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ARYA Atheroscler. 2011 Winter; 6(4): 149–152.
PMCID: PMC3347829

Anomalous Origin of the Left Coronary Artery from the Right Sinus of Valsalva and Sever Mitral Stenosis

Abstract

Congenital coronary anomalies are presented in approximately1% of patient referred for cardiac catheterization. Among the congenital coronary anomalies, a separated anomalous origin of all the coronary arteries from the right sinus of valsalva is very uncommon. We report a rare occurance of simultaneous occurence of mitral stenosis with ectopic origin of left main stem coronary artery from right sinus of Valsalva.

Keywords: Anomalous Origin of Coronary Arteries, Mitral Stenosis.

Introduction

Among all the congenital coronary anomalies, anomalous origin of the left coronary artery from the right coronary sinus of valsalva is rare.1Congenital coronary anomalies are presented in approximately 1% of patient referred for cardiac cathetrization.2 Among the congenital coronary anomalies, a separated anomalous origin of all the coronary arteries from the right sinus of valsalva is very uncommon.3

We report a case of severe mitral stenosis with a rare anomaly of the left coronary artery.

Case report

A 41 year-old female patient with a history of rheumatic fever, since 30 years ago presented with chief complaint of dyspnea on exertion. Vital signs of the patient were normal. In cardiac examination a 3/6 diastolic rumbling murmurs followed by opening snap with presystolic accentuation and loud S1 was heard. ECG showed normal sinus rhythm with signs of left atrial enlargement.

In transthoracic echocardiography mitral valve showed severe stenosis (MVA=1cm2 by planimetery). Mitral valve score for PTMC was 6. Ejection fraction was normal and mild to moderate pulmonary hypertension was present (pulmonary artery pressure was about 45 mm Hg). There was no left atrial clot in transesophageal echocardiographic examination.

The patient was scheduled for precutaneous transluminal mitral commiserotomy (PTMC). At first, we tried to perform coronary angiography because her age was above 40, but we couldnot find the orifice of the left coronary artery with judkins left catheter No 4.

On right coronary injection, left main stem and right coronary artery were seen to arise separately from right coronary sinus and left coronary vessel crossed posterior to pulmonary artery(posterior course) (Figure 1,,22,,33).

Figure 1
Anomalous origin of left main stem from right sinus of Valsalva (LAO view)
Figure 2
Anomalous origin of left main stem from right sinus of Valsalva(Lateral view)
Figure 3
Anomalous origin of left main stem from right sinus of Valsalva (RAO view)

PTMC was done with 26-Innoue balloon and left atrial pressure decreased from 32 mm Hg to 20mm Hg. No significant gradient was present between left atrial and left ventricular end diastolic pressure (LVEDP) at the termination of the procedure. No mitral regurgitation was seen at the final ventriculography (Figure 4).

Figure 4
PTMC with Innoue No 26 balloon

Discussion

Congenital coronary anomalies are uncommon.4 Ectopic origin of a coronary artery from the conteralateral sinus of valsalva may have important clinical manifestation, most notably exertional sudden cardiac death particulary in youth.5 , 6 , 7

A single coronary artery arising from the right sinus of valsalva has four anatomical courses;4

1- Anterior course: main stem turns anterior in front of RVOT.

2- Interarterial course: main stem turn between great vessels,aorta and pulmonary artery.

3- Septal course, main stem had interamyocardial septal continuation.4-Posterior, main stem turn posteriorly behind the aorta.

The interarterial course has been known to have the worst prognosis with respect to symptoms and incidence of sudden cardiac death (SCD).8 Other types can also present with myocardial ischemia, heart failure or sudden cardiac death.

In the interarterial course, ischemia or SCD are assumed to be caused by vascular compression or kinking.9

Since the overall number of these patients is small, the treatment strategy varies and is not clearly defined.The possible treatment of choice to prevent SCD in particular interarterial course is CABG using the internal mammary artery as a conduit.10

In this case we report a rare occurance of simultaneous occurence of mitral stenosis with ectopic origin of left main stem coronary artery from right sinus of Valsalva.

Conflict of Interests

Authors have no conflict of interests.

References

1. Cho HO, Cho KH, Jeong YS, Ahn SG, Choi SJ, Yoo JY, et al. Anomalous Origin of the Left Coronary Artery from the Right Sinus of Valsalva, which Presented as Acute Myocardial Infarction. Korean Circulation Journal. 2006;36:817–9.
2. Kardos A, Babai L, Rudas L, Gaal T, Horvath T, Talosi L, et al. Epidemiology of congenital coronary artery anomalies: a coronary arteriography study on a central European population. Cathet Cardiovasc Diagn. 1997;42(3):270–5. [PubMed]
3. Yang DG, Cha KS, Kim BK, Lee SH, Park TH, Kim ET, et al. Anomalous Origin of the Left Main Coronary Artery from the Right Sinus of Valsalva-Combined Ostial Spasm and Separate Anterior Course of the Left Anterior Descending and Circumflex Coronary Arteries. Korean Circ Journal. 2000;30(9):1165–9.
4. El-Sherief A, Liberthson RR, Ahmed W, Rojas CA, Ghoshhajra BB, Abbara S. Left Main Coronary Artery Arising From the Right Sinus of Valsalva. MGH Division of Cardiology. 2010;23
5. Leberthson RR, Dinsmore RE, Bharati S, Rubenstein JJ, Caulfield J, Wheeler EO, et al. Aberrant coronary artery origin from the aorta. Diagnosis and clinical significance. Circulation. 1974;50(4):774–9. [PubMed]
6. Cheitlin MD, De Castro CM, McAllister HA. Sudden death as a complication of anomalous left coronary origin from the anterior sinus of Valsalva, A not-so-minor congenital anomaly. Circulation. 1974;50(4):780–7. [PubMed]
7. Liberthson R. Congenital anomalies of the coronary arteries. In: Gatzoulis MA, Webb GD, Daubeney PEF, editors. Diagnosis and Management of Adult Congenital Heart Disease. Philadelphia: Churchill Livingstone; 2003. pp. 425–31.
8. Taylor AJ, Byers JP, Cheitlin MD, Virmani R. Anomalous right or left coronary artery from the contralateral coronary sinus: "high-risk" abnormalities in the initial coronary artery course and heterogeneous clinical outcomes. Am Heart J. 1997;133(4):428–35. [PubMed]
9. Taylor AJ, Rogan KM, Virmani R. Sudden cardiac death associated with isolated congenital coronary artery anomalies. J Am Coll Cardiol. 1992;20(3):640–7. [PubMed]
10. Alibegovica J, Hendiria T, Didierb D, Camenzind E. Single coronary artery originating from the right sinus Valsalva, Kardiovaskul? re Medizin. 2006;9(5):198–200.

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