Background: Surgical repair of interrupted aortic arch (IAA) remains challenging and is associated with significant mortality and incidence of late arch obstruction, as recently reported by the Congenital Heart Surgeons' Society (CHSS). In particular, the CHSS reported that any technique other than direct anastomosis with patch augmentation is a risk factor for arch reintervention. The experience at Texas Children's Hospital with IAA repair using an aortic arch advancement technique without a patch was examined.
Methods: Between July 1995 and December 2005, 60 patients underwent IAA repair using aortic arch advancement without a patch. Selective cerebral perfusion was used in 25 patients (42%). Cox proportional hazards models were used to analyze 20 variables to determine risk factors for death, arch reintervention, and left ventricular outflow tract (LVOT) reintervention.
Results: Median age was 8 days (range, 2 to 271 days) and weight was 3.0 kg (range, 1.7 to 6.1 kg). IAA types were A in 18 (30%) and B in 42 (70%). Associated anomalies were multiple congenital anomalies in 30 (50%) patients, DiGeorge syndrome in 21 (35%), LVOT obstruction in 26 (43%), a single ventricle in 11 (18%), and truncus arteriosus in 6 (10%). Mean follow-up was 3.0 +/- 2.6 years. Five-year freedom from aortic arch reintervention was 100%. Survival at 30 days, 1 year, and 5 years was 93%, 78%, and 76%, respectively. Since July 2000, two of 32 patients have died for an overall survival of 94%. Risk factors for death are older age, multiple congenital anomalies, DiGeorge syndrome, and bicuspid aortic valve. Selective cerebral perfusion was an independent protective variable for survival. Survival for an IAA patient with a ventricular septal defect and no complicating cardiac anomalies was 100%.
Conclusions: Aortic arch advancement without a patch can be applied to IAA patients, with the expectation of a minimal need for arch reintervention. This technique affords an excellent survival, to which selective cerebral perfusion may be a contributing factor.