title: Bidirectional cavopulmonary shunt with additional pulmonary blood flow: a failed or successful strategy?
abstract: OBJECTIVE
  In patients with single ventricle physiology, Fontan circulation is considered as the optimal surgical approach, although it entails a growing incidence of late complications. It has been speculated that the association of bidirectional cavopulmonary shunt (BCPS) and additional pulmonary blood flow (APBF) might provide long-lasting palliation. The present study was undertaken to assess the long-term outcome of this strategy.
  
  
  METHODS
  A group of 70 patients with single ventricle physiology, who underwent BCPS with APBF between 1990 and 2000, were reviewed retrospectively. Median age at operation was 2 years (range: 0.1-27 years). Unilateral BCPS was performed in 60 patients (86%), bilateral BCPS in 9 and the Kawashima procedure in 1. APBF was provided through antegrade pulmonary outflow tract in 43 patients (61%) and by aortopulmonary shunt in 27 (39%). Two patients died early and three were lost to follow-up. Mean follow-up of the 65 remaining patients was 13.5 ± 4 years. End-points were death, need for heart transplantation (HTx) or Fontan completion and functional outcome.
  
  
  RESULTS
  Five patients died (two after HTx, three from ventricular failure); overall actuarial survival was 89 ± 4% at 15 years. Six patients underwent HTx (one after Fontan completion) with two early deaths and no late mortality. Fifty-one patients underwent Fontan completion (11 with additional palliative procedures before completion); there was no early or late mortality following Fontan completion; one patient underwent HTx. Among the remaining six patients with BCPS and APBF, two were not suitable for Fontan completion and four remained suitable. Overall, clinical failure (mortality, HTx, unsuitability for Fontan completion) occurred in 13 patients (19%). Risk factors for clinical failure were older age at BCPS (P = 0.01) and postoperative complications after BCPS (P = 0.001). Considering late mortality, HTx and Fontan completion as strategic failures, the actuarial freedom from these events was 20 ± 5% at 10 years.
  
  
  CONCLUSIONS
  BCPS with APBF approach: (i) fails as a strategy for definitive palliation, (ii) provides a high survival rate, (iii) does not preclude a successful Fontan completion and (iv) may delay the long-term deleterious consequences of Fontan circulation. Palliation by BCPS with APBF should be achieved early in life.
authors.affiliation: Department of Pediatric Cardiac Surgery,  University Paris Descartes, Sorbonne Paris Cité and Sick Children Hospital, Paris,  France.
authors.forename: Sébastien
authors.initials: S
authors.lastname: Gérelli
authors.forename: Célia
authors.initials: C
authors.lastname: Boulitrop
authors.forename: Mathieu
authors.initials: M
authors.lastname: Van Steenberghe
authors.forename: Davide
authors.initials: D
authors.lastname: Maldonado
authors.forename: Mirela
authors.initials: M
authors.lastname: Bojan
authors.forename: Olivier
authors.initials: O
authors.lastname: Raisky
authors.forename: Daniel
authors.initials: D
authors.lastname: Sidi
authors.forename: Pascal R
authors.initials: PR
authors.lastname: Vouhé
mesh_terms: D000293:Adolescent; D000328:Adult; D002423:Cause of Death; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000379:methods* / Q000401:mortality*; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging / Q000401:mortality / Q000601:surgery*; D006352:Heart Ventricles / Q000002:abnormalities*; D006439:Hemodynamics / Q000502:physiology; D006801:Humans; D007223:Infant; D008297:Male; D010166:Palliative Care / Q000379:methods; D018579:Patient Selection; D011651:Pulmonary Artery / Q000502:physiology; D011652:Pulmonary Circulation / Q000502:physiology*; D012189:Retrospective Studies; D018570:Risk Assessment; D015996:Survival Rate; D013997:Time Factors; D016896:Treatment Outcome; D014463:Ultrasonography; D055815:Young Adult