Download and Right Descending Aorta

Vascular Ring Due to Left Aortic Arch
and Right Descending Aorta
WILLIAM BERMAN, JR., M.D., STEVEN M. YABEK, M.D., TERRENCE DILLON, M.D.,
JOE F. NEAL, M.D., BECHARA AKL, M.D., AND JEROME BURSTEIN, M.D.
SUMMARY Two children presented with symptoms suggestive of a vascular ring. Barium esophagrams in
each case showed a posterior impression caused by a retroesophageal aortic arch. Angiographic studies defined
a rare form of vascular ring resulting from an ascending left-sided aorta, a descending right-sided aorta and a
right-sided ligamentum arteriosum. At surgery, the diagnosis was confirmed and the anomaly was corrected in
each patient. Left pulmonary anomalies were present in each patient.
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A vascular anomaly of the great vessels was
suspected, so cardiac catheterization was performed.
There was mild right ventricular and pulmonary
artery hypertension (40-mm Hg systolic pressure). No
intra- or extracardiac shunts were detected. Angiography revealed no anatomic intracardiac defects. Left
ventriculograms showed that the aorta arose normally
from the left ventricle and ascended anteriorly and to
the left in a normal fashion. In its terminal portion,
the aortic arch turned medially, crossed the midline
behind the esophagus, which it compressed, and
descended to the right of the thoracic spine (fig. 2).
There was an aberrant right subclavian artery arising
from the descending thoracic aorta (fig. 3). The left
pulmonary artery was smaller in caliber than the right.
Surgery was performed 2 days later through a right
lateral thoracotomy. The surgical findings confirmed
the presence of a vascular ring. A ligamentum
arteriosum, which originated from the descending aortic arch together with the anomolous right subclavian
artery and inserted into the right pulmonary artery,
completed the vascular ring and tethered the distal
aortic arch anteriorly against the esophagus. The
ligamentum was divided, and the esophagus was dissected and mobilized. Since surgery, the infant has
been free of feeding difficulties or other symptoms and
has gained weight appropriately.
VASCULAR RINGS surrounding the trachea and
esophagus are a cause of dysphagia and respiratory
distress during infancy. Most symptomatic vascular
rings result from a double aortic arch system or the
coexistence of a right-sided aortic arch with a leftsided patent ductus arteriosus or ligamentum
arteriosum. 1 2
We report the cases of two infants with marked
dysphagia associated with an extremely unusual form
of vascular ring. Feeding problems were managed
successfully by surgery.
Case Reports
Case 1
This 1-month-old male infant was born after an
uneventful, full-term gestation. Birth weight was 6
pounds, 3 ounces. He presented with a 2-week history
of regurgitation, choking and nasopharyngeal reflux
after all bottle feedings. He had no history of
respiratory distress or cyanosis.
On physical examination, vital signs were normal
and weight was 8 pounds. The patient was acyanotic
and the lungs were clear. Cardiac examination
revealed normal pulses, a quiet precordium, a normal
liver size and a grade 1I/VI high-pitched systolic murmur along the upper right and left sternal borders. The
infant choked and regurgitated formula repeatedly
through the nose and mouth during feeding.
Laboratory evaluation showed normal electrolytes,
blood count and urinalysis. A chest x-ray showed a
normal heart size, normal pulmonary vascular markings, and scant right perihilar and right upper lobe infiltrates. An ECG was normal. A barium esophagram
showed marked reflux of contrast material into the
nasopharynx and extrinsic, posterior compression of
the proximal esophagus (fig. 1).
Case 2
This 3½/2-year-old female had a history of chronic
dysphagia, recurrent lower respiratory infections and
easy fatiguability. Physical examination showed normal peripheral pulses and blood pressures, a right ventricular lift, an increased intensity of the pulmonic
component of the second heart sound and a grade
1I/VI systolic ejection murmur. A chest x-ray showed
right ventricular enlargement and pulmonary venous
congestion. The ECG showed right atrial and right
ventricular hypertrophy. A barium esophagram
showed a posterior impression on the esophagus. Cardiac catheterization revealed no intracardiac shunts;
the pulmonary artery pressure was 72/25 mm Hg.
The left pulmonary artery was not seen (fig. 4). The
aortic arch ascended to the left, but descended on the
right; the right subclavian artery arose from a diverticulum on the descending aorta.
Through a right thoracotomy, a right-sided liga-
From the Departments of Pediatrics, Surgery and Radiology,
University of New Mexico School of Medicine, Albuquerque, New
Mexico.
Address for correspondence: William Berman, Jr., M.D., University of New Mexico School of Medicine, Department of Pediatrics,
Albuquerque, New Mexico 87131.
Received April 23, 1980; accepted June 4, 1980.
Circulation 63, No. 2, 1981.
458
RETROESOPHAGEAL AORTIC ARCH/Bernan et al.
459
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PiGURE[ 3. Left ventricular angiogran JrtoI case 1, in
lateral projectionshow ing anomalous origin of right suhclavian arter /rofnt7i des cending aorta.
FIGURE 1. Barium esophagram from case 1, in anteroposterior and lateral projections showing posterior impression
from retroesophageal aortic arch.
FiG URi. 4. Puilmzonary arteriogram from case 2, showing
ahs ence o/ the leJt pulmlionaryi, arteryv. Dots outline the courve
of a retrograde catheter traversing the anomalous aortic
arch into the left ventricle.
mentum arteriosum was divided and the esophagus
was mobilized. The patient recovered from surgery
uneventfully and no longer complains of dysphagia.
Discussion
FIGURI. 2. Left ventricular angiograni fJrom
anteropo.vlerior projection defining the course
trathoracic aorta.
1, in
the in-
case
oJ
In 1948, Edwards hypothesized that a left-sided aortic arch with a retroesophageal component and a
right-sided descending aorta, in conjunction with an
CIRCULATION
460
ANOMALOUS RIGHT
SUBCLAVIAN ARTERY
RETROESOPHAGEAL
ARCH
IAORTIC
1
.
/: -
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DESCENDING AORTA
PULMONARY ARTERY
A.SCENDING AORTA
FIGURE 5. Illustration of arterial anomaly.
anomalous right subclavian artery and a ligamentum
arteriosum joining the origin of the right subclavian
artery to the right pulmonary artery, could form a
vascular ring about the trachea and esophagus3 (fig.
5). Later that year, Paul4 reported two children (ages 7
and 111/2 years) in whom a left-sided aortic arch and
descending right-sided aorta were documented for the
first time; however, normal origin of the right subclavian artery was present in each case and a complete
vascular ring was not present. In 1948, Edwards also
reported the autopsy findings from a 17-month-old
male who died from small bowel obstruction after abdominal surgery.5 Although that infant had no
dysphagia or respiratory distress during life,
pathologic examination of the heart and great vessels
revealed a left-sided aortic arch with a retroesophageal component, a right-sided descending aorta, anomalous origin of the right subclavian artery and
a right-sided ligamentum arteriosum. Since 1948, nine
additional cases have been reported in which a leftsided aortic arch has coexisted with a right-sided descending aorta.6"10 Four of the patients were adults,
in whom the aortic anomaly was suspected from fluoroscopic examination. Five of these patients were
children. Although each had a left-sided aortic arch
and a right-sided descending aorta, a symptomatic
vascular ring of the type described by Edwards was
present in only one of the cases.10
Our cases had a true vascular ring about the trachea
and esophagus. The aortic arch arose from the left
ventricular outflow tract and coursed leftward,
anterior to the trachea, in the normal fashion. Follow-
VOL 63, No 2, FEBRUARY 1981
ing
a course that was somewhat higher than normal
into the upper thoracic cavity, the aorta then coursed
posteriorly and to the right behind the esophagus. The
esophageal impression from the descending portion of
the aortic arch was evident on the barium esophagram. The aorta then coursed inferiorly, descending on the right side of the spine. The great vessel
branching pattern was abnormal. The first branch of
the ascending aorta was the right common carotid
artery, followed by the left common carotid and the
left subclavian arteries. The right subclavian artery
arose from the descending, right-sided portion of the
thoracic aorta. It appeared to arise from a
diverticulum-like outpouching of the anterior aortic
wall, from which the ligamentum arteriosum also
arose. The ligamentum arteriosum attached to the
right pulmonary artery and completed the vascular
ring about the trachea and esophagus (fig. 5). The left
pulmonary artery was hypoplastic in case 1 and absent
in case 2. Left pulmonary artery anomalies have not
been reported in association with this pattern of aortic
arch malformation.
The clinical findings in these two cases were
suggestive of a vascular ring. The catheterization and
angiographic findings detailed the anomalous course
of the aortic arch. Because the ductus arteriosus was
not patent, all the structures that contributed to the
vascular ring could not be visualized angiographically.
Hypothetical and autopsy reports by Edwards,
however, alerted us to the possibility that these infants' symptoms were caused by this extraordinarily
rare condition.
These case reports emphasize the complexity and
diversity of vascular malformations that may constrict the trachea and esophagus. They also point out
the contributions made to clinical medicine by
detailed and meticulous studies of the embryology and
pathology of congenital malformations.
References
1. Binet JP, Langlois J: Aortic arch anomalies in children and infants. J Thorac Cardiovasc Surg 73: 248, 1977
2. Eklof 0, Ekstrom G, Eridsson BO, Michaelsson M, Stephensen
0, Soderlund S, Thoren C, Wallgren G: Arterial anomalies
causing compression of the trachea and/or the oesophagus.
Acta Paediat Scand 60: 81, 1971
3. Edwards JE: Anomalies of the derivatives of the aortic arch
system. Med Clin North Am 32: 925, 1948
4. Paul RN: a new anomaly of the aorta - left aortic arch with
right descending aorta. J Pediatr 32: 19, 1948
5. Edwards JE: Retro-esophageal segment of the left aortic arch,
right ligamentum arteriosum and right descending aorta causing a congenital vascular ring about the trachea and esophagus.
Mayo Clin Proc 23: 108, 1948
6. Heinrich WD, Tamayo RP: Left aortic arch and right descending aorta. Am J Roentgenol 76: 762, 1956
7. de Balsac RH: Left aortic arch (posterior or circumflex type)
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Occurrence of right aortic arch in various types of congenital
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Vascular ring due to left aortic arch and right descending aorta.
W Berman, Jr, S M Yabek, T Dillon, J F Neal, B Akl and J Burstein
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Circulation. 1981;63:458-460
doi: 10.1161/01.CIR.63.2.458
Circulation is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231
Copyright © 1981 American Heart Association, Inc. All rights reserved.
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