THE ROENTGENOLOGICAL DIAGNOSIS OF SYPHILITIC

AORTITIS-A REVIEW OF FORTY PROVED CASES”

DAVID STEEL, M.D. CLEVELAND, 01-110

T HE data for this paper are taken from a study of forty cases of luetic aortitis observed roentgenologically and proved by autopsy.

No case of aneurysm is included. The roentgen method varied some- what but included in every case a fluoroscopic examination and tele- roentgenograms taken for the most part in the postero-anterior posi- tion and in many cases also in both oblique positions.

It is needless to point out that the course of the aorta should be thoroughly understood in the various positions. The diagrams (Figs. 1, 2, 3) bring out the so-called normal silhouette in the average case, but there are in addition normal factors which play an important part in this configuration. These are concerned mainly with increased and decreased spatial relations of the thoracic cage. With a high position of the diaphragm (expiration, ascites, abdominal tumors, etc.) the cage is small and the aorta is spread, appearing more prominent to the right and left and increasing slightly in height. With low position of the diaphragm the vascular shadow is narrow, and bulging to the right or left is minimal. With foreshortening of the thoracic cage, as in caries of the spine, spreading again takes place. With advancing age the aorta elongates and in accommodating itself to spatial rela- tions becomes spiral with an increase in its height.

In addition, the silhouette is not always sharply defined, anatomical relations are many times obscure, and the superior vena cava might add to, and the brilliant trachea subtract from, the apparent silhou- ette, especially in tk.e first oblique view. Even with special care it is difficult to obtain plates in an exact. postero-anterior view. These fac- tors, combined with the spatial relations of the thorax, must all be evaluated as closely as possible. We therefore often have s,everal fac- tors present, the value of which is almost impossible to estimate and hence various proposed measurements have a decided limitat,ion and can often lead to error. Nevertheless, if these limitations are recog- nized, measurements have a clinical value. Of the various methods, perhaps the simplest and most ingenious is t,hat proposed by Kreuz- fuchs; namely, measurement of the aort,ic width after barium visual- ization of the aortic bed of the esophagus. This method has been ac- curate at aut,opsy ir. the few cases I have proved. Unfortunately, the

*From the Roentgenological am3 Pathological Departments of the Clevelanrl City Hospital.

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60 THE AMERICAN HEmART JOURNAL

method measures the aorta near the isthmus rather than near the root where syphilis is more common and more import,ant physiologically. Just as proficiency grows with experience in other fields of medicine,

Fig. l.-Diagram of the normal heart in the anteroposterior position. S.V.C., SUPeriOr Vena cava: I.V., innominate vein; A.u., ascending aorta; p..4., pulmonary artery: A.w., left auricular appendage; R.V., right ventricle ; R.A., right atrium : L.V., left ventricle.

Fig. 2. Fig. 3.

Fig. Z.-Diagram of the normal heart in the first oblique position. I.V.C., Inferior vena cava ; LA., left atrium : Tr., trachea : Ad., ascending aorta: Tr. P..A., CI‘OSS- section of the pulmonary artery; P.A., pulmonary artery: R.V., right ventricle; L.V., left ventricle ; &f, manubrium : 8, sternum ; H, head of humerus.

Fig. 3.-Diagram of the normal heart in an exaggerated second oblique POSitiOn. R.A., Eight atrium; S.V.C., superior veno cava: 1.V.: innominate vein : A.u., ascend- ing aorta ; P.A., pulmonary artery ; L.V., left ventricle ; Tr., trachea; H, humerus ; R.V., right ventricle.

so does ability to judge aortic width fluoroscopically increase after many examinations in the normal and pathological. For this reason it seems logical to depend upon experience and general impression

STEEL : ROENTGENOLOGICAL DIAGNOSIS OF SYPHILITIC AORTITIS 61

after fluoroscopy rather than on a number of figures subject to many unknown factors.

The differential diagnosis of the pathological aorta is one of the im- portant problems awaiting a possible roentgenological solution. We have to differentiate, therefore, between the dilatation of atherosclero- sis, advancing age, hypertension, aortic insufficiency and stenosis of the isthmus. I have had no experience with isthmus stenosis. A re- view of these syphilitic cases gives the definite impression that these lesions cannot always be safely differentiated roentgenologically. Yet a diagnosis can be safely made when t.he lesion is well marked, and this margin of safety increases notably when the clinical facts are used. Therefore, the roentgen diagnosis should always be accepted by the clinician with this condition-“provided the history and physi- cal findings agree or at least do not oppose.”

Of the forty cases there are three considered as early. These will be discussed as a separate group. The remaining cases showed changes which we all associate with a pathological aorta, namely:

1. A dense shadow often with hazy borders, 2. A high, dense and prominent aortic knob. 3. Irregular and also general dilatation. 4. Increased puls,ation. 5. Assos:iat8ion with aortic insufficiency.

In many of the cases the shadow was unusually dense, supporting previous reports tha-t the luetic aorta produces a denser shadow than other lesions. This density is due for the most part to the increased diameter of the blood column incident to dilatation, and probably also in part to the changes in the wall. The changes in the height of the knob are most likely secondary to the changed spaeial relations due to aort,ic elongation and dilatation. The most important sign is irreg- ular dilatation. This presents itself as one or more localized spindle- shaped areas, not aneurysmal in dimension and involving most eom-

monly the root. In the postero-anterior view, such a change appears as a dense shadow of the upper right cardiac a,rch (Fig. 4). Its bor- der if extended would not conform to renmining portions of the sil- houette. In the first oblirjue the anterior and posterior borders of the ascending aort’a do not run parallel as normally, but, because of the greater dilatation of the root, they converge toward the sortie knob. IIence the silhouette is triangular with the base down. If the loeal- ized dilatation is near the knob, then the walls converge toward the heart and the silhouette becomes club-shaped. In the second oblique vielv, because the course of the aorta is now at right angles to the path of the rays, making the entire silhouette visible especially in the pathological case, hea1 dilatations can easily be made out (Fig. 5). These local dilatations are to be expected, ancl they are easily ex- nlainecl by the disruption of the elastic fibers so common in syphilitic

aortitis. With the dilatation there is a localiwd incrcasr in pulwt~ion which should always be looked for and used as a11 index to dilatation.

In the later stages dilatation may beeomr tlif?usc~ (Fig. 6). This can become extreme, and since marked dilat,ation is seldom swn in

Fig. 4.-Localized dilatation of thr ascending aorta as sew in the postero-anterior view.

Fig. 5.+om,lized dilatation of the ascending portion. Thq ar’c of this portion Mhm,contmued (----) do,es not conform to the remaining portlons of the arch far-

other lesions, it has considerable diagno..tic importance. The pulsa- tions are decreased in amplitude, probably due to cicatricial changes. Increasing dilatation on repeated observat,ion tends to rule out athero- sclerosis in favor of syphilitic aortitis.

STEEL : ROENTGENOLOGIC’AL DIAGNOSIS OF R1’PHILmITI( AORTITIS 63

To summarize the well-marked cases of syphilitic aortit,is, it can be said that the findings of a high, dense and prominent knob, a slight haziness in outline, A cardiac silhouette consistent with aortic insuffl-

Fig. 6. Fig. 7.

FiIi‘ip. ti.-Diffuse dilatation of the aorta with aortic insufficiency. Fig. T.--Early- case. .Yortic insufficiency.

tion. ilutopsy seven days after this examina-

Fig. 8 Fig-. 9.

Fig. H.-Early case. Fluoroscopically the ascending portion of the aorta was locally dilated and showed increased pulsations. examination.

see next figure for follow-up

Fig 9 -The sane ca:w showng (liffuse llilatation of the aorta anal canliac ~lecom- wnsation.

ckucy, generalized dilatation, unless excessive, and increase in ampli- tude of pulsation are not in themselves pathognomonic signs for the

64 TBEs AMERIPAS HEART JOURNAL

roentgenological diagnosis of syphilis. d marked degree of general-

ized clila.tation is more ~~inmon in syphilis and can be taken as pre- sumptive evidence, especially if the heart shadow is small or if it occurs in a person under fort,y. When the various segments are vari- ably dilated the diagnosis is almost cert,ain. I sa) ’ ’ almost ’ ’ because

Va’guez and Bordet report local dilatation of the aortic root in Iiodg- kin’s disease. Itheumatic dilatat,ion has been rrported also. The

roentgen findings should always be correlated with the clinical and laboratory findings. IIowever. too great reliance must not be placed on the Wassermann test, because in this series of thirty-six cases so examined, fourteen, or about 39 per cent, were negative and four, or 11 per cent, were reported as ant,icomplementary.

The three cases referred to above rrpresent t,he earliest, lesions of the series and are characterized by minimal or questionable roent- genological changes in spite of the fact that at, autopsy the lesion was well marked. Each case shows an increase in the height of the knob and an increased density, changes which ANY .SP are found in tllc other common lesions. Wl len thesr arc present. alone, which is true of two of these three, the diagnosis of syphilis is not possible. How- ever, in each case, the right upper cardiac arch has a hazy outline and shows an indefinite or questionable bulge to the right. In one case there was present in the second oblique position a slight localized dilatation with increased pulsat,ion in t,he ascending aorta (Fig. 7). The significance of the hazy outline and the very questionable bulging of the right upper arch in the postero-ant,erior view is questionable, but seeing such a change should call for very careful fluoroscopic ob- servation in t,he second oblique view for localized dilatat,ion and pulsa- tion. When these findings are demonstrable, the diagnosis rests upon a firm founclation. Fig. 4 rt>presents a well-marked localized dilata- tion of the ascending aorta in the postero-anterior view, and Fig. 5 represents similar changes in the second oblique position. If the arc of the dilated portion is schematically contjinuetl, it will be noticed that it does not conform to the remaining outline of the arch. Wc must conclude, therefore, that early syphilitic aortit,is is not always demonstrable roentgenologically and that its earliest sign is localized dilatation associated with a localized increase in the amplitude of the pulsations (Figs. 8 and 9).

There are cases of syphilitic aortitis which present definite roent- genological changes and which are indistinguishable from the other common lesions of the aorta. The lesions can be suspected when (1) a diffuse dilatation is present and is associated with a normal sized heart silhouette, (2) a dense, high aorta is present in a young individual without a previous hypertension. It can be safely diagnosed when

STEEL : ROE.NTGENOLOGICAL DfAGNOSIS OF SYPHILITIC AORTITIS 65

localized dilatation a.ssociated with localized increased pulsation is demonstrable.

REFERENCEtS

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Assmann, Herbert: Die klinische Roentgendiagnost,ik der iuneren Erkrankungen, ed. 3, Leipzig, Vcgel, 1924.

Eisler, F., and Erouzfuchs, S.: Die Roentgendiagnose der Aortensyphilis, Deutsche med. Wchnschr. 39: 214.5, 1913.

Groedel, Franz M.: Lehrbuch der R,Gntgendiagnostik, Miiuchen. J. F. Lehmann, 1924.

Holmes, 0. W., and Ruggles, 1-I. E.: Roentgen Interpretation, ed. 3, Phil- adelphia, Lea & Fehiger, 1926.

Holzhnecht, G.: Zum radiographischen Verhalten pathologischer Processe der Brustaorta, Wien. klin. Wchnschr. 13: 573, 1900.

Holzknecht, G. : Da3 radiographische Verhalten der normalen Brustaorta, Wien. klin. Wchnschr. 13: 225, 1900.

Karshner, R. G., and Ken&ott, R. H.: A Practical Method of Roentgen Exam- ination of Heart Based Upon a Study of 100 Consecutive Normal and Ab- normal Cases, Am. J. Rocitgenol. 9: 305, 1922.

Karsner, H. T.: Human Pathology, ed. 2, Philadelphia, Lippincott, 1929. Kb;hler, Alban : Ro2ntgenologp, Translation from Fifth German Edition, New

York, Wm. Wood, 1928. Kreuzfurhs, S.: Ucber eine neuc Methode der Aortenmessung, Med. Kliu. 16:

36. 1920. Lippmann, A., and f&iring, W.: Die RGntgenuntersuchung der Aortenerkrank-

ungen mit spezieller Beriicksichtigung der Aortenlues, Fortschr. a. d. Geb. d. Rijntgcnstrahlen 19: 253, 1912.

Vaquez, H., and Bo-det, E.: Radiologie du cocur ct des vaisseaux de la base, cad. 4, Paris, Ball &e, 1938.

Zehbe : Beobachtungen am Herzen und der Aorta, Deutsche med. Wchnschr. 42: 315, 1916.

CISCUCSION

Dr. Lre, Washington, D. C.--Dr. Steel referred to the size of the heart. Does

he use the measurement3 of Bordet and Vaquez, and in what phase of respiration

does he take the pictures?

Dr. Lrl~is 8. Con?ley, New York, N. Y.-I do not feel that I can let this oppor-

tunity pass without adding a warning concerning the roentgeuological diagnosis of

syphilitic aortic disease. I think Dr. Steel has been very conservative in his state-

ments. It seems to me: however, that it would be a pity for us to go away with

the idea that this is a rsafe method of establishing a diagnosis. I have frequently

seen the grossest sort of mistakes made because of too great reliance upon the

roentgenographic and fluoroscopie appearances of the aorta. I do not think one is often justified in making that diagnosis on the basis of the roentgenological findings

alone, and I am sure Dr. Steel does not think so. This is no criticism of his

statements. Elongation of the aorta begins very early and is very frequent in the

early fifties even without any outstanding hypertension, and doubtless there are

also variations in the curves of the right and left side of the heart, but most of us are not skilled enough to use these slight signs as important factors in the diagnosis.

I still believe, as I have for many years, that clinical diagnosis of syphilitic a.ortitis,

in the absence of aneurysm and other gross deformities of the aorta, cannot safely

bc established before the> advent of aortic insufficiency.

Dr. TT. S. Tkayw, Baltimorr, Md.-One word in connection with Dr. Conner’s remarks. The only ma?- we ran establish a diagnosis is by finding out all that wc

ran from every source, and then by putting our heads together. The man who is attempting to make a definite diagnosis on the basis of one single method of ex-

amination, is doing a lery rash thing. I think this method is an admirablr one,

but I would like to say more than Dr. Conner, and that. is that we clinicians should

66 THE AMICRTCAN IIE~ART JOURSAI,