Intermediate outcome for the single-stage surgical repair of aortopulmonary window associated with interrupted aortic arch

OBJECTIVES: Aortopulmonary window (APW) associated with an interrupted aortic arch (IAA) is a rare cardiac malformation with significant mortality and morbidity. The goal of this study was to report the intermediate outcomes of single-stage repair concentrating on the surgical techniques and postoperative reintervention for this rare cardiac lesion. METHODS: Eleven patients were diagnosed with IAA-associated APW and underwent single-stage surgical repair at Vietnam National Children’s Hospital. RESULTS: The APW anatomy types were types I, II and III in 1, 4 and 6 patients, respectively. The IAA morphology was type A in 9 patients and type B in 2 patients. The median age was 27 [interquartile range (IQR) 6–79] days, and the median weight was 3.5 (IQR 2.8–4.5) kg. The aortic arch was repaired using direct end-to-side tissue anastomosis in 7 patients, and patch aortoplasty was performed in 4 patients. Six patients underwent APW closure with an intra-aortic baffle, and 5 patients required right pulmonary artery detachment and reimplantation. One early death occurred. Four patients required reinterventions: 1 patient required reoperation due to aortic stenosis and 3 required balloon angioplasty for either recurrent aortic arch stenosis (n = 1) or right pulmonary stenosis (n = 2) with a mean follow-up time of 3.1 years (IQR 0.5–4.3 years). CONCLUSIONS: Single-stage repair of IAA-associated APW can be achieved with good survival outcomes in children. However, the need for repeat reintervention or reoperation remains high, and the growth of both the aorta and pulmonary arteries should follow carefully as the patient grows. CLINICAL REGISTRATION NUMBER: VNCH-RICH-18–96