Aortic origin of the RPA: immediate resolution of severe pulmonary artery hypertension by surgical repair.

Aortic origin of the right pulmonary artery (AORPA) is a rare condition requiring a high index of suspicion for diagnosis as the usual signs of structural heart disease are often absent. Ultrasound examination can strongly suggest the condition but can easily be misinterpreted. Infants should be considered "operable" even when the resistance calculations predict otherwise as there appears to be a large component of immediately reversible pulmonary artery hypertension. With technical skills learned from arterial switch operations (for transposition of the great arteries), surgical repositioning of the RPA should be possible in nearly all cases without the use of a tube graft.