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Syphilitic Aortitis and Aneurysm *
SYPHILITIC AORTITIS AND ANEURYSM'" AARON ARKIN, Ph.D., M.D., FAC.P.t CARDIOVASCULAR SYPHILIS IS PREVENTABLE
SYPHILITIC heart disease is today the only fonn of heart disease which is preventable. A more general recognition of this fact will lead to a careful search of all patients infected with syphilis for the early manifestations of this most fatal fonn of the disease. A diagnosis in the primary seronegative stage by darkfield examination, followed by two years of proper treatment, would prevent at least 90 per cent of cases of cardiovascular syphilis. I wish in this clinic to discuss the pathology and diagnosis of syphilitic aortitis. Syphilis is responsible for about 20 per cent of cases of chronic cardiac disease found in adults. About onefifth of all persons who acquire syphilis develop cardiovascular disease. The average length of time from infection to the onset of symptoms is about fifteen years. In luetic aortic regurgitation the average latent period is twenty years, in aneurysm twentytwo years. At necropsy we find that 75 per cent of persons with visceral lues have an aortitis. It is therefore obvious that many of these individuals do not develop clinical evidence of heart disease, and are not diagnosed during life. We shall emphasize the methods at our disposal for the early diagnosis of uncomplicated syphilitic aortitis. SEX AND RACE INCIDENCE
There is a marked preponderance of the male sex in the entire picture of syphilis of the heart and aorta. About 80 per cent of the patients are males, yet there is no marked difference in the frequency of luetic aortitis in the two sexes. In females the disease takes a more benign course. The severe supravalvular fonns .. From the Department of Medicine, Cook County Hospital. of Medicine and Chairman of Department of Medicine, Cook County Graduate School; Associate Professor of Medicine (Rush), University of Illinois College of Medicine; Attending Physician, Cook County and Mt. Sinai Hospitals; Staff, Wesley Memorial Hospital.
t Professor
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with aortic regurgitation, stenosis of the coronary ostia, and aneurysm are much more common in men. Syphilitic heart disease is about four times as frequent in the Negro as in the white. Aneurysms are twice as common as in the white race. The greater number of hard laborers, high incidence of hypertension, and more frequent neglect of proper therapy may explain this difference. I believe that there is a more marked involvement of the media with more extensive necrosis in the colored race. While aneurysm is rare in white women we find it not infrequently in Negro women. PATHOLOGY OF SYPHILITIC AORTITIS
Syphilitic aortitis is the basic lesion from which the manifestations of cardiovascular syphilis develop. It leads to aneurysm formation, widening of the aortic ring with separation of the cusps at the commissures and aortic regurgitation, and stenosis of one or both coronary ostia. Luetic infection usually occurs in the twenties, yet the highest incidence of symptoms due to aortitis is near the forty-fifth year. The reason for this long latent period of twenty years is the very slow progress of the chronic inflammatory process which begins in the vasa vasorum of the adventitia and media. These small vessels undergo obliterative changes which furnish partial anaerobic conditions for the growth of the spirochetes in the media. There is an infiltration by round and plasma cells. The elastic fibers are destroyed by the development of miliary gummas. The destructive and inflammatory changes in the media lead to intimal wrinkling and puckering. The inner surface of the aorta becomes wrinkled and depressed by small and large fibrous scars of pinkish or white color. Atheromatous changes are often combined with the luetic aortitis in an amount increasing with age. The mouths of the large branches of the aorta, such as the innominate, carotid, and subclavian, are often fibrosed and greatly constricted or almost occluded. This stenosis of the ostia of the large arteries is of great value in diagnosis, leading to pulse differences, thrills and murmurs along the course of the vessels. Sometimes there is a complete absence of the pulse in one or more large arteries. ANEURYSM FORMATION
As the aortic wall (in some cases also the innominate, carotid, subclavian or other large artery) loses its elastic layer the wall
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becomes weakened. It stretches under the influence of the blood pressure. If the mesaortitis is widespread there may be a diffuse dilatation of the ascending, arch, descending or entire thoracic aorta. When the destruction is limited to a small area a saccular aneurysm may develop. Such aneurysms of the thoracic aorta are practically all luetic, whether atheromatosis be present or absent. Multiple aneurysms of the thoracic aorta are quite common. Aneurysms 'occur in about 20 per cent of cases of syphilitic aortitis. They are most common in the ascending aorta and arch, and decrease in frequency toward the bifurcation. They tend to occur along the path which receives the impact of the blood column. This pathway runs in the middle of the anterior wall of the supravalvular portion, then to the right in the ascending, horizontally in the arch, and along the posterior left aspect of the descending arch, and in the middle of the posterior wall of the descending aorta. They may erode the spine, ribs or sternum. ,. We shall see that the demonstration of a widening of any part of the thoracic aorta is the earliest diagnostic sign of syphilitic aortitis. Such a widening can often be found before the development of aortic regurgitation, aneurysm, or angina pectoris. Syphilitic aortitis without dilatation of the aorta, regurgitation, or coronary stenosis cannot be diagnosed clinically. When the orthodiagraphic measurement of the aorta exceeds the normal values, syphilitic aortitis must be suspected. This is especially so when the patient has a positive serologic test, Argyll-Robertson pupils, absent patellar reflexes, or any other evidence of lues. Hypertension, rheumatic aortic regurgitation, isthmus stenosis, or thyrotoxicosis may lead to increased cardiac force or output with moderate widening of the aorta. With old age the aorta elongates, the arch rises and the vessel becomes more tortuous. There is evidence of atheromatosis, and the process is quite uniform. These conditions can usually be distinguished from luetic aortitis. Aneurysm may be caused by bacterial emboli in bacterial endocarditis. It is very rare in rheumatic fever. Periarteritis nodosa, suppuration, tuberculosis and trauma are rare causes. Aortic rupture or dissecting aneurysm is usually the result of cystic media necrosis, first described by Erdheim. Abdominal aneurysm may be due to arteriosclerosis.
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AORTIC REGU RGITATlON
Perhaps the most important pathologic change is aortic regurgitation. This is found in about 75 per cent of the cases diagnosed clinically, and in about 30 per cent of cases of luetic aortitis at autopsy. Syphilis has a special affinity for the sinuses of Valsalva and the aortic ring. The increased strain Upon this region caused by the closure of the aortic cusps in diastole is a factor in producing the stretching of the ring. The cusps become thickened at the commissures and are separated from each other. No vegetations are produced, hence there is never a stenosis of the aortic ostium. The ring is stretched, the cusps are too small to close the lumen in diastole and regurgitation results. There is later a regurgitation at each of the commissures, and in the center of the lumen. Syphilitic aortitis, therefore, causes only one valvulttr lesion, aortic regurgitation, never a stenosis. Rarely, syphilitic pulmonary arteritis causes a pulmonary regurgitation. The mitral and tricuspid valves are not affected. With regurgitation there is a progressive dilatation and hypertrophy of the left ventricle, often leading to great cardiac enlargement. The average length of life is about three years. STENOSIS OF THE CORONARY OSTIA
Another important change is stenosis of the ostia of the coronary arteries. This is a very frequent finding, a part of the aortitis in the sinuses of Valsalva. It leads to anoxemia of the heart muscle with angina pectoris. The anginal attacks are usually of long duration. The T wave is often negative in Leads I and 11 of the electrocardiogram. Myocardial failure which reacts poorly to all medication is the end result. The stenosis is so gradual that one ostium may be totally occluded without producing clinical symptoms or any gross myocardial changes. During the years that elapse there is plenty of time for the collaterals to enlarge so that the heart muscle gets its nourishment from the other coronary. When both coronaries are affected the heart may be unable to undergo much hypertrophy. The heart muscle may still receive some blood supply from the thebesian channels, or from anastomoses between the coronaries and vasa vasorum or bronchial arteries. Sometimes the coronary ostium is covered by a small flaplike thickening of the intima. The presence of an aortic regurgita-
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tion reduces the blood flow in the coronaries still further by lowering the diastolic blood pressure. Sudden death is not uncommon in such cases of aortic regurgitation with stenosis of the coronary ostia. In very rare cases a supernumerary vessel may furnish blood to the myocardium. It often appears as if the stenosis of the coronary ostia protects them against atheromatosis by reducing the blood flow and pressure in these vessels. The arteries themselves are not affected in syphilis, hence coronary thrombosis with infarction
Fig. l2.-Early syphilitic aortitis, with characteristic. convex prominence of the ascending aorta. There was a typical tambour aortic second sound, heard best in the second right interspace. There was no regurgitation, hence the heart is normal in size. The· Kahn test was positive.
is uncommon. When it does occur it is usually the result of sclerosis or subintimal hemorrhage. The heart muscle is only rarely affected by syphilis. Seldom is a gumma or diffuse syphilitic mY9carditis found. In the bundle of His it may lead to heart block. In a papillary muscle rupture, may occur. The fibrous changes found in older luetic individuals are more readily explained as a result of arteriosclerotic nonluetic coronary disease, or ischemia of the muscle from the stenosis of the ostia.
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It must be emphasized here that aneurysm alone does not cause cardiac enlargement, regardless of its size. In the absence of aortic .regurgitation, coronary disease, hypertension or other cause, the heart remains normal (Figs. 13, 15, 20). Aneurysms of the supravalvular portion of the aorta may rupture into the pericardial sac, causing death from heart tamponade. Syphilitic aneurysm is not likely to lead to' a dissecting aneurysm because of the inflammatory changes in the media and adventitia. It often compresses the trachea, left bronchus, esophagus, pleura, or lung. It may rupture into any adjacent structures. DYSPHAGIA AND LEFTHEMOTHORAX
I have in the past few months seen three cases of aneurysm of the arch with co~pression of the esophagus and marked dys-
Fig. 13.
Fig. 14.
Fig. H.-Aneurysm of the arch of the aorta with marked compression and displacement of the trachea and esophagus. The patient had marked dyspnea, an asthmatoid wheeze, cyanosis and dysphagia. He could not even swallow liquids. There was no aortic regurgitation, hence heart is normal in size. The diaguosis was made clinically from the history and physical examination. Fig. 14.-Same patient as Figure 13 with barium in esophagus. Note the marked displacement of the esophagus to the right with a complete occlusion. The patient died three weeks later of a perforation of the aneurysm into the esophagus (autopsy).
phagia (Figs. 13, 14, 15, 20). In two of the cases swallowing became impossible. .One patient died of perforation into the esophagus, another of tracheal compression (Figs. 14, 15).
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We have had five cases of aneurysm of the descending aorta with dysphagia. In three there was also erosion of the spine. Four years ago I had under observation a patient with a syphilitic aneurysm of the right subclavian artery with marked erosion of the right clavicle, and clubbed fingers of the right hand (Fig. 25). We have had five cases of aneurysm of the arch and descending aorta with left hemothorax. In one there was an accompany-
Fig. 15.-Aneurysm of the arch of the aorta with marked dysphagia. Note the marked dilatation of the aortic arch, with an anel,lrysm of the descending portion pointing to the right and occluding the esophagus. There was no aortic regurgitation. The diagnosis was made clinically from the history of dysphagia, a tracheal tug, and visible pulsation in the left sternoclavicular region. The case is quite similar to that in Figures 13 and 14. Both were in my ward at the same time.
ing atelectasis of the left lung from occlusion of the left bronchus (Figs. 22, 26, 27). I have for several years recognized this as a definite clinical entity. One interesting case of aneurysm involved the left sinus of Valsalva. There was an aortic regurgitation; The film revealed a prominent pulmonic knob due to displacement of the pulmonary artery by the aneurysm (Fig. 24). The diagnosis was confirmed at autopsy. In such cases there is usually an aortic
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Fig. 16. Fig. 17. Figs. 16 and 17.-Syphili.tic aortitis with aortic regurgitation and huge aneurysm of the lower thoracic aorta. The aneurysm eroded the ribs posteriorly on 'the left side producing a large pulsating mass. It dissected the diaphragm. Note the erosion and sclerosis of the tenth. eleventh and twelfth dorsal vertebrae with destruction of the attachment of the eleventh and twelfth ribs. The .twelfth rib is. displaced downward.
Fig. 19. Fig. 18. Fig. lS.-Large calcified aneurysm of the descending arch of the aorta. Npte the widened and calcified descending thoracic aorta just below the large sac. Fig. 19.-Aneurysm of the innominate artery. associated with early luetic aortic regurgitation and slight cardiac eulargement. The diagnosis was made from the physical findings. There was a pulsating mass ih the right supraclavicular fossa. a tracheal tug. and marked dyspnea.
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regurgitation. The pulmonary artery, interventricular septum, right or left atrium may be displaced or compressed. Another case of aneurysm of the abdominal aorta led to a . very marked hypertension. The aneurysm, in a colored man aged twenty-one, was produced by a gumma of the media. The hypertension was most likely due to partial occlusion of one renal artery by the aneurysm. SYMPTOMS OF ANEURYSM
The most common symptom of aneurysm is pain. This is usually due to erosion of bony structures, or pressure on rierve trunks (Figs. 17, 21, 25). When a stenosis of the coronary ostia develops, angina pectoris also occurs. I have often emphasized in clinics that there are two important causes of persistent thoracic
Fig. 20. Fig. 21. Fig. 20.-Large saccular aneurysm of the descending thoracic aorta with backache and dysphagia. Note the dilated aorta' below the sac, visible through the heart shadow. There was no aortic regurgitation, hence no cardiac enlargement. . Fig.. 21.-Same patient as Figure 20, lateral view. Note the large aLleurysmal sac, and the definite erosion of the seventh and eighth thoracic vertebrae.
pain in adults, namely pulmonary neoplasm and aneurysm. In many cases the diagnosis of aneurysm is made by mere inspection of the anterior and posterior chest wall. There may .be a visible pulsation in the painful area in the sternal, parasternal, or left paravertebral regions. This plus a tracheal tug or difference
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in the radial pulses, or an ·aortic regurgitation, makes the diagnosis very easy. Yet the roentgenographic findings based only on a film may be very confusing, especially with atelectasis of an entire lung or a hemorrhagic pleuritis, or unsharp outline of the aneurysm due to infiltration of adjacent structures with blood (Figs. 22, 26). Dy spnea due to tracheal or bronchial compression is the next most common symptom. Not only is the trachea or bronchus displaced or compressed, but the cartilages may be destroyed with collapse and death from asphyxia. Oit"en the patient assumes
Fig. 22. Fig. 23. Fig. 22.-Large aneurysm of the entire a~ch of the aorta with left hemothorax. Aspiration yielded bloody fluid. A diagnosis of slow bleeding into the pleura was made clinically and confirmed (autopsy). Fig. 23.-Large saccular aneurysm of the ascending aorta, with diffuse dilatation and some atheromatosis of the arch and descending thoracic aorta. There was no aortic regurgitation, hence the heart was not enlarged.
an odd position in which he gets some relief. Recently I had a patient with an aneurysm of the arch who was more comforable on his stomach in an opisthotonos position, which he occupied most of the time. Others lie on the left side, or assume. a knee-chest position. Cougb is the third most Jrequent symptom, often accompanied by hoarseness. The cough is persistent, leading to marked cyanosis. I have found dyspbagia much more common than textbooks and articles on aortitis indicate, Hoarseness due to involvement of the left recurrent laryngeal nerve often occurs with large -aneurysms of the aortic arch.
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Fig. 24. Fig. 25. Fig. 24.-Syphilitic aortitis with aortic regurgitation and aneurysm of. the left sinus of, Valsalva. Note the prominent pulmonary artery, displaced by the aneurysm (autopsy). Fig. 25.-Aneurysm of the right axillary artery due' to · syphilitic arteritis. Note the circular erosion of the clavicle. There was marked clubbing of the fingers of the right hand. Operation was performed with ligation of the artery.
Fig. 26. Fig. 27. Fig. 26...c..Large aneurysm of the aortic arch with compression of the left bronchus, atelectasis of the left lung, and left hemothorax. Fig. 27.-Findingsafter aspiration of the left chest. Note the hlrge aneurysm of the arch and the atelectatic left lung.
A roentgenographic study consisting of careful fluoroscopy and films in various positions is of the greatest value. The ·pres-
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ence of dilatation of other parts of the aorta may be decisive in a differential diagnosis (Fig. 20). Aneurysm of the innownate artery may be diagnosed by finding the throbbing in the right supraclavicular fossa, a palpable pulsating mass, a thrill, often a diminished right radial pulse, pressure upon the trachea, and a tracheal tug (Fig. 19). Aneurysm of the abdominal aorta will be diagnosed more frequently if the abdomen is carefully palpated in all luetics, especially those with thoracic aneurysm. Although some may be arteriosclerotic, the majority of abdominal aneurysms are due to syphilis. Abdominal pain, a pulsating expansile mass, and roent'genographic evidence when accompanied by atheromatosis make the diagnosis easy in most cases. The aneurysm may lead to hypertension by causing renal ischemia, may erode the spine, compress or split the diaphragm, lead to embolism of the mesenteric artery, or may rupture. DIAGNOSIS OF SYPHILITIC AORTITIS
In luetic aortitis the diagnosis is of greatest value to the patient in the early uncomplicated stage, before aortic regurgitation, aneurysm, or angina pectoris has developed. I shall, therefore, stress the signs which I have found most useful in the diagnosis of uncomplicated aortitis. In the first few ,years after the primary infection a clinical diagnosis of syphilitic aortitis is usually not possible, although histologic changes may already be present. The earliest and most important diagnostic sign is a widening of the ascending aorta, or any other part of the aorta, on fiuoroscopic exannnation. An examination of hundreds of normal as well as syphilitic aortas will give the observer a good idea of the normal size of the aorta in persons of various size and weight. The entire contour of the ascending, arch, and part of the descending aorta can be very quickly observed by the trained examiner. Unfortunately we are unable to visualize the first inch or more of the aorta, including the aortic ring, where syphilis does its greatest harm . . A convex prominence of the ascending aorta, often with increased pulsation in this area, is quite characteristic (Fig. 12). An ascending aorta more than 3 to 4 cm. in diameter in an adult should be suspected of being luetic. This is especially true when t~e patient h;tS it. positive serologic test, Argyll~RobertsOn pupils,
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absent patellar reflexes, tabes or paresis. Unfortunately, only about 75 per cent of patients with cardiovascular syphilis have a positive Wassennann or Kahn test, a negative test thus occuring in about one fourth of the cases. In the right oblique position the ascending aorta can be measured quite accurately. Its increased diameter, convex prominence to the right, and increased density proportional to its dilatation make the diagnosis. The aortic knob on the left is often enlarged and extends farther upward and to the left. Giving barium paste enables one to measure the descending portion of the arch, from the concavity in the esophagus wall to the left border (Kreuzfuch's method). The normal symmetry is often disturbed, and the arch becomes asymmetrical. It must be .remembered that aortitis and atherosclerosis are frequently combined. It is never safe to assume a marked dilatation as due to atheromatosis or hypertension. A definite aneurysm of the ascending aorta or arch is practically always luetic. The patient should be rotated in the left as well as right oblique position. Any departure frot,n the cylindrical form is significant. , The second most important sign in aortitis without regurgitation is the increased and widened manubrial dulness on percussion, especially to the right. There may be a slight pulsation in the second or third right interspace on deep exhalation, or the second or third left interspace. The third most frequent finding is the tambour or bell-like, ringing, aortic second sound first described by Potain. This peculiar musical quality may be due to the increased tension on the aortic cusps produced by stretching of the ring. The heart sounds are often unusually well transmitted because of the thinning of the lung by the expanding aorta. The aorta lies in con, tact with the chest wall. The tambour second sound is heard in a small area, usually to the right of the sternum. To the trained ear the tambour second sound is diagnostic of uncomplicated syphilitic aortitis. It may be present before there is any demonstrable widening of the aorta, thus being the earliest sign of aortitis. A fourth frequent finding is a systolic murmur at the base found in about two thirds of the cases of uncomplicated aortitis: and in practically all the cases of aortic regurgitation accompanying the diastolic murmur. The murmur is often harsh and
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heard best in the second right or left intercostal space near the sternum. It must be remembered that a systolic murmur at the base is a frequent finding in patients past middle life, due to atheromatosis or sclerosis of the cusps in the absence of aortic syphilis. Therefore, a systolic 'murmur alone is not diagnostic of aortitis. I believe that the systolic murmur in syphilitic aortitis is more likely due to the stretching of the aortic ri~g with increased tension upon the cusps in systole. This prevents them from approxiplating the wall of the aorta. The projection of the cusps under tension produces the systolic murmur. It persists after separation of the cusps, hence the double murmur of aortic regurgitation. . A fifth sign in the diagnosis of syphilitic aortitis is the pulsus differens, or even total absence of pulsation in one or more of the main branches of the aorta (innominate, left carotid, left subclavian). When the left carotid and subclavian pulse, or only the subclavian, is weaker than the right an isthmus stenosis 'must be considered. This is usually not difficult to diagnose, especially when the blood pressure in the lower extremity is low and the ribs reveal the characteristic erosions. Inequality of the radial pulses is common in aortitis. All three ostia of the main arteries may be involved, as in one of my cases. There was no pulsation in' either arm or the right carotid. There was a systolic murmur and thrill over the left carotid. Our diagnosis was a luetic aortitis with occlusion of the ostium of the innominate and left subclavian arteries, and stenosis of the ostium of the left carotid. This was confirmed at autopsy. There was also an anomalous vertebral artery coming from the aortic arch. ' A sixth sign of syphilitic aortitis is the presence of an aneurysm of any part of the thoracic aorta. We have seen a number of aneurysms of the innominate artery. Angina pectoris is quite frequent in luetic aortitis. It is usually of a severe type and progressive. The pain is due to the stenosis or occlusion of the coronary ostia. Another factor is the lowering of the diastolic blood pressure when aortic regurgitation deyelops. A certain number of the patients have hypertension with cardiac hypertrophy. The increase in the volume of the heart muscle causes a still greater demand for oxygen at the. same time that the blood supply is being slowly reduced. Another sign of value is the presence of a suprasternal pulsa-
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tion, due to dilatation and elongation of the aortic arch. At times the dilated aortic arch may be palpable in the episternal region. DIAGNOSIS OF AORTIC REGURGITATION
Aortic regurgitation, the only valvular disease caused by syphilis, except for the rare pulmonary regurgitation, is seen most often in the fifth and sixth decades. Occasionally we find it before the age of thirty-five years. The diagnosis of aortic insufficiency is usually an easy matter. First and most characteristic is the diastolic rmlrrmlr at the base. In the early stage it may be missed unless one listens carefully during forced exhalation with the patient leaning forward. It may be heard in the aortic area, in the second or third right or left interspace near the sternum. As the disease progresses and the aorta dilates, the latter lies close to the chest wall to the right of the sternum, hence the murmur in advanced cases is often loudest in the second and third right interspaces. There is usually an accompanying systolic m:urnrur, which I believe is best explained as due to tension on the cusps so that they cannot approximate the aortic wall during the systole. Later there is a disappearance of the aortic second sound and only the two murmurs are heard. The pulse pressure increases, and the heart enlarges especially to the left. In the majority of cases the lesion can be suspected from the elevated systolic and low'ered diastolic pressures. There are few other causes of increased pulse pressure, namely, thyrotoxicosis, senile arteriosclerosis, and total heart block. Frequently the diagnosis can be made at a distance by noting the hopping carotid arteries with synchronous vibration of the head. The Corrigan pulse and Duroziez sign on compression of the femoral or other large artery are quite characteristic. A capillary pulse is often present, but not diagnostic as we see it in other conditions. In the past twenty years I have seen three cases of luetic aortic regurgitation with an unusually loud musical diastolic murmur asso.ciated with a diastolic thrill. The thrill was most marked in the second or third right intercostal space. I have offered as one explanation for this unusual murmur and thrill the presence of a fenestrum of one or more of the aortic cusps.
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Differential Diagnosis between Luetic and Rheumatic Regurgitation
The differential diagnosis between luetic and rheumatic aortic regurgitation may be difficult in exceptional cases. The presence of an associated organic mitral lesion speaks for rheumatic endocarditis. The same is true when an aortic stenosis is present. An, aortic regurgitation, therefore, in the absence of any other organic valvular disease in an adult, is most likely luetic. The presence of aortic dilatation or aneurysm makes the diagnosis quite certain, even in the 25 per cent with a negative Wassermann or Kahn test. I have seen three cases of combined rheumatic and luetic disease in which the diagnosis of both types of lesion was possible. In two there was a definite stenosis of the aortic ostium, which is never luetic, associated with an aortic aneurysm. In one there was an old rheumatic mitral stenosis and regurgitation, a history of chancre twenty-five years ago, and a luetic aortic regurgitation with an aneurysm of the ascending aorta. In rare cases a bacterial endocarditis may be engrafted on a syphilitic aortic valve. In the absence of a widening of the aorta, of a positive serologic test or other evidence of syphilis, or of other valvular disease, it would be impossible to say whether the lesion is luetic or rheumatic. To state that syphilitic and rheumatic or syphilitic and subacute bacterial disease do not occasionally occur in the same patient is incorrect. I have seen such combinations several times both clinically and at necropsy. CONCLUSION
In conclusion, I wish to emphasize the importance of a periodic careful physical examination and roentgenographic study, for the presence of luetic aortitis, of every patient with evidence of syphilis. The diagnosis of uncomplicated aortitis is possible in a considerable percentage of all cases. The most valuable aids are (1) careful fluroscopic examination, (2) tambour aortic second sound, (3) increased manubrial dulness especially in the second and third intercostal space, (4) visible pulsation in the second or third intercostal spaces or suprasternal, (5) difference in the radial or carotid pulses, (6) angina pectoris in a young adult in the absence of other cause. An early diagnosis followed by intensive antiluetic therapy will prevent most cases of aortic regurgitation and aneurysm.
SYPHILITIC heart disease is today the only fonn of heart disease which is preventable. A more general recognition of this fact will lead to a careful search of all patients infected with syphilis for the early manifestations of this most fatal fonn of the disease. A diagnosis in the primary seronegative stage by darkfield examination, followed by two years of proper treatment, would prevent at least 90 per cent of cases of cardiovascular syphilis. I wish in this clinic to discuss the pathology and diagnosis of syphilitic aortitis. Syphilis is responsible for about 20 per cent of cases of chronic cardiac disease found in adults. About onefifth of all persons who acquire syphilis develop cardiovascular disease. The average length of time from infection to the onset of symptoms is about fifteen years. In luetic aortic regurgitation the average latent period is twenty years, in aneurysm twentytwo years. At necropsy we find that 75 per cent of persons with visceral lues have an aortitis. It is therefore obvious that many of these individuals do not develop clinical evidence of heart disease, and are not diagnosed during life. We shall emphasize the methods at our disposal for the early diagnosis of uncomplicated syphilitic aortitis. SEX AND RACE INCIDENCE
There is a marked preponderance of the male sex in the entire picture of syphilis of the heart and aorta. About 80 per cent of the patients are males, yet there is no marked difference in the frequency of luetic aortitis in the two sexes. In females the disease takes a more benign course. The severe supravalvular fonns .. From the Department of Medicine, Cook County Hospital. of Medicine and Chairman of Department of Medicine, Cook County Graduate School; Associate Professor of Medicine (Rush), University of Illinois College of Medicine; Attending Physician, Cook County and Mt. Sinai Hospitals; Staff, Wesley Memorial Hospital.
t Professor
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with aortic regurgitation, stenosis of the coronary ostia, and aneurysm are much more common in men. Syphilitic heart disease is about four times as frequent in the Negro as in the white. Aneurysms are twice as common as in the white race. The greater number of hard laborers, high incidence of hypertension, and more frequent neglect of proper therapy may explain this difference. I believe that there is a more marked involvement of the media with more extensive necrosis in the colored race. While aneurysm is rare in white women we find it not infrequently in Negro women. PATHOLOGY OF SYPHILITIC AORTITIS
Syphilitic aortitis is the basic lesion from which the manifestations of cardiovascular syphilis develop. It leads to aneurysm formation, widening of the aortic ring with separation of the cusps at the commissures and aortic regurgitation, and stenosis of one or both coronary ostia. Luetic infection usually occurs in the twenties, yet the highest incidence of symptoms due to aortitis is near the forty-fifth year. The reason for this long latent period of twenty years is the very slow progress of the chronic inflammatory process which begins in the vasa vasorum of the adventitia and media. These small vessels undergo obliterative changes which furnish partial anaerobic conditions for the growth of the spirochetes in the media. There is an infiltration by round and plasma cells. The elastic fibers are destroyed by the development of miliary gummas. The destructive and inflammatory changes in the media lead to intimal wrinkling and puckering. The inner surface of the aorta becomes wrinkled and depressed by small and large fibrous scars of pinkish or white color. Atheromatous changes are often combined with the luetic aortitis in an amount increasing with age. The mouths of the large branches of the aorta, such as the innominate, carotid, and subclavian, are often fibrosed and greatly constricted or almost occluded. This stenosis of the ostia of the large arteries is of great value in diagnosis, leading to pulse differences, thrills and murmurs along the course of the vessels. Sometimes there is a complete absence of the pulse in one or more large arteries. ANEURYSM FORMATION
As the aortic wall (in some cases also the innominate, carotid, subclavian or other large artery) loses its elastic layer the wall
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becomes weakened. It stretches under the influence of the blood pressure. If the mesaortitis is widespread there may be a diffuse dilatation of the ascending, arch, descending or entire thoracic aorta. When the destruction is limited to a small area a saccular aneurysm may develop. Such aneurysms of the thoracic aorta are practically all luetic, whether atheromatosis be present or absent. Multiple aneurysms of the thoracic aorta are quite common. Aneurysms 'occur in about 20 per cent of cases of syphilitic aortitis. They are most common in the ascending aorta and arch, and decrease in frequency toward the bifurcation. They tend to occur along the path which receives the impact of the blood column. This pathway runs in the middle of the anterior wall of the supravalvular portion, then to the right in the ascending, horizontally in the arch, and along the posterior left aspect of the descending arch, and in the middle of the posterior wall of the descending aorta. They may erode the spine, ribs or sternum. ,. We shall see that the demonstration of a widening of any part of the thoracic aorta is the earliest diagnostic sign of syphilitic aortitis. Such a widening can often be found before the development of aortic regurgitation, aneurysm, or angina pectoris. Syphilitic aortitis without dilatation of the aorta, regurgitation, or coronary stenosis cannot be diagnosed clinically. When the orthodiagraphic measurement of the aorta exceeds the normal values, syphilitic aortitis must be suspected. This is especially so when the patient has a positive serologic test, Argyll-Robertson pupils, absent patellar reflexes, or any other evidence of lues. Hypertension, rheumatic aortic regurgitation, isthmus stenosis, or thyrotoxicosis may lead to increased cardiac force or output with moderate widening of the aorta. With old age the aorta elongates, the arch rises and the vessel becomes more tortuous. There is evidence of atheromatosis, and the process is quite uniform. These conditions can usually be distinguished from luetic aortitis. Aneurysm may be caused by bacterial emboli in bacterial endocarditis. It is very rare in rheumatic fever. Periarteritis nodosa, suppuration, tuberculosis and trauma are rare causes. Aortic rupture or dissecting aneurysm is usually the result of cystic media necrosis, first described by Erdheim. Abdominal aneurysm may be due to arteriosclerosis.
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AORTIC REGU RGITATlON
Perhaps the most important pathologic change is aortic regurgitation. This is found in about 75 per cent of the cases diagnosed clinically, and in about 30 per cent of cases of luetic aortitis at autopsy. Syphilis has a special affinity for the sinuses of Valsalva and the aortic ring. The increased strain Upon this region caused by the closure of the aortic cusps in diastole is a factor in producing the stretching of the ring. The cusps become thickened at the commissures and are separated from each other. No vegetations are produced, hence there is never a stenosis of the aortic ostium. The ring is stretched, the cusps are too small to close the lumen in diastole and regurgitation results. There is later a regurgitation at each of the commissures, and in the center of the lumen. Syphilitic aortitis, therefore, causes only one valvulttr lesion, aortic regurgitation, never a stenosis. Rarely, syphilitic pulmonary arteritis causes a pulmonary regurgitation. The mitral and tricuspid valves are not affected. With regurgitation there is a progressive dilatation and hypertrophy of the left ventricle, often leading to great cardiac enlargement. The average length of life is about three years. STENOSIS OF THE CORONARY OSTIA
Another important change is stenosis of the ostia of the coronary arteries. This is a very frequent finding, a part of the aortitis in the sinuses of Valsalva. It leads to anoxemia of the heart muscle with angina pectoris. The anginal attacks are usually of long duration. The T wave is often negative in Leads I and 11 of the electrocardiogram. Myocardial failure which reacts poorly to all medication is the end result. The stenosis is so gradual that one ostium may be totally occluded without producing clinical symptoms or any gross myocardial changes. During the years that elapse there is plenty of time for the collaterals to enlarge so that the heart muscle gets its nourishment from the other coronary. When both coronaries are affected the heart may be unable to undergo much hypertrophy. The heart muscle may still receive some blood supply from the thebesian channels, or from anastomoses between the coronaries and vasa vasorum or bronchial arteries. Sometimes the coronary ostium is covered by a small flaplike thickening of the intima. The presence of an aortic regurgita-
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tion reduces the blood flow in the coronaries still further by lowering the diastolic blood pressure. Sudden death is not uncommon in such cases of aortic regurgitation with stenosis of the coronary ostia. In very rare cases a supernumerary vessel may furnish blood to the myocardium. It often appears as if the stenosis of the coronary ostia protects them against atheromatosis by reducing the blood flow and pressure in these vessels. The arteries themselves are not affected in syphilis, hence coronary thrombosis with infarction
Fig. l2.-Early syphilitic aortitis, with characteristic. convex prominence of the ascending aorta. There was a typical tambour aortic second sound, heard best in the second right interspace. There was no regurgitation, hence the heart is normal in size. The· Kahn test was positive.
is uncommon. When it does occur it is usually the result of sclerosis or subintimal hemorrhage. The heart muscle is only rarely affected by syphilis. Seldom is a gumma or diffuse syphilitic mY9carditis found. In the bundle of His it may lead to heart block. In a papillary muscle rupture, may occur. The fibrous changes found in older luetic individuals are more readily explained as a result of arteriosclerotic nonluetic coronary disease, or ischemia of the muscle from the stenosis of the ostia.
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It must be emphasized here that aneurysm alone does not cause cardiac enlargement, regardless of its size. In the absence of aortic .regurgitation, coronary disease, hypertension or other cause, the heart remains normal (Figs. 13, 15, 20). Aneurysms of the supravalvular portion of the aorta may rupture into the pericardial sac, causing death from heart tamponade. Syphilitic aneurysm is not likely to lead to' a dissecting aneurysm because of the inflammatory changes in the media and adventitia. It often compresses the trachea, left bronchus, esophagus, pleura, or lung. It may rupture into any adjacent structures. DYSPHAGIA AND LEFTHEMOTHORAX
I have in the past few months seen three cases of aneurysm of the arch with co~pression of the esophagus and marked dys-
Fig. 13.
Fig. 14.
Fig. H.-Aneurysm of the arch of the aorta with marked compression and displacement of the trachea and esophagus. The patient had marked dyspnea, an asthmatoid wheeze, cyanosis and dysphagia. He could not even swallow liquids. There was no aortic regurgitation, hence heart is normal in size. The diaguosis was made clinically from the history and physical examination. Fig. 14.-Same patient as Figure 13 with barium in esophagus. Note the marked displacement of the esophagus to the right with a complete occlusion. The patient died three weeks later of a perforation of the aneurysm into the esophagus (autopsy).
phagia (Figs. 13, 14, 15, 20). In two of the cases swallowing became impossible. .One patient died of perforation into the esophagus, another of tracheal compression (Figs. 14, 15).
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We have had five cases of aneurysm of the descending aorta with dysphagia. In three there was also erosion of the spine. Four years ago I had under observation a patient with a syphilitic aneurysm of the right subclavian artery with marked erosion of the right clavicle, and clubbed fingers of the right hand (Fig. 25). We have had five cases of aneurysm of the arch and descending aorta with left hemothorax. In one there was an accompany-
Fig. 15.-Aneurysm of the arch of the aorta with marked dysphagia. Note the marked dilatation of the aortic arch, with an anel,lrysm of the descending portion pointing to the right and occluding the esophagus. There was no aortic regurgitation. The diagnosis was made clinically from the history of dysphagia, a tracheal tug, and visible pulsation in the left sternoclavicular region. The case is quite similar to that in Figures 13 and 14. Both were in my ward at the same time.
ing atelectasis of the left lung from occlusion of the left bronchus (Figs. 22, 26, 27). I have for several years recognized this as a definite clinical entity. One interesting case of aneurysm involved the left sinus of Valsalva. There was an aortic regurgitation; The film revealed a prominent pulmonic knob due to displacement of the pulmonary artery by the aneurysm (Fig. 24). The diagnosis was confirmed at autopsy. In such cases there is usually an aortic
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Fig. 16. Fig. 17. Figs. 16 and 17.-Syphili.tic aortitis with aortic regurgitation and huge aneurysm of the lower thoracic aorta. The aneurysm eroded the ribs posteriorly on 'the left side producing a large pulsating mass. It dissected the diaphragm. Note the erosion and sclerosis of the tenth. eleventh and twelfth dorsal vertebrae with destruction of the attachment of the eleventh and twelfth ribs. The .twelfth rib is. displaced downward.
Fig. 19. Fig. 18. Fig. lS.-Large calcified aneurysm of the descending arch of the aorta. Npte the widened and calcified descending thoracic aorta just below the large sac. Fig. 19.-Aneurysm of the innominate artery. associated with early luetic aortic regurgitation and slight cardiac eulargement. The diagnosis was made from the physical findings. There was a pulsating mass ih the right supraclavicular fossa. a tracheal tug. and marked dyspnea.
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regurgitation. The pulmonary artery, interventricular septum, right or left atrium may be displaced or compressed. Another case of aneurysm of the abdominal aorta led to a . very marked hypertension. The aneurysm, in a colored man aged twenty-one, was produced by a gumma of the media. The hypertension was most likely due to partial occlusion of one renal artery by the aneurysm. SYMPTOMS OF ANEURYSM
The most common symptom of aneurysm is pain. This is usually due to erosion of bony structures, or pressure on rierve trunks (Figs. 17, 21, 25). When a stenosis of the coronary ostia develops, angina pectoris also occurs. I have often emphasized in clinics that there are two important causes of persistent thoracic
Fig. 20. Fig. 21. Fig. 20.-Large saccular aneurysm of the descending thoracic aorta with backache and dysphagia. Note the dilated aorta' below the sac, visible through the heart shadow. There was no aortic regurgitation, hence no cardiac enlargement. . Fig.. 21.-Same patient as Figure 20, lateral view. Note the large aLleurysmal sac, and the definite erosion of the seventh and eighth thoracic vertebrae.
pain in adults, namely pulmonary neoplasm and aneurysm. In many cases the diagnosis of aneurysm is made by mere inspection of the anterior and posterior chest wall. There may .be a visible pulsation in the painful area in the sternal, parasternal, or left paravertebral regions. This plus a tracheal tug or difference
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79
in the radial pulses, or an ·aortic regurgitation, makes the diagnosis very easy. Yet the roentgenographic findings based only on a film may be very confusing, especially with atelectasis of an entire lung or a hemorrhagic pleuritis, or unsharp outline of the aneurysm due to infiltration of adjacent structures with blood (Figs. 22, 26). Dy spnea due to tracheal or bronchial compression is the next most common symptom. Not only is the trachea or bronchus displaced or compressed, but the cartilages may be destroyed with collapse and death from asphyxia. Oit"en the patient assumes
Fig. 22. Fig. 23. Fig. 22.-Large aneurysm of the entire a~ch of the aorta with left hemothorax. Aspiration yielded bloody fluid. A diagnosis of slow bleeding into the pleura was made clinically and confirmed (autopsy). Fig. 23.-Large saccular aneurysm of the ascending aorta, with diffuse dilatation and some atheromatosis of the arch and descending thoracic aorta. There was no aortic regurgitation, hence the heart was not enlarged.
an odd position in which he gets some relief. Recently I had a patient with an aneurysm of the arch who was more comforable on his stomach in an opisthotonos position, which he occupied most of the time. Others lie on the left side, or assume. a knee-chest position. Cougb is the third most Jrequent symptom, often accompanied by hoarseness. The cough is persistent, leading to marked cyanosis. I have found dyspbagia much more common than textbooks and articles on aortitis indicate, Hoarseness due to involvement of the left recurrent laryngeal nerve often occurs with large -aneurysms of the aortic arch.
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Fig. 24. Fig. 25. Fig. 24.-Syphilitic aortitis with aortic regurgitation and aneurysm of. the left sinus of, Valsalva. Note the prominent pulmonary artery, displaced by the aneurysm (autopsy). Fig. 25.-Aneurysm of the right axillary artery due' to · syphilitic arteritis. Note the circular erosion of the clavicle. There was marked clubbing of the fingers of the right hand. Operation was performed with ligation of the artery.
Fig. 26. Fig. 27. Fig. 26...c..Large aneurysm of the aortic arch with compression of the left bronchus, atelectasis of the left lung, and left hemothorax. Fig. 27.-Findingsafter aspiration of the left chest. Note the hlrge aneurysm of the arch and the atelectatic left lung.
A roentgenographic study consisting of careful fluoroscopy and films in various positions is of the greatest value. The ·pres-
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ence of dilatation of other parts of the aorta may be decisive in a differential diagnosis (Fig. 20). Aneurysm of the innownate artery may be diagnosed by finding the throbbing in the right supraclavicular fossa, a palpable pulsating mass, a thrill, often a diminished right radial pulse, pressure upon the trachea, and a tracheal tug (Fig. 19). Aneurysm of the abdominal aorta will be diagnosed more frequently if the abdomen is carefully palpated in all luetics, especially those with thoracic aneurysm. Although some may be arteriosclerotic, the majority of abdominal aneurysms are due to syphilis. Abdominal pain, a pulsating expansile mass, and roent'genographic evidence when accompanied by atheromatosis make the diagnosis easy in most cases. The aneurysm may lead to hypertension by causing renal ischemia, may erode the spine, compress or split the diaphragm, lead to embolism of the mesenteric artery, or may rupture. DIAGNOSIS OF SYPHILITIC AORTITIS
In luetic aortitis the diagnosis is of greatest value to the patient in the early uncomplicated stage, before aortic regurgitation, aneurysm, or angina pectoris has developed. I shall, therefore, stress the signs which I have found most useful in the diagnosis of uncomplicated aortitis. In the first few ,years after the primary infection a clinical diagnosis of syphilitic aortitis is usually not possible, although histologic changes may already be present. The earliest and most important diagnostic sign is a widening of the ascending aorta, or any other part of the aorta, on fiuoroscopic exannnation. An examination of hundreds of normal as well as syphilitic aortas will give the observer a good idea of the normal size of the aorta in persons of various size and weight. The entire contour of the ascending, arch, and part of the descending aorta can be very quickly observed by the trained examiner. Unfortunately we are unable to visualize the first inch or more of the aorta, including the aortic ring, where syphilis does its greatest harm . . A convex prominence of the ascending aorta, often with increased pulsation in this area, is quite characteristic (Fig. 12). An ascending aorta more than 3 to 4 cm. in diameter in an adult should be suspected of being luetic. This is especially true when t~e patient h;tS it. positive serologic test, Argyll~RobertsOn pupils,
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absent patellar reflexes, tabes or paresis. Unfortunately, only about 75 per cent of patients with cardiovascular syphilis have a positive Wassennann or Kahn test, a negative test thus occuring in about one fourth of the cases. In the right oblique position the ascending aorta can be measured quite accurately. Its increased diameter, convex prominence to the right, and increased density proportional to its dilatation make the diagnosis. The aortic knob on the left is often enlarged and extends farther upward and to the left. Giving barium paste enables one to measure the descending portion of the arch, from the concavity in the esophagus wall to the left border (Kreuzfuch's method). The normal symmetry is often disturbed, and the arch becomes asymmetrical. It must be .remembered that aortitis and atherosclerosis are frequently combined. It is never safe to assume a marked dilatation as due to atheromatosis or hypertension. A definite aneurysm of the ascending aorta or arch is practically always luetic. The patient should be rotated in the left as well as right oblique position. Any departure frot,n the cylindrical form is significant. , The second most important sign in aortitis without regurgitation is the increased and widened manubrial dulness on percussion, especially to the right. There may be a slight pulsation in the second or third right interspace on deep exhalation, or the second or third left interspace. The third most frequent finding is the tambour or bell-like, ringing, aortic second sound first described by Potain. This peculiar musical quality may be due to the increased tension on the aortic cusps produced by stretching of the ring. The heart sounds are often unusually well transmitted because of the thinning of the lung by the expanding aorta. The aorta lies in con, tact with the chest wall. The tambour second sound is heard in a small area, usually to the right of the sternum. To the trained ear the tambour second sound is diagnostic of uncomplicated syphilitic aortitis. It may be present before there is any demonstrable widening of the aorta, thus being the earliest sign of aortitis. A fourth frequent finding is a systolic murmur at the base found in about two thirds of the cases of uncomplicated aortitis: and in practically all the cases of aortic regurgitation accompanying the diastolic murmur. The murmur is often harsh and
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83
heard best in the second right or left intercostal space near the sternum. It must be remembered that a systolic murmur at the base is a frequent finding in patients past middle life, due to atheromatosis or sclerosis of the cusps in the absence of aortic syphilis. Therefore, a systolic 'murmur alone is not diagnostic of aortitis. I believe that the systolic murmur in syphilitic aortitis is more likely due to the stretching of the aortic ri~g with increased tension upon the cusps in systole. This prevents them from approxiplating the wall of the aorta. The projection of the cusps under tension produces the systolic murmur. It persists after separation of the cusps, hence the double murmur of aortic regurgitation. . A fifth sign in the diagnosis of syphilitic aortitis is the pulsus differens, or even total absence of pulsation in one or more of the main branches of the aorta (innominate, left carotid, left subclavian). When the left carotid and subclavian pulse, or only the subclavian, is weaker than the right an isthmus stenosis 'must be considered. This is usually not difficult to diagnose, especially when the blood pressure in the lower extremity is low and the ribs reveal the characteristic erosions. Inequality of the radial pulses is common in aortitis. All three ostia of the main arteries may be involved, as in one of my cases. There was no pulsation in' either arm or the right carotid. There was a systolic murmur and thrill over the left carotid. Our diagnosis was a luetic aortitis with occlusion of the ostium of the innominate and left subclavian arteries, and stenosis of the ostium of the left carotid. This was confirmed at autopsy. There was also an anomalous vertebral artery coming from the aortic arch. ' A sixth sign of syphilitic aortitis is the presence of an aneurysm of any part of the thoracic aorta. We have seen a number of aneurysms of the innominate artery. Angina pectoris is quite frequent in luetic aortitis. It is usually of a severe type and progressive. The pain is due to the stenosis or occlusion of the coronary ostia. Another factor is the lowering of the diastolic blood pressure when aortic regurgitation deyelops. A certain number of the patients have hypertension with cardiac hypertrophy. The increase in the volume of the heart muscle causes a still greater demand for oxygen at the. same time that the blood supply is being slowly reduced. Another sign of value is the presence of a suprasternal pulsa-
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tion, due to dilatation and elongation of the aortic arch. At times the dilated aortic arch may be palpable in the episternal region. DIAGNOSIS OF AORTIC REGURGITATION
Aortic regurgitation, the only valvular disease caused by syphilis, except for the rare pulmonary regurgitation, is seen most often in the fifth and sixth decades. Occasionally we find it before the age of thirty-five years. The diagnosis of aortic insufficiency is usually an easy matter. First and most characteristic is the diastolic rmlrrmlr at the base. In the early stage it may be missed unless one listens carefully during forced exhalation with the patient leaning forward. It may be heard in the aortic area, in the second or third right or left interspace near the sternum. As the disease progresses and the aorta dilates, the latter lies close to the chest wall to the right of the sternum, hence the murmur in advanced cases is often loudest in the second and third right interspaces. There is usually an accompanying systolic m:urnrur, which I believe is best explained as due to tension on the cusps so that they cannot approximate the aortic wall during the systole. Later there is a disappearance of the aortic second sound and only the two murmurs are heard. The pulse pressure increases, and the heart enlarges especially to the left. In the majority of cases the lesion can be suspected from the elevated systolic and low'ered diastolic pressures. There are few other causes of increased pulse pressure, namely, thyrotoxicosis, senile arteriosclerosis, and total heart block. Frequently the diagnosis can be made at a distance by noting the hopping carotid arteries with synchronous vibration of the head. The Corrigan pulse and Duroziez sign on compression of the femoral or other large artery are quite characteristic. A capillary pulse is often present, but not diagnostic as we see it in other conditions. In the past twenty years I have seen three cases of luetic aortic regurgitation with an unusually loud musical diastolic murmur asso.ciated with a diastolic thrill. The thrill was most marked in the second or third right intercostal space. I have offered as one explanation for this unusual murmur and thrill the presence of a fenestrum of one or more of the aortic cusps.
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Differential Diagnosis between Luetic and Rheumatic Regurgitation
The differential diagnosis between luetic and rheumatic aortic regurgitation may be difficult in exceptional cases. The presence of an associated organic mitral lesion speaks for rheumatic endocarditis. The same is true when an aortic stenosis is present. An, aortic regurgitation, therefore, in the absence of any other organic valvular disease in an adult, is most likely luetic. The presence of aortic dilatation or aneurysm makes the diagnosis quite certain, even in the 25 per cent with a negative Wassermann or Kahn test. I have seen three cases of combined rheumatic and luetic disease in which the diagnosis of both types of lesion was possible. In two there was a definite stenosis of the aortic ostium, which is never luetic, associated with an aortic aneurysm. In one there was an old rheumatic mitral stenosis and regurgitation, a history of chancre twenty-five years ago, and a luetic aortic regurgitation with an aneurysm of the ascending aorta. In rare cases a bacterial endocarditis may be engrafted on a syphilitic aortic valve. In the absence of a widening of the aorta, of a positive serologic test or other evidence of syphilis, or of other valvular disease, it would be impossible to say whether the lesion is luetic or rheumatic. To state that syphilitic and rheumatic or syphilitic and subacute bacterial disease do not occasionally occur in the same patient is incorrect. I have seen such combinations several times both clinically and at necropsy. CONCLUSION
In conclusion, I wish to emphasize the importance of a periodic careful physical examination and roentgenographic study, for the presence of luetic aortitis, of every patient with evidence of syphilis. The diagnosis of uncomplicated aortitis is possible in a considerable percentage of all cases. The most valuable aids are (1) careful fluroscopic examination, (2) tambour aortic second sound, (3) increased manubrial dulness especially in the second and third intercostal space, (4) visible pulsation in the second or third intercostal spaces or suprasternal, (5) difference in the radial or carotid pulses, (6) angina pectoris in a young adult in the absence of other cause. An early diagnosis followed by intensive antiluetic therapy will prevent most cases of aortic regurgitation and aneurysm.