title: The impact of ventricular morphology on midterm outcome following completion total cavopulmonary connection.
abstract: OBJECTIVE
  This study was undertaken to compare the early and midterm outcome following completion total cavopulmonary connection (TCPC) in patients with a single functional ventricle of left or right morphology.
  
  
  METHODS
  Between August 1996 and July 2001, 103 patients underwent completion TCPC following an interim superior cavopulmonary connection. The single functional ventricle was of left (n=44, 42%) or right ventricular morphology (n=59, 58%). The TCPC was performed using an extracardiac conduit (n=84, 82%) or a lateral atrial tunnel (n=19, 18%), and was fenestrated in 53 patients (51%). Outcomes studied included duration of pleural effusions and in-patient hospitalisation; early mortality, reoperation and reintervention; actuarial survival, freedom from reoperation and reintervention; and current functional status. These were assessed according to a series of preoperative, operative and postoperative variables. Follow-up was complete with a median interval of 17 months (range, 21 days-5.2 years).
  
  
  RESULTS
  Early mortality was 1.9% (n=2) and one other patient required takedown of the Fontan circulation. There was one late death. Five-year survival with a Fontan circulation (+/-1 SEM) was 95.6+/-2.5%. Forty-two patients (41%) had prolonged pleural drainage (> or =14 days) and 41 patients (40%) had a prolonged hospital stay. Five-year freedom from reoperation and reintervention (+/-1 SEM) were 92.2+/-5.0 and 73.4+/-6.0%, respectively. The Fontan procedure was associated with an improved functional class (P<0.005) and all current survivors (n=99) are in either New York Heart Association classes I or II. Multivariate analysis identified left atrial isomerism as the single risk factor for death (P<0.05). Independent risk factors for prolonged hospital stay included a morphologic right ventricle (P<0.05), increased postoperative pulmonary artery pressures (P<0.005) and an unfenestrated Fontan procedure (P<0.01).
  
  
  CONCLUSIONS
  In this contemporary series, the modified Fontan procedure was characterised by low early mortality, excellent midterm survival, and improved functional class independent of the morphology of the single functional ventricle. Nevertheless, a morphologic right ventricle was a risk factor for prolonged in-patient hospitalisation and may yet influence long term survival.
authors.affiliation: Department of Cardiac Surgery, Diana, Princess of Wales Children's Hospital, Steelhouse Lane, Birmingham B4 6NH, UK.
authors.forename: Simon P
authors.initials: SP
authors.lastname: McGuirk
authors.forename: David S
authors.initials: DS
authors.lastname: Winlaw
authors.forename: Stephen M
authors.initials: SM
authors.lastname: Langley
authors.forename: Oliver F
authors.initials: OF
authors.lastname: Stumper
authors.forename: Joseph V
authors.initials: JV
authors.lastname: de Giovanni
authors.forename: John G
authors.initials: JG
authors.lastname: Wright
authors.forename: William J
authors.initials: WJ
authors.lastname: Brawn
authors.forename: David J
authors.initials: DJ
authors.lastname: Barron
mesh_terms: D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D004812:Epidemiologic Methods; D005260:Female; D018729:Fontan Procedure; D006330:Heart Defects, Congenital / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D006352:Heart Ventricles / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D007223:Infant; D007902:Length of Stay; D008297:Male; D010996:Pleural Effusion; D016896:Treatment Outcome