Ann Thorac Surg 1998;65:1458-1459
© 1998 The Society of Thoracic Surgeons
Case Reports
Unruptured Aneurysm of the Sinus of Valsalva Into the Pulmonary Artery
Shoichiro Shiraishi, MDa,
Shoji Watarida, MDa,
Kazuhiko Katsuyama, MDa,
Yasuhiko Nakajima, MDa,
Masato Imura, MDa,
Takao Nishi, MDa,
Atsumi Mori, MDa
a Department of Surgery, Shiga University of Medical Science, Shiga, Japan
Accepted for publication December 8, 1997.
Address reprint requests to Dr Shiraishi, Second Department of Surgery, Shiga University of Medical Science, Seta-Tsukinowa, Ohtsu, Shiga 520-21, Japan
e-mail: (shiro{at}belle.shiga-med.ac.jp)
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Abstract
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Congenital aneurysms of the sinus of Valsalva are rare lesions. Because the aortic root is central, the aneurysm can rupture into any cardiac chamber, and virtually all combinations of sinus and chamber fistulas have been described. Rupture into the pulmonary artery, however, is very rare. We encountered a 14-year-old boy with conal ventricular septal defect and right coronary cusp prolapse with an unruptured aneurysm of the sinus of Valsalva into the pulmonary artery.
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Introduction
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Congenital aneurysms of the sinus of Valsalva are rare lesions characterized morphologically by thin-walled, tubular sacs resembling a windsock. These aneurysms are caused by a deficiency of the aortic media and follow predictable intracardiac courses. When they rupture into an adjacent, low-pressure chamber, the results include aortocardiac fistulas, a large shunt, and congestive heart failure. However, rupture into the pulmonary artery is very rare.
This patient was born by normal delivery. A heart murmur was detected at the age of 1 month. He was diagnosed with conal ventricular septal defect and was followed up by another hospital. When the patient was 14 years of age, echocardiography revealed a conal ventricular septal defect and prolapse of the right coronary cusp with mild aortic regurgitation. Left ventriculography and aortography revealed a conal ventricular septal defect and prolapse of the right aortic sinus with trivial aortic regurgitation (Fig 1).

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Fig 1. Aortogram showing a prolapse of the right sinus of Valsalva (asterisk) and mild regurgitation of the aortic valve.
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The patient underwent an elective operation. Median sternotomy was performed, and then the patient was cannulated with an ascending aorta and biatrial cannulas, and cardiopulmonary bypass was started. Moderate hypothermia was induced and the ascending aorta was cross-clamped. After cardioplegic arrest, a longitudinal arteriotomy was made in the pulmonary artery. A vein retractor was placed across the pulmonary valve to expose the conal ventricular septal defect (about 10 mm in diameter). We closed the ventricular septal defect with a Dacron patch. When the aortic cross-clamp was released, we found a defect in the posterior wall of the main pulmonary artery. The defect measured about 15 mm in diameter and was located just above the right aortic sinus of Valsalva (Fig 2). The defect of the pulmonary artery was enveloped by the right aortic sinus. We closed the defect with a prosthetic patch. Then the pulmonary artery was closed. The patient was easily weaned from cardiopulmonary bypass. The postoperative course was good. On postoperative echocardiograms, neither residual shunt nor progression of regurgitation was detected.

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Fig 2. Operative photograph showing the right sinus of Valsalva (V) through the partial defect of the pulmonary artery. (P = pulmonary valve; VSD = ventricular septal defect.)
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Comment
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Congenital aneurysms of the sinus of Valsalva are rare lesions, with an incidence of 0.1% [1]. However, such aneurysms are five times more common in Asia, based on a comparison of current surgical series [2]. About 75% of the patients are male [1, 3]. Two thirds of the aneurysms are located in the right aortic sinus, one quarter in the noncoronary sinus, and the rest in the left aortic sinus [1, 3]. The aneurysms may be isolated, or in 30% to 50% of cases may be associated with a ventricular septal defect [1, 3], especially defects of the outlet septum [1], as in our patient. The proportion of patients with a ventricular septal defect is higher when the aneurysm arises from the right sinus [1]. With an associated ventricular septal defect, particularly if it is subpulmonary, there is often prolapse of the aortic valve cusp and aortic incompetence, so that this combination overlaps the association of conal ventricular septal defect with aortic incompetence without an aneurysm of the sinus of Valsalva.
The aneurysms follow predictable intracardiac courses. When they rupture into an adjacent, low-pressure chamber, the result is an aortocardiac fistula, a large shunt, and congestive heart failure. Because the aortic root is central, aneurysms can rupture into any cardiac chamber, and virtually all combinations of sinus and chamber fistulas have been described. Rupture most often involves right sinus aneurysm into the right ventricle, particularly if there is an outlet ventricular septal defect [1]. Rupture into the pericardium or pulmonary artery is very rare. The incidence of rupture into the pulmonary artery is less than 1% [1], and there is only 1 case report [4]. It is conceivable that our case involved an unruptured aneurysm of the sinus of Valsalva that would have ruptured into the pulmonary artery if it had progressed.
The basic lesion appeared to involve a thinning of the wall of the aortic sinus just above the annulus [5]. Pathologically, this was caused by the total absence of normal elastic fibers in the media [1]. The defect in the pulmonary artery in our patient was associated with the origins of the conal septum, right ventricular outflow, and great arteries.
We surgically closed the defect in the pulmonary artery with a prosthetic patch. Because of the risk of deformity in the sinus of Valsalva and progression of aortic insufficiency, the patient must be kept under close long-term observation.
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References
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- Kirklin J.W., Barratt-Boyes B.E. Congenital aneurysm of the sinus of Valsalva. In: Kirklin J.W., Barratt-Boyes B.E., eds. Cardiac surgery, 2nd ed. New York: Churchill Livingstone, 1993:825-839.
- Chu S.H., Hung C.R., How S.S., et al. Ruptured aneurysms of the sinus of Valsalva in Oriental patients. J Thorac Cardiovasc Surg 1990;99:288-298.[Abstract]
- Nowicki E.R., Aberdeen E., Friedman S., et al. Congenital left aortic sinusleft ventricle fistula and review of aortocardiac fistulas. Ann Thorac Surg 1977;23:378-388.[Abstract]
- Heilman KJd, Groves B.M., Campbell D., et al. Rupture of left sinus of Valsalva aneurysm into the pulmonary artery. J Am Coll Cardiol 1985;5:1005-1007.[Abstract]
- Roberts W.C., Honing H.S. The spectrum of cardiovascular disease in the Marfan syndrome: a clinico-morphologic study of 18 necropsy patients and comparison to 151 previously reported necropsy patients. Am Heart J 1982;104:115-135.[Medline]
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