Vladimiro L. Vida, Kimberlee Gauvreau, Adam Dorfman, Alesandro Larrazabal, Frank Pigula, Emile A. Bacha, John E. Mayer, Pedro J. Del Nido, Francis Fynn-Thompson; Children's Hospital of Boston, Boston, MA
Objective: Although the overall surgical results for the management of hypoplastic left heart syndrome (HLHS) have improved considerably over the last decade, patients with aortic atresia and mitral stenosis (AA/MS) continue to present a particularly high surgical risk. We sought to define specific anatomic and preoperative characteristics that placed this anatomic subgroup at increased perioperative risk.Methods: From January 2001 to December 2005, all patients with the AA/MS variant of HLHS who underwent surgical palliation were retrospectively reviewed. We evaluated preoperative echocardiographic data, operative characteristics and postoperative factors associated with death. Kaplan-Meier analysis was used to assess survival.
Results: Thirty-two of 143 patients (22%) who underwent first stage palliative procedure for HLHS had AA/MS. Hospital mortality for patients with AA/MS was significantly higher than for other anatomic subgroups (p=0.004). Coronary artery-to-left ventricle fistulae (CAF) were present in 14 of the 32 patients (44%) and were associated with a significantly higher hospital (p=0.04) as well as inter-stage (p=0.02) mortality. At first stage surgical palliation, 21 patients (64.5%) had a modified Blalock-Taussig shunt while 11 patients (35%) had a right ventricle to pulmonary artery (Sano) conduit. No difference in outcome was demonstrated between these two groups. Twenty-four patients (75%) had delayed sternal closure, and 6 (19%) required post-operative extra-corporeal membrane oxygenation (ECMO) support. A longer cardio-pulmonary bypass time (p=0.006), longer circulatory arrest time (p=0.006), and the need for postoperative ECMO support (p=0.001) were associated with a higher mortality rate. Inter-stage and mid-term survival after initial surgical palliation were significantly better (p=0.008 and p=0.001 respectively) in patients who did not have CAF.
Conclusion: Surgical management for patients with AA/MS variant of HLHS continues to be challenging. The presence of CAF seems to be associated with a higher hospital, inter-stage and mid-term mortality after surgical palliation.
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