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.