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Ann Thorac Surg 1996;62:1205-1207
© 1996 The Society of Thoracic Surgeons

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Case Report

Coronary Artery Aneurysm Associated With Adult Supravalvular Aortic Stenosis

Ahmet T. Yilmaz, MD, Mehmet Arslan, MD, Ertuörul Özal, MD, Hakan Bngöl, MD, Harun Tatar, MD, Ömer Yüksel Öztürk, MD

Department of Cardiovascular Surgery, Gülhane Military Medical Academy, Ankara, Turkey

Accepted for publication April 25, 1996.

	Abstract

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Two patients, aged 20 and 21 years, with supravalvular aortic stenosis and aneurysms of the coronary arteries are described. In supravalvular aortic stenosis, dilatation of the sinuses of Valsalva and multiple abnormalities of one or both coronary arteries are common. Aneurysm of coronary artery has not been well recognized as a lesion associated with supravalvular aortic stenosis. The operation in these patients was limited to relief of the supravalvular obstruction.

	Introduction

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I n congenital supravalvular aortic stenosis, coronary artery abnormality is one of the major associated cardiovascular lesions. The coronary arteries may be dilated and tortuous because the ostia are below the site of obstruction and exposed to elevated left ventricular pressure, or they may become narrowed by the overhanging, stenosing ring or even completely obstructed should the valve cusp become adherent to the aortic wall [1]. The majority of these anomalies are obstructing lesion of the coronary arteries, particularly of the left main coronary trunk. Aneurysm of the proximal coronary artery, although less frequent, has also been described [2]. This report concerns 2 adult patients with supravalvular aortic stenosis and aneurysm of coronary arteries. The role of coronary sinus cardioplegia in minimizing the risk of distal embolization from the coronary aneurysm during surgical repair was also emphasized.

	Case Reports

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Patient 1
A 21-year-old man with supravalvular aortic stenosis was seen at GATA Cardiology Department because of effort angina and dyspnea. Cardiac catheterization demonstrated discrete supravalvular aortic stenosis with left ventricular outflow tract gradient of 98 mm Hg. Left ventricular end-diastolic pressure was elevated to 35 mm Hg. No pulmonary artery stenosis was seen in selective pulmonary angiograms. Coronary angiography revealed a 1.5-cm aneurysm of the proximal left coronary and a 2-cm aneurysm of the proximal right coronary artery (Fig 1). Operation was performed using hypothermic cardiopulmonary bypass with retrograde cardioplegic hypothermic myocardial preservation. The repair was made by single sinus aortoplasty. No attempt was made for coronary artery aneurysms. Recovery was uneventful. The patient was placed on anticoagulant therapy (warfarin sodium), and continues to do well 3 years after operation.

View larger version (71K): [in this window] [in a new window]   Fig 1. . Aortography showed the pitting of the ascending aorta and aneurysms in both coronary arteries (A). Left and right coronary arteriograms revealed aneurysmal dilatation of the left and right coronary arteries with no dilatation of the distal coronary system (B, C).

View larger version (133K): [in this window] [in a new window]   Fig 2. . Aortography showed supravalvular aortic stenosis and aneurysms in proximal right and left coronary arteries.

	Comment

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Congenital aneurysms of the coronary arteries are unusual and should not be confused with aneurysm-like dilatation of a coronary artery secondary to the existence of coronary hypertension due to supravalvular aortic stenosis [4]. An isolated aneurysm of a single vessel with otherwise normal coronary arteries would suggest either a congenital aneurysm or an isolated inflammatory process. The observations show that supravalvular aortic stenosis in most, if not all, patients is part of a much more widespread and unrecognized abnormality of the cardiovascular system involving major conducting arteries and left ventricular myocardium [5].

The decision to undertake surgical repair (whether to bypass the coronary arteries and the ligate the aneurysms or to leave them alone) in the presence of significant coronary artery aneurysm depends to a large extend on the experience and the results obtained by the surgeon in the treatment of coronary artery aneurysm associated with supravalvular aortic stenosis. Because only a few cases have been reported, it is difficult to formulate a treatment for those cases. Further follow-up has to be done to evaluate what is the best treatment. Our patients had only aneurysmal dilatation of the proximal left and right coronary arteries. The left anterior descending, circumflex, and distal right coronary arteries were normal. The operation in these patients was limited to relief of supravalvular obstruction. The patients continue to be asymptomatic with warfarin alone as therapy, 1 and 3 years after operation. It is not clear whether isolated proximal dilatation of the both coronary arteries are related to supravalvular aortic stenosis or to another congenital aneurysm of the coronary artery associated with supravalvular aortic stenosis.

	Footnotes

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Address reprint requests to Dr Yilmaz, GATA Loj Numan Apt No. 5, 06018 Etlik, Ankara, Turkey.

	References

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1. Van Son JAM, Danielson GK, Puga FJ, et al. Supravalvular aortic stenosis. Long-term results of surgical treatment. J Thorac Cardiovasc Surg 1994;107:103–15.[Abstract/Free Full Text]
2. Gupta MP, Zoneraich S, Aintablain A, Mehta J. Congenital aneurysm of the left ventricle associated with supravalvular aortic stenosis and aneurysm of the left main coronary artery: case report and review of the literature. Angiology 1975;26:269–75.[Free Full Text]
3. Landes RG, Zavoral JH, Emery RW, Moller JH, Lindsay WG, Nicoloff DM. The surgical management of vascular abnormalities associated with supravalvular aortic stenosis. J Thorac Cardiovasc Surg 1978;75:80–6.[Medline]
4. Braunstein PW Jr, Sade RM, Crawford FA Jr, Oslizlok PC. Repair of supravalvar aortic stenosis: cardiovascular morphometric and hemodynamic results. Ann Thorac Surg 1990;50:700–7.[Abstract]
5. Payne RM, Johnson MC, Grant JW, Strauss AW. Toward a molecular understanding of congenital heart disease. Circulation 1995;91:494–504.[Abstract/Free Full Text]

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This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Harun Tatar Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Yilmaz, A. T. Articles by Öztürk, O. Y. Search for Related Content PubMed PubMed Citation Articles by Yilmaz, A. T. Articles by Öztürk, O. Y.