Long-term results after primary one-stage repair of transposition of the great arteries and aortic arch obstruction

OBJECTIVES: The study was designed to evaluate perioperative and late results after primary, single-stage arterial switch operation (ASO) associated with aortic arch obstruction repair. Outcome of patients with more than five years of follow-up were analyzed. BACKGROUND: The treatment of patients with transposition of the great arteries, or other forms of ventriculoarterial discordance suitable for an ASO, with coexisting arch obstruction is a difficult task. Single-stage repair has become the treatment of choice at many institutions, but large series with long-term results are seldom reported. METHODS: Between 1990 and 1998, a primary operation including aortic arch repair through a midline sternotomy was performed in 38 patients. The relief of arch obstruction was accomplished during a period of hypothermic circulatory arrest, employing a wide pericardial patch to enlarge the inner curvature of the entire arch in most patients. RESULTS: There were nine (24%) hospital deaths. None could be directly related to aortic arch repair, but additional risk factors for an ASO were common (right ventricular hypoplasia, complex coronary anatomy, uncommon relationship between the great vessels or severe pulmonary hypertension). There were no late deaths. Four patients required cardiac reoperation, whereas three underwent successful treatment of recurrent coarctation with balloon angioplasty. CONCLUSIONS: Infants with ventriculoarterial discordance and aortic arch obstruction represent a high-risk subgroup of candidates for an ASO. Despite a non-negligible operative mortality, single-stage primary repair represents the treatment of choice, and follow-up of operative survivors is favorable. Pericardial patch enlargement is a reliable technique for arch obstruction repair.