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lytes were normal. The chest X-Ray alsowas normal. ECG showed sinus rhythm with a normal axis and incomplete right bundle branch block. Holter ECG.

CASE REPORT

Imaging of a non-coronary sinus of Valsalva

aneurysm with transthoracic echocardiography and multislice computed tomography H. Al Hashimi, K Nieman, F.J. ten Cate, D.V. Drunen, M.L. Simoons

A 46-year-old female with a giant aneurysm ofthe non-coronary sinus of Valsalva, associated with moderate aortic valve regurgitation, is described. The aneurysm was detected by echocardiography in a patient who was complaining of paroxysmal palpitations. The patient was accepted for aneurysmectomy. In this case report the possible aetiologies and clinical aspects ofthe aneurysm are reviewed. Treatment and the role of multi-sliced computed tomography (MSCT) in heart disease are discussed. (Neth HeartJ2002;10:203-6.) Key words: sinus of Valsalva aneurysm, echocardiography and MSCT

ongenital aneurysm ofthe aortic sinus ofValsalva

Wis an uncommon anomaly that occurs three times more often in males than in females.' The malformation consists of a separation or lack of fusion between the media of the aorta and annulus fibrosis ofthe aortic valve. This deficiency in the aortic media seems to be congenital. Detection of this disorder in infants and children is, however, rare because the resulting progressive aneurysmal dilatation of the weakened area may not be recognised until the 3rd or 4th decade of life. This anomaly usually presents with a complication, such as rupture into a cardiac chamber.' We describe a case in which a non-specific symptom led to the detection of sinus of Valsalva aneurysm.

H. Al Hashimi. K. Nieman. F.J. ten Cate. D.V. Drunen. M.L Simoons.

University Hospital Rotterdam, Dr. Molewaterplein 40, 3015 GD Rotterdam. Address for correspondence: H. Al Hashimi. E-mail: [email protected]

Netherlands Heart Journal, Volume 10, Number 4, April 2002

Case report History A 46-year-old female was referred by her general practitioner (GP) to the outpatient department ofthe Thorax Centre because of paroxysmal palpitations. There were no relieving or exacerbating factors and no other associated symptoms. There was no relevant past medical history or significant family history. She was a non-smoker and a social drinker. She was prescribed sotalol 2 x 80 mg for her palpitations.

Examination There were no peripheral stigmata indicative of connective tissue disease (such as Marfan's syndrome) or other chronic diseases. Her blood pressure was 140/80 mmHg in both arms, pulse rate 65 bpm, respiratory rate 20 per minute. Jugular venous pressure was not raised. The apex beat was normal in character, at the 5th intercostal space midclavicular line. The heart sounds were normal. An early blowing diastolic murmur, grade II, was detected at the left sternal border. The rest of the physical examination including chest, abdomen and the central nervous system were unremarkable.

Investigations Full blood count, liver function tests, urea and electrolytes were normal. The chest X-Ray also was normal. ECG showed sinus rhythm with a normal axis and incomplete right bundle branch block. Holter ECG revealed sinus rhythm throughout the 24 hours, with sporadic premature atrial complexes. On echocardiography, a large aneurysm was observed, arising from the non-coronary sinus ofValsalva and compressing the left atrium (figure 1). Moderate aortic valve regurgitation (grade II) was present (figure 2). The rest ofthe echocardiographic study was normal including left ventricular function and the aorta. Multislice computed tomography (MSCT) confirmed the presence of an aneurysm, originating from the noncoronary sinus of Valsalva and compressing the left atrium. The aorta appeared normal (figure 3).

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Imagng of a nonrcoonary sinus of Valsalva aneurysm with transthoracic echocardiography and multslice computed tomography

Figure 1. Echocardiograpby de ntrating the non-coronary sinus of Vaalwva aneurwm (NCGA) compessing the left atrium (LA). Ain the diastolic phas; B: in te systolc phas and c: sbowing xeflow into the aneurysm (F).

Coronary angiography revealed a normal origin and distribution of the coronary arteries. No significant stenoses were seen. The aortagram showed the sinus of Valsalva aneurysm, which was about 7 cm in diameter (figure 4). Discu5sion The sinuses of Valsalva are pouch-like dilatations behind each ofthe three aortic valve cusps (left, right and non-coronary cusp). The left and right sinuses give rise to the left and right coronary artery sinuses of Valsalva.2 They are confined proximally by the bases of the semilunar valve leaflets and distally by the

sinotubular ridge.3 They accommodate the open leaflets providing space behind them and, as such, preventing occlusion of the coronary artery orifices. They allow formation of eddy currents within them which prevents the leaflets striking the aortic wall and promotes valve closure.4 Sinus ofValsalva aneurysm is an uncommon defect. It is a saccular pathological dilatation ofthe aortic sinus outlined by a thin wall, caused by a lack of elastic or muscular tissue in the aortic root. This leads to separation of the fibrous annulus of the aortic valve from the media of the aortic root. Sinus of Valsalva aneurysms are frequently found in the right sinus of Vaisalva, but are less frequent in the non-coronary sinus (about 5-15%). The left aortic sinus is rarely involved.' Sinus of Valsalva aneurysms are often associated with other anomalies such as a bicuspid aortic valve (10%), ventricular septal defects (30-60% of cases) and coartation ofthe aorta." 56 These aneurysms can be solitary or multiple, involving multiple sinuses of Valsalva (10%), the ascending aorta and sometimes an extensive portion of the aortic root.6 Aneurysms ofthe sinus ofValsalva are asymtomatic and usually undetected until they rupture; with echcardiography they are now being diagnosed with increasing frequency. Occasionally, an unruptured aneurysm can cause serious complications such as: (a) infection; (b) conduction abnormalities due to the proximity ofthe right coronary cusp to the common bundle of His and proximal portions of the right bundle and left anterior fascicle, which may lead to complete heart block; (c) right ventricular outflow tract obstruction; (d) extension of the inter-ventricular septum leading to malignant arrhythmias; (e) intramural thrombus formation and rarely (f) myocardial ischaemia may be caused by coronary artery compression. Acute large rupture, which often occurs in men aged between 30-40 year, can have significant and serious consequences. It often involves rupture into 7

f

Figure 2. Echocardiography showing the associated aortic incompetence (AOI), grade two.

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Nethelands Heart Journal, Volume 10, Number 4, April 2002

Imaging of a non-coronary sinus of Valsalva aneurysm with transthoracic echocardiography and multi-slice computed tomography

Figure 3 (A, B, C). MSCT confirming the presence of an aneurysmatic mass, ortiinatingfrom the non-coronary sinus of Valsalva and compressing the eft atrium.

the right heart cavities, creating continuous arteriovenous shunting and volume loading of both right and left heart chambers. This in turn results in gradual or more commonly acute heart failure and even pulmonary oedema culminating in death after a short period of time. On the other hand, a small perforation may initially go unnoticed. Gradual progression will

Figure 4. Aortagram showing the non-coronary sinus of Valsalva aneurysm with a normal aorta.

Netherlands Heart journal, Volume 10, Number 4, April 2002

permit a better haemodynamic adjustment. Rupture is characterised by the sudden appearance of a continuous murmur in an otherwise healthy individual. Finally, aortic root pathology is more common than primary aortic valve disease and accounts for more than halfof all patients undergoing valve replacement. In these cases, aortic insufficiency is most likely the result of progressive dilatation of the aortic sinuses and distortion of the annulus.4 A sinus of Valsalva aneurysm is an uncommon cardiac anomaly, which requires appropriate diagnosis either by echocardiography, magnetic resonance imaging (MRI), MSCT, or contrast angiography. The development of a new generation of MSCT scanners with retrospective ECG-gated reconstruction algorithms has created a range ofopportunities in the field of non-invasive CT-based angiography. MSCT offers high-contrast ECG synchronised images of the heart and great vessels. The high-resolution 3D datasets consisting of near isotropic voxels are ideal for volume rendered post-processing. In this particular case, a non-triggered scan protocol with thicker slices (4 x 2.5 mm) would probably have been sufficient to diagnose the abnormality. Nevertheless, this optimnised protocol offers detailed visualisation of the coronary sinus and coronary arteries without motion artefacts related to cardiac contraction.

Management Symptomatic or complicated aneurysms can be treated surgically with excellent results. The aim of surgery is to close and amputate the aneurysmal sac; then the aortic wall is reunited with the heart, either by direct suture or with a prosthesis. If the aortic valve is involved and the coronary ostia originate in the aneurysm, then aortic valve and the aortic root replacement may be necessary.5 The pre-operative medical management involves relieving cardiac failure and treating coexistent arrhythmias or endocarditis, if present. Management ofasymptomatic and uncomplicated sinus ofValsalva aneurysms is controversial. There are 205

Imaging of a non-coronary sinus of Valsalva aneurysm with transthoracic echocardiography and multi-slice computed tomography

two schools of thought about managing these patients involving a conservative and an invasive approach. The conservative group advocates regular follow-up ofthese patients and surgery is seen as a last resort. The other group is for an invasive approach, postulating that surgical treatment is necessary to prevent the development of potentially fatal complications.8-'0 Up until now, there is no set approach to treating asymtomatic and uncomplicated sinus of Valsalva aneurysms. Furthermore, there are no follow-up studies of these patients that could lead to a standard treatment for these anomalies. * Abbreviations: LAA=left atrial appendage. PA=pulmonary artery. LCA=left coronary artery. LCC=left coronary sinus of Valsalva. RCC=right coronary sinus of Valsalva. RCA=right coronary artery. NCCA=non-coronary sinus ofValsalva aneurysm. AO=aorta. LA=left atrium. LV=left ventricle.

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References Friedman WF. Aortic sinus aneurysm and fistula. In: Braunwald E, ed. Heart disease. A text book of cardiovascular medicine. Philadelphia: WB Saunders & Co, 1997;910-1. 2 Wayne Alexander R, et al. Anatomy ofthe heart. The heart. Chapter 2;28-31. 3 Sutton JP, et al. The forgotten inter-leaflet triangles: a review of the surgical anatomy of the aortic valve. Ann Thorac Surg 1995;59:419-27. 4 Spitaels SEC. Congenital aortic sinus ofValsalva aneurysm and prolapse. ThoraxcentreJournal200;13-I. 5 Simic 0, et al. Unruptured aneurysms ofthe non-coronary and left sinuses ofValsalva accompanied by severe aortic valve regurgitation. EurJ Cardiothorac Surg 1996;10:1030-2. 6 Holman WL. Aneurysms of the sinuses ofValsalva. In: Sabiston DC, Spencer FC, eds. Surgery ofthe chest. Philadelphia: WB Saunders & Co, 1995;1316-26. 7 Ribeiro A, et al. Non-coronary sinus ofValsalva aneurysm diagnosed after a road traffic accident. Heart 1999;82:5. 8 Hiyamuto K, et al. Aneurysm of the left aortic sinus causing acute myocardial infarction. Circulation 1983;67:1151-4. 9 Jebara VA, et al. Isolated extra-cardiac unruptured sinus of Valsalva aneurysms. Ann Thorac Surg 1992;54:323-6. 10 Williams TG, William BT. Isolated unruptured aneurysm of the left coronary sinus ofValsalva. Ann Thorac Surg 1983;35:556-9. 1

Netherlands Heart Journal, Volume 10, Number 4, April 2002

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