Comment
In 1969, Van Praagh and Van Praagh first reported a PFAA in a male patient7. PFAA is usually located below the fourth aortic arch. As a result, the distal aortic arch takes a bifurcated shape, which is also called a double-lumen aortic arch. Embryologically, mammals have six pairs of primitive pharyngeal arches, which form the aortic arch and the head and neck vessels. After normal development, the left fourth aortic arch develops as the normal left aortic arch, and both sixth aortic arches develop as the pulmonary arteries. Ductus arteriosus originates from the distal portion of the left sixth aortic arch. Both fifth aortic arches normally degenerate without leaving any remnants. However, when the fifth aortic arch persists and forms an additional aortic arch, it results in a double-lumen aortic arch without a known mechanism. In a review of the clinical practice of PFAA, PFAA was classified into four types according to the connection of the vessels and the direction of the blood flow: systemic-to-systemic, systemic-to-pulmonary, pulmonary-to-systemic, and bilateral types8. A review of 26 cases of PFAA by Lambert et al.1 found that 76% of the cases presented with systemic-to-systemic connections via the fifth aortic arch. Of them, 38% and 19% of the cases were associated with the aortic coarctation and interruption of the fourth aortic arch, respectively.
Various surgical methods for the PFAA with aortic coarctation have been reported previously including reconstruction of the fourth aortic arch using PFAA after ligation of the ductus arteriosus2, interposition of a Gore-Tex tube graft (W. L. Gore & Assoc, Flagstaff, AZ, USA) between the ascending and descending aorta6, patch aortoplasty augmenting both the fourth and fifth aortic arches6, side-to-side anastomosis of the left common carotid artery and left subclavian artery and patch augmentation of the coarcted segment5, end-to-end anastomosis of the PFAA and descending aorta after making one aortic arch from the fourth and fifth aortic arches1,3, and end-to-end anastomosis between the fifth aortic arch and the descending aorta4. In our case, the aortic arch was reconstructed using end-to-side anastomosis between the fifth aortic arch and the descending aorta after complete resection of the ductal tissue (Fig. 2). There were several advantages to our strategy. First, because the anastomosis is simply performed between the fifth aortic arch and the descending aorta, this method is easy and effective. Second, this technique can be performed using the patient’s own tissue without any artificial patching material. Therefore, it has growth potential and no additional surgery is needed in the future.
In conclusion, PFAA with aortic coarctation is a rare congenital cardiovascular malformation, which can be repaired by various surgical methods. Among them, our surgical treatment option without the use of any artificial material showed good postoperative results and some benefits.