title: Mitral disease: the real burden for Ross-Konno procedure in children.
abstract: BACKGROUND
  Aortic valve replacement and management of complex left ventricular outflow tract obstruction in early life remains a surgical challenge. We assessed our experience with the Ross-Konno procedure.
  
  
  METHODS
  Since 2000, 49 consecutive patients (24 neonates and infants) underwent the Ross-Konno procedure. Anatomic and clinical risk factors were analyzed.
  
  
  RESULTS
  Median age was 12.2 months (0.0 to 194.4); 82% had previous valvotomy (surgical, n = 26; balloon, n = 7; balloon then surgical, n = 7) and 35% preceding arch repair (n = 17). Fifteen patients (31%) required concomitant procedures: mitral valve repair (n = 11), replacement (n = 1), endocardial fibroelastosis resection (n = 4), and aortic arch repair (n = 2). There were 5 hospital deaths (10.2%). Median follow-up was 63 ± 47 months. There were 4 late deaths (8.1%), all because of persistent pulmonary hypertension despite subsequent mitral procedures. Five-year actuarial survival and freedom from reoperation were 79.7% ± 6.1% and 68.6% ± 9.3%, respectively. Preoperative lower shortening fraction (p = 0.005) was associated with early mortality, while concomitant mitral surgery and pulmonary hypertension (p = 0.002) were associated with late mortality. Sixteen patients underwent 26 reoperations. Autograft function was normal in 30 of the 37 late survivors (81.1%), 4 (10.8%) had grade 2 regurgitation, and 3 (8.1%) required valve replacement.
  
  
  CONCLUSIONS
  Ross-Konno in children remains a high-risk procedure. Preoperative ventricular dysfunction is associated with significant early mortality and should favor conservative options. Mitral involvement substantially affects late outcome, survival for these patients relies on the efficacy in relieving mitral disease. Efforts to improve mitral repair in these infants are critically required as an alternative to univentricular pathways.
authors.affiliation: Centre Chirurgical Marie Lannelongue-M3C, Department of Pediatric and Congenital Heart Disease, Université Paris-Sud, Orsay, France. Electronic address: mvergnat@gmail.com.
authors.forename: Mathieu
authors.initials: M
authors.lastname: Vergnat
authors.affiliation: Centre Chirurgical Marie Lannelongue-M3C, Department of Pediatric and Congenital Heart Disease, Université Paris-Sud, Orsay, France.
authors.forename: Francois
authors.initials: F
authors.lastname: Roubertie
authors.affiliation: Centre Chirurgical Marie Lannelongue-M3C, Department of Pediatric and Congenital Heart Disease, Université Paris-Sud, Orsay, France; Centre Chirurgical Marie Lannelongue, INSERM U999, Le Plessis Robinson, France.
authors.forename: Virginie
authors.initials: V
authors.lastname: Lambert
authors.affiliation: Centre Chirurgical Marie Lannelongue-M3C, Department of Pediatric and Congenital Heart Disease, Université Paris-Sud, Orsay, France.
authors.forename: Daniela
authors.initials: D
authors.lastname: Laux
authors.affiliation: Centre Chirurgical Marie Lannelongue-M3C, Department of Pediatric and Congenital Heart Disease, Université Paris-Sud, Orsay, France.
authors.forename: Mohamedou
authors.initials: M
authors.lastname: Ly
authors.affiliation: Centre Chirurgical Marie Lannelongue-M3C, Department of Pediatric and Congenital Heart Disease, Université Paris-Sud, Orsay, France.
authors.forename: Régine
authors.initials: R
authors.lastname: Roussin
authors.affiliation: Centre Chirurgical Marie Lannelongue-M3C, Department of Pediatric and Congenital Heart Disease, Université Paris-Sud, Orsay, France.
authors.forename: Alban-Elouen
authors.initials: AE
authors.lastname: Baruteau
authors.affiliation: Centre Chirurgical Marie Lannelongue, INSERM U999, Le Plessis Robinson, France; Centre Chirurgical Marie Lannelongue, Department of Physiology, Paris-Sud University, Le Plessis Robinson, France.
authors.forename: André
authors.initials: A
authors.lastname: Capderou
authors.affiliation: Centre Chirurgical Marie Lannelongue-M3C, Department of Pediatric and Congenital Heart Disease, Université Paris-Sud, Orsay, France; Centre Chirurgical Marie Lannelongue, INSERM U999, Le Plessis Robinson, France.
authors.forename: David
authors.initials: D
authors.lastname: Kalfa
authors.affiliation: Centre Chirurgical Marie Lannelongue-M3C, Department of Pediatric and Congenital Heart Disease, Université Paris-Sud, Orsay, France.
authors.forename: Emre
authors.initials: E
authors.lastname: Belli
mesh_terms: D000293:Adolescent; D006348:Cardiac Surgical Procedures / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D005260:Female; D005500:Follow-Up Studies; D006349:Heart Valve Diseases / Q000000981:diagnostic imaging / Q000601:surgery*; D006352:Heart Ventricles / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008943:Mitral Valve / Q000601:surgery*; D012189:Retrospective Studies; D016896:Treatment Outcome