title: Surgery for infants with a hypoplastic systemic ventricle and severe outflow obstruction: early results with a modified Norwood procedure.
abstract: OBJECTIVE
  Prospective audit of the first year of implementation of a modified approach to palliation for infants with hypoplastic systemic ventricle and severe systemic outflow obstruction.
  
  
  METHODS
  Tertiary referral centre for neonatal and infant cardiac surgery.
  
  
  METHODS
  17 of 19 infants (aged < 35 days) presenting to Birmingham Children's Hospital in 1993 with hypoplastic systemic ventricle and severe outflow obstruction underwent surgery. This was performed using a new modification of the Norwood-type arch repair, without the use of exogenous material, and a 3.5 mm Gore-tex shunt between the innominate and right pulmonary arteries. The Gore-tex shunt was replaced by a cavopulmonary shunt between 3 and 5 months later. Clinical, morphological, and functional determinants of outcome were examined.
  
  
  RESULTS
  10 (59%) infants survived initial surgery. All proceeded to cavopulmonary shunt without further loss. Significant atrioventricular valve regurgitation seemed to be the main risk factor for poor outcome. If this was excluded, the morphology of the dominant ventricle seemed to have little effect on the outcome of initial surgery.
  
  
  CONCLUSIONS
  Early survival was achieved in 59% of patients in the first year of implementation of a protocol for surgery in infants with hypoplastic systemic ventricle and severe outflow obstruction. The construction of a neoaorta without the use of exogenous material may allow improved later growth of the neoaorta. Early cavopulmonary shunt can be performed safely and should reduce mid-term complications from cyanosis and systemic ventricular volume loading.
authors.affiliation: Heart Unit, Birmingham Children's Hospital.
authors.forename: F A
authors.initials: FA
authors.lastname: Bu'Lock
authors.forename: O
authors.initials: O
authors.lastname: Stümper
authors.forename: R
authors.initials: R
authors.lastname: Jagtap
authors.forename: E D
authors.initials: ED
authors.lastname: Silove
authors.forename: J V
authors.initials: JV
authors.lastname: De Giovanni
authors.forename: J G
authors.initials: JG
authors.lastname: Wright
authors.forename: B
authors.initials: B
authors.lastname: Sethia
authors.forename: W J
authors.initials: WJ
authors.lastname: Brawn
mesh_terms: D005260:Female; D018610:Heart Bypass, Right; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000601:surgery*; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008485:Medical Audit; D010166:Palliative Care; D011446:Prospective Studies; D015996:Survival Rate; D016896:Treatment Outcome; D014694:Ventricular Outflow Obstruction / Q000601:surgery*