title: Surgical strategy for atrioventricular septal defect and tetralogy of Fallot or double-outlet right ventricle.
abstract: BACKGROUND
  Tetralogy of Fallot, or double-outlet right ventricle with atrioventricular (AV) septal defect (TOF/DORV-AVSD), is rare, with limited long-term data available. We report our institutional experience and outcome over a 50-year period.
  
  
  METHODS
  From January 1961 to January 2011, 73 patients (50 males [68%]), with a mean age of 6.8 ± 4.4 years (range, 1 month to 35 years), underwent surgical repair of TOF/DORV-AVSD. Symptoms included cyanosis in 50 (69%) and heart failure in 12 (17%). Down syndrome was present in 25 (34%). Rastelli type A, B, and C was seen in 12%, 7%, and 81% of patients, respectively. Moderate or more common AV valve (AVV) regurgitation was present in 40%. Forty-nine patients (67%) had previous palliation, including 36 with a systemic-to-pulmonary arterial shunt.
  
  
  RESULTS
  Surgical management included two-ventricle complete repair (CR) in 35 (48%) and single-ventricle (SV) palliation in 38 (52%). Overall, early mortality was 31% for CR and 34% for SV; after 1990, mortality was 6% for CR and 14% for SV. Repair before 1990 (p = 0.008) and the presence of significant common AVV regurgitation (p = 0.016) were univariate risk factors for early death in both groups. Median follow-up was 9.8 years (maximum, 32 years). Late mortality rate was 12% in CR (n = 6) and 18% (n = 9) in SV (p = 0.95). The presence of significant right AVV regurgitation was associated with late death (p = 0.02). Overall survival at 1, 5, and 15 years was 92%, 77%, and 77% in CR, and 83%, 79%, 70% in SV (p = 0.9). Freedom from reoperation at 1, 5, and 15 years was 95%, 85%, 67% in CR and 96%, 91%, 82% in SV (p = 0.1). Reoperations were most common for right ventricular outflow tract pathology, Fontan revision, and AVV intervention. Right AVV regurgitation (p = 0.018) and repair before 1990 (p = 0.041) were risk factors for late reoperation in both groups.
  
  
  CONCLUSIONS
  Complete repair of TOF/DORV-AVSD is standard of care and associated with low early mortality rate in the current era, with reasonable long-term outcome. SV palliation continues to have significant risk. The presence of AVV regurgitation is a significant risk factor for death and reoperation.
authors.affiliation: Division of Cardiovascular Surgery, Mayo Clinic and Foundation, Rochester, Minnesota, USA.
authors.forename: Vijayakumar
authors.initials: V
authors.lastname: Raju
authors.forename: Harold M
authors.initials: HM
authors.lastname: Burkhart
authors.forename: Natalie
authors.initials: N
authors.lastname: Rigelman Hedberg
authors.forename: Benjamin W
authors.initials: BW
authors.lastname: Eidem
authors.forename: Zhuo
authors.initials: Z
authors.lastname: Li
authors.forename: Heidi
authors.initials: H
authors.lastname: Connolly
authors.forename: Hartzell V
authors.initials: HV
authors.lastname: Schaff
authors.forename: Joseph A
authors.initials: JA
authors.lastname: Dearani
mesh_terms: D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging / Q000401:mortality / Q000601:surgery; D000046:Academic Medical Centers; D000293:Adolescent; D000328:Adult; D006348:Cardiac Surgical Procedures / Q000009:adverse effects / Q000379:methods* / Q000401:mortality*; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D003131:Combined Modality Therapy; D004310:Double Outlet Right Ventricle / Q000000981:diagnostic imaging / Q000401:mortality / Q000601:surgery*; D015150:Echocardiography, Doppler; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging / Q000401:mortality / Q000601:surgery; D006343:Heart Septal Defects / Q000000981:diagnostic imaging / Q000401:mortality / Q000601:surgery*; D017052:Hospital Mortality; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D016015:Logistic Models; D008297:Male; D008910:Minnesota; D015999:Multivariate Analysis; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D018570:Risk Assessment; D015996:Survival Rate; D013771:Tetralogy of Fallot / Q000000981:diagnostic imaging / Q000401:mortality / Q000601:surgery*; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult