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UNUSUAL TRUNCUS ARTERIOSUS COMMUNIS/Angelini et al.
22. Harrison TR: Harrison's Principles of Internal Medicine, ed 8, edited by
Thorn GS. New York, McGraw-Hill, 1977, p 429
23. Hogg GR: Congenital acute lupus erythematosis associated with subendocardial fibroelastosis. Am J Clin Pathol 28: 648, 1957
24. Brigden W, Bywaters EGL, Lassof MH, Ross IP: The heart in systemic
lupus erythematosus. Br Heart J 22: 1, 1960
25. Griffith GC, Vural IL: Acute and disseminated lupus erythematosis. Circulation 3: 492, 1951
26. Nora JJ: Multifactorial inheritance hypothesis for the etiology of congenital heart diseases. Circulation 38: 604, 1968
27. Nora JJ, Weishuhn EJ, Bourland BJ, Watson SC: Fluorescent antiheart
28.
29.
30.
31.
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IgM and raised levels of serum IgM in newborns with congenital heart
diseases. Br Heart J 36: 167, 1974
Aitken JK: Congenital heart block. Lancet 2: 1375, 1932
Morgan WS: The probable systemic nature of Mikulicz's disease and its
relation to Sjogren's syndrome. N Engl J Med 251: 5, 1954
James TN, Spencer MS, Kloepfer JC: Adult-onset syncope with comments on the nature of congenital heart block and the morphogenesis of
the human atrioventricular septal junction. Circulation 54: 1001, 1976
Levy AM, Camm AJ, Keane JF: Multiple arrhythmias detected during
nocturnal monitoring in patients with congenital complete heart block.
Circulation 55: 247, 1977
Truncus Arteriosus Communis
Unusual Case Associated with Transposition
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PAOLO ANGELINI, M.D., ALFREDO LLOVET VERDUGO, M.D., JAIME PEY ILLERA, M.D.,
AND ROBERT D. LEACHMAN, M.D.
SUMMARY A child with truncus arteriosus communis, characterized by the posterior origin of an individualized pulmonary trunk is
presented. This relationship between the great arteries is unusual in
truncus arteriosus communis and the spatial orientation resembles that
seen in transposition of the great vessels. A brief discussion is proposed about a proper terminology in this type of complex anomaly.
VARIETIES OF COMMON TRUNCUS have been previously discussed in the literature.1'5 For classification purposes, the length of the main pulmonary trunk and its point
of origin from the common trunk have been most frequently
utilized. It has been suggested' that the type of common
truncus, with a persisting segment of the pulmonary artery
(type I of Collett and Edwards), is embryologically derived
from the partial failure of completion of truncal septation.
Depending upon the length of the main pulmonary artery
segment and its position with reference to the aortic portion
of common trunk, it might be possible to identify truncus
arteriosus in which the aorta and pulmonary artery remnants are in the position usually identified as transposition of
the great arteries. This type of great vessel arrangement has
not been reported in truncus arteriosus.
The present case report is illustrative of what we believe to
be the simultaneous presence of "common truncus" and
transposition of the great vessels.
mur began immediately following an ejection click and was
heard best in the pulmonary area. The second heart sound
was single. No diastolic murmurs or sounds were heard. The
electrocardiogram was interpreted as regular sinus rhythm
with evidence of right ventricular hypertrophy (fig. 1). By
X-ray examination, the heart was slightly enlarged, without
selective chamber enlargement. The aortic arch was on the
left side. The vascular pedicle was narrow. The pulmonary
vascular shadows were large near the mediastinum, but
small near the periphery of the lungs (fig. 2).
Heart catheterization data are presented in table 1.
A large ventricular septal defect was seen in the angiograms below a single overriding semilunar valve. A single
arterial vessel of short length emerged from the heart and
divided into two vessels; one with the characteristics of an
ascending aorta and one with those of a pulmonary artery.
The main pulmonary artery arose posteriorly from the common trunk and had a 2 cm long undivided segment that was
obscured by the ascending aorta in the postero-anterior projection and was seen to be completely posterior to the aorta
in the lateral projection (fig. 3).
The final diagnosis was trunco-conal septal defect (common truncus arteriosus) with transposition of the divided
portion of the great vessels and pulmonary vascular obstructive disease.
In view of the high pulmonary resistances (ratio of
pulmonary to systemic resistances equal to 0.78) this child
was not considered a suitable candidate for corrective surgery.
Case Report
A five-year-old child was admitted to Texas Children's
Hospital for evaluation of congenital heart disease. He was
essentially asymptomatic, but known to have a complicated
heart anomaly from previous venous angiographic study.
On physical examination the child was well developed
and had no signs of congestive heart failure. The blood
pressure was 90/60 in both arms. There was evidence of mild
cardiomegaly with a right ventricular heave palpable at the
left lower parasternal area. A grade 2 systolic ejection murFrom the Texas Heart Institute, Houston, Texas, and Escuela Nacional
Enfermedades de Torax, Madrid, Spain.
Address for reprints: Dr. Robert D. Leachman, St. Luke's Episcopal
Hospital, P.O. Box 20269, Houston, Texas 77025.
Received May 9, 1977; revision accepted July 6, 1977.
Discussion
In most anatomic specimens of common truncus
arteriosus, type I of Collett-Edwards,2 the longer the main
pulmonary artery trunk, the more lateral and anterior is its
56, No 6, DECEMBER 1977
VOL
CIRCULATION
1108
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FIGURE
Electrocardiogram
1.
admission,
on
showing signs of right ventricular hypertrophy.
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V
2
1/V3
V4
ly
1
V5
V6
origin from the common trunk. Similarly, when there is a
very short pulmonary artery trunk. the origin is more often
from the posterior wall of the truncus, a finding that makes
the differentiation of type I from type II (Collett-Edwards)
clinically difficult.4 This was not the finding in the present
_'a
V7
relatively long
case, in which a
originated posterior
main
and did not
trunk
pulmonary artery
cross
the aortic trunk in the
lateral view.
This unusual heart stimulates
bryologic
hypothesis
Two
truncus.
might
defects that
The
error
hypothesis
trunco-conusi1
second
The
possible
to
explain
that
states
hypothesis3
common
only
of the
that
states
infundibular atresia occurred at the
pulmonary
em-
the
incomplete septation
the
is
review of
suggested
have been
traditional
developmental
a
have caused it.
same
time
that the truncus and its valve failed to divide.
It is
our
heart. It
cus
belief that there
arteriosus
resulted
trunco-conal septum.
completed,
position
of
from
ings
narrow
prominent
failure of
left
faults in this
completion
of the
that, had trunco-conal septation
arteries
is further
typical
(figs. 4, 5).
transposition of the great
septal defect,
of
embryologic
the arteries would have been in the
transposed great
This view,
conal
two
Further, the spatial arrangement of
the great vessels is such
been
are
reasonable to believe that in this case, trun-
seems
arteries with trunco-
supported by
the clinical find-
superior mediastinum and absence of
pulmonary
artery
shadow.
Further
TABLFE 1. Pressure and Oxygen Saturation Data
FIGURE 2. Chest roentgenogram showing mild cardiomegaly, increased hilar pulmonary vascular markings, decreased at the
periphery.
Right atrium
Right ventricle
Left atrium
Left ventricle
Aorta
Pulmonary artery
Pulmonary vein
Pulmonary-to-systemic flow ratio
Pulmonary resistance
Pressure
02 Sat (%)
5
70
70
98/0-5
7
98/0-8
98/58,80
98/45,75
6
1.2:1
78%
93
93
81
86
95
a
the
1109
UNUSUAL TRUNCUS ARTERIOSUS COMMUNIS/Angelini et al.
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FIGUJRE 3. Retrograde aortogram in the frontal (3a) and lateral (3b) projections, showing the unulsual originz of thse
puglmonary trunk posterior to the common arterial trunk.
b~~~~~
a
bS (4
c
FIGUJRE 4. Diagrammatic representation of normally crossed great veisels with complete (left side) aznd incomplete6
(right side) truncal septation. (a =frontal view; b =lateral view). This refers to the usualform of trun7cus arteriosus communis.
FIGURE 5. Diagrammatic representation of transposed great vessels with complete (a) and incomplete (b)
tion. Panel b is thecase of the present report.
trugncal septa-
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CIRCULATION
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hemodynamic observation that blood in the pulmonary
artery contained more oxygen than that in the aorta is contrary to that in the usual truncus arteriosus, but similar to
that in transposition of the great vessels.
We believe that the recognition of these variants is conceptually important, even though the practical importance
may be limited. Surgically it may be of some relevance. The
pulmonary trunk posterior to the aorta would require a
variation of the Rastelli technique for correction.8
Hallerman and colleagues4 reported in their series of 27
cases of truncus type I (Collett-Edwards), eight cases in
which the pulmonary trunk arose posteriorly from the undivided truncus. Unfortunately the authors do not clarify the
length of the pulmonary trunk in these cases. It is conceivable that most of them had a short trunk, as expected in
the case of an isolated anomaly of the trunco-conal septation. A case reported by Testelli7 as interrupted aortic arch
and common truncus appears to be similar to our case with
the aorta completely anterior, immediately above the truncal valve, as in transposition complexes.
The possibility of other trunco-conal anomalies, such as
transposition, co-existing with common truncus, is further
evidence of the inadequacy of the traditional terminology
"truncus arteriosus communis persistens." The recently
suggested terminology of trunco-conal septal defect5 reflects
the need for description of additional identifying character-
VOL 56, No 6, DECEMBER 1977
istics in order to avoid confusion, namely extension of the
defect, unequal partition, abnormal spiraling of the truncoconal septum, inversion of the bulboventricular loop, lateral
positions of the trunco-conus, and associated malformations
of the aortic arch. From this perspective, the classification of
this case as transposition of the great vessels in "common
truncus" is easily understood and conveys an image of the
abnormality not made clear by the simple diagnosis of truncus arteriosus.
References
1. Humphreys EM: Truncus arteriosus communis persistens: Criteria for
identification of common arterial trunk, with report of a case with four
semilunar cusps. Arch Pathol 14: 671, 1932
2. Collett RW, Edwards JE: Persistent truncus arteriosus. A classification
according to anatomic types. Surg Clin North Am 29: 1245, 1949
3. Van Praagh R, Van Praagh S: The anatomy of the common aorticpulmonary trunk (truncus arteriosus communis) and its embryologic implications. A study of 57 necropsy cases. Am J Cardiol 16: 406, 1965
4. Hallerman FJ, Kincaid OW, Tsakinis AG, Ritter DG, Titus JL: Persistens
truncus arteriosus; a radiographic and angiographic study. Am J
Roentgenol 107: 827, 1969
5. Angelini P, Leachman RD: Trunco-conal septal defects. An anatomic and
embryologic discussion of common truncus and related malformations.
Eur J Cardiol 2/1: 11, 1974
6. McGoon DC, Rastelli GC, Ongley PA: An operation for the correction of
truncus arteriosus. JAMA 205: 69, 1968
7. Testelli MR: Tronco arterioso comun con interrupcion del arco aortico.
Informe de un caso con supervivencia en edad adulta. Arch Inst Cardiol
Mex 42: 122, 1972
Truncus arteriosus communis. Unusual case associated with transposition.
P Angelini, A L Verdugo, J P Illera and R D Leachman
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Circulation. 1977;56:1107-1110
doi: 10.1161/01.CIR.56.6.1107
Circulation is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231
Copyright © 1977 American Heart Association, Inc. All rights reserved.
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