Rev Bras Cardiol Invasiva.

2017;25(1-4):12-15

Original Article

Right coronary artery anomaly originating in the contralateral sinus of Valsalva

interpreted by the angiographic rapid identification method
Fernando Roberto de Fazzio, Fernando Matheus, Carlos M. Campos, Adriano Ossuna Tamazato,
Gabriel Dodo Buchler, Santiago Raul Arrieta, José Mariani, Pedro A. Lemos*
Serviço de Hemodinâmica e Cardiologia Intervencionista, Instituto do Coração, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil.

ARTICLE INFO ABSTRACT

Article history: Background: This study aimed to determine the correspondence between the anterior dot sign at the
Received 4 May 2016 angiography and the malignant interarterial course, defined by the gold standard coronary angiography (angio-
Accepted 2 March 2017 CT), in individuals with right coronary artery originating in the contralateral sinus of Valsalva.
Methods: This was an observational, retrospective, and single-center study. All individuals who had both coronary
angio-CT and invasive coronary angiography (ICA), and a diagnosis of right coronary artery anomaly originating
in the contralateral sinus of Valsalva were screened. ICA images were retrieved and analyzed for angiographic
Keywords: findings, searching for the presence of the anterior dot sign, characteristic of the interarterial course.
Coronary vessel anomalies
Diagnostic imaging Results: Between January 2010 and April 2015, 1,410 patients who underwent angio-CT and ICA were identified.
Cardiovascular diagnostic techniques Of these, 13 patients (0.92%) had a diagnosis of right coronary anomaly originating in the contralateral sinus of
Valsalva. The mean age was 59.6 ± 18.3 years, and 61.5% were males. In all 13 cases, the right coronary artery
originating in the left coronary sinus showed an interarterial course (between the aorta and the pulmonary
trunk) at the angio-CT. In all cases with appropriate acquisition (9/13), the ICA showed the presence of the
anterior dot sign.
Conclusions: The presence of the anterior dot sign at the ICA confirmed the interarterial course of the right
coronary artery originating in the contralateral sinus of Valsalva.
© 2017 Sociedade Brasileira de Hemodinâmica e Cardiologia Intervencionista.
This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Anomalia da artéria coronária direita com origem no seio de Valsalva

contralateral interpretada pelo método de identificação rápida angiográfico

RESUMO

Palavras-chave: Introdução: O objetivo deste trabalho foi determinar a correspondência entre o sinal angiográfico dot anterior
Anomalias dos vasos coronários e o trajeto interarterial maligno, definido pelo padrão-ouro por meio de angiotomografia coronária (angio-TC),
Diagnóstico por imagem em indivíduos com coronária direita com origem no seio de Valsalva contralateral.
Técnicas de diagnóstico cardiovascular Métodos: Estudo observacional, retrospectivo, unicêntrico. Foram rastreados todos os indivíduos que possuíam
simultaneamente angio-TC de coronárias e angiografia coronária invasiva (CINE), e que tinham diagnóstico
de anomalia de coronária direita com origem no seio de Valsalva contralateral. As imagens de CINE foram
recuperadas e analisadas para avaliação dos achados angiográficos, buscando-se presença do sinal dot anterior,
característico do trajeto interaterial.
Resultados: Entre janeiro de 2010 e abril de 2015, foram identificados 1.410 pacientes que possuíam angio-TC e
CINE. Destes, 13 pacientes (0,92%) apresentaram o diagnóstico de anomalia de coronária direita com origem no
seio de Valsalva contralateral. A idade média foi de 59,6 ± 18,3 anos, e 61,5% eram do sexo masculino. Em todos
os 13 casos, a artéria coronária direita com origem no seio coronário esquerdo apresentava trajeto interarterial
(entre a aorta e o tronco pulmonar), pela angio-TC. Em todos os casos com aquisição apropriada (9/13), a CINE
apresentava o sinal do dot anterior.
Conclusões: A presença do sinal dot anterior à CINE confirmou o trajeto interarterial da artéria coronária direita
com origem no seio de Valsalva contralateral.
© 2017 Sociedade Brasileira de Hemodinâmica e Cardiologia Intervencionista.
Este é um artigo Open Access sob a licença de CC BY-NC-ND (http://creativecommons.org/licenses/by-nc-nd/4.0/).

doi: 10.31160/JOTCI2017;25(1-4)A0004
* Corresponding author: Av. Dr. Enéas Carvalho de Aguiar, 44, Cerqueira Cesar, CEP: 05403-000, São Paulo, SP, Brasil.
E-mail: pedro.lemos@incor.usp.br (P.A. Lemos).
Peer review under the responsibility of Sociedade Brasileira de Hemodinâmica e Cardiologia Intervencionista.

0104-1843/© 2017 Sociedade Brasileira de Hemodinâmica e Cardiologia Intervencionista. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
 F. Fazzio et al. / Rev Bras Cardiol Invasiva. 2017;25(1-4):12-15 13

Introduction

Coronary artery course anomalies occur in less than 1% of the

population.1 In most cases, the presence of anomalies does not cor-
respond to clinical manifestations or major prognostic implications.2
However, the subgroup of patients with anomalous coronary arte-
ries originating in the contralateral sinus of Valsalva and with an
interarterial course (coronary trajectory between the aorta and the
pulmonary trunk) appears to be more prone to arrhythmias, myocar-
dial infarction, syncope, and sudden cardiac death.3-5 In the United
States, this condition is the second leading cause of sudden cardiac
A B
death among young athletes6 and the leading cause among military
recruits.7 Although it is difficult to estimate its exact prevalence, pu-
blished data suggest that the incidence of this malignant anomaly
ranges from 0.1 to 1.07%. It is estimated that the right coronary ano-
maly originating in the contralateral sinus of Valsalva is 6 to 10-fold
more common than that of the left coronary artery,8,9 also being more
prevalent among the Japanese.2
In the past, angiographic methods were proposed to allow the
diagnosis of such origin and course anomalies. The so-called dot-
and-eye method assumed that contrast-enhanced radiographic im-
pressions in the right anterior oblique view (“dot” sign), when pre-
sent at the anterior or posterior border of the aorta, respectively C D
diagnosed the interarterial or retroaortic course of the coronary
vessel. Similarly, the eye-shaped image could determine the pre-pul- Figure 1. (A) Nonselective injection in the right coronary artery, in the right an-
terior oblique view, demonstrating the origin of the right coronary artery in the
monary and septal courses.10 contralateral coronary sinus and its anterior relation with the aorta (Ao). (B) Com-
However, after the advent of coronary angiography (angio-CT), puted angiotomography with reconstruction in the sagittal view, reproducing the
this method has become more prominent in this scenario, and it is anterior dot sign (arrow), defining the interarterial relation of the right coronary
artery between the Ao and the pulmonary artery. (C) Computed angiotomography
the exam of choice for the precise definition of the origin and course with 3-D reconstruction, demonstrating the origin of the right coronary artery in
of coronary anomalies.11,12 the left coronary sinus and interarterial course. (D) Ventriculography performed
Interestingly, there are no conclusive studies in the literature to in the right anterior oblique view showing the anterior dot image in relation to the
Ao (arrow). RCA: right coronary artery; PT: pulmonary trunk; LAD: left anterior
date that validate the traditional angiographic dot-eye method ver- descending artery.
sus the current gold standard, coronary angio-CT. Such validation is
clinically relevant, since in a significant number of cases the diag­
nosis of coronary anomaly occurs incidentally during cardiac cathe-
terization (invasive coronary angiography - ICA),2 without previous
diagnosis or suspicion. The main objective of this study was to de­ findings. Particularly, as previously described by Serota et al.,10 angio-
termine the correspondence between the anterior dot angiographic graphic projections in the right anterior oblique view were carefully
pattern and the interarterial course defined by coronary angiography analyzed to assess the presence of the anterior dot sign, characteris-
in individuals with right coronary artery originating in the contrala- tic of the interarterial course (Figure 1).
teral sinus of Valsalva.

Methods
This was an observational, retrospective, single-center study car-
ried out in a high-complexity, tertiary Cardiology service. The research
was based on database analysis and chart review.
Study population
The database of the Cardiology Service of Instituto do Coração of
the Hospital das Clínicas of Faculdade de Medicina of Universidade
de São Paulo (USP) was searched with the assistance of the Medi-
cal and Hospital Information Unit, and all individuals who simulta­
neously had angio-CT and ICA with a diagnosis of anomalous right
coronary artery originating in the contralateral sinus of Valsalva at
the angio-CT were selected.
Data collection and image analysis
All reports and original images of angio-CT were retrieved and re-
viewed to confirm the anomalous coronary artery origin and cour­se,
and new reconstructions were performed when necessary. All ICA
images were retrieved and analyzed to evaluate the angiographic
Results
Between January 2010 and April 2015, 1,410 patients who under­
went to angio-CT and ICA were identified. The individual evaluation
of each of the charts, through the electronic medical record system,
resulted in the selection of 13 patients (0.92%) with a diagnosis of
anomalous right coronary artery originating in the contralateral sinus
of Valsalva.
Of the 13 patients that comprised the present study population,
8 (61.5%) were males and 12 (92.3%) were Caucasian. The mean age
was 59.6 ± 18.3 years, ranging from 27.9 years to 95.1 years.
In all 13 cases, the right coronary artery originating in the left
coronary sinus presented an interarterial course (between the aorta
and the pulmonary trunk) at the angio-CT. Of these, four cases did
not have ICA with ventriculography or aortography in the right ante-
rior oblique view. In the remaining nine cases, in which such projec-
tions were systematically performed, the anterior dot was present.
Of the total number of patients, six (46.2%) remained in clinical
treatment, two cases (15.4%) underwent percutaneous coronary in-
tervention with stent implantation, one case (7.7%) was treated surgi-
cally with reimplantation of the right coronary artery, and four cases
(30.8%) were submitted to surgical revascularization with grafting
to the right coronary artery (Figure 2).
14 F. Fazzio et al. / Rev Bras Cardiol Invasiva. 2017;25(1-4):12-15
Figure 2. Angiotomography showing a patent saphenous vein graft (SVG) in anasto-
mosis with the right coronary artery (RCA) originating in the left sinus of Valsalva.
LMCA: left main coronary artery.
Discussion
Over two decades ago, Serota et al.10 described the dot-and-eye
method for the angiographic diagnosis of coronary anomalies, in
which the anterior dot represented the impression caused by the an-
gulated left sinus outflow of the right coronary artery, with a cour­se
between the aorta and the pulmonary artery. The main finding of the
present study is the validation that the presence of the anterior dot
sign in the coronary angiography confirms the interarterial course
of the right coronary artery originating in the left sinus of Valsalva.
With the implementation and increasing availability of 64-channel
computed tomography, coupled with ECG-synchronized protocols
and new algorithms for interactive image reconstruction, the method
became the gold standard for the assessment of coronary anomalies,
whose main advantages are the fact that it is not an invasive exami-
nation, having quick image acquisition, greater temporal and spatial
resolution, and post-procedural 3-D reconstruction. Its disadvanta-
ges, such as the higher dose of radiation and contrast volume, have
also been overcome with the development of the aforementioned
techniques.13,14
In previous studies, coronary anomalies were classified as benign
(80.6% in an analyzed series) or potentially serious anomalies (19.4%),
including: (1) ectopic origin from the pulmonary artery, (2) ectopic
origin from the contralateral sinus of Valsalva, (3) single coronary ar-
tery, and (4) large coronary fistula.15 In the present study, the study
population was restricted to individuals with right coronary artery
originating in the left sinus, because this anomaly is frequently asso-
ciated with an interarterial course,15,16 which consequently in­creases
its malignancy potential – an underestimated finding in daily clinical
practice.
The present results demonstrate that this anomaly is unusual, in
line with the prevalence of 0.019 to 0.92% identified in previous stu-
dies,1,2 which, in turn, emphasizes the need to maintain a high degree
of clinical suspicion for the correct angiographic detection of its
morphological details. It is noteworthy that, in most of the present
cases, angio-CT was performed after ICA, reflecting the lack of confi-
dence in conventional angiography, reinforcing the need for diagnos-
tic validation of angiographic criteria.
In theory, the coronary vessel originating in the contralateral si-
nus can have four distinct courses – posterior (retroaortic), septal
(subpulmonary), anterior (pre-pulmonary), and interarterial – until
it reaches and irrigates the territory for which it is responsible. In
fact, the terminology “origin” might have been used in an inappro-
priate way, since more recent studies on coronary embryology report
the ingrowth theory, in which the coronary artery would develop
from a tangle of epicardial vessels, and its proximal portion would
fuse in the peritruncal ring.17 However, as observed in the present
study, most individuals with right coronary artery originating in
the left sinus have an interatrial course.15,16 The main high-risk cha­
racteristics of this type of anomalous coronary course are lateral
luminal compression of the intramural portion of the anomalous
vessel, coronary compression between the aorta and the pulmonary
artery, slitlike orif ice, and origin with acute exit angle.12 Further-
more, these arteries have an intramural course (defined histologi-
cally by the coronary artery sharing the same media with the aorta,
without adventitia interposition).18,19 Although established, the ex-
planation of a scissors-like mechanism, created by the close proxi-
mity of the aorta and pulmonary artery, especially during exertion,
is still controversial.20
Several risk stratifications have been suggested, including non­
invasive assessment using provocative stress tests (myocar­ dial
scin­tigraphy, echocardiography),21 analysis of angiotomographic ana­
tomic criteria,22 or even through measurements obtained by the
in­travascular ultrasound (IVUS) and fractional flow reserve (FFR)
values associated with dobutamine infusion;23 however, all methods
present limitations.
Although the surgical treatment for the correction of coronary
artery anomalies with an interarterial course is almost a consensus
among specialists,21,24 even without specific guidelines to guide such
management, the best way of managing cases of malignant corona­
ry anomalies is not well defined, especially in asymptomatic indi­
vi­duals. Among the several surgical techniques the unroofing pro-
cedure can be highlighted representing the strategy of choice when
there is an intramural component of the initial course; reimplan­
tation; coronary artery bypass grafting surgery using venous or ar-
terial grafts; pulmonary artery translocation; and the creation of a
new ostium.15,18,25
Conservative treatment is recommended by some authors26 in si-
tuations of negative results in provocative tests after drug treatment
implementation (commonly with beta-blockers). More recently, per-
cutaneous coronary intervention has been presented as an alterna-
tive technique to the limitations and risks of the surgical procedure,
with good results described in the short-term follow-up.25
The use of drug-eluting stents and a careful selection of the guide-
catheter, together with other devices that provide greater therapeu-
tic support, and a IVUS-guided procedure, can contribute to maintain
long-term success.27,28
Study limitations
This was an observational, pioneering study analyzing a series of
cases with a rare coronary anomaly using two methods of cardiovas-
cular imaging. However, its retrospective nature, based on database
information and patient files, as well as the small number of patients,
does not allow drawing definitive and comprehensive conclusions on
the subject, and further studies with a larger number of individuals
are required.
Conclusions
The presence of the anterior dot sign on the coronary angiogra-
phy confirmed the interarterial course of right coronary arteries
originating in the contralateral sinus of Valsalva. This angiographic
sign can be a valuable tool for the rapid identification of this cour­se,
considered to be high risk and often underestimated in daily clini-
cal practice.
 F. Fazzio et al. / Rev Bras Cardiol Invasiva. 2017;25(1-4):12-15
Sources of funding
None to be declared.
Conflicts of interest
The authors declare no conflicts of interest.
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