Surgical strategy for atrioventricular septal defect and tetralogy of Fallot or double-outlet right ventricle

Ann Thorac Surg. 2013 Jun;95(6):2079-84; discussion 2084-5. doi: 10.1016/j.athoracsur.2013.02.016. Epub 2013 Apr 18.

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.

Publication types

  • Comparative Study
  • Evaluation Study

MeSH terms

  • Abnormalities, Multiple / diagnostic imaging
  • Abnormalities, Multiple / mortality
  • Abnormalities, Multiple / surgery
  • Academic Medical Centers
  • Adolescent
  • Adult
  • Cardiac Surgical Procedures / adverse effects
  • Cardiac Surgical Procedures / methods*
  • Cardiac Surgical Procedures / mortality*
  • Child
  • Child, Preschool
  • Cohort Studies
  • Combined Modality Therapy
  • Double Outlet Right Ventricle / diagnostic imaging
  • Double Outlet Right Ventricle / mortality
  • Double Outlet Right Ventricle / surgery*
  • Echocardiography, Doppler
  • Female
  • Follow-Up Studies
  • Heart Defects, Congenital / diagnostic imaging
  • Heart Defects, Congenital / mortality
  • Heart Defects, Congenital / surgery
  • Heart Septal Defects / diagnostic imaging
  • Heart Septal Defects / mortality
  • Heart Septal Defects / surgery*
  • Hospital Mortality*
  • Humans
  • Infant
  • Kaplan-Meier Estimate
  • Logistic Models
  • Male
  • Minnesota
  • Multivariate Analysis
  • Proportional Hazards Models
  • Retrospective Studies
  • Risk Assessment
  • Survival Rate
  • Tetralogy of Fallot / diagnostic imaging
  • Tetralogy of Fallot / mortality
  • Tetralogy of Fallot / surgery*
  • Time Factors
  • Treatment Outcome
  • Young Adult

Supplementary concepts

  • Atrioventricular Septal Defect