title: Individualized approach to the surgical treatment of tetralogy of Fallot with pulmonary atresia.
abstract: BACKGROUND
  Tetralogy of Fallot with pulmonary atresia is a heterogeneous group of defects, characterised by diverse sources of flow of blood to the lungs, which often include multiple systemic-to-pulmonary collateral arteries. Controversy surrounds the optimal method to achieve a biventricular repair with the fewest operations while basing flow to the lungs on the native intrapericardial pulmonary arterial circulation whenever possible. We describe an individualized approach to this group of patients that optimizes these variables.
  
  
  METHODS
  Over a consecutive 10-year period, we treated 66 patients presenting with tetralogy of Fallot and pulmonary atresia according to the source of the pulmonary arterial flow. Patients were grouped according to whether the flow of blood to the lungs was derived exclusively from the intrapericardial pulmonary arteries, as seen in 29 patients, exclusively from systemic-to-pulmonary collateral arteries, as in 5 patients, or from both the intrapericardial pulmonary and collateral arteries, as in the remaining 32 patients. We divided the latter group into 9 patients deemed simple, and 23 considered complex, according to whether the pulmonary arterial index was greater than or less than 90 millimetres squared per metre squared, and whether the number of collateral arteries was less than or greater than 2, respectively.
  
  
  RESULTS
  We achieved complete biventricular repair in 58 patients (88%), with an overall mortality of 3%. Repair was accomplished in a single stage in all patients without systemic-to-pulmonary collateral arteries, but was staged, with unifocalization, in the patients lacking intrapericardial pulmonary arteries. Complete repair without unifocalization was achieved in all patients with the simple variant of the mixed morphology, and in 56% of patients with the complex variant. The average number of procedures per patient to achieve complete repair was 1, 2.2, 3.8, and 2.6 in patients with exclusively native intrapericardial, simple and mixed, complex and mixed and exclusively collateral pulmonary arterial flow, respectively.
  
  
  CONCLUSIONS
  An individualized approach based on the morphology of the pulmonary arterial supply permits achievement of a high rate of complete intracardiac repairs, basing pulmonary arterial flow on the intrapericardial pulmonary arteries in the great majority of cases, and has a low rate of reoperation and mortality.
authors.affiliation: Department of Pediatric Cardiothoracic Surgery, Rainbow Babies and Children's Hospital, Case Western Reserve University, 11100 Euclid Avenue, Cleveland, OH 44106, USA.
authors.forename: Ahmed
authors.initials: A
authors.lastname: Farouk
authors.forename: Kenneth
authors.initials: K
authors.lastname: Zahka
authors.forename: Ernest
authors.initials: E
authors.lastname: Siwik
authors.forename: Francine
authors.initials: F
authors.lastname: Erenberg
authors.forename: Yasser
authors.initials: Y
authors.lastname: Al-Khatib
authors.forename: Alex
authors.initials: A
authors.lastname: Golden
authors.forename: Mohsen
authors.initials: M
authors.lastname: Karimi
authors.forename: Minhaz
authors.initials: M
authors.lastname: Uddin
authors.forename: Hani A
authors.initials: HA
authors.lastname: Hennein
mesh_terms: D003097:Collateral Circulation; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011651:Pulmonary Artery; D018633:Pulmonary Atresia / Q000150:complications / Q000401:mortality / Q000601:surgery*; D015996:Survival Rate; D013771:Tetralogy of Fallot / Q000150:complications / Q000401:mortality / Q000601:surgery*; D016896:Treatment Outcome