title: Atrio-ventricular valve regurgitation in univentricular hearts: outcomes after repair†.
abstract: OBJECTIVE
  The aim was to describe the early and mid-term outcome after atrio-ventricular valve (AVV) repair in patients with univentricular hearts (UVHs) and to identify risk factors for AVV reoperation and death.
  
  
  METHODS
  This study is a retrospective review of patients undergoing valve repair for AVV regurgitation at any stage of univentricular palliation from 1998 to 2014. Patient- and procedure-related variables were analysed.
  
  
  RESULTS
  A total of 31 consecutive patients underwent 38 procedures for ≥ moderate AVV regurgitation at a median age of 3.6 years. Thirty-two percent of patients had a common AVV, 26% had two AVVs, 22% had a dominant tricuspid valve and 19% had a dominant mitral valve. All patients underwent valve repair as a first procedure without early mortality. At discharge, patients preserved their ventricular function (fractional shortening <30%: preoperative 16% vs postoperative 22.5%, NS). In 19% (n = 6) of patients, the procedure was considered as failed because of significant residual regurgitation. There were three late deaths [median delay: 1 year (range 0.7-13.6)] and three heart transplantations. Six patients underwent seven AVV reoperations [median delay: 2 years (range 0.2-7.6)]. Longer intensive care stay (P = 0.022), longer total postoperative hospital stay (P = 0.039), higher total number of surgeries (P = 0.039), lower body mass index (P = 0.042) and higher preoperative mean pulmonary pressure (P = 0.047) were univariate risk factors for death/transplantation. Failed first AVV repair (P = 0.01), higher total number of surgeries (P = 0.026), lower body mass index (P = 0.031), male gender (P = 0.031) and need for valve repair before bidirectional cavopulmonary connection (P = 0.036) were univariate risk factors for AVV reoperation. In multivariate analysis, no univariate risk factor reached statistical significance. Freedom from death/transplantation was 84% (CI 95%: 70%-98%) at 5 and 10 years. Survival free from AVV reoperation was 72% (CI 95%: 52%-92%) at 5 years and 62% at 10 years (CI 95%: 36%-88%). Mean follow-up of survivors was 4.7 years (SD ± 4.3; range 0.2-15.6). At last visit, 96% of survivors were in NYHA Class I-II. Ninety-two percent had a ≤ mild residual regurgitation.
  
  
  CONCLUSIONS
  In patients with a UVH and ≥ moderate AVV regurgitation, AVV repair is feasible without postoperative deterioration of their ventricular function. Nevertheless, these patients remain at increased risk for death/transplantation and AVV reoperation.
authors.affiliation: Department of Pediatric and Congenital Heart Disease-M3C, Marie Lannelongue Hospital, University Paris Sud, Le Plessis Robinson, France d.laux@ccml.fr.
authors.forename: Daniela
authors.initials: D
authors.lastname: Laux
authors.affiliation: Department of Pediatric and Congenital Heart Disease-M3C, Marie Lannelongue Hospital, University Paris Sud, Le Plessis Robinson, France.
authors.forename: Mathieu
authors.initials: M
authors.lastname: Vergnat
authors.affiliation: Department of Pediatric and Congenital Heart Disease-M3C, Marie Lannelongue Hospital, University Paris Sud, Le Plessis Robinson, France.
authors.forename: Virginie
authors.initials: V
authors.lastname: Lambert
authors.affiliation: Department of Pediatric and Congenital Heart Disease-M3C, Marie Lannelongue Hospital, University Paris Sud, Le Plessis Robinson, France.
authors.forename: Marielle
authors.initials: M
authors.lastname: Gouton
authors.affiliation: Department of Pediatric and Congenital Heart Disease-M3C, Marie Lannelongue Hospital, University Paris Sud, Le Plessis Robinson, France.
authors.forename: Mohamed
authors.initials: M
authors.lastname: Ly
authors.affiliation: Department of Pediatric and Congenital Heart Disease-M3C, Marie Lannelongue Hospital, University Paris Sud, Le Plessis Robinson, France.
authors.forename: Marianne
authors.initials: M
authors.lastname: Peyre
authors.affiliation: Department of Pediatric and Congenital Heart Disease-M3C, Marie Lannelongue Hospital, University Paris Sud, Le Plessis Robinson, France.
authors.forename: Regine
authors.initials: R
authors.lastname: Roussin
authors.affiliation: Department of Pediatric and Congenital Heart Disease-M3C, Marie Lannelongue Hospital, University Paris Sud, Le Plessis Robinson, France.
authors.forename: Emre
authors.initials: E
authors.lastname: Belli
mesh_terms: D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D015150:Echocardiography, Doppler; D005260:Female; D005500:Follow-Up Studies; D005602:France; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging / Q000601:surgery; D019918:Heart Valve Prosthesis Implantation / Q000009:adverse effects / Q000379:methods* / Q000401:mortality*; D006352:Heart Ventricles / Q000002:abnormalities*; D017052:Hospital Mortality; D006801:Humans; D007223:Infant; D008297:Male; D008944:Mitral Valve Insufficiency / Q000000981:diagnostic imaging / Q000401:mortality / Q000601:surgery*; D011183:Postoperative Complications / Q000401:mortality / Q000503:physiopathology / Q000601:surgery; D012086:Reoperation / Q000379:methods; D012189:Retrospective Studies; D018570:Risk Assessment; D016019:Survival Analysis; D013997:Time Factors; D016896:Treatment Outcome
keywords: Atrio-ventricular valve; Univentricular heart; Valve repair