title: Outcome after repair of atrioventricular septal defect with tetralogy of Fallot.
abstract: BACKGROUND
  Long-term outcomes of repair of tetralogy of Fallot associated with complete atrioventricular septal defect are seldom reported. We report our survival and reintervention outcomes over a 29-year time period.
  
  
  METHODS
  Between March 1979 and April 2008, 61 patients with the combined cardiac defect of atrioventricular septal defect and tetralogy of Fallot were surgically managed. Trisomy 21 was present in 49 (80%) patients. Primary repair was performed in 36 patients at a median age of 9 months (range, 1 month to 16 years), whereas 25 patients had initial palliation by systemic-pulmonary shunt at a median age of 21 months (range, 0 days to 36 years). Thirty-one (51%) patients had a transannular patch. Fifty-three patients required right ventriculotomy for relief of the right ventricular outflow tract obstruction. Four patients had a right ventricle-pulmonary artery conduit with a homograft. Relationships between patient characteristics and outcome variables were examined using Kaplan-Meier survival curves; comparisons were performed using the log-rank test.
  
  
  RESULTS
  Median follow-up was 4.7 years. A total of 12 patients died during the course of follow-up: 4 (7%)deaths within 30 days of surgery and 8 late deaths (range, 4 months to 9.9 years after repair). Since 2000, there have been no early deaths and 1 late death, 5 months after the operation. The estimated survival at 5 years after definitive repair was 82% (95% confidence interval, 69%, 90%). Time to death was not associated with any patient or surgical variables examined. Overall, 30% of the survivors required a reoperation. The type of reoperations was on the mitral valve (4 repairs, 4 replacements) and 7 pulmonary valve replacements. We did not find an effect of era on mortality (P = .23 for comparison of 1979-1989, 1990-1999, and 2000-2008). The percentage of patients with primary repair did not change during the different quartiles. The estimated freedom from reoperation at 5 years was 80% (65%, 90%). Time to reoperation was shorter for patients with a conduit (P = .01).
  
  
  CONCLUSIONS
  Excellent long-term survival was achieved after repair of tetralogy of Fallot associated with complete atrioventricular septal defect. Palliation and primary repair resulted in comparable outcomes; as such, primary repair is favored. The choice of right ventricular outflow tract reconstruction affects the need for reoperation.
authors.affiliation: Department of Cardiovascular Surgery, Children's Hospital Boston, Boston, Mass., USA. jeffrey.shuhaiber@gmail.com
authors.forename: Jeffrey H
authors.initials: JH
authors.lastname: Shuhaiber
authors.forename: Barbara
authors.initials: B
authors.lastname: Robinson
authors.forename: Kimberlee
authors.initials: K
authors.lastname: Gauvreau
authors.forename: Roger
authors.initials: R
authors.lastname: Breitbart
authors.forename: John E
authors.initials: JE
authors.lastname: Mayer
authors.forename: Pedro J
authors.initials: PJ
authors.lastname: Del Nido
authors.forename: Frank
authors.initials: F
authors.lastname: Pigula
mesh_terms: D000015:Abnormalities, Multiple; D000293:Adolescent; D000328:Adult; D001900:Boston; D006348:Cardiac Surgical Procedures / Q000009:adverse effects / Q000401:mortality; D002648:Child; D002675:Child, Preschool; D005260:Female; D006344:Heart Septal Defects, Atrial / Q000150:complications / Q000401:mortality / Q000601:surgery*; D006345:Heart Septal Defects, Ventricular / Q000150:complications / Q000401:mortality / Q000601:surgery*; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D008297:Male; D010166:Palliative Care; D012086:Reoperation; D018570:Risk Assessment; D012307:Risk Factors; D017741:Survivors; D013771:Tetralogy of Fallot / Q000150:complications / Q000401:mortality / Q000601:surgery*; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult