Journal of Cardiovascular Computed Tomography 9 (2015) 593e596

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Journal of Cardiovascular Computed Tomography

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Short communication

Pictorial review of coronary anomalies in Tetralogy of Fallot

Sangita Kapur a, *, Gunjan Aeron b, 1, Christopher N. Vojta a
a
Department of Radiology, University of Cincinnati, 234 Goodman St, Cincinnati, OH 45219, USA
b
Department of Radiology, VAMC Cincinnati, Cincinnati, OH 45220, USA

a r t i c l e i n f o a b s t r a c t

Article history: Tetralogy of Fallot (TOF) classically consists of four characteristic features-right ventricular outflow
Received 21 February 2014 obstruction, right ventricular hypertrophy, ventricular septal defect and an overriding aorta. In addition
Received in revised form there are multiple other associated cardiac anomalies, including coronary artery anomalies. In this re-
24 December 2014
view, the role of CT angiography and the spectrum of coronary anomalies will be discussed along with
Accepted 26 January 2015
Available online 6 August 2015
importance of such anomalies in the context of surgery.
© 2015 Society of Cardiovascular Computed Tomography. Published by Elsevier Inc. All rights reserved.
Keywords:
Tetralogy of Fallot
Coronary anomalies

1. Objective 3. Coronary arterial anomalies in TOF

To illustrate the coronary anatomy in Tetralogy of Fallot (TOF)
and the utility of coronary CT angiography (CCTA) in pre-operative
evaluation of such anomalies.
2. Introduction
3.1. Anomalies of orifice
High takeoff of a coronary artery may be present in TOF and
can be associated with an intramural course at its origin.4 This
may give rise to difficulties during aortic cross clamping at

Tetralogy of Fallot (TOF) is the most common cyanotic congen-

ital heart disease (3.5% of infants born with congenital heart dis-
ease) and consists of right ventricular outflow obstruction, right
ventricular hypertrophy, ventricular septal defect and an overriding
aorta.1,2 In addition, there may be other associated abnormalities,
often including coronary anomalies, which have a reported inci-
dence of 4e36%.3e7 The reason for the frequent association of
coronary anomalies with TOF is unknown. Since the coronaries
normally form by connection of coronary buds from the aorta with
an already established myocardial vascular network, it has been
suggested that the anterior position of the aorta may predispose to
anomalous coronary anatomy, but this is probably not a sufficient
explanation.8 In this pictorial review, typical coronary anomalies in
patients with TOF will be discussed along with their importance in
the context of corrective surgery.

* Corresponding author.
E-mail addresses: Sangita.kapur@uchealth.com (S. Kapur), Gunjan.aeron@va.gov
(G. Aeron), chrisvojta@gmail.com (C.N. Vojta).
1
Present address: Department of Radiology, VAMC Providence, RI 02908, USA. Fig. 1. Clockwise rotation of the aortic root in a patient with Tetralogy of Fallot (AoR).

http://dx.doi.org/10.1016/j.jcct.2015.01.018
1934-5925/© 2015 Society of Cardiovascular Computed Tomography. Published by Elsevier Inc. All rights reserved.
594 S. Kapur et al. / Journal of Cardiovascular Computed Tomography 9 (2015) 593e596

Fig. 2. 52 year old male, status post TOF repair. A. Anomalous origin of the LAD from the right coronary cusp (arrowhead). B. The anomalous LAD courses anterior to the RVOT
(arrows).

operation, as well as produce problems during selective arte-
riography.4 Intramural course and acute angulation at the
origin of a coronary artery have been implicated in sudden
death during exercise.4 Furthermore, there may be atresia of
the proximal aspects of coronary vessels in TOF patients.
3.2. Anomalous origin
An anomalous coronary origin has been reported with a fre-
quency of 8.2% in TOF.3 However, clockwise rotation of the aortic
root may create difficulties coronary ostia (Fig. 1). A left anterior

Fig. 3. 44 year old female with TOF and pulmonary conduit, now with atrial fibrillation. Cardiac CTA demonstrates interarterial course of the anomalous LAD. It arises from the right
coronary cusp and courses between the aorta and the pulmonary artery towards the anterior interventricular groove (arrows).
 S. Kapur et al. / Journal of Cardiovascular Computed Tomography 9 (2015) 593e596
Fig. 4. A 63 year old male with repaired TOF. Anomalous origin of the RCA (arrows)
from the left main that crosses anterior to the RVOT. There is extensive atherosclerotic
disease in the visualized coronary tree. Note bicuspid pulmonic valve (PV).
descending coronary artery arising from the right coronary
artery (RCA) has been found to be the most common anomaly
(4.2 to 6.4%)3,5,7,9 (Figs. 2, 3, 6). In angiography, this condition
may be confused with a large conus branch of the RCA, which
is also frequently present. The RCA may in turn arise from the
left cusp (Figs. 4, 7). There may be an accessory left anterior
descending artery (LAD) arising from the RCA (Fig. 5). A single
coronary artery has been reported as the second most frequent
anomaly (1.8 to 4.2%)3,5,7 (Fig. 6). Other less common anomalies
are as follows:
Left circumflex artery (LCX) arising from the RCA in 0.4 to 0.8% of
cases5,6 and RCA from the pulmonary trunk 0.9%.3
3.3. Anomalous course
The most clinically relevant course is that of an anomalous
coronary arising from the contralateral sinus of Valsalva and
coursing in front of the RVOT, alternatively behind the RVOT and in
front of the aorta. The frequency of an aberrant artery that crosses
the RVOT has been found to range from 6e14% (Figs. 2e4, 7)3e6 and
this is a condition which may cause considerable problems, espe-
cially if unrecognized, in corrective surgery.
Fig. 5. 44 year old female with TOF and a pulmonary conduit, now with atrial fibrillation. Duplicated LAD with a small native LAD (black arrow). Anomalous LAD (white arrow) in
the anterior inter-ventricular groove.
595
Fig. 6. Common origin of RCA, Conus artery & LAD from right sinus of Valsalva.
3.4. Anomalous termination
In 7e11% of the patients, a coronary artery-to-pulmonary artery
or right atrium fistula may be seen.5,6
3.5. Others
The incidence of left dominance (20- 33%) is greater in tetralogy of
Fallot3e5 and a prominent conus artery has been reported with a high
frequency of 19e92%.3e5 Anastomoses between coronary and bron-
chial arteries are present in 26%of individuals.5 Obviously, once TOF
patients are older, incidental coronary artery disease may be seen.
4. Significance of coronary anatomy in pre-operative
planning
Knowledge of aberrant coronary arterial supply is essential for
pre-operative planning.9 The incidence of coronary injury in the
presence of anomalous course is approximately 8%.10 The 2008
ACC/AHA recommendations state that coronary artery anatomy
596 S. Kapur et al. / Journal of Cardiovascular Computed Tomography 9 (2015) 593e596
Fig. 7. 62 year old male postoperative TOF. There is an anomalous origin of the RCA
from left cusp with a course anterior to RVOT. Also note atherosclerotic changes in the
coronary vasculature.
should be determined before any intervention on RV outflow tract.9
Consequently, the most relevant situation is that of an anomalous
coronary vessel crossing anterior to the right ventricular outflow
tract, usually the left coronary artery arising from the right. It is at
risk for injury during any repair involving the right outflow tract
including transannular repair of the infundibulum, as it may be
difficult to identify at surgery due to overlying myocardium,
epicardial fat or adhesions7,11,12 (Figs. 2B, 4A and 7). It may in fact, be
necessary to change the location of ventriculotomy and to avoid
RVOT conduit placement to prevent injury and compression of an
anomalous coronary.13e15
As is apparent from discussion above, lack of recognition of
coronary anomalies in TOF preoperatively can have disastrous
consequences.6 On the other hand, CT permits identification and
exact delineation of anomalous coronary arteries and operative
outcomes in patients with anomalous coronary arteries diag-
nosed prior to surgery are similar to those in other patients with
TOF.13,16
Conflict of interest
No conflicts of interest for any of the authors.
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