Surgical Strategy for Atrioventricular Septal Defect and Tetralogy of Fallot or Double-Outlet Right Ventricle

摘要

Statistical AnalysisResultsSurvivalReoperationRisk Factors for Death and ReoperationFollow-UpTetralogy 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.MethodsFrom 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.ResultsSurgical 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.ConclusionsComplete 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.CTSNet classification:21Tetralogy of Fallot (TOF), or double-outlet right ventricle (DORV) with complete atrioventricular (AV) septal defect (AVSD), is an uncommon complex congenital heart anomaly (Fig 1Fig 1). In general, complete repair of TOF/DORV-AVSD should be considered whenever possible because palliative procedures may be associated with excessive volume loading of the ventricle and progression of common AV valvular (AVV) regurgitation (CAVVR). However, complete repair may not always be?possible due to the complexity of the lesion and other?associated cardiac anomalies. Controversy exists regarding the best surgical approach in patients with TOF/DORV-AVSD with respect to the timing of complete repair and the role of palliation. Few data are available on long-term outcome of complete repair of TOF/DORV-AVSD, with little discussion of surgical strategy.Fig 1Diagram of tetralogy of Fallot/double-outlet right ventricle-atrioventricular septal defect (AVSD) shows overriding of the aorta, right ventricular outflow obstruction, and complete atrioventricular septal defect. (OPASD?= ostium primum atrial septal defect; PT?= pulmonary trunk; VSD?= ventricular septal defect; ZOA = zone of apposition.)View Large Image | Download PowerPoint SlideWe reviewed our 50-year surgical experience with TOF/DORV-AVSD. Particular attention was given to the surgical strategies of complete repair (CR) vs single-ventricle (SV) palliation as well as long-term outcome.Jump to SectionStatistical AnalysisResultsSurvivalReoperationRisk Factors for Death and ReoperationFollow-UpCommentDiscussionReferencesThe Mayo Foundation Institutional Review Board approved this study, and all patients or their families gave