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: yin.wh@msa.hinet.net

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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