title: Management and outcome of patients with abnormal ventriculo-arterial connections and mitral valve cleft.
abstract: BACKGROUND
  In patients with abnormal ventriculo-arterial connections, a mitral valve cleft different from an atrioventricular canal is occasionally associated. It may cause outflow obstruction, mitral regurgitation, and complicate biventricular repair.
  
  
  METHODS
  A retrospective review identified 21 patients operated upon with mitral valve cleft, abnormal ventriculo-arterial connections, and two well-developed ventricles. Eight patients had a ventricular outflow obstruction due to the mitral valve, whereas 2 had more than mild mitral regurgitation. One patient required initial mitral valve surgery. Eleven patients underwent biventricular repair, associated with mitral valve repair in 2 cases: arterial switch operation (n = 4), Senning operation (n = 3) associated with an arterial switch operation in one case, intraventricular repair (n = 3), and Rastelli-type extracardiac conduit repair (n = 1). Single-ventricle palliation was preferred in 10 patients with major mitral valve straddling (n = 5), outflow tract obstruction (n = 2), and noncommitted or multiple VSDs (n = 3).
  
  
  RESULTS
  There were three hospital deaths, two of which occurred after biventricular repair and one after an early reoperation after a bidirectional cavopulmonary anastomosis. Postoperatively after biventricular repair, 1 patient required permanent pacemaker implantation and 3 patients were reoperated on for subaortic stenosis (n = 1) and mitral regurgitation (n = 2), with one late death. By multivariate analysis, patients with a double-outlet right ventricle were at greater risk of death (p = 0.04). After a mean follow-up period of 60.7 months (+/- 68.6 months), 16 patients are in New York Heart Association (NYHA) class I. One patient with a moderate mitral regurgitation on Doppler study is in NYHA class II.
  
  
  CONCLUSIONS
  The surgical management remains controversial in patients with abnormal ventriculo-arterial connections and mitral valve cleft. Biventricular repair may not always be feasible, especially in cases of complex intracardiac anatomy associated with mitral valve straddling. Single-ventricle palliation can be achieved in these patients, although it is unknown whether the long-term results are as good as those obtained with biventricular repair.
authors.affiliation: Service de Cardiologie Pédiatrique, Hôpital Necker Enfants Malades, Paris, France. afraisse@mail.ap-hm.fr
authors.forename: Alain
authors.initials: A
authors.lastname: Fraisse
authors.forename: Tony Abdel
authors.initials: TA
authors.lastname: Massih
authors.forename: Pascal
authors.initials: P
authors.lastname: Vouhé
authors.forename: Bernard
authors.initials: B
authors.lastname: Kreitmann
authors.forename: Jean
authors.initials: J
authors.lastname: Gaudart
authors.forename: Daniel
authors.initials: D
authors.lastname: Sidi
authors.forename: Damien
authors.initials: D
authors.lastname: Bonnet
mesh_terms: D000293:Adolescent; D002423:Cause of Death; D002648:Child; D002675:Child, Preschool; D005260:Female; D006330:Heart Defects, Congenital / Q000401:mortality / Q000601:surgery*; D017052:Hospital Mortality; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008943:Mitral Valve / Q000002:abnormalities* / Q000601:surgery; D011183:Postoperative Complications / Q000401:mortality; D012086:Reoperation / Q000401:mortality; D012189:Retrospective Studies; D014694:Ventricular Outflow Obstruction / Q000151:congenital* / Q000401:mortality / Q000601:surgery