Asian Cardiovasc Thorac Ann 2004;12:75-77
© 2004 Asia Publishing EXchange Ltd
Sinus of Valsalva Aneurysm with Congenital Aortic Stenosis and Aortic Coarctation
Toshihiro Ohata, MD,
Yuji Miyamoto, MD,
Toshiki Takahashi, MD,
Hajime Ichikawa, MD,
Koji Kagisaki, MD,
Hikaru Matsuda, MD
Department of Surgery, Osaka University Graduate School of Medicine, Suita, Japan
For reprint information contact: Toshihiro Ohata, MD Tel: 81 729 650 731 Fax: 81 729 652 022 Email: TOHATA{at}aol.com Department of Cardiovascular Surgery, Kawachi General Hospital, 28 Yokomakura-Higashi, Higashi-Osaka 578-0955, Japan.
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ABSTRACT
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A 52-year-old female was diagnosed with the extremely rare combination of sinus of Valsalva aneurysm, congenital unicuspid aortic valve stenosis, and coarctation of the aorta. She was successfully treated in a two-stage operation. The first operation consisted of a bypass graft to correct the coarctation. The second, performed 31 days later, included aortic valve replacement, patch closure of the aneurysm, and translocation of the right coronary artery.
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INTRODUCTION
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A combination of aortic coarctation and congenital aortic valve stenosis is frequently reported.1 However, we did not find any published report of the association of sinus of Valsalva aneurysm (SVA) with this combination. We describe here a patient with all of these 3 lesions, who was successfully treated in a two-stage operation.
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CASE REPORT
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A 52-year-old woman was admitted to Osaka University Hospital complaining mainly of a sudden onset of dyspnea on exertion. Despite the presence of heart murmurs since childhood, she had had no medical history of note until a congenital disorder was discovered by chance when she was 45 years old. Her upper-limb blood pressure was 150/60 mmHg, while her lower-limb systolic pressure was 96 mmHg. A systolic murmur of Levine IV/VI was audible at the second intercostal space on the sternal border. No pretibial edema was observed, nor were there any remarkable laboratory findings except for a serum C-reactive protein level of 1.4 mg·dL-1. Chest radiography showed cardiac enlargement (cardiothoracic ratio, 64%) and dilation of the ascending aorta. Electrocardiography indicated sinus rhythm without any sign of hypertrophy, but a QS pattern was seen in lead III and an abnormal Q wave in lead aVF. Echocardiography revealed aortic stenosis with severe calcification, mild aortic regurgitation, and SVA. The pressure gradient across the aortic valve was estimated to be 70 mmHg, and a large aneurysmal cavity of 65 mm in diameter communicating to the aorta was located in front of the atrioventricular sulcus. Computed tomography and magnetic resonance imaging showed a large aneurysm, slow blood flow inside it without any thrombus, dilation of the ascending aorta, and coarctation in the elongated descending aorta. Finally, thallium cardiac scintigraphy showed inferior wall ischemia and redistribution. Cardiac catheterization (Figure 1
) revealed a normal left coronary artery, but the right coronary artery (RCA) was blocked from view by the hugely expanded sinus of Valsalva. Aortic regurgitation was mild, and the pressure gradient across the aortic coarctation was 50 mmHg. The cardiac index was 3.9 L·min-1·m-2.
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Figure 1. Aortograms showing (A) the unruptured extracardiac sinus of Valsalva aneurysm and (B) aortic coarctation. AN = aneurysm; AR = aortic regurgitation; CoA = coarctation of the aorta.
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As the pressure gradient was significantly high, it was decided to repair the aortic coarctation before correcting the aneurysm and the aortic stenosis in order to avoid ischemia of the lower extremities during cardiopulmonary bypass (CPB).
During the first operation through a left thoracotomy, it was found that the left subclavian artery was 18 mm in diameter and the aorta proximal to the ligamentum arteriosum had become elongated. Grafting between the distal aortic arch and the descending aorta was performed using an 18-mm Hemashield (Meadox Medicals, Oakland, NJ, USA) with end-to-side fastening on both sides. The pressure gradient between the ascending and descending aorta disappeared after the grafting. The postoperative course was uneventful.
During the second operation, performed 31 days after the first, a little effusion and slight inflammatory change inside the pericardial cavity were seen. The aneurysm was 85 x 60 mm in size, on which the RCA could be seen clearly. The ascending aorta showed poststenotic dilatation (38 mm in diameter). Under moderately hypothermic CPB and cold cardioplegic arrest, the aneurysm was incised vertically. The aortic valve was found to be unicuspid and severely calcified (Figure 2). The aneurysm had an orifice of 30 x 20 mm located on the anterior wall of the ascending aorta where the right coronary cusp is usually found, and the right coronary ostium had become included in the aneurysmal wall. The aortic valve was replaced with a 23-mm St. Jude Medical valve (St. Paul, MN, USA). The orifice of the aneurysm was then closed with a 36 x 18 mm Dacron patch reinforced with the patient’s pericardium. After shortening of the aneurysm, the right coronary ostium was translocated to the anterior wall of the ascending aorta (Figure 3). The postoperative course was uneventful.
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Figure 3. Schematic illustrations of the second operation, intra- and postoperatively, which consisted of aortic valve replacement, patch closure and shortening of the sinus of Valsalva aneurysm, and translocation of the right coronary artery.
#1 = aortic unicuspid valve;
#2 = ostium of right coronary artery;
#3 = orfice of the aneurysm of the sinus of Valsalva.
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Cardiac catheterization after the second operation showed no aneurysm of the sinus of Valsalva and good patency of the RCA (Figure 4). The bypass graft of the coarctation functioned well without any pressure gradient. The patient was discharged 30 days after the operation and has been followed up as an outpatient.
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Figure 4. Postoperative (A) aortogram and (B) right coronary angiogram, showing good patency of the bypass graft and the right coronary artery.
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DISCUSSION
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A combination of coarctation of the aorta and congenital aortic valve stenosis (coarctation complex) is common in congenital heart anomalies. The incidence of association of a bicuspid aortic valve with this complex was observed to be 27% to 40%.1 However, we could find no report of the association of a unicuspid aortic valve, which is rare even as an isolated anomaly, occurring at a rate of 0.9% to 4% among cases of congenital aortic stenosis.2 The combination of SVA and aortic coarctation is very rare too, reported at a rate of only 0.9%.3 The presence of SVA with congenital bicuspid aortic stenosis is even rarer, with only 2 cases having been described so far.4 Finally, we did not find any account of the association of SVA with unicuspid aortic stenosis and coarctation of the aorta.
Large unruptured SVAs have been found to cause coronary artery obstruction, right or left ventricular outflow tract obstruction, or atrioventricular node compression.5 In the present patient, preoperative cardiac scintigraphy detected ischemia resulting from the RCA being stretched and obstructed by the huge aneurysm. To relieve this ischemia and to remove the expanded aneurysmal wall completely, revascularization or reconstruction of the RCA was required. Of 29 reports of surgical correction for unruptured SVAs, 7 concerned cases with myocardial ischemia due to coronary artery obstruction, which was treated by coronary artery bypass grafting with a saphenous vein.6–8 Following the procedure of Okita and colleagues,8 we chose to translocate the right coronary ostium, which tends to provide better patency of the RCA than does saphenous vein grafting. With this method, the reduction of the SVA was easy and complete.
The aortic coarctation was corrected prior to the operation for the SVA and aortic stenosis in order to avoid hypoperfusion of the lower extremities during CPB or other complications associated with repair of the aortic valve and SVA. Aortic coarctation could now be treated by the less invasive percutaneous stent-graft implantation, which was not available at that time, followed by repair of the other 2 lesions in the same operation. Careful follow-up on an outpatient basis is recommended in case the resected SVA become enlarged again.
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REFERENCES
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ABSTRACT
INTRODUCTION
