Download CORONARY ARTERIES IN TOF

CORONARY ARTERIES IN TOF/Dabizzi et al.
26. Neches WH, Mathews RA, Park SC, Lenox CC, Zuberbuhler
JR, Siewers RD, Bahnson HT: Anomalous origin of the left
coronary artery from the pulmonary artery. A new method of
surgical repair. Circulation 50: 582, 1974
27. Shubrooks SJ Jr, Naggar CZ: Spontaneous near closure of coronary
artery fistula. Circulation 57: 197, 1978
28. Noren GR, Raghib G, Moller JH, Amplatz K, Adams P Jr,
Edwards JE: Anomalous origin of the left coronary artery from
the pulmonary trunk with special reference to the occurrence of
mitral insufficiency. Circulation 30: 171, 1964
29. Sabiston DC Jr, Orme SK: Congenital origin of the left coronary
artery from the pulmonary artery. J Cardiovasc Surg 9:
543, 1968
30. Johnson AD, Detwiler JH, Higgins CB: Left coronary artery
anomaly in patients with bicuspid aortic valves. Br Heart J 40:
489, 1978
Distribution and Anomalies of Coronary Arteries
in Tetralogy of Fallot
ROBERTO PIERO DABIZZI, M.D., GIUSEPPE CAPRIOLI, M.D., LUIGI AIAZZI,
M.D.,
CARLO CASTELLI, M.D., GIORGIo BALDRIGHI, M.D., LuCIo PARENZAN, M.D.,
AND VINCENZO BALDRIGHI, M.D.
SUMMARY The surgical management of tetralogy of Fallot (TF) may be complicated by
anomalies in the
course and distribution of coronary arteries. Selective coronary angiography was
performed in 119 cases of TF
in order to prevent injury of aberrant vessels. In 11 patients, anomalies in the origin of
coronary branches were
revealed (five patients with a single coronary ostium, five with the anterior descending
coronary artery arising
from the right coronary artery or right sinus of Valsalva, and one with the circumflex
artery arising from the
right coronary artery). Other findings include anastomoses between coronary and
bronchial arteries, small
fistulas between coronary arteries and the pulmonary artery or right atrium, and
hypoplasia of the coronary
tree.
The onset of left anterior hemiblock after total correction of TF could possibly be
explained, in cases with a
dominant left pattern, by injury to the coronary system after closure of the interventricular
septal defect,
rather than by injury to the conduction system.
THE RESULT of the total correction of tetralogy of
Fallot (TF) depends not only on the seriousness of the
functional changes, but also on the possible presence
of certain unfavorable anatomic features.
Anomalies in the course and distribution of the coronary
arteries may be present in TF. In a number of
patients, the left anterior descending coronary artery
(LAD) arises from the right coronary artery (RCA)
and crosses over the right ventricular outflow tract,
thus complicating the surgical correction.'-6 Such an
anomalous vessel is not always detectable at the time
of ventriculotomy, particularly when it is obscured by
the overlying myocardium,l "-9 by epicardial fat9 1' or
by pericardial-epicardial adhesions,4 5 in patients who
have previously undergone palliative surgery.
The anomalous origin of the LAD from the RCA is
not the only variant found in these patients.1 2, 4, 6, 8-13
From the Division of Cardiology, General Hospital, Legnano, the
Division of Pediatric Cardiac Surgery, General Hospital, Bergamo,
and the Department of Medicine, Section of Cardiology, University
of Florence, Florence, Italy.
Address for correspondence: Roberto Piero Dabizzi, M.D.,
Cattedra di Malattie dell'Apparato Cardiovascolare, Universita di
Firenze, Viale Morgagni, 85, 50134 Firenze, Italy.
Received March 1, 1979; revision accepted July 13, 1979.
Circulation 61, No. 1, 1980.
In order to prevent possible consequences that may
follow the division of an aberrant vessel,1 2, 5, 14, 15 the
course and distribution of the coronary arteries must
be known before operation. For this reason, we
usually carry out selective coronary angiography in
patients with TF before corrective surgery. This report
describes our observations in 119 patients with TF.
Materials and Methods
Studies were performed on 119 cases with TF, 79
males and 40 females, ages 5 months to 26 years. Cardiac
catheterization and selective coronary angiography
were carried out. Initially, right ventricular
angiocardiography was performed in two projections
(sitting-up and hepatoclavicular), with a #6F or #7F
NIH catheter introduced into the saphenous or axillary
vein. Subsequently, selective coronary
angiography was carried out via the right axillary
artery, using a Sones 6.5F catheter in patients
weighing under 13 kg and a Sones 7.5F catheter in
those weighing over 13 kg. For the left coronary artery
(LCA), anteroposterior and right anterior oblique
projections were always used, and for the RCA,
usually the anteroposterior projection was used. In
patients in whom an aberrant artery was detected, we
also used the left anterior oblique or lateral projections.
Injections were carried out by manual pressure,
95
Downloaded from http://circ.ahajournals.org/ by guest on March 24, 2015
VOL 61, No 1, JANUARY 1980
TABLE 1. Distribution of Coronary Circulation in Tetralogy
of Fallot
nN
Dominant right type of
coronary distribution 64 53.8
Dominant left type of
coronary distribution 39 32.8
Balanced coronary distribution 16 13.4
Total 119
TABLE 2. Coronary Artery Anomalies in Tetralogy of Fallot
nN
Single coronary ostium 5 4.2
LAD from RCA or right sinus
of Valsalva 5 4.2
LCX from RCA 1 0.8
"Large" conus artery 23 19.3
Fistulas between coronary arteries
and PA or right atrtim 14 11.7
Anastomoses between coronary and
bronchial arteries 31 26.0
Hypoplastic coronary arteries 1 0.8
Abbreviations: LAD = left anterior descending artery;
RCA = right coronary artery; RSV = right sinus of Valsalva;
LCX = left circumflex artery; PA = pulmonary artery.
using 0.25-0.33 ml/kg of contrast material
(Urographin 76%).
Results
The results are summarized in tables 1 and 2.
The "large" conus artery was considered as an
anomaly because of the importance this vessel has
when it is greatly enlarged and courses over the right
ventricular infundibulum.
Distribution of Coronary Circulation
In 64 patients (53.8%) a dominant right pattern was
observed. The RCA gave rise to the artery for the
atrioventricular (AV) node and to the posterior
descending artery (PDA). The left circumflex artery
(LCX) was hypoplastic, and in some cases did not pass
into the AV groove, but ended on the posterolateral
surface of the left ventricle. In one patient, the LCX
was absent. This type of dominant right distribution
was more frequent in cases of TF with marked hypoplasia
of the pulmonary artery truck.
A dominant left coronary distribution was present
in 39 patients (32.8%). A large obtuse marginal branch
originated from the LCX, which also gave rise to the
PDA, to the branches for the posterior wall of the left
ventricle and sometimes to the artery for the AV node.
The RCA ended at the crux after giving off a large
conus artery or an anterior ventricular branch.
Sixteen patients (13.4%) had a balanced coronary
circulation. A small PDA and the artery for the AV
node originated from the RCA; the LCX supplied
large branches for the posterior wall of the left ventricle
and an artery for the obtuse margin of the heart.
Single Coronary Ostium
We considered a single coronary ostium as being
right or left depending on its location on the right or
left coronary sinus, respectively. A single coronary ostium
can be further subdivided, depending on whether
the anomalous coronary artery passes in front of or
behind the pulmonary artery. We observed five cases
of single coronary ostium, two from the left sinus and
three from the right.
Single Left Coronary Ostium
In two patients, the LCA had a normal origin in the
left sinus of Valsalva. The RCA arose from the proximal
portion of the LCA. The RCA passed behind the
aorta, reached the AV groove and coursed along it to
the crux. The PDA, the two small branches for the
posterior wall of the left ventricle and the artery for
the AV node originated at the crux. The LAD and the
LCX had normal courses. In figures IA and B, the
LAD does not supply the first septal branch and the
diagonal branches are poorly developed.
Single Right Coronary Ostium
This anomaly was seen in three patients. The first
patient, 2½/2 years old, had attacks of cyanosis and
marked hypoplasia of the pulmonary artery trunk.
The second patient, 12 months old, had anoxic spells.
We examined the third patient, 3 years old, after a
total correction with insertion of a valved conduit had
been performed owing to the presence of the LAD
coursing over the right ventricular outflow tract. We
performed selective coronary angiography to ensure
that the anomalous vessel was intact (figs. 2A and B).
In these patients, the right sinus of Valsalva, which
presented a marked anterior displacement to the left
owing to the considerable clockwise rotation of the
aorta, gave rise to a common branch that divided into
three arteries. One artery followed the normal course
of the RCA, while the second one turned to the left
and, passing across the infundibulum of the right ventricle,
ran into the anterior interventricular groove.
The third branch, passing between the aorta and the
pulmonary artery, reached the posterior wall of the
left ventricle. The lateral projection (fig. 3) shows that
the LAD is posteriorly displaced because of the considerable
clockwise rotation of the interventricular
septum. In two patients, the LAD seemed to have no
first septal branch and few diagonal branches.
Left Anterior Descending Coronary Artery Arising
from the Right Coronary Artery
or the Right Sinus of Valsalva
In accordance with Meng,8 we subdivided this
anomaly into two types, depending on the relationship
of the LAD to the base of the pulmonary artery.
96 CIRCULATION
Downloaded from http://circ.ahajournals.org/ by guest on March 24, 2015
CORONARY ARTERIES IN TOF/Dabizzi et al.
FIGURE 1. A) Cineangiograms ofa single left coronary ostium
in the anteroposterior projection. B) Same anomaly in
the left anterior oblique projection. The left anterior descending
artery is devoid of the first septal branch and poor in
diagonal branches. The first portion of the right coronary
artery runs behind the aorta.
Type 1: In three patients, the LAD arose from the
proximal portion of the RCA and reached the anterior
interventricular groove passing just below the base of
the pulmonary artery trunk. In these patients, the
LAD did not give off the first septal branch, while the
diagonal arteries were very small. One patient had an
anomalous origin of the LAD from the right sinus of
Valsalva (fig. 4).
Type 2: In one patient, the LAD arose from the
proximal portion of the hypoplastic RCA and entered
the anterior interventricular groove passing across the
outflow tract of the right ventricle, about 2-3 cm
below the base of the pulmonary artery trunk.
In all five cases, the LCA originated normally from
B , as
FIGURE 2. Cineangiograms of a single right coronary ostium
in a patient with a valved conduit. A) Anteroposterior
projection. The left anterior descending artery (LAD), right
coronary artery and branch for the posterior wall of the left
ventricle originate from the right sinus of Valsalva. B)
Lateral projection. The LAD is posteriorly displaced.
the left sinus of Valsalva, giving off only the LCX,
which ran along the AV groove. In two patients, a
small, short accessory LAD arose from the LCA and
ended before it could enter the anterior interventricular
groove.
Left Circumflex Artery from Right Coronary Artery
In one patient, 4 years of age, a common right coronary
ostium gave rise both to the RCA, with a normal
course along the AV groove, and to a large LCX,
which bent to the right and passed behind the aorta
and the pulmonary artery, ending in a long branch for
the posterolateral wall of the left ventricle. The LAD
originated normally from the left sinus of Valsalva
and ran along the anterior interventricular groove
97
Downloaded from http://circ.ahajournals.org/ by guest on March 24, 2015
VOL 61, No 1, JANUARY 1980
FIGURE 3. A) Cineangiograms of a single right coronary
ostium in the anteroposterior projection. B) Same anomaly
in the lateral projection. The left circumflex artery is
hypoplastic. The left anterior descending artery is
posteriorly displaced and poor in septal and diagonal
branches. The posterior descending artery is short.
supplying septal and diagonal branches (figs. SA and
B). In accordance with other authors,'6 we have
observed this anomaly even in normal subjects.
Large Conus Artery
In 23 patients, this vessel was remarkably
developed, but its size was not related to the type of
coronary distribution. A large conus artery was, in
fact, present in both right and left dominant patterns.
It usually originated from the proximal portion of the
RCA, but in two cases, it originated from a separate
ostium in the right sinus of Valsalva (fig. 6). In 10
patients, this vessel had a diameter equal to or greater
than that of the RCA and, crossing over the outflow
tract of the right ventricle, reached the heart apex, so
that in anteroposterior projections it could be mistaken
for the LAD (figs. 7A and B).
Fistulas from Coronary Arteries to the
Pulmonary Artery or Right Atrium
Small fistulas between the coronary arteries and the
pulmonary artery were seen in 13 patients. In eight
patients, the fistulas originated from the left main coronary
artery or the initial portion of the LCA or LAD.
In three cases they arose from the RCA and in two
from both the RCA and LCA. Figure 8 shows a case
of TF with a left dominant pattern whose left main
coronary artery gives rise to a branch that communicates
with the pulmonary artery trunk. A communication
between the coronary arterial bed
(anterior branch arising from the hypoplastic RCA)
and the right atrium was present in one patient (fig. 9).
In all cases the fistulas were small.
Anastomoses Between the Bronchial and Coronary Arteries
We observed anastomotic channels between the
RCA or LCA and the bronchial arteries in 31 cases of
severe TF with hypoplasia of the pulmonary artery
branches. Figure 10 shows a case of TF in which the
LCA gives rise to branches running superiorly and
posteriorly to anastomose with the bronchial arteries.
This patient had agenesia of the left branch of the
pulmonary artery. Figure 11 shows the RCA in the
emptying phase. Anastomoses between the RCA and
the bronchial arteries are also evident.
Hypoplasia of the Coronary Artery Tree
One patient had hypoplasia of all the coronary
branches (fig. 12), and died of a low cardiac output
syndrome during surgical correction.
Discussion
The low statistical incidence of coronary anomalies
in TF reported by surgical teams'-5 can be explained
by the fact that many of these anomalies are difficult
to detect intraoperatively. Postmortem examinations7
indicate a higher incidence, probably due to a
greater possibility of carefully examining the coronary
arteries.
Angiographic studies of the coronary arteries in TF,
apart from one of the two cases reported by White,9
have been perforfned by aortography only. Fellows,6
in his study of 84 patients with TF, visualized the coronary
arteries by injecting contrast material into the
aorta (79 cases) or into the right ventricle (five cases).
He found a 5% incidence of anomalies in coronary distribution
(4% LAD from the RCA, 1% single coronary).
98 CIRCULATION
Downloaded from http://circ.ahajournals.org/ by guest on March 24, 2015
CORONARY ARTERIES IN TOF/Dabizzi et at.
FIGURL 4. Cineangiograms of anomalous origin of the left
anterior descending artery (LAD) from the right sinus of
Valsalva (RSV). A and B) Left selective coronary
angiography in the anteroposterior and lateral projections. FIGURE 5. C
The left circumflex artery runs posteriorly in the lateral1 pro- (LCX) from th
jection. C and D) Right selective coronary angiography. The onary angiogr(
right coronary artery, conus artery and anomalous LAD The LCX is at,
from the RSV are evident in anteroposerior (C) and lateral in the lateral p
(D) projections. the aorta.
7ineangiograms of the left circumflex artery
he right coronary artery. A) Left selective coraphy
in the right anterior oblique projection.
sent. B) Right selective coronary angiography
Irojection. The LCX is evident and runs behind
99
Downloaded from http://circ.ahajournals.org/ by guest on March 24, 2015
VOL 61, No 1, JANUARY 1980
FIGURE 6. Cineangiogram of a "large" conus artery arising
from the anteriorly displaced right sinus of Valsalva.
The use of selective coronary angiography allowed
us to visualize anomalies that cannot be shown by surgical
examination and aortography, such as coronarybronchial
anastomoses or small fistulas between the
coronary arteries and the pulmonary artery.
In TF, for the reason mentioned previously, the
anomalies in distribution and origin of the coronary
arteries involving the anterior wall of the right ventricle
become very significant. These anomalies, if not
detected by angiography, may lead to errors at operation.
Thus, an aberrant vessel that courses over the outflow
tract of the right ventricle is important.
We observed a large conus artery in 23 patients
(19.3%), in 10 of whom the vessel was as large as or
larger than the RCA. This anomaly should therefore
be sought for by the surgeon.
The three patients with a single right coronary ostium
and the five with the LAD arising from the RCA
confirmed that the LAD runs over the outflow tract of
the right ventricle in 6.7% of our cases.
Eleven patients (9.2%) had anomalies in the origin
of the coronary arteries. Of these, five had a single
coronary ostium, five had the LAD originating from
the RCA or the right sinus of Valsalva and one patient
had an anomalous origin of the LCX from the RCA.
The absence of the first septal branch and the scarcity
of diagonal arteries in almost all the patients with
the LAD arising from the RCA or with a single coronary
ostium are quite interesting. In all the other
patients, we found that the first septal branch was
more or less long. A clear visualization of the other
septal branches was not always possible, but the blood
supply of the interventricular septum was similar to
that of normal subjects.
As already described by James,'7 the interventricular
septum and the conduction system can be
FIGURE 7. A "large" conus artery. A) Left selective coronary
angiography in the right anterior oblique projection.
B) Right selective coronary angiography in the left anterior
oblique projection. The conus artery arising from the right
sinus of Valsalva reaches the apex of heart and has a
diameter greater than that of the right coronary artery.
divided into two portions. The distal tract of the AV
node, the bundle of His, and the proximal portion of
the two branches of the bundle of His lie in the
superior and posterior portion of the interventricular
septum. These structures are supplied by the artery of
the AV node, by the septal branches arising from the
PDA and by the first septal branches of the LAD. The
lower and apical portion of the interventricular sep100 CIRCULATION
Downloaded from http://circ.ahajournals.org/ by guest on March 24, 2015
CORONARY ARTERIES IN TOF/Dabizzi et al.
FIGURE 8. Left selective coronary angiography in the
anteroposterior projection, showing a fistula between the
coronary and pulmonary arteries.
tum includes the two branches of the bundle of His
and the most distal portion of the bundle branches.
These structures are mostly supplied by septal
branches of the LAD and, to a lesser extent, by septal
branches of the PDA.
In patients with dominant left pattern, the LCA
supplies the whole interventricular septum. In the
presence of a subcristal interventricular septal defect
(as often occurs in TF), the septal branches arising
from the LAD can be displaced on the upper and
anterior margin of the defect, while those arising from
the PDA and coursing in a posteroanterior direction
can be displaced on the lower and posterior margin of
the defect.
FIGURE 9. A fistula between an anterior ventricular
branch, arising from a hypoplastic right coronary artery,
and the right atrium.
FIGURE 10. Left selective coronary angiography in the
anteroposterior projection, showing coronary-bronchial
artery anastomoses.
Considering this type of blood supply of the interventricular
septum, an injury to the arterial system
supplying the septum could probably explain some of
the left anterior hemiblocks that occur after total correction
of TF, without necessarily implying an injury
to the conduction pathways.
Finally, the incidence of the dominant left pattern
(32.8%) is higher than in normal subjects (10-12%).'8 19
FIGURE 1 1. Right selective coronary angiography in the
anteroposterior projection during the emptying phase, showing
anastomoses between the right coronary artery and the
bronchial arteries.
101
Downloaded from http://circ.ahajournals.org/ by guest on March 24, 2015
VOL 61, No 1, JANUARY 1980
FIGURE 12. Hypoplasia of the coronary tree. A) Left
selective coronary angiography. B) Right selective coronary
angiography.
Conclusion
We agree with other authors4' 5, 20 that preoperative
selective coronary angiography should be performed
in all patients with complex congenital heart diseases.
Reliable preoperative knowledge of a possible coronary
anomaly allows the surgeon to choose the most
appropriate operative technique. Aortography in two
projections can show the major variations of the coronary
arterial tree. The aortic root injection does not
ensure uniform contrast filling of the coronary
arteries, which is necessary for reliable interpretation,
but selective coronary angiography does. In doubtful
cases, when an expected coronary branch is not
visualized by selective coronary angiography, aortography
may be advisable. There were no complications
from selective coronary angiography during
our investigation. Selective coronary angiography,
moreover, requires injecting only small amounts of
contrast material.
References
1. Kirklin JW, Ellis FJ Jr, McGoon DC, Dushane JW, Swan
HJC: Surgical treatment for the tetralogy of Fallot by open intracardiae
repair. 3 Thorac Cardiovasc Surg 37: 33, 1959
2. Senning A: Surgical treatment of right ventricular outflow tract
stenosis combined with ventricular septal defect and right-left
shunt (Fallot's tetralogy). Acta Chir Scand 117: 73, 1959
3. Howe A, Rastelli GC, Ritter DG, Dushane JW: Management
of right ventricular outflow tract in severe tetralogy of Fallot. J
Thorac Cardiovasc Surg 60: 131, 1970
4. Berry BE, McGoon DC: Total correction for tetralogy of Fallot
with anomalous coronary artery. Surgery 74: 894, 1973
5. Meyer 3, Chiariello L, Hallman GL, Cooley DA: Coronary
artery anomalies in patients with tetralogy of Fallot. J Thorac
Cardiovasc Surg 69: 374, 1975
6. Fellows KE, Freed MD, Keane JF, Van Praagh R, Bernhard
WF, Castaneda AC: Resuilts of routine preoperative coronary
angiography in tetralogy of Fallot. Circulation 51: 561, 1975
7. Longenecker CG, Reemtsma K, Creech 0 Jr: Anomalous coronary
artery distribution associated with tetralogy of Fallot: a
hazard in open cardiac repair. J Thorac Cardiovasc Surg 42:
258, 1961
8. Meng L, Eckner DAO, Lev M: Coronary artery distribution in
tetralogy of Fallot. Arch Surg 90: 363, 1965
9. White RI, Frech RS, Castaneda A, Amplatz K: The nature and
significance of anomalous coronary arteries in tetralogy of
Fallot. Am J Roentgenol Rad Ther Nucl Med 114: 350, 1972
10. Friedman S, Ash R, Klein D, Johnson J: Anomalous single coronary
artery complicating ventriculotomy in a child with
cyanotic congenital heart disease. Am Heart J 59: 149, 1960
11. Ogden JA, Goodyer AV: Patterns of distribution of single coronary
artery. Yale J Biol Med 43: 11, 1970
12. Robicsck F, Sanger PW, Daugherty HK, Galucci V: Origin of
the anterior interventricular (descending) coronary artery and
vein from the left mammary vessels. A previously unknown
anomaly of the left coronary system. J Thorac Cardiovasc Surg
53: 602, 1967
13. Bjork L: Angiographic demonstration of extracardiac
anastomoses to coronary arteries. Radiology 87: 274, 1966
14. Gadboys HL, Slonim R, Litwak RS: The treacherous
anomalous coronary artery. Am J Cardiol 8: 854, 1961
15. Kirklin JW, Payne WS, Theye RA, Dushane JW: Factors
affecting survival after operation for tetralogy of Fallot. Ann
Surg 152: 485, 1960
16. Baltaxe HA, Amplatz K, Levine DC: Coronary Angiography.
Springfield, Illinois, Charles C Thomas, 1973, p 111
17. James TN: Blood supply of the human interventricular septum.
Circulation 17: 391, 1958
18. Helfant RH, Banka VS: A Clinical and Angiographic Approach
to Coronary Heart Disease. Philadelphia, FA Davis
Company, 1978, p 23
19. Gensini GG: Coronary Arteriography. Mt. Kisco, New York,
Futura Publishing Company Inc, 1975, p 168
20. Formanek G: Les arttres coronaires de l'enfant dans les cardiopathies
cong6nitales et les possibilit6s d'une arttriographie
coronaire stlective. Ann Radiol 12: 327, 1959