title: Surgical outcome in pediatric patients with Ebstein's anomaly: A multicenter, long-term study.
abstract: OBJECTIVE
  Surgical outcomes of pediatric patients with Ebstein's anomaly are often described as part of all-age-inclusive series. Our objective is to focus on patients treated surgically in childhood (0-18 y). We study the intended treatment (biventricular or 1.5 ventricle repair or univentricular palliation), freedom from unplanned reoperation and survival of this specific age group, in a nationwide study.
  
  
  METHODS
  Records of all Ebstein's anomaly patients born between 1980 and 2013 were reviewed. Demographic variables, intraoperative procedures and postoperative outcomes were analyzed.
  
  
  RESULTS
  Sixty-three patients underwent 109 operations. Median follow-up after diagnosis was 121 months (range 0-216 months). Twenty-nine (46%) patients required surgery in the first year of life, including 21 who required neonatal surgery. The intended treatment was biventricular (n = 37, 59%) and 1.5 ventricle (n = 5, 8%) repair or univentricular (n = 21, 33%) palliation. The one-, five-, and 10-year freedom from unplanned reoperation was 89%, 79%, and 75% respectively. There were nine (14%) in hospital deaths (within 30 d after surgery). Causes of death were low cardiac output syndrome, cardiac failure, hypoxemia, pulmonary hypertension or an unknown cause. There were no late deaths.
  
  
  CONCLUSIONS
  Surgery in childhood represents the worse spectrum of disease, biventricular repair is often not applied. Patients often face revision surgery. Mortality is limited to the immediate postsurgical period.
authors: Department of Pediatric Cardiology, Radboud University Medical Center, Nijmegen, the Netherlands.|Department of Pediatric Cardiology and Intensive Care Medicine, Hannover Medical School, Hannover, Germany.
authors: Lianne M
authors: LM
authors: Geerdink
authors: Center for Congenital Heart Diseases, University Medical Center Groningen, Groningen, the Netherlands.
authors: Gideon J
authors: GJ
authors: du Marchie Sarvaas
authors: Department of Pediatric Cardiology, Academic Medical Center, Amsterdam, the Netherlands.
authors: Irene M
authors: IM
authors: Kuipers
authors: Department of Pediatric Cardiology, Erasmus Medical Center, Rotterdam, the Netherlands.
authors: Willem A
authors: WA
authors: Helbing
authors: Department of Pediatric Cardiology, Maastricht University Medical Center, Maastricht, the Netherlands.
authors: Tammo
authors: T
authors: Delhaas
authors: Department of Pediatric Cardiology, University Medical Center Utrecht, Utrecht, the Netherlands.
authors: Henriette
authors: H
authors: Ter Heide
authors: Department of Pediatric Cardiology, Leiden University Medical Center, Leiden, the Netherlands.
authors: Lieke
authors: L
authors: Rozendaal
authors: Department of Radiology, Medical Ultrasound Imaging Center, Radboud University Medical Center, Nijmegen, the Netherlands.
authors: Chris L
authors: CL
authors: de Korte
authors: Department of Cardiothoracic Surgery, Isala Clinics, Zwolle, the Netherlands.
authors: Sandeep K
authors: SK
authors: Singh
authors: Department of Cardiothoracic Surgery, University Medical Center Groningen, Groningen, the Netherlands.
authors: Tjark
authors: T
authors: Ebels
authors: Department of Cardiothoracic Surgery, Leiden University Medical Center, Leiden, the Netherlands.
authors: Mark G
authors: MG
authors: Hazekamp
authors: Department of Cardiothoracic Surgery, University Medical Center Utrecht, Utrecht, the Netherlands.
authors: Felix
authors: F
authors: Haas
authors: Department of Cardiothoracic Surgery, Erasmus Medical Center, Rotterdam, the Netherlands.
authors: Ad J J C
authors: AJ
authors: Bogers
authors: Department of Pediatric Cardiology, Radboud University Medical Center, Nijmegen, the Netherlands.|Department of Pediatric Cardiology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.
authors: Livia
authors: L
authors: Kapusta
mesh_terms: D000293:Adolescent; D006348:Cardiac Surgical Procedures / Q000009:adverse effects / Q000401:mortality; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D004437:Ebstein Anomaly / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D005260:Female; D017052:Hospital Mortality; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D009426:Netherlands; D010166:Palliative Care; D011183:Postoperative Complications / Q000209:etiology / Q000401:mortality / Q000601:surgery; D012086:Reoperation; D012189:Retrospective Studies; D012307:Risk Factors; D017741:Survivors; D013997:Time Factors; D016896:Treatment Outcome
keywords: Ebstein's anomaly; congenital; neonates; outcome; surgery