S INUS of Valsalva aneurysms are an uncommon entity with less than 300 cases reported in the literature prior to

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1 VoL. 122, No. 4 CALCIFIED NONSYPHILITIC ANEURYSMS OF THE SINUSES OF VALSALVA By ROBERT T. REINKE, M.D.,* MARC N. COEL, M.D.,t and CHARLES B. HIGGINS, M.D. LA JOLLA, CALIFORNIA S INUS of Valsalva aneurysms are an uncommon entity with less than 300 cases reported in the literature prior to These aneurysms may be discrete aneurysms of a single sinus which are usually congenital, or may be due to an aneurysm of the aortic root which extends through the aortic annulus and causes generalized dilatation of all 3 sinuses of Valsalva,1 which is usually acquired. Calcified sinus of Valsalva aneurysms are even more unusual.3 In the past these have usually been associated with syphilis. We report here 2 cases of calcified sinus of Valsalva aneurysms, unassociated with syphilis. REPORT OF CASES CASE I. J.S. (#i I), a 53 year old white man, was diagnosed as having subacute bacterial endocarditis in 1944, and was told he had the murmurs of aortic valvular insufficiency and stenosis. He received penicillin therapy for 3 months. During the 1950S and I960s he was hospitalized multiple times for ethanol abuse, but had no cardiac symptomatology. He denied ever having had syphilis or a penile lesion. A VDRL in November 1950 was nonreactive. In 1955 curvilinear calcifications were first noted on a chest roentgenogram. The VDRL was still nonreactive in June Because of his murmur and progressive calcification of the aneurysms of the sinuses of Valsalva (Fig. I, A-C) he was treated with penicillin and streptomycin in July 1972, although his cardiac symptoms were unchanged clinically. Physical examination revealed a blood pressure of 120/70 mm. Hg and murmurs of aortic valvular stenosis and insufficiency. The VDRL and the Fluorescent Treponemal Antibody-Absorption test were nonreactive. Roentgenograms of the sacroiliac joints were normal. Cardiac catheterization demonstrated moderate aortic valvular insufficiency and the discrete aneurysms of the sinuses of Valsalva (Fig. 2, A and B). After a 2 year follow-up, the patient remains asymptomatic. CASE II. H.J. (#4972), a 53 year old white man, had 3 episodes of acute rheumatic fever in his early thirties and was told he had the murmur of aortic valvular insufficiency. He denied ever having syphilis or a penile lesion. He did well until age 43 at which time he had progressive dyspnea on exertion which was relieved by digitalization. During September 1972 he was admitted for evaluation of chest pain. Physical examination revealed a wide pulse pressure and the peripheral signs of aortic insufficiency. He had the murmurs of aortic valvular stenosis and aortic valvular insufficiency. There was also an Austin- Flint murmur and a S3 gallop. The neurologic examination was normal. A routine roentgenogram of the chest during September 1972, demonstrated a large calcified ascending aortic aneurysm (Fig. 3, A-C) contiguous with calcified aneurysmal dilatation of the sinuses of Valsalva. There was moderate cardiomegaly with biventricular enlargement. An electrocardiogram showed left axis deviation, left ventricular hypertrophy with a strain pattern, and left bundle branch block. Cholesterol, triglycerides, latex fixation and antinuclear antibody tests were normal. The VDRL and Fluorescent Treponemal Antibody-Absorption tests were nonreactive. Roentgenograms of the sacroiliac joints were normal. Right and left heart catheterization demonstrated normal pressures in all chambers, and normal cardiac index and arteriovenous oxygen difference. There was moderate aortic regurgitation and a markedly dilated aortic root with aneurysmal dilatation of the sinuses of Valsalva and ascending aorta extending to and involving the arch vessels. Surgery was not attempted. Recent roent- From the Division of Cardiovascular Radiology at University Hospital,5 and the Department of Radiology at the Veterans Administration Hospital,t University of California at San Diego, School of Medicine, La Jolla, California. 783

2 784 R. T. Reinke, M. N. Coel and C. B. Higgins DECEMBER, 1974 genograms of the chest have shown no change in the size of the ascending aortic aneurysm. DISCUSSION Aneurysms of the sinus of Valsalva may be congenital on acquired. Several excellent reviews have thoroughly dealt with the congenital sinus of Valsalva aneurysms and their relationships to ventricular septal defects and acquired aortic valvular insufficiency Congenital aneurysms of the sinus of Valsalva are usually discrete; i.e., involve a single sinus. They follow a definite pattern in site of origin and direction of development; 4 over 95 per cent of aneurysms originate in the right coronary sinus and the right part of the noncoronary sinus, and project into the right ventricle or right atrium. Edwards et al.,2 have proposed that the fundamental

3 VOL. 122, No. Calcified Nonsyphilitic Aneurysms of the Sinuses of Valsalva 785 FIG. 2. Case I. Retrograde aortic root injections in (A) lateral and (B) left anterior oblique projections demonstrate the sinuses of Valsalva (right [RI, left EL] and noncoronary [NC) cusps). There are 2 aneurysmal dilatations extending from them. One (arrowheads) is posterior-inferiorly located between the noncoronary cusp and the left coronary cusp, and is best seen on the lateral projection (A). The second, best seen on the left anterior oblique projection (B), is higher and is at the level of the junction of the aortic annulus with the right coronary cusp, and does not fill (arrow), probably because it is filled with thrombus. cause of congenital aneurysms is due to a separation of the aortic media and the annulus of the aortic valve. Acquired sinus of Valsalva aneurysms may be discrete9 (localized) or generalized (involving all the sinuses), developing as a consequence of a localized inflammatory process or as a generalized lesion of the aortic root which extends through the aortic annulus and causes dilatation of all 3 sinuses of Valsalva. Generalized dilatation of the sinus of Valsalva is usually acquired secondary to bacterial endocarditis, syphilis,8 0 5 or atherosclerosis.6 Marfan s syndrome is reponsible for those few generalized sinus of Valsalva aneurysms which arise on a congenital basis The diagnosis of an aneurysm of the sinus of Valsalva on the plain roentgenogram is facilitated by the presence of calcification, which is a relatively rare finding. The majority of those reported in the past have occurred in syphilis The literature indicates that when aortic calcification extends into the intracardiac origin of the aorta and outlines the aortic sinuses, the diagnosis of syphilitic aortic sinus aneurysm becomes almost a certainty Only 2 cases of calcified aneurysm of the sinuses unassociated with syphilis 2 have been previously described and both of these were congenital in origin. The current cases indicate that acquired aneurysms of the sinuses of Valsalva may

4 786 R. T. Reinke, M. N. Coel and C. B. Higgins DECEMBER, 1974 FIG. 3. Case II. Roentgenograms of the chest in the (A) posteroanterior, (B) lateral and (C) left anterior oblique projections demonstrate the large calcified aneurysm of the ascending aorta which extends to and involves the sinuses of Valsalva. Calcification in the dilated sinuses of Valsalva is indicated by arrows. calcify in the absence of a syphilitic etiology. The other plain film roentgenographic findings are relatively nonspecific as to etiology and include: (i) a prominent pulmonary arch segment due to enlargement of the sinus of Valsalva to the left;7 (2) a prominent localized bulge of the lower right cardiac margin;3 (3) a dilated ascending aorta;8 7 2#{176} and (.) presence of a notch sign between the ascending aorta and the heart noted on the posteroanterior or left anterior oblique views. 5 Linear calcification of the aorta, which has long been recognized as an important sign of syphilitic aortitis, 6 24 recently has been found not to be a pathognomonic roentgenographic sign of syphilis as previously thought In this study the most common cause of ascending aortic calcification was atherosclerosis.4 These 2 cases show that nonsyphilitic inflammatory disease may cause calcification of aneurysms of the sinus of Valsalva. With the decrease in cardiovascular syphilis, and the attendant increase in longevity, other causes of

5 VoL. 222, No. Calcified Nonsyphilitic Aneurysms of the Sinuses of Valsalva 787 calcification may become relatively more common. SUMMARY Calcification of a sinus of Valsalva aneurysm has been considered specific for a syphilitic aortitis. The current report contains 2 cases of calcified aneurysms of the sinuses of Valsalva due to nonsyphilitic inflammatory disease. These 2 cases along with 2 cases previously reported to be congenital in origin comprise a total of 4 reported cases with calcified aneurysms of the sinuses of Valsalva of nonsyphilitic etiology. Thus, calcified sinus of Valsalva aneurysms are not specific for syphilitic aortitis, but may rarely be associated with any of the etiologic factors responsible for these aneurysms. Robert T. Reinke, M.D. Department of Radiology University of California, San Diego University Hospital 225 West Dickinson Street San Diego, California REFERENCES i. BJORK, V. 0., and BJORK, L. Aneurysm of sinus of Valsalva. 7. Thoracic & Cardiovasc. Surg., 1965, 50, EDWARDS, J. E., BURCHELL, H. B., and CHRIS- TENSEN, N. A. Specimen exhibiting essential lesion in aneurysm of aortic sinus. Proc. Staff Meet. Mayo C/in., 1956, 3!, HARRIS, E. J. Aneurysm of sinus of Valsalva. AM. J. ROENTGENOL., RAD. THERAPY & NUCLEAR MED., 1956, 76, HIGGINS, C. H., and REINKE, R. T. Non-syphilitic etiology of linear calcification of ascending aorta. To be published.. HOPE, J. A. Treatise on the Heart and Great Vessels. 1840, London. 6. KAWASAKI, I. A., and SCHULTZ, F. B. Aneurysm of sinus of Valsalva with rupture into right auricle. Am. Heart 7., 1951, 4!, 149-I MCKUSICK, V. A. Cardiovascular aspects of Marfan s syndrome: heritable disorder of connective tissue. Circulation, 1955, II, MERTEN, C. W., FINBY, N., and STEINBERG, I. Ante mortem diagnosis of syphilitic aneurysm of aortic sinuses. Am. 7. Med., 1956, 20, ORAM, S., and EAST, T. Rupture of aneurysm of aortic sinus into right side of heart. Brit. Heartj., 1955, 17, OSTRUM, H. W., RoBINsoN, B. D., NICHOLS, C. F., ani WIDMAN, B. P. Aneurysm of aortic sinuses or sinus of Valsalva. Aid. J. ROENT- GENOL. & RAD. THERAPY, 1938, 40, II. PRIAN, G. W., and DIETRICH, E. B. Sinus of Valsalva abnormalities. Vasc. Surg., 1973, 7, ROWLEY, J. C., and HOLMES, R. B. Sinus of Valsalva aneurysms. 7. Canad. A. Radiologists, 1965, 16, SAKAKIBARA, S., and KONNO, S. Congenital aneurysm of sinus of Valsalva associated with ventricular septal defect. Am. Heart 7., 1968, 75, SAKAKIBARA, S., and KONNO, S. Congenital aneurysm of sinus of Valsalva: anatomy and classification. Am. Heart 7., 1962, 63, SCHNEIDER, H. J., and SPITZ, H. B. Unruptured aortic sinus aneurysm : plain film diagnosis. Dis. Chest, 1968, 53, i6. SHIPP, J. C., CROWLEY, L. V., and WIGH, R. Aortic sinus aneurysm : production of intracardiac calcification and pulmonary artery fistula. Am. 7. Med., 1955, i8, i6o-i STEINBERG, I., and GELLER, W. Aneurysmal dilatation of aortic sinuses in arachnoidactyly: diagnosis during life in 3 cases. Ann. mt. Med., 1955, 43, STEINBERG, I., and FINBY, N. Clinical manifestation of unperforated aortic sinus aneurysm. Circulation, 1956, 14, STEINBERG, I. Dilatation of aortic sinuses in Marfan s syndrome: roentgen findings in new cases. AM. J. ROENTGENOL., RAD. THERAPY & NUCLEAR MED., 1960, 83, STEINBERG, I., and FINBY, N. Roentgen manifestations of unperforated aortic sinus aneurysms. AM. J. ROENTGENOL., RAD. THERAPY & NUCLEAR MED., 1957, 77, SYMBAS, P. N., RAIZNER, A. E., TYRAS, D. H., HATCHER, C. R., INGLE5BY, T. V., and BALD- WIN, B. J. Aneurysms of all sinuses of Valsalva in patient with Marfan s syndrome. Ann. Surg., 1971, 174, TAGUCHI, K., SASAKI, N., MATSUURA, Y., and UEMURA, R. Surgical correction of aneurysm of sinus of Valsalva. Am. 7. Cardiol., 1969, 23, TRIANO, G. J. Calcified congenital left coronary sinus aneurysm. Radiology, 1968, 9!, WOLKIN, A. Significance of calcification in ascending portion of aortic arch. Radiology, 1954, 62,

6 This article has been cited by: 1. Arul Dominic Furtado, Devananda Nijagal Shivanna, Syed Murfad Peer, Jayaranganath Mahimarangaiah Calcified Unruptured Sinus of Valsalva Aneurysm in a Child. Pediatric Cardiology 32:8, [CrossRef] 2. Bibliography [CrossRef]