Syphilitic Aortitis

SYPHILITIC AORTITIS, a chronic diffuse inflammation of the aortic wall caused by the causative agent of syphilis, the pale spirochete. As a disease sui generis, it was described in 1878 by the Norwegian Heiberg, and in 1889 by Dohle and Heller. Syphilitic aortitis is the most common disease in the group of visceral syphilis. Etiology. The etiology of syphilitic aortitis was finally established in 1906 by the discovery of the causative agent in inflammatory foci. Penetrating either through the vasa vasorum or, possibly, directly from the lumen of the vessel, the pale spirochete settles in the muscular layer of the aorta. Other etiological factors, such as trauma and various intoxications, are only predisposing causes. Localization. The favored localization of the process is the ascending aorta (a. ascendens), especially its initial part (immediately above the valves), the inner surface of which takes on a very characteristic appearance in syphilitic aortitis (see article 137). The main changes develop in the middle layer of the vessel (hence the name "syphilitic mesoortitis"); in connection with the disruption of the integrity of the middle layer, there is a gradual stretching of the aortic lumen by the pressure of the blood, which can lead to the formation of an aortic aneurysm (see). The inflammatory process can spread from the aortic wall to the orifices of the large vessels branching from it; however, unlike atherosclerosis, in syphilitic aortitis the coronary vessels are not affected along their entire course, but their orifices are often subject to stenosis. Due to its localization in the initial part of the aorta, in syphilitic aortitis the fibrous ring to which the semilunar valves are attached is often stretched, the consequence of which can be their relative insufficiency. More often, the syphilitic process leads to organic insufficiency of the aortic valves, due to the transition of the specific inflammatory process to the semilunar valves of the aorta and their subsequent contraction. In contrast to the endocarditic process, syphilis leaves the free edges of the valves and their surface facing the aortic ring lumen untouched. Clinical picture. The clinical picture of syphilitic aortitis is diverse. In the simple uncomplicated form, the disease may proceed without symptoms. More often, patients complain of dull pains behind the sternum radiating to the shoulder girdle. Some authors call these pains aortalgia, explaining their origin by the involvement of the endings of sensitive nerves in the adventitia (periarteriitis Huchard's). From dull aching pains behind the sternum, which do not appear prognostically severe, one should distinguish typical attacks of angina pectoris, the basis of which usually lies in the aforementioned stenosis of the orifices of the coronary arteries. Less often in syphilitic aortitis there are attacks of cardiac asthma. Their pathogenesis is analogous to that of cardi sclerosis. Along with a diastolic murmur on the aorta depending on aortic insufficiency, one usually also finds a systolic murmur depending on the thickening and rigidity of the valves and the unevenness of the aortic wall itself. The second tone on the aorta is accentuated, often has a metallic tint, explained by the hardening of the aortic wall. On the pulse, in syphilitic aortitis the expression of p. celer is less pronounced, and the diastolic pressure is usually not as low as in aortic insufficiency of endocarditic origin. Clinically, syphilitic aortitis proceeds in the form of a slowly increasing cardiac insufficiency. The causes of the latter are insufficient nutrition of the cardiac muscle (due to stenosis of the coronary artery orifices), overstrain of the cardiac muscle due to valvular defect, and combined with syphilitic aortitis often occurring damage to the cardiac muscle. Finally, with the development of an aneurysm, the clinical picture depends on its localization and size, being conditioned by the mechanical conditions created. In all forms of syphilitic aortitis, sometimes there are increases in temperature of subfebrile type, often making differential diagnosis difficult. Associated syphilitic lesions of other organs. Among the accompanying syphilitic lesions of other organs, one should point out the relative frequency of lesions of the central nervous system. In practical diagnosis, the following rules are important. In the presence of typical angina pectoris phenomena in relatively young age (35-50 years), and in the absence of indications of syphilis in the anamnesis, one must perform a Wassermann reaction and a roentgen examination of the chest, specifically of the aorta. In every case of aortic insufficiency, starting from the age of thirty, one should bear in mind syphilitic aortitis and clarify its presence by the same methods. Almost every significant diffuse and every sac-like expansion of the aorta is the consequence of syphilitic aortitis. The diagnosis of syphilitic aortitis is based on the following data. Subjectively: pains behind the sternum, attacks of angina pectoris or cardiac asthma in relatively young age, progressive increase of cardiac weakness, shortness of breath, palpitations, edema, difficulty swallowing, aphonia, etc. Objectively: dullness on the sternum, metallic accent on the second tone of the aorta, the presence of signs of aortic insufficiency without acute rheumatic polyarthritis in the anamnesis. Often a single roentgenological examination allows, based on significant or uneven expansion of the aorta, even in the complete absence of other symptoms, to recognize the disease and that in relatively early stages. Differential diagnosis with endocarditis lenta and with atherosclerosis is sometimes difficult. For syphilitic aortitis, enlargement of the spleen, focal glomerulonephritis, anemia, Bittorf's symptom and the Endothelsymptom are not characteristic; these, on the contrary, speak for the presence of subacute endocarditis. On the other hand, the latter is not characterized by expansion of the aorta, positive Wassermann reaction. Atherosclerosis gives on the roentgen an elongation and intensification of the aortic shadow, whereas for syphilitic aortitis characteristic is its expansion. Prognosis. In uncomplicated cases, with early recognition and with energetic and prolonged therapy, syphilitic aortitis may subside and even be completely cured. More often, especially with late recognition, however, irreversible anatomical changes remain, since in late stages there is already significant destruction of the aorta and its valves, with replacement of elastic and muscular tissue by scar tissue. In far-advanced and actively progressing cases, or with valvular insufficiency, aneurysm, and especially with stenosis of the coronary artery orifices, the prognosis is significantly aggravated, becoming hopeless in cases with already developed severe cardiac insufficiency. The usual duration of the disease from the onset of its manifestation to death is from 2 to 5 years. Treatment. In non-severe cases, in the absence of signs of cardiac insufficiency, treatment is outpatient. In principle it should be cautious and at the same time energetic and prolonged. Treatment is recommended as combined, in the form of courses of salvarsan-mercury or salvarsan-bismuth therapy, repeated 2 or 3 times a year. The total dose of Neo is about 4.5-5 g, a single dose from 0.075-0.15 to 0.45 g. Mercury in the form of soluble or insoluble salts intramuscularly, or in the form of Novosuropl, Suagva and calomel intravenously. Bismuth in the form of Bijochinol'H (10-12 injections of 3 cc) or Bismogenol'H (15 injections of 1 cc) intramuscularly. In addition, potassium iodide in large doses and Bieta's mixture are recommended. In cases where there is no cardiac insufficiency and angina pectoris or aneurysm, resort treatment in Pyatigorsk and Kislovodsk may be indicated. Contraindications to energetic specific therapy are decompensation, nephrosclerosis, hemorrhages. Epidemiology. Syphilitic aortitis, it seems, like syphilis in general, is more common among the urban population. Among all organic cardiovascular diseases, syphilitic aortitis, syphilitic aortic insufficiency, and aneurysms make up about 25% (Romberg). Almost 75% (Wittgenstein) of all aortic insufficiencies are of syphilitic etiology. Among visceral syphilis, aortitis occurs in 70% (Romberg) - 82% (Stadler). According to Pletnev's data, out of 362 cases of cardiovascular syphilis, in 44 cases cardiovascular lesions were noted as monovisceral lesions, in the rest as plurivisceral in combination with specific changes in other internal organs. In congenital syphilis, changes in the aorta occur in 67%. Most often syphilitic aortitis is revealed in the age of 40-60 years (Romberg, Arnoldi). On average, between infection and clinical onset of the disease, 15-20 years pass (21.9-Romberg, 23.5-Hubert). Angina pectoris is observed in 14% (Romberg). Men are affected in a/s of women in Vs of cases (Hubert). In 56% of cases there is an indication in the anamnesis of lues, only in 11% of them was treatment carried out at the beginning of the disease (Wittgenstein). Of 325 men - 50%, of 128 women - 70% (Pletnev) did not know about the syphilis they had (lues ignorata) and, consequently, thanks to this, did not carry out a single course of specific treatment. The Wassermann reaction is positive in 67% (Wittgenstein) - 85% (Romberg). The outcome in aneurysm is observed in 16% (Romberg) - 22% (Arnoldi). The cause of death in 50% of cases is intercurrent infections, in a/s of cases death occurs from cardiac insufficiency, in x/v from angina pectoris, in x/8 from rupture of the aorta (Romberg).