BACKGROUND/ PURPOSE: Aortopulmonary window (APW) is a rare congenital heart disease and surgical correction is advised as early as possible. However, few studies have focused on infants. This study sought to define the clinical features and outcomes in patients who underwent surgical repair of APW in early infancy. METHODS: Between 1983 and 2004, there were 14 patients (0.15%) with APW out of 9414 patients with congenital heart disease. Ten underwent surgical repair of APW when they were younger than 4 months of age and constituted the study population. RESULTS: There were four patients with type I APW, three with type II and three with type III. Concomitant cardiovascular anomalies were present in all patients, mainly aortic arch anomalies (80%, including right aortic arch, interrupted aortic arch [IAA] and severe coarctation of the aorta [CoA]). Patients were further grouped according to the presence (n = 5) or absence (n = 5) of IAA or severe CoA. All patients underwent surgical repair of APW with various techniques, including direct ligation, division and patch or flap closure. There was one early postoperative death. None of the survivors died during a median follow-up of 34 months (range, 8-116 months). Patients with IAA or severe CoA had longer intubation time, intensive care unit stay and hospital stay. Early reintervention was required only in patients with IAA or severe CoA (n = 3, 60%). Among them, two had significant residuals and both had type III APW. CONCLUSION: Long-term outcomes are generally good in patients with APW who have undergone surgical correction in early infancy. However, associated IAA or severe CoA may predict a prolonged hospital course and an increased risk of early reintervention. In patients with type III APW requiring early reintervention, significant hemodynamic residuals may be common even after reintervention.
BACKGROUND/ PURPOSE: Aortopulmonary window (APW) is a rare congenital heart disease and surgical correction is advised as early as possible. However, few studies have focused on infants. This study sought to define the clinical features and outcomes in patients who underwent surgical repair of APW in early infancy. METHODS: Between 1983 and 2004, there were 14 patients (0.15%) with APW out of 9414 patients with congenital heart disease. Ten underwent surgical repair of APW when they were younger than 4 months of age and constituted the study population. RESULTS: There were four patients with type I APW, three with type II and three with type III. Concomitant cardiovascular anomalies were present in all patients, mainly aortic arch anomalies (80%, including right aortic arch, interrupted aortic arch [IAA] and severe coarctation of the aorta [CoA]). Patients were further grouped according to the presence (n = 5) or absence (n = 5) of IAA or severe CoA. All patients underwent surgical repair of APW with various techniques, including direct ligation, division and patch or flap closure. There was one early postoperative death. None of the survivors died during a median follow-up of 34 months (range, 8-116 months). Patients with IAA or severe CoA had longer intubation time, intensive care unit stay and hospital stay. Early reintervention was required only in patients with IAA or severe CoA (n = 3, 60%). Among them, two had significant residuals and both had type III APW. CONCLUSION: Long-term outcomes are generally good in patients with APW who have undergone surgical correction in early infancy. However, associated IAA or severe CoA may predict a prolonged hospital course and an increased risk of early reintervention. In patients with type III APW requiring early reintervention, significant hemodynamic residuals may be common even after reintervention.