Asian Cardiovasc Thorac Ann 1998;6:138-140
© 1998 Asia Publishing EXchange Pte Ltd
Extracardiac Unruptured Sinus of Valsalva Aneurysm Presenting with Aortic Incompetence
Berent Discigil, MD,
Mehmet Boga, MD1,
Ugur Gürcün, MD1,
Cahide Soydas, MD2,
Münevver Yüksel, MD1
Department of Cardiovascular Surgery Adnan Menderes University Aydin, Turkey
1 Department of Cardiovascular Surgery
2 Department of Cardiology Ege University Medical Faculty  zmir, Turkey
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For reprint information contact: Berent Discigil, MD Department of Cardiovascular Surgery Adnan Menderes University Aydin, Turkey Tel: 90 256 212 1850 Fax: 90 256 212 0146
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ABSTRACT
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We report a case of extracardiac unruptured aneurysm of the noncoronary sinus of Valsalva presenting with massive aortic regurgitation and high fever. The preoperative evaluation, echocardiographic and cardiac catheterization findings, and surgical management of this rare condition are discussed.
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INTRODUCTION
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Congenital aneurysm of the sinus of Valsalva is an uncommon entity. Its diagnosis is rare in childhood because most cases are asymptomatic. This condition is usually diagnosed in adults incidentally or because of an aneurysm.1,2 Congenital aneurysms are usually located in one sinus of Valsalva, they are intracardiac and often rupture into adjacent cardiac chambers, mostly into a low pressure area.3 Unruptured sinus of Valsalva aneurysms are rarely extracardiac and usually develop in the left coronary sinus.4–6 Isolated extracardiac aneurysms of the noncoronary sinus of Valsalva are extremely rare.1,7 We report the case of an adolescent patient with an unruptured extracardiac noncoronary sinus of Valsalva aneurysm who presented with massive aortic regurgitation and high fever.
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CASE REPORT
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A previously healthy 15-year-old boy with a 2-month history of sudden onset high fever, dyspnea, palpitation, and progressive effort intolerance was treated for subacute bacterial endocarditis in another hospital. He was given high-dose antibiotic therapy although blood cultures were negative. When his temperature did not fall, he was referred to Ege University Hospital. On admission, the patient was orthopneic, his temperature was 38°C, his pulse regular and 110 beats per minute, and the blood pressure was 110/60 mm Hg in both arms. There were no petechiae, splinter hemorrhages, or organomegaly. On auscultation, a grade 4/6 holosystolic murmur was heard at the apex as well as a loud early diastolic decrescendo murmur on the left sternal border associated with a prominent thrill. Clinical findings did not suggest a connective issue disorder such as Marfan's or Ehlers-Danlos syndrome.
A teleroentgenogram showed left ventricular enlargement and the cardiothoracic ratio was 0.65. The electro-cardiogram showed normal sinus rhythm. Laboratory studies revealed a white blood cell count of 5600 per mm3, packed cell volume of 26%, platelet count of 201,000 per mm3, and an erythrocyte sedimentation rate of 110 mm•h–1. Blood urea nitrogen was 730 mg•L–1, plasma creatinine was 16.8 mg•L–1, and other blood chemistry and urine analysis were normal. M-mode and two-dimensional transthoracic echocardiography showed a dilated left ventricle with internal diameters in systole and diastole of 58 mm and 66 mm, respectively. There was global hypokinesia with an ejection fraction of 35%. The aortic valve was trileaflet without thickening or vegetation. Massive aortic regurgitation was seen by color-flow imaging and by continuous-wave Doppler in the parasternal long-axis view (Figure 1
). Two-dimensional echocardiography in the parasternal short-axis revealed a periaortic aneurysm (Figure 2). At cardiac catheterization the left ventricular end-diastolic pressure was found to be elevated. Left ventriculography demonstrated dilatation of the cavity, aortography showed grade 4+ aortic regurgitation and a lobulated aneurysm originating from noncoronary sinus of Valsalva (Figure 3).
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Figure 1. Color-flow imaging of the left ventricle in the parasternal long-axis view showing massive aortic regurgitation. AO = aorta, LA = left atrium, LV = left ventricle, RV = right ventricle.
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Figure 2. A periaortic aneurysm was visualized by transthoracic echocardiography in the parasternal short-axis view. AO = aorta, LA = left atrium, LAA = left atrial appendage, RA = right atrium, PA = pulmonary artery.
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Figure 3. Aortic root angiography demonstrating the aneurysmal pouch originating from the noncoronary sinus of Valsalva (A) in the left anterior oblique view, and (B) in the right anterior oblique view.
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The patient was given antimicrobial and antifungal therapy for 2 weeks following his admission. However, he had a persistent high fever rising especially in the afternoon. Frequent blood cultures during this period were all negative. His temperature gradually returned to normal in the 3rd week of his admission. He was given 2 units of whole blood. A few weeks before surgery, he suffered 2 attacks of ventricular tachycardia and pulmonary edema.
At operation, a median sternotomy was performed. On opening the pericardium, a normal aorta was seen anteriorly. However, posterior to the aortic root, there was a lobulated (two lobules) oval-shaped aneurysm, 4 x 6 cm in size, corresponding to the noncoronary sinus of Valsalva. The heart, especially the left ventricle, was noticeably enlarged. Cardiopulmonary bypass was instituted between the ascending aorta and both venae cavae. After aortotomy, the orifice of the aneurysm in a fish mouth shape was found at the noncoronary sinus of Valsalva. The aneurysmal pouch extended towards the retroaortic area and the noncoronary part of the aortic annulus was dilated along with the aneurysm. There was a tear along the annular site of noncoronary cusp of the aortic valve extending from one commissure to the other, detaching the cusp entirely from its annulus. Only a tiny amount of leaflet tissue was left at the annular site of the tear and the cusp was flail and attached to the annulus only at the commissures. The rest of the aortic valve appeared normal. There was no vegetation, degeneration, or thickening at the leaflets. Aortic wall reconstruction was carried out with pledgetted mattress sutures by stitching the dilated aortic noncoronary annulus to the normal part of the aorta excluding the aneurysmal pouch. The noncoronary cusp was reattached to the annulus with pledgetted sutures, however, because the coaptation of the aortic cusps was found to be insufficient, the aortic valve was replaced with a St. Jude (no. 21) prostheses (St. Jude Medical, Inc., St. Paul, MN, USA). The patient's postoperative course was uneventful and he was discharged from the hospital on the 10th postoperative day. After 20 months of follow-up, the patient is well and asymptomatic.
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DISCUSSION
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Aneurysms may originate in one or more of the sinuses of Valsalva of the aortic valve. Most aneurysms are single and usually involve the right coronary sinus.4,5 Because of the central position of the aorta, these aneurysms can rupture into any of the cardiac chambers, frequently into the right ventricle or right atrium.3 Not all aneurysms of the sinus of Valsalva rupture and they are generally asymptomatic.5 Extracardiac unruptured aneurysms of one sinus of Valsalva are extremely rare, especially in the noncoronary sinus.1 Congenital aneurysms of the noncoronary sinus usually originate from its anterior portion and project into the right atrium.3 Rare cases of extracardiac development of congenital aneurysms have been reported but these were always posterior, corresponding to the left coronary sinus. The case reported here is unusual in that an unruptured aneurysm originating from the noncoronary sinus of Valsalva developed extracardiac and posterior to the aortic root, forming a large lobulated aneurysm, which caused severe aortic incompetence because of detachment of the aortic valve from its annulus. The mechanism of this detachment was the tear at the noncoronary cusp, which was probably caused by the shear stress of the high-pressure turbulent flow in the huge aneurysm of the noncoronary sinus of Valsalva.
Aneurysms of the aortic sinuses of Valsalva may be congenital or acquired. The acquired type form in young age and may result from bacterial endocarditis.4,8 Since congenital aneurysms may also be complicated by endo-carditis, especially when associated with aortic incompetence or a ventricular septal defect, it may be difficult to distinguish between the two as in our case.8 The fact that our patient was an adolescent and presented with fever and aortic incompetence without any evidence of bacterial endocarditis is of interest. Unfortunately, no biopsy was taken from the aortic wall so it was not established whether there was a congenital loss of elastic fibers in the aortic wall. Although all attempts failed to isolate an organism in blood cultures, it is possible that the aneurysm might have been secondary to mycotic destruction of the aortic wall in the noncoronary sinus. Our case confirms the reported difficulties in the diagnosis and surgical correction of this rare entity.
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REFERENCES
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ABSTRACT
INTRODUCTION
