A rare, congenital anomaly of the great arteries characterized by an extrapericardial vessel arising from the ascending aorta proximal to the brachiocephalic artery and terminating either in the dorsal aorta or in pulmonary arteries via a persistently patent arterial duct. The resulting connection is a systemic-to-systemic or systemic-to-pulmonary. Clinical manifestation include exercise intolerance, reduced femoral pulses, cyanosis with or without pulmonary hypertension and heart failure. Other congenital cardiovascular anomalies are often present and influence the clinical presentation.
The state in which profound and widespread reduction of effective tissue perfusion leads first to reversible, and then if prolonged, to irreversible cellular injury.
A case report of persistent fifth aortic arch presenting with severe left ventricular dysfunction.
Nakashima K, Oka N, Hayashi H, Shibata M, Kitamura T, Itatani K, Miyaji K. Int Heart J. 2014;55(1):87-8.
According to several previous reports on persistent fifth aortic arch (PFAA), the presentation of the patients was usually either very mild when diagnosed by physical examination including upper body systemic hypertension and systolic murmur, or severe with ductal shock in the neonatal period.
