title: Atrioventricular discordance: results of repair in 127 patients.
abstract: OBJECTIVE
  The conventional management of patients with atrioventricular discordance is directed at associated lesions, taking advantage of physiologic "correction"; however, the morphologic right ventricle and tricuspid valve support the systemic circulation. Questions surrounding survival using this approach led us to analyze our institutional results.
  
  
  METHODS
  All patients with atrioventricular discordance undergoing biventricular repair were analyzed (n = 127, 1959-1997), excluding those with functionally univentricular hearts. The ventriculoarterial connection associated with atrioventricular discordance varied and was most commonly discordant (87%), but occasionally concordant (6%), double-outlet right ventricle (6%), or double-outlet left ventricle (1%). At initial presentation, the most common lesions associated with atrioventricular discordance were ventricular septal defect (86%), pulmonary stenosis (64%), tricuspid regurgitation (28%), and atrioventricular block (12%). Nine patients underwent a double switch procedure to create ventriculoarterial concordance and the remainder were managed conventionally without correcting discordant connections.
  
  
  RESULTS
  Operative mortality was 6% and did not vary by associated lesion. Twenty years after repair, survival was 48%. Within 20 years, 56% of patients required reoperation, usually for atrioventricular valve incompetence (n = 16), pulmonary stenosis (n = 16), or both (n = 3). Pacemakers were required in 50 patients, 4 before repair, 40 within 2 months of repair, and 6 remotely after repair. In early follow-up, the double switch procedure (n = 9) had equivalent mortality and a high pacemaker requirement for atrioventricular block.
  
  
  CONCLUSIONS
  Analysis of conventional management of atrioventricular discordance revealed cumulative increases in mortality, systemic atrioventricular valve (tricuspid) replacement, complete atrioventricular block, and incidence of reoperation. Alternative management should be examined.
authors.affiliation: Division of Cardiovascular Surgery, Hospital for Sick Children, Toronto Congenital Cardiac Centre for Adults, University of Toronto, Toronto, Ontario, Canada.
authors.forename: T
authors.initials: T
authors.lastname: Yeh
authors.forename: M S
authors.initials: MS
authors.lastname: Connelly
authors.forename: J G
authors.initials: JG
authors.lastname: Coles
authors.forename: G D
authors.initials: GD
authors.lastname: Webb
authors.forename: P R
authors.initials: PR
authors.lastname: McLaughlin
authors.forename: R M
authors.initials: RM
authors.lastname: Freedom
authors.forename: P B
authors.initials: PB
authors.lastname: Cerrito
authors.forename: W G
authors.initials: WG
authors.lastname: Williams
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008875:Middle Aged; D011183:Postoperative Complications; D012086:Reoperation; D012189:Retrospective Studies; D015996:Survival Rate