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CLINICAL PROGRESS Clinical Manifestations of the Unperforated Aortic Sinus Aneurysm By ISRAEL STEINBERG, M.D., AND NATHANIEL FINBY, M.D. ANEURYSMS of the aortic sinuses (of Valsalva) are rare and are either con- genital or acquired. The acquired types are chiefly due to syphilis or bacterial endo- carditis. The congenital aneurysms are thought to be due to a developmental defect in either the aorticopulmonary septum or the elastic tissue of the aortic sinuses. Morgan Jones and Langley in 1949,' in a classic paper, reviewed the literature; they recorded 43 autopsied cases of aortic sinus aneurysms and added 4 new ones. Venning in 19512 reported 3 additional cases, and Falholt and Thompson in 19533 described a case of un- ruptured congenital right aortic sinus aneurysm diagnosed by retrograde aortography and in- creased the number of congenital sinus of Valsalva aneurysms to 34. In 1954, Besabe and associates4 added a case of congenital aortic sinus aneurysm with rupture into the right atrium due to bacterial endocarditis, which also caused embolic gangrene of a foot. Nineteen cases of syphilitic aneurysm of the aortic sinuses have been reported in the English literature since 1914; the antemortem diagnosis was made only once. However, the advent of contrast visualization of the aorta has made possible the recognition of aortic sinus aneu- rysms in 9 additional cases.5 In a 10-year period, during which over 2,500 patients were studied by intravenous angio- cardiography, 19 cases of unruptured aortic sinus aneurysms have been diagnosed at this center. Seventeen cases have been reported previously and of these, 8 were congenital; 3, From the Departments of Medicine and Radi- ology, The New York Hospital-Cornell Medical Center, New York, N. Y. Aided by a grant from the Mallinckrodt Chemical Works. associated with coarctation of the aorta6; 1, with coarctation of the aorta and bacterial endocarditis7; 1 with pseudocoarctation of the aorta8; and 3 with arachnodactyly.9 The 9 acquired cases were due to syphilis: of these, 6 were associated with aneurysms of the ascend- ing aorta; while 3 had aortitis with localization of the aortic sinuses.5 The 19 cases of unperforated aortic sinus aneurysms observed by us form the basis of this report. Experience gained from their study indicates that there are significant clinical clues that should suggest the diagnosis. Confirma- tion may then be had by angiocardiography. NORMAL ANATOMY OF THE AOiRTIC SINUSES The aortic sinuses are 3 small dilatations in the wall of the aorta immediately above the aortic valves. Each sinus lies just above the attachment of an aortic cusp. The right and left coronary arteries usually originate within 2 of the aortic sinuses; however, they may emerge immediately distal to the aortic sinuses. The aortic sinuses and their corresponding aortic valves are named according to the source of the coronary arteries. Thus, the right and left coronary arteries emerge from the right and left aortic sinuses, respectively. The remaining aortic sinus usually lies posteriorly, does not contain a coronary artery and, according to the newest nomenclature, is designated the nion- coronary sinus.' The aortic sinuses are intracardiac and can- not be identified on conventional roent- genography. During angiocardiography they appear as small dilatations at the root of the aorta immediately above the aortic valves, and are best visualized in the left anterior oblique projection.10 Inconstant filling of the coronary arteries during angiocardiography 15 Circulation, Volume XIV, July, 1956 by guest on May 11, 2018 http://circ.ahajournals.org/ Downloaded from

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Page 1: CLINICAL Clinical Manifestations of the Unperforated ...circ.ahajournals.org/content/14/1/115.full.pdf · CLINICAL PROGRESS Clinical Manifestations of the Unperforated Aortic Sinus

CLINICAL PROGRESS

Clinical Manifestations of the UnperforatedAortic Sinus Aneurysm

By ISRAEL STEINBERG, M.D., AND NATHANIEL FINBY, M.D.

ANEURYSMS of the aortic sinuses (ofValsalva) are rare and are either con-genital or acquired. The acquired types

are chiefly due to syphilis or bacterial endo-carditis. The congenital aneurysms are thoughtto be due to a developmental defect in eitherthe aorticopulmonary septum or the elastictissue of the aortic sinuses.Morgan Jones and Langley in 1949,' in a

classic paper, reviewed the literature; theyrecorded 43 autopsied cases of aortic sinusaneurysms and added 4 new ones. Venning in19512 reported 3 additional cases, and Falholtand Thompson in 19533 described a case of un-ruptured congenital right aortic sinus aneurysmdiagnosed by retrograde aortography and in-creased the number of congenital sinus ofValsalva aneurysms to 34. In 1954, Besabe andassociates4 added a case of congenital aorticsinus aneurysm with rupture into the rightatrium due to bacterial endocarditis, which alsocaused embolic gangrene of a foot.

Nineteen cases of syphilitic aneurysm of theaortic sinuses have been reported in the Englishliterature since 1914; the antemortem diagnosiswas made only once. However, the advent ofcontrast visualization of the aorta has madepossible the recognition of aortic sinus aneu-rysms in 9 additional cases.5

In a 10-year period, during which over 2,500patients were studied by intravenous angio-cardiography, 19 cases of unruptured aorticsinus aneurysms have been diagnosed at thiscenter. Seventeen cases have been reportedpreviously and of these, 8 were congenital; 3,

From the Departments of Medicine and Radi-ology, The New York Hospital-Cornell MedicalCenter, New York, N. Y.

Aided by a grant from the Mallinckrodt ChemicalWorks.

associated with coarctation of the aorta6; 1,with coarctation of the aorta and bacterialendocarditis7; 1 with pseudocoarctation of theaorta8; and 3 with arachnodactyly.9 The 9acquired cases were due to syphilis: of these, 6were associated with aneurysms of the ascend-ing aorta; while 3 had aortitis with localizationof the aortic sinuses.5The 19 cases of unperforated aortic sinus

aneurysms observed by us form the basis ofthis report. Experience gained from their studyindicates that there are significant clinical cluesthat should suggest the diagnosis. Confirma-tion may then be had by angiocardiography.

NORMAL ANATOMY OF THE AOiRTIC SINUSES

The aortic sinuses are 3 small dilatations inthe wall of the aorta immediately above theaortic valves. Each sinus lies just above theattachment of an aortic cusp. The right and leftcoronary arteries usually originate within 2 ofthe aortic sinuses; however, they may emergeimmediately distal to the aortic sinuses. Theaortic sinuses and their corresponding aorticvalves are named according to the source of thecoronary arteries. Thus, the right and leftcoronary arteries emerge from the right and leftaortic sinuses, respectively. The remainingaortic sinus usually lies posteriorly, does notcontain a coronary artery and, according to thenewest nomenclature, is designated the nion-coronary sinus.'The aortic sinuses are intracardiac and can-

not be identified on conventional roent-genography. During angiocardiography theyappear as small dilatations at the root of theaorta immediately above the aortic valves,and are best visualized in the left anterioroblique projection.10 Inconstant filling of thecoronary arteries during angiocardiography

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CLINICAL PROGRESS

does not allow complete identification of thesinuses. However, in the left anterior obliqueview, the right coronary sinus is anterior andjust behind the sternum. The aortic sinuses ofValsalva are in close relation to all the cardiacchambers, particularly the right atrium andventricle. The superior vena cava and thepulmonary artery, the left ventricle and leftatrium are adjacent.

CLINICAL FEATURES OF UNPERFORATEDCONGENITAL AORTIC SINUS ANEURYSMSAge, Sex, and Race. The ages in 9 congenital

unperforated aortic sinus aneurysm patientsvaried from 7 to 47 years. All but 2 were under30 years; the older patients had associated

arachnodactyly. Six were male and 3 werefemale. Only 1 was Negro.

Associated Anomalies. Four patients withaortic sinus aneurysms had the classical find-ings of coarctation of the aorta, i.e., hyper-tension of the upper extremities, rib notching,and absent or diminished blood pressures of thelower extremities.6 One of the patients withcoarctation of the aorta had subacute bacterialendocarditis and aortic regurgitation. Threehad the classical findings of arachnodactyly.9One patient had pseudocoarctation of theaorta8; and 1, the youngest, aged 7 years, alsohad congenital mitral and aortic stenosis withgeneralized hypertrophy and dilatation of thecardiac chambers.

FIG. 1. Aneurysm of the right coronary sinus and coarctation of the aorta. Left anterior obliqueangiocardiogram of a 27-year-old woman demonstrates an enlarged left ventricle, a dilated ascendingaorta with an aneurysm of the right coronary sinus (arrow), coarctation of the aorta (arrow), andenlarged brachiocephalic vessels with prominent collateral arteries. (Previously published,7 repro-duced with the permission of the New England Journal of Medicine.)

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U-N 1E'RItF'OtATI1) AORTIC SINUS ANU'STlMYSIS

Skynptoms. All the patients with coarctationof the aorta were symptomatic; however, thesyim-ptoims were not due to the aortic sinusanieuiysm but to the associated aortic disease.Headache, dizziness, epistaxis, and Jacksonianepilepsy led to physical examination and thediagnosis of coarctation of the aorta in 3; feverand boutts of dyspnea resulted in the discoveryof a complicated subacute bacterial endocarditisin the fourth patient.Two of the patients with arachnodactyly

were free of cardio-vascular complaints; thethird had sudden onset of chest pain at the ageof 47 years, apparently due to the onset ofaortic regurgitation and mild heart failure. Thepatient with pseudlo(oarctatioln complainied ofnervousness alIlld backache, piol)al)ly due to a

neurotic disposition; while the ninth case, a7-year-old child, 1was chronically ill withdyspnea and easy fatigability.

lurmnurs. All patients with congenital aorticsinus aneurysms had heart murnmurs. Commonto all was a loud, rough (grade 2 to 4) systolicmurmur at the apex of the heart, heard all overthe precordium and especially to the left of thesterIumIn.7-9 In 3 instances, aortic valvularlesions were also present and produced diastolicand systolic murmurs at the base of theheart.7 9

In the 4 patients with coarctation of theaorta, there were in addition, interscapularbruits due to arterial collaterals. Resection ofthe coarcted segment and re-aniastomosis of theaorta completely relieved the hypertension and

1 'i(:. 2. Ancti'nsinal dilatat ion of thc aortic sinuses and p)seudocoarctlation. IFront al angiocardio-granm of a 26- ear-ol0 woman shows aneurysmal dilatation of the coronary sinuses (arrow) anl pseu-(locoarciat ion of the aorta (arrow). Note the details of the l)sexudocoatrctationi and the (lilated as-cending and1 (lescending aorta. In addition to normal blood pressure readiings in upper and lowerextremities, direct readings of 1)rachial and feInoral arterial p)ressures showed no dellav in blood flow,establ)lishi ng the (liagnosis of pseudocoarctation. (Previously plul)lishedl,8 re)ro(ducedI wsith the per-mission of Ihei British Heart .Journal.)

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did away with the interscapular murmurs, butthe cardiac murmurs were unaltered.

Blood Pressure. Systolic and diastolie hyper-tension was present in 3 of the patients withcoarctation of the aorta; the fourth with aorticregurgitation had a systolic pressure of 190mm. Hg but the diastolie blood pressure was 0.Two patients in this group had absent bloodpressures in the legs and in 2, the lower ex-tremity pressures were present but diminished.The patient with aira(chniodactyly and aorticregurgitation had an elevated pulse pressure,(140/60 mnm. IIg); in the other 2 cases theblood pressure was nornmal9

Electrocardiographly. ILeft ventricular hyper-trophy on electrocardiography was present in 2of the 4 patients with coaretation of the aorta.Two others and the patient with pseudoco-arctation had normal electrocardiograins. In thegroup with Mlarfain's syndrome, 1 had a normalelectrocardiographic tracing, although con-ventional and angi ocardiographic roentgeno-grams showed left ventricular hypertrophy;

another had partial heart block with a normal-sized left venitricle; the third had left axis devi-ation and left, ventricular hypertrophy.9 Thechild with generalized cardiac chamber en-largemnents showed peak P waves indicative ofatrial enlargement and inverted S-T and Twaves suggestive of myocardial cdisease.

Rlocttyenography of (.oqenlifalt I upeforratedA ortic Sinus Anieurlysnms(Cotrintional 1?ociitgcnograph,1y. On fluor-

oscopy, unusual pulsation of the roott andascending aorta was frequent. However, thehyperitension and(ldilated ascend(linig aorta due tocoarctation of the aorta an(l the aortic valveinvolvement ill the majority (of thle (c.a.ses 11mitedthe value of this observation.An abnormal cardiac silhouettte w-as present

in all ruietg~enogranis of patienlts with ('Co-gcenital aortic sinus aneurysmis except in 1instance; a patient with ara(chno(lac(tyly had anormal heart. A.11 cases with coarctation of theaorta, in addition, exhibited rib notchingsand

Fi(x. 3. Syphilitic aortitis with localized aneurysm of the aortic sinuses. Frontal roentgenogramof a 64-year-old man show-ing enlargement of the heart, l)ulmonary artery segment, an(l a massivecalcified mass occupying most of the right upper cardiac silhouette (a.rroxw-s).

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UNPERAFORATJL) AORTIC SINUS ANEURYSM-S1\9IS

an enlarged left ventricle. Evidence of post-stenotic enlargement of the descending aortaweas seen and verified by indentation of theesophagus in 3 instances. The patient withpseudocoarctation had a prominent shadow inthe region of the aortic knob and a dilateddescending aorta; howeverI,no rib notching wasrecognized. Two of the patients with arach-nodactyly had enlarged left -ventricles. In thepatient with aortic and mitral valve stenosisthere was also enlargement of cardiac chambers.A ngiocardiography. Angiocardiography pro-

vided the definitiv?e diagiiosis of aortic sinlusaneurysm. Gleneralized aneurysinal dilatation ofthe aortic sinuses was present in all b)ut 1instance. In that case (fig. 1), only the rightaortic sinus was involved. In the cases of co-arctation of the aorta, the point of coarctat on,the dilated b)ra(chiio(ephali( and collateralarteiial branches as well as the enlarged left

ventricle were clearly outlined (fig. 1). In con-trast, in the case of pseudocoarctation (fig. 2),although there was an unusually dilated leftsubclaviaIn artery, an apparent point of coarcta-tion1, and a dilated descending aorta, there w-asno collateral arterial circulation.

In summary, the only clue to the presence ofan unperforated congenital aortic sinus aneu-rysm is a grade s to 4 systolic inurmur over theheart, especially to the left of the sternum.Such a murmur if associated with coarctationof the aorta, anomalies of the aortic valves orarch, or arachnodactyly should arouse sus-picion of an aortic sinus aneurysim. Angio-cardiography w-ill establish the diagnosis.

SYPHILITIC INIEFIWO RATE I) AORTICSIN-US ANEURYSMS

Pathologic Aspects. Syphilitic involvement ofthe cardiovascular system is commonest at the

Fi(;. 4. Left anterior oblique angiocardiogram reveals the localized aortic sinus aneurysm (arrow)surrounded b)y calcified thrombus. The left ventricle is enlarged. (Previously 1l)l)lisledl,5 reproducedOweit}1 the J)erluiSSi of t be Americam.Joinriial of i\Icdici 1e

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aortic valve and ascenidinig aorta. It begims withwseakeniing and destruction of the intimalsurface of the aorta and elastic fibers of themedia. The aortitis becomes manifest as eithera diffuse aneurysmal dilatation or a localizedsaccular aneurysm of the ascending aorta.Calcium plaques are frequently deposited inthe damaged initima; their recognition in theroot and ascending aorta becomes an importantclue of syphilis."' 12 If the aneurysm is large,stasis of blood occurs and a laminated thrombuspartially fills the lumen. The aortic sinusesmay similarly he involved and aineurysmaldilatation or sacculatiorn of the sinuses results.Dilatation of the aortic ring and the productionof aortic insufficiency are usual.5

Uiilike congenital and mycotic aneurysms of

the aortic sinuses, which are paper thin andprone to rupture,' syphilitic aneurysms usuallybecome very large and laminated before theyperforate.'' 12 Any of the aortic sinuses may bethe site of origin of a syphilitic aneurysm. 'l'hisis in contrast to the congenital type, -which isbelieved to involve chiefly the right and non-coroniary sinuses.' Contrary to reports, there is11o predictable relation between the sinus ofOrigin and the stru(ture ilit(O which an aneurysmperforates.'3 E1ight of the 11 perforated cases re-ported in the literature (73 per cent) rupturedinto the lesser circulation (pulmionary artery 4,right atriunm 2, and right v'entricle 2), and pro-(luced a left to right shuniit.' Syphilitic aorticSiums aneurysms, unlike the (coingeniital, mayalso Irtiptuire outside the heri t. This is probably

Fni(.. 5. Syphilitic saecular aneurysm of the ascending aorta with aneurysmal involvement of t lieaortic sinuses. Conventional lateral roentgenogram of a 53-year-ol0( man shows enlargement of theleft ventricle and a large saccular aneurysm of the asceidiiig aorta, lined b)y calcium (arrow'). Notethat the linear calcification extends into the intracardiac l)ortion of the aorta and clearly outliiiesthe dilated aortic sinuses.

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UNPERFORATED AORTIC SINUS ANEURYSMS12

because the syphilitic aneurysms attain such alarge size.

IIi the literature, the most common cause ofdeath in patients with syphilitic aortic sinusaneurysm, even with perforation, was heartfailure. Rupture in 8 cases with the establish-ment of aorticocardiac or aorticopulmonaryshunts hastened failure; none survived morethan 2 months. On the other hand, ruptureoutside of the heart caused sudden death. In 3instances, a coronary artery occlusion due topressure of the adjacent aortic sinus aneurysmwas present. In 1 case, myocardial infarctionwas found distal to the occluded left coronaryartery and caused sudden death. In another,the aneurysm was intact but bulged into theinterventricular septum and pulmonary conuscausing pulmonary stenosis and delayed atrio-ventricular conduction, presumably due topressure on the bundle of His.5

Clinical A spects of Syphilitic UnperforatedAortic Sinus Aneuirysms

Age, Sex, and Race. In our series of 10 casesof syphilitic aortic sinus aneurysms, all theaneurysms were unperforated. The ages rangedfrom 44 to 60 years and averaged 54 years.MIen predominated; only 1 patient was awoman. Five were white and 5 Negro.Symptoms and Signs. As a rule, prior to

rupture, aortic sinus aneurysms do not bythemselves produce symptoms. Rather, syph-ilitic involvement of the aortic valvular ringcauses aortic insufficiency and heart failure._Most of our 10 patients were moderately toseverely ill; all had aortic incompetence. AortiCdiastolic and systolic murmurs, poundingpulses, and wide pulse pressures were usual. In3, heart failure was present in addition. Pre-

FIG.r6. Left lateral angiocardiogram demonstrates the aneurysm of the aortic sinuses, the dilatedascending aorta (aortitis) and the sacculated retrosternal aneurysm of the aorta. (Previously pub-lished,5 reproduced with the permission of the American Journal of Medicine.)

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cordial pain probably due to coronary ostialstenosis occured in several.

Blood Pressure. Prominent features in thereported cases and in our cases of syphiliticaortic sinus aneurysm were systolic hyperten-sion, high pulse pressures, and low diastolicpressures, which were related to the aortic in-sufficiency. Two of our patients, however, hadnormal blood pressures.

Electrocardiography. Electrocardiographic ex-amination in 9 living cases showed either leftaxis deviation or left ventricular hypertrophy.These findings reflect the enlargement of theleft ventricle due to aortic regurgitation andhypertension. In 1 patient (fig. 3 and 4), therewas right axis deviation and first degree heartblock. In this instance compression of thepulmonary conus, pulmonary artery, and itsright branch by the aneurysm was demon-strated, and is believed to have caused chroniccor pulmonale.5 In the literature, 2 cases alsoexhibited right axis deviation of the electro-cardiogram, but this was associated with per-foration of the aortic sinus aneurysm into thelesser circulation. The right axis deviation inthose instances was probably due to acute corpulmonale from the overloading and hyper-tension of the right heart.5

Roentgenography of Syphilitic UnperforatedAortic Sinus AneurysmsConventional Roentgenography. The diagnosis

of syphilitic aortic sinus aneurysm can oftenbe made after conventional roentgenography.A large boot-shaped heart due to the large leftventricle caused by the isolated aortic insuffi-ciency was present in all but 1 case. Pulsatiledilatation of the ascending aorta and fusiformor saccular aneurysms of the ascending aortaare other frequent findings. Linear calcificationof the aorta is common and has long beenrecognized as an important sign of syphiliticaortitis."1' 12 When the calcification extendsinto the intracardiac origin of the aorta andoutlines the aortic sinuses (fig. 5 and 6), or be-comes massive in this region (fig. 3 and 4), thediagnosis of syphilitic aortic sinus aneurysmbecomes almost a certainty.

Rarely, a patient with right heart enlarge-ment, right axis deviation of the electrocardio-

gram, and calcification near the left atriummay be mistaken for rheumatic heart diseasewith mitral and aortic valvular involvementand left atrial calcification.5 12 However, thepresence of linear calcification in the ascendingaorta should suggest the syphilitic etiology.

Angiocardiography. Aortic sinus aneurysmsare readily demonstrated by angiocardiog-raphy. In the syphilitic, 2 types are recognized:in 7 instances, the aneurysms were continu-ations of huge fusiform or saccular aneurysmsof the ascending aorta (fig. 6); in 3, the aneu-rysms were localized at the aortic sinuses andassociated with aortitis (fig. 4). In all in-stances, the syphilitic aneurysms were con-siderably larger than the congenital types and,therefore, created pressure effects in the sur-rounding cardiac chambers and great vessels.In 1 case (fig. 3 and 4), the deformity of the pul-monary conus and the pulmonary artery and itsleft branch was sufficient to cause right ventricu-lar hypertrophy and chronic cor pulmonale.5

In summary, the presence of a syphiliticaortic sinus aneurysm should be suspectedwhenever a fusiform or saccular aneurysm ofthe ascending aorta seems to extend into theheart. If, in addition, there is linear calcifica-tion of the ascending aorta reaching into theintracardiac origin of the aorta and, especially,if there is linear or massive calcification in theregion of the aortic sinuses, the diagnosis of asyphilitic sinus of Valsalva aneurysm becomesalmost a certainty. Angiocardiography by out-lining the root of the aorta will make the defin-itive diagnosis and will establish whether theaortic sinus aneurysm is continuous with anascending aorta aneurysm or localized, pro-ducing pressure on the neighboring cardiacchambers and great vessels.

RUPTURE OF AORTIC SINUS ANEURYSMS

Sudden onset of severe dyspnea, right heartoverloading and failure, enlargement of thepulmonary artery, and evolution of machinery-like murmurs may mean rupture of an aorticroot aneurysm into the pulmonary artery orright heart chambers.'4 Confirmation may behad by cardiac catheterization.'5' 16 A recentattempt to repair a ruptured aortic sinus wasnot successful.17

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UNPERFORATED AORTIC SINUS ANEURYSMS

TREATMENT AND PROGNOSIS OF AORTICSINUS ANEURYSMS

Surgical repair of syphilitic sinus of Valsalvaaneurysms is formidable because of their largesize and serious pressure effects on the neighbor-ing cardiovascular structures. However, in thevery rare congenital type (fig. 1) when theaneurysm is localized to a single aortic sinus,approach through the right atrium appearsfeasible. In our case, surgery of the aneurysmappeared especially dangerous because of theassociated aortic regurgitation, coarctation ofthe aorta, and the recent bacterial endocarditis.However, the patient withstood surgical cor-rection of the aortic coarctation very well.7Even though congenital sinus of Valsalva aneu-rysms are paper thin and tend to rupture, surgi-cal repair of the diffuse aneurysmal types ofunruptured congenital sinus of Valsalva aneu-rysms does not seem warranted.

Early diagnosis and treatment of syphiliswill prevent aortitis and aneurysms. Evidenceis being accumulated that penicillin treatmentof aortitis, either simple or complicated byaneurysm, is also beneficial. Of our 10 cases ofsyphilitic aortic sinus aneurysm, 3 are dead, 3were lost to observation, and 4 are alive andwell and have been under medical care forperiods up to 5 years.The 9 patients with congenital types of aortic

sinus aneurysms are all alive. The 4 with co-arctation of the aorta had resection of the co-arcted segments with complete relief of hyper-tension after end-to-end anastomosis of theaorta; all but the patient with recent subacutebacterial endocarditis are well and working.One of the 3 patients with arachnodactyly isstill troubled by mild congestive heart failure;the other 2 continue to be free of cardiovascularcomplaints. The patient with pseudocoarctationis also free of cardiovascular symptoms and isworking; while the child with the multiplevalve disease and heart enlargement remainsdisabled.The long-term prognosis of patients with un-

perforated aortic sinus aneurysms is unknown.However, even though there have been only afew years of observation, the general well beingof the majority of our patients suggests that

the prognosis is not immediately fatal. Once theaneurysm ruptures, the situation is usuallyhopeless. Therefore, even though the surgicalapproach is difficult and hazardous and thecirculatory hemodynamics are desperate, an at-tempt to repair the perforation should be made.

SUMMARY

There are no diagnostic features for therecognition of unperforated aortic sinus aneu-rysms except for a grade 3 to 4 cardiac murmur.Linear or massive calcifications in the intra-cardiac portion of the root of the aorta con-tinuous with the linear calcifications of theascending aorta sometimes occurs in syphiliticaortic sinus aneurysms. Accordingly, a highindex of suspicion and angiocardiography areadvocated in order to establish the diagnosisduring life.

Surgical repair of a localized congenital aorticsinus aneurysm through the right atrial ap-proach seems feasible if there are no seriouscomplicating aortic or cardiac lesions. Thediffuse unruptured aneurysmal congenital andsyphilitic aortic sinus aneurysms are betterleft alone. If rupture occurs, surgical repair,even though heroic, should be attempted.

ADDENDUMSince this review was submitted for publication

5 more congenital aneurysmal dilatations of theaortic sinuses have been discovered. Four were asso-ciated with Marfan's syndrome (2 in identical twins2 years old), the fifth with coarctation of the aorta.Two recent reports from England also describe

operations in 2 patients for closure of perforatedcongenital aortic sinus aneurysms."8 19

SUMMARIO IN INTERLINGUA

Ii non ha characteristicas diagnostic pro lerecognition de non-perforate aneurysmas delsino aortic, excepte un murmure cardiac degrado III a IV. Calcificationes linear o massivein le portion intracardiac del radice aortic,continuante in le calcificationes linear del aortaascendente, occure a vices in syphilitic aneurys-mas del sino aortic. Ergo, un alte grado desuspicion e le uso de methodos angiocardio-graphic es recommendabile si on vole establirle diagnose durante le vita del patiente.

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In le absentia de serie aortic o cardiac lesionescomplicatori, il pare practicabile reparar unlocalisate e congenite aneurysma del sino aorticvia le atrio dextere. In casos de diffuse non-rupturate aneurysmas del sino aortic, tantocongenite como etiam syphilitic, il vale melioabstener se de omne intervention. Si un rupturase effectua, le tentativa de un reparo chirurgicdebe esser interprendite, ben que un tal esalora heroic.

REFERENCES1 JONES, A. M., AND LANGLEY, F. A.: Aortic sinus

aneurysms. Brit. Heart J. 11: 325, 1949.2'VENNING, G. R.: Aneurysms of the sinus of Val-

salva. Am. Heart J. 42: 57, 1951.3FALHOLT, W., AND THOMSEN, G.: Congenital

aneurysms of the right sinus of Valsalva, diag-nosed by aortography. Circulation 8: 549, 1953.

4BASABE, H., HOJMAN, D., AND R6SEMBILT, E.:Bicuspid aortic valve and aortic (Valsalva) sinusaneurysm opening into right auricle: Embolicgangrene of the left foot. Rev. Asoc. m6d.argent. 68: 173, 1954.

5MERTEN, C. W., FINBY, N., AND STEINBERG, I.:The antemortem diagnosis of syphilitic aneu-rysm of the aortic sinuses: Report of nine cases.Am. J. Med. 20: 345, 1956.

6 DUBILIER, W. H., TAYLOR, T. L., AND STEINBERG,I.: Aortic sinus aneurysm associated with coarc-tation of the aorta. Am. J. Roentgenol. 73: 10,1955.

7 STEINBERG, I., AND FINBY, N.: Congenital rightaortic sinus aneurysm associated with coareta-tion of the aorta: Antemortem report of a case.New England J. Med. 253: 549, 1955.

8-: Aneurysm of the aortic sinuses with pseudo-coarctation of the aorta. Brit. Heart J. 18:85, 1956.

9 , AND GELLER, W.: Aneurysmal dilatation ofaortic sinuses in arachnodactyly: diagnosis dur-ing life in three cases. Ann. Int. Med. 43: 120,1955.

10 DOTTER, C. T., AND STEINBERG, I.: Angiocar-diography. New York, Paul B. Hoeber, Inc.,1951.

WALKIN, A.: The significance of calcification inthe ascending portion of the aortic arch.Radiology 62: 101, 1954.

12 SHIPP, J. C., CROWLEY, L. V., AND WIGH, R.:Aortic sinus aneurysm: Production of intra-cardiac calcification and pulmonary arteryfistula. Am. J. Med. 18: 160, 1955.

13 OSTRUM, H. W., ROBINSON, B. D., NICHOLS, C. F.,AND WIDMAN, B. P.: Aneurysms of the aorticsinuses or sinus of Valsalva. Am. J. Roentgenol.40: 828, 1938.

14HERRMANN, G. R., AND SCHOFIELD, N. D.: Thesyndrome of rupture of aortic root or sinus ofValsalva aneurysm into the right atrium. Am.Heart J. 34: 87, 1947.

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17KIRKLIN, J. W.: Quoted by Burchell, H. B.,Unusual forms of heart disease. Circulation 10:574, 1954.

18 ORAM, D., AND EAST, T.: Rupture of aneurysm ofaortic sinus (of Valsalva) into the right side ofthe heart. Brit. Heart J. 17: 541, 1955.

19 BROWN, J. WV., HEATH, D., AND WHITAKER, W.:Cardioaortic fistula: A case diagnosed duringlife and treated surgically. Circulation 12: 819,1955.

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ISRAEL STEINBERG and NATHANIEL FINBYClinical Manifestations of the Unperforated Aortic Sinus Aneurysm

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