Original articleCardiovascularAnalysis of Surgical Outcome in Complex Double-Outlet Right Ventricle With Heterotaxy Syndrome or Complete Atrioventricular Canal Defect
Section snippets
Patients
From January 1992 to May 1999, 96 patients (50 males, 46 females) who had complex DORV with heterotaxy or CAVC defect and were admitted to Children’s Hospital, Boston, Massachusetts, for surgery were eligible for entry into the study. Patients were retrospectively identified by review of the cardiac surgical, echocardiography, and cardiac catheterization laboratory databases. Assignment to the asplenia or polysplenia group was done according to the findings at echocardiography using criteria
Results
Between January 1992 and May 1999, 96 children (50 males, 46 females) with complex DORV (with heterotaxy or CAVC) were admitted to Children’s Hospital, Boston, and underwent surgery. Median follow-up was 16 months (range, 1 day to 7.4 years). As depicted in Table 1, of these 96 children, 68 patients had heterotaxy syndrome, 83 patients had CAVC defect, including 37 patients with unbalanced form of AV canal defect, and 30 patients had TAPVC. Fifty-six patients had both heterotaxy syndrome and
Comment
In this study, we found that AVVR, pulmonary venous obstruction, and neonatal presentation, but not TAPVC, are independent risk factors for mortality in patients with complex DORV with heterotaxy and/or CAVC.
Double-outlet right ventricle encompasses a broad spectrum of anatomic variants and associated malformations. Heterotaxy syndrome, which is often associated with TAPVC and CAVC, has been considered a risk factor for surgical repair of DORV. The early and mid-term outcomes for children with
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References (17)
- et al.
The modified Fontan operation in children less than 4 years old
J Am Coll Cardiol
(1990) - et al.
Factors associated with marked reduction in mortality for Fontan operations in patients with single ventricle
J Thorac Cardiovasc Surg
(1992) - et al.
Fontan operation in five hundred consecutive patientsfactors influencing early and late outcome
J Thorac Cardiovasc Surg
(1997) - et al.
Extending the limits for modified Fontan procedures
J Thorac Cardiovasc Surg
(1986) - et al.
Total anomalous pulmonary venous drainage in newborns with visceral heterotaxy
Ann Thorac Surg
(1994) - et al.
Long-term outcome of infants with single ventricle and total anomalous pulmonary venous connection
J Thorac Cardiovasc Surg
(1999) - et al.
Improving outcome in functional single ventricle and total anomalous pulmonary venous connection
Ann Thorac Surg
(2004) - et al.
Modified Fontan procedure in ninety-nine cases of atrioventricular valve regurgitation
J Thorac Cardiovasc Surg
(1997)