日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

Persistent fifth aortic arch (PFAA) is a rare congenital anomaly and may cause the same clinical symptom as coarctation of the aorta. We experienced two neonatal cases with PFAA and interrupted fourth aortic arch. A 15-day-old boy was referred to our hospital because of ductal shock. He underwent the emergent aortic arch repair. A 7-day-old boy was admitted to our institute for suspected coarctation of the aorta. There was reverse blood flow from the left subclavian artery to the descending aorta. He underwent the elective aortic arch repair at 14 days of age. The ductal tissue was resected, and a proximal part of PFAA was used for aortic arch reconstruction in both patients. Although one patient required balloon dilatation 2 months after the surgery, neither patient had a pressure gradient between the upper and lower extremities 11 and 10 months after surgery, respectively. Histological findings of PFAA wall showed three normal layers as observed in a normal aortic wall. This suggested that PFAA wall could be used for aortic auch reconstruction.