CASE REPORT
Tetralogy of Fallot with Persistent Fifth Aortic Arch:Echocardiographic Diagnosis
Gerard Holmes • Joanna L. Holmes •
William Berman Jr. • Steven Yabek
Received: 9 September 2009 / Accepted: 12 October 2009 / Published online: 10 November 2009
� Springer Science+Business Media, LLC 2009
Abstract Although persistent, fifth aortic arch (P5A) is
an uncommon anomaly. Its incidence is unknown due
largely to failure of recognition. Fundamental to its diag-
nosis is an adequate understanding of the various settings
in which P5A may manifest. One of the more frequently
reported manifestations of this unusual anomaly is a sys-
temic-to-pulmonary connection in the presence of pul-
monary atresia, with or without a ventriculoseptal defect.
This report describes a new case of P5A in an infant with
tetralogy of Fallot but not pulmonary atresia, which was
diagnosed echocardiographically. The echo-Doppler char-
acteristics of P5A in the presence of right ventricular
outflow obstruction are described, and the implications
regarding surgery for the accompanying defects are dis-
cussed. To the authors’ best knowledge, the described case
is the first reported instance of P5A in this specific setting.
Keywords Echocardiography � Persistent 5th aortic arch �Tetralogy of fallot
Persistent fifth aortic arch (P5A) was first described in 1969
as a double aortic arch lumen [9]. Since then, P5A has been
recognized in a variety of different settings, albeit in a
limited number of cases [2–7, 10]. Most diagnoses of this
condition are based on angiography or direct examination.
In one of its more frequently reported presentations,
P5A manifests as a systemic-to-pulmonary connection in
the presence of pulmonary atresia, with or without a ven-
triculoseptal defect (VSD).
We report a unique case of P5A with tetralogy of fallot
(TOF) but not pulmonary atresia, which was diag-
nosed echocardiographically. We describe the echo-
Doppler characteristics of P5A with a systemic-to-pul-
monary connection and right ventricular outflow obstruc-
tion and discuss implications regarding a surgical approach
to the accompanying cardiac defects.
Case Report
A 7-week-old girl was seen in consultation for a heart
murmur. Her birth history was unremarkable, but she had
experienced increased work of breathing and poor feeding
for a few weeks before evaluation. Physical evaluation
showed a non-dysmorphic infant with mildly increased
respiratory effort. No cyanosis, edema, or hepatomegaly
was noted. The baby’s pulses were brisk, and she had
slightly increased precordial activity without a palpable
thrill. The second sound was narrowly split and loud, and
there was a harsh medium pitch pan systolic murmur at
the mid left sternal border. No diastolic abnormalities
were noted.
A two-dimensional echo-Doppler study demonstrated
TOF with narrowing at the right ventricular os infundibu-
lum and infundibulum but a normal pulmonary valve
(Fig. 1). A tubular connection, the persistent fifth aortic
arch, was seen between the ascending aorta and the distal
main pulmonary artery just proximal to the origin of the
left pulmonary artery (Fig. 2). Diastolic flow reversal was
seen in the transverse aortic arch (Fig. 3).
Electronic supplementary material The online version of thisarticle (doi:10.1007/s00246-009-9573-4) contains supplementarymaterial, which is available to authorized users.
G. Holmes (&) � J. L. Holmes � W. Berman Jr. � S. Yabek
Pediatrix Cardiology Associates of New Mexico,
201 Cedar SE, Suite 700, Albuquerque, NM 87106, USA
e-mail: [email protected]
123
Pediatr Cardiol (2010) 31:280–282
DOI 10.1007/s00246-009-9573-4
The baby was taken to the operating room 1 week later.
After median sternotomy, the fifth aortic arch was over-
sewn and divided. Next, a cardiopulmonary bypass (CPB)
was created, and the VSD was closed with a patch. The
infundibular muscle was resected, and a right ventricular
outflow patch was placed. The infant’s postoperative
course was unremarkable, and she returned home on the
fifth postoperative day with no medications.
Discussion
The incidence of P5A is not known due largely to a failure
of recognition [3]. Gerlis et al. [3] reviewed 2,000 speci-
mens from the combined congenital heart disease collec-
tions of the Royal Brompton and Killingbeck hospitals
(UK). They reported three covert cases of P5A from this
database misdiagnosed previously as ductus arteriosus or
aortopulmonary window and estimated an incidence of one
in 330 cases.
Four different types of connections, namely, systemic to
systemic, systemic to pulmonary, pulmonary to systemic, and
combined, have been described together with a variety of
accompanying cardiac defects in cases of P5A [8]. In addi-
tion, P5A has been reported as an isolated anomaly [8]. The
variations in connections and their accompanying defects are
summarized well by Peirone et al. [8]. An understanding of
these variations is fundamental to recognizing P5A because
the nature of the connection and the accompanying cardiac
lesion determine how it appears on images such as echocar-
diograms and how it presents clinically.
A systemic-to-pulmonary connection has been reported
in at least eight previous cases of P5A with accompanying
pulmonary atresia with or without VSD [2, 3, 5, 8]. The
reported case had a right ventricular outflow obstruction
due to infundibular stenosis but not pulmonary atresia. To
the best of our knowledge, this is the first report of P5A in
this specific setting. However, if our case is grouped with
previous cases of pulmonary atresia, it seems that P5A with
systemic-to-pulmonary connection and right ventricular
outflow obstruction generally constitutes one of the more
frequent manifestations of this relatively unusual anomaly.
The reported patient had two cardinal echo-Doppler
features with accompanying TOF. First, the persistent fifth
arch appeared as a tubular connection between the ascending
aorta and the distal main pulmonary artery just proximal to
the origin of the left pulmonary artery. Second, there was a
diastolic flow reversal in the transverse aortic arch.
The tubular nature of the connection and its position in
our patient is in contrast to an aortopulmonary window,
which is broad based and distinctly nontubular and usually
involves the proximal right pulmonary artery. It also is
distinct from a ductus arteriosus, which usually would arise
Fig. 1 Parasternal short-axis view through the cardiac base showing
a large ventriculoseptal defect (VSD), anterior malalignment of the
conal septum, and os infundibular and infundibular narrowing, but a
relatively normal pulmonary valve. See movie clip in the online
version of this article
Fig. 2 More superior angulation from the cardiac base in the
parasternal short-axis sweep showing a tubular connection between
the ascending aorta and the distal main pulmonary artery just
proximal to the left pulmonary artery origin. Color shows aortic-to-
pulmonary flow. See movie clip in the online version of this article
Fig. 3 Spectral Doppler in the distal transverse aortic arch showing
diastolic flow reversal
Pediatr Cardiol (2010) 31:280–282 281
123
from the base of a subclavian or innominate artery or from
the inferior aspect of the distal aortic arch in instances of
pulmonary atresia.
Diastolic flow reversal in the transverse aortic arch is
known to occur in an aortopulmonary window and in sig-
nificant aortic valve insufficiency. It also was found in our
patient, representing runoff of blood flow from the high-
resistance systemic to the low-resistance pulmonary cir-
culations through the fifth arch. Thus, P5A also should be
considered in the differential diagnosis of TOF with P5A
diastolic flow reversal in the aortic arch, particularly when
there is right ventricular outflow obstruction.
Clinical recognition of P5A also can be important in
planning the surgical approach to repair accompanying
defects [1, 4–7]. In our patient, its recognition allowed the
surgeon first to oversew and divide the fifth arch before
creating a bypass to close the VSD, resecting the infun-
dibular musculature, and patching the right ventricular
outflow. However, if the fifth arch had been misdiagnosed
as an aortopulmonary window, the surgical approach may
have been much more complicated and risky.
Accurate diagnosis of P5A rather than an aortopulmonary
window in our patient also allowed consideration of a staged
approach. If a staged approach had been chosen, the fifth arch
would first have been ligated through a lateral thoracotomy
and the TOF repaired at a later time when the child was larger.
This approach may have been favored at other centers.
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