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Anomalous Right Coronary Artery Arising from
Left Coronary Sinus in Two Brothers
Hung-Yu Chang and Wei-Hsian Yin
We report two brothers aged 61 and 69 respectively with abnormal right coronary artery (RCA) origin ating from the
left sinus of Valsalva. Both of them presented with exertional dyspnea and chest tightness in their seventh decade.
Another younger brother of theirs suffered from sudden cardiac death at the age of 60. Other symptomatic family
members were screened by multi-slice computed tomography (MSCT), but no coronary artery anomalies were
found. Considering different risk factors of these two brothers, one patient underwent coronary artery bypass
surgery, while another took medical therapy. Previously reported familial cases of anomalous coronary arteries are
very rare.
Key Words: Anomalous coronaries � Computed tomography � Familial clustering
INTRODUCTION
Coronary artery anomalies are reported in 1.3% of
patients undergoing coronary angiography,1 and may be
associated with sudden death, myocardial ischemia, ar-
rhythmia and syncope. Some anatomic presentations of
coronary anomalies are considered to be high-risk
group.2,3 However, many patients were asymptomatic
before their presentation of sudden cardiac death,4 indi-
cating that early detection of potential lethal cases is
difficult. Familial cases of anomalous coronary arteries
have been very rare. In this case report, we report two
brothers with abnormal right coronary artery (RCA)
origin from the left coronary cuspid with identical
courses of RCA, and we suggest clinicians pay atten-
tion to the possibility of familial clustering of coronary
anomalies.
CASE REPORT
A 61-year-old man (patient 1) presented to our insti-
tution because of exertional dyspnea and chest distress.
He had hypercholesterolemia and was a casual smoker.
He received treadmill exercise test, which revealed ST
segment depression of 1 mm at leads V4-V6 during the
stage III exercise. Exercise thallium-201 myocardial per-
fusion scan with dipyridamole also demonstrated re-
versible perfusion defect in the proximal inferior walls
of the left ventricle. Cardiac catheterization showed ab-
errant origin of the right coronary artery (RCA) from the
left coronary cuspid. There were also mild atherosc-
lerotic changes in the coronary arteries (Figure 1A).
Multi-slice computed tomography (MSCT) proved the
diagnosis of anomalous RCA arising from the left sinus
of Valsalva and taking an interarterial course between
the aorta and pulmonary artery (Figures 1B&C). The pa-
tient received anti-atherosclerotic medication and his
condition remained stable during 3 years of follow-up.
A 69-year-old brother (patient 2) of patient 1 was
admitted to our hospital 2 years after his brother’s coro-
nary examination. Another brother of the two had died
suddenly 3 months previous to this hospitalization at his
age of 60, but the diagnosis was uncertain. Patient 2 suf-
Acta Cardiol Sin 2011;27:124�7 124
Anomalous Coronary Artery in BrothersCase Report Acta Cardiol Sin 2011;27:124�7
Received: September 22, 2010 Accepted: December 30, 2010
Division of Cardiology, Cheng-Hsin General Hospital, Taipei,
Taiwan.
Address correspondence and reprint requests to: Dr. Wei-Hsian Yin,
Cheng Hsin General Hospital, No. 45, Cheng-Hsin Street, Pei-Tou,
Taipei 112, Taiwan. Tel: 886-2-2826-4400; Fax: 886-2-2826-1242;
E-mail: [email protected]
fered increasing shortness of breath and dyspnea on
exertion for months. His past medical history were sig-
nificant for hypertension and dyslipidemia. Treadmill
exercise test showed marked 2 mm ST segment depres-
sion at inferior leads and leads V4-V6 during the stage III
exercise and recovery phases. Exercise thallium-201
myocardial perfusion scan with dipyridamole demon-
strated significant myocardial ischemia involving the left
ventricular anteroseptal wall and apex. Cardiac cathe-
terization showed total occlusion at the proximal portion
of the left anterior descending artery (LAD) and tortuous
atherosclerotic left circumflex artery. The RCA arose
from the left coronary cuspid, and a narrow orifice was
suspected (Figure 1D). MSCT revealed abnormal orifice
of the RCA from the left coronary sinus, with inter-
arterial course and narrowed ostium (Figures 1E&F).
Coronary artery bypass surgery was performed and di-
rect compression of the RCA proximal portion by the
pulmonary artery was found during the operation. There-
fore, the LAD and RCA were revascularized. The patient
was in stable status during 6 months of follow-up. Other
symptomatic family members were screened by MSCT,
but no coronary artery anomalies were found.
DISCUSSION
Coronary artery anomalies were reported in 1.3% of
patients undergoing coronary angiography by Yamanaka.1
Important clinical problems of coronary anomaly are
sudden cardiac death, myocardial ischemia, arrhythmia
and syncope. Sudden death mostly happens before 35
125 Acta Cardiol Sin 2011;27:124�7
Anomalous Coronary Artery in Brothers
Figure 1. (A) Coronary angiography in left anterior oblique projection reveals aberrant origin of RCA (arrow) from left coronary cuspid in patient
1. (B) Reconstructed contrast-enhanced computed tomography shows anomalous RCA (arrow) arising from the left sinus of Valsalva and taking an
interarterial course between aorta and pulmonary artery in patient 1. (C) Reconstructed computed tomography of patient 1. Anomalous RCA is
marked by arrow. (D) Coronary angiography in left anterior oblique projection reveals aberrant origin of RCA (arrow) from left coronary cuspid in
patient 2. A narrow orifice of RCA was suspected. (E) Reconstructed contrast-enhanced computed tomography shows anomalous RCA (arrow)
arising by the side of left main coronary artery and taking an interarterial course in patient 2. Total occlusion of LAD is also noted (arrowhead). (F)
Reconstructed computed tomography of patient 2. Note the narrowed RCA ostium (arrow) and total occlusion of LAD (arrowhead). Ao, aorta; PA,
pulmonary artery; LMCA, left main coronary artery.
A B C
D E F
years of age. Increased risk of sudden cardiac death with
this anomaly has been associated with four risk factors:
interarterial course between the aorta and pulmonary ar-
tery; slit-like coronary orifice; acute angle of take-off of
the anomalous coronary artery from the aorta; and the
presence of aortic intramural coronary arteries.2 Symp-
toms premonitory to a fatal event, such as exertional
syncope, chest pain or palpitations, are common in pa-
tients at risk, so surgical correction is indicated in symp-
tomatic patients at any age. However, only about 20% of
fatal anomalous coronary artery cases had prodromic
symptoms. RCA arising from the left coronary sinus is
more common than left coronary artery (LCA) from the
right sinus. In Yamanaka’s report, RCA arising from the
left coronary cuspid was noted in 0.17% of coronary
angiographies, while LCA from the right coronary
cuspid was seen in 0.047%. Data from Garg reported
that 15 out of 4100 patients (0.37%) had abnormal ori-
fice of RCA from the left sinus of Valsalva, but only 1
patient (0.02%) had LCA from the right sinus.5 How-
ever, the clinical course of LCA from the right coronary
sinus is more malignant than that of RCA from left coro-
nary sinus. Basso reported 27 sudden deaths in young
athletes. Of these, 23 had LCA arising from the right
coronary sinus and only 4 had RCA from the left coro-
nary sinus.4 Thus, surgical repair is recommended in pa-
tients with anomalous LCA.6 Nevertheless, treatment in
patients with anomalous RCA is still controversial be-
cause most conditions of this disease are benign. MSCT
can be used to evaluate the orifice and the course of
anomalous coronary artery, which can guide the treat-
ment policy.
Familial clustering of anomalous coronary arteries
was only described in five previous reports.7-11 De-
vanagondi reported one 10-year-old boy with aberrant
origin of LCA from the right sinus of Valsalva present-
ing with sudden cardiac death, while his 8-year-old
brother, who had aberrant RCA from the left coronary si-
nus with interarterial course and slit-like orifice, was
asymptomatic. Un-roofing operation was performed for
this 8-year-old boy, with good short-term outcome.7
Laureti demonstrated two brothers with anomalous LCA
from the right coronary sinus. The junior was found to
have interarterial course and was treated by reimplan-
tation of LCA, while the senior with retroaortic course
was treated medically.8 Horan described a father with ab-
normal origin of RCA from the circumflex artery and his
daughter with abnormal LCA arising from the RCA.9 In
a study of combined coronary and perfusion cardiovas-
cular magnetic resonance imaging, Bunce demonstrated
two sisters having anomalous coronary arteries. One had
abnormal RCA arising from the left coronary cuspid, and
the other sister had single coronary artery arising from
the right sinus of Valsalva.10 Rowe reported anomalous
origin of the left circumflex artery in a father and his son
and daughter.11 In these previous reports, only one mor-
tality was noted.7 A brother of our index cases suffered
sudden cardiac death, but no autopsy was done, so the
definitive diagnosis was undetermined. Comparing to
other reports, our patients also had atherosclerotic coro-
nary disease in addition to coronary anomalies, making
the cause of clinical symptoms uncertain. Patient 2 had
several risk factors: severe atherosclerotic coronary ar-
tery disease, narrow RCA orifice, interarterial course of
RCA, and suspected family history of sudden death. Sur-
gical intervention thus is reasonable for him. The treat-
ment of patient 1 is relatively questionable. Since most
anomalous RCA with interarterial courses are benign,
medical treatment was decided.
According to our cases and other previous reports,
we believe that familial clustering of coronary anomalies
does exist. Recent study demonstrated that gene Tbx1
defect is associated with anomalous coronary artery in
mice, though definitive gene of humans is unknown yet.
In this case report, we suggest that if family members of
a patient with coronary anomalies present with exer-
tional syncope or chest pain, the possibility of familial
clustering of anomalous coronaries must still be kept in
mind. Coronary angiogram or MSCT can be arranged in
symptomatic family members with positive stress test.
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Anomalous Coronary Artery in Brothers