Surgical outcome in aortopulmonary window beyond the neonatal period.

OBJECTIVE: Aortopulmonary window (APW) is a rare congenital cardiac defect accounting for 0.1% to 0.2% of all congenital cardiac defects. We here present the current midterm outcome of surgical repair of APW in patients more than 3 months of age. METHODS: The retrospective study was conducted to identify all the patients more than 3 months of age at presentation who underwent surgical repair of APW between June 2010 and August 2018 at our tertiary care institute and their outcome was analyzed. RESULTS: We found 14 patients of APW operated at the age of more than 3 months over a period of 8 years. Mean age of the cohort was 2.29 ± 2.96 years ranging from 3 months to 10 years with 57.14% being males. There were 11 (78.57%) patients with isolated APW and 3 (21.43%) had associated cardiac defects including tetralogy of Fallot (n = 1), ventricular septal defect (n = 1), subaortic membrane causing subaortic stenosis (n = 1), and one had extracardiac malformations. Two patients had type I, nine had type II, and three had type III APW as per Jacobs' classification. The mean size of the defect was 14.14 ± 4.33 mm. Mean duration of mechanical ventilation was 26.91 ± 16.65 hours (range, 12.25-67 hours). There was one in-hospital mortality and no late mortality over a mean follow-up of 3.06 ± 2.19 years. None of the patients required any kind of reintervention. CONCLUSION: Good results can be obtained even on late presentation with adequate perioperative care of the patients with the reversible pulmonary hypertensive disease.

A Tubular Aortopulmonary Window: An Embryological Curiosity.

We describe, in this report, an unusually shaped aortopulmonary communication observed in a six-month-old infant who presented with an associated ventricular septal defect. The defect was tubular, measuring 7 mm in length, and located intrapericardially between the proximal ascending aorta and the pulmonary trunk. It was well defined by transthoracic echocardiogram and was suitable for surgical ligation. We share our dilemma in naming this defect appropriately. We base our explanation on our understanding of normal development of the intrapericardial arterial trunks. There is initially an extensive aortopulmonary foramen. This is closed by apposition of a protrusion from the dorsal wall of the aortic sac, the aortopulmonary septum, with the distal margins of the outflow cushions. The spiral nature of formation of the aortopulmonary septum provides an understanding of the configuration of our tubular aortopulmonary window.