authors: Richard J
authors: RJ
authors: Czosek
authors: Children's Hospital of Wisconsin, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI.
authors: Joshua
authors: J
authors: Kovach
authors: C.S. Mott Children's Hospital, Department of Pediatrics, University of Michigan Medical School, Ann Arbor, MI; Children's Hospital Colorado, Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO.
authors: Johannes C
authors: JC
authors: von Alvensleben
authors: Texas Children's Hospital, Department of Pediatrics, Baylor College of Medicine, Houston, TX.
authors: Santiago
authors: S
authors: Valdes
authors: Primary Children's Hospital, Department of Pediatrics, University of Utah School of Medicine, Salt Lake City, UT.
authors: Susan P
authors: SP
authors: Etheridge
authors: Mayo Clinic, Department of Pediatric and Adolescent Medicine, Mayo Clinic College of Medicine, Rochester, MN.
authors: Michael J
authors: MJ
authors: Ackerman
authors: Children's Healthcare of Atlanta, Department of Pediatrics, Emory University School of Medicine, Atlanta, GA.
authors: Debbie
authors: D
authors: Auld
authors: Children's Healthcare of Atlanta, Department of Pediatrics, Emory University School of Medicine, Atlanta, GA.
authors: Jeryl
authors: J
authors: Huckaby
authors: Children's Healthcare of Atlanta, Department of Pediatrics, Emory University School of Medicine, Atlanta, GA.
authors: Courtney
authors: C
authors: McCracken
authors: Children's Healthcare of Atlanta, Department of Pediatrics, Emory University School of Medicine, Atlanta, GA. Electronic address: campbellr@kidsheart.com.
authors: Robert
authors: R
authors: Campbell
mesh_terms: D000293:Adolescent; D002312:Cardiomyopathy, Hypertrophic / Q000150:complications; D002648:Child; D002675:Child, Preschool; D003330:Coronary Vessel Anomalies / Q000150:complications; D016757:Death, Sudden, Cardiac / Q000453:epidemiology / Q000209:etiology; D005260:Female; D006323:Heart Arrest / Q000175:diagnosis* / Q000453:epidemiology / Q000209:etiology; D006801:Humans; D008133:Long QT Syndrome / Q000150:complications; D008297:Male; D012189:Retrospective Studies; D012307:Risk Factors; D017180:Tachycardia, Ventricular / Q000150:complications; D000098850:Polymorphic Catecholaminergic Ventricular Tachycardia
keywords: catecholaminergic polymorphic ventricular tachycardia; congenital coronary artery anomalies; family history; hypertrophic cardiomyopathy; long QT syndrome; patient history; sudden cardiac arrest