Association of interrupted aortic arch, aortopulmonary window with anomalous origin of the right pulmonary artery from the aorta, one-stage repair and postoperative outcomes: A case report

Özet

Association of interrupted aortic arch (IAA), aortopulmonary window (APW) with aortic origin of the right pulmonary artery (RPA), first described by Berry and colleagues in 1982, is an extremely rare congenital heart disease (1). IAA is seen in 0.7 to 1%–4% of congenital heart disease. Additionally, it has been reported that APW can be associated with IAA at a rate of 4% (2). Though rare presentations may be seen in adolescents, clinical presentation mostly occurs in the neonatal or infancy period. Early diagnosis plays an important role in the management of this disease, and, with late presentation, hemodynamics may be impaired as a result of ductus arteriosus closure. Surgery is mandatory as this is fatal. One-stage surgery can be performed, even on a low birth weight neonate (3). We report a rare case of a newborn requiring complex surgery who presented with shock and was diagnosed as having an association with type A IAA, type 3 APW, with RPA arising from the aorta.