Cardiovascular syphilis is the most frequent basic cause of death from acquired syphilis. Indeed, it is preventable to a great extent and its progress into a fatal lesion can be either warded off or controlled ordinarily. Is it, then, creditable that such a preventable condition should be a common occurrence? Early diagnosis and early institutions of adequate specific therapy in every patient with syphilis will prevent its occurrence in a great majority of luetic persons. I no other aspect of late syphilis is this truism of greater need of appreciation. Failing this ideal, the aim of the clinician should be to recognise its existence in a stage of uncomplicated aortitis when the tissue involvement is at a minimum. No opportunity should be lost to impress the patient that every primary sore carries a risk of this dangerous complication and that early and efficient treatment averts its possibility.
The effect of syphilis on the cardiovascular system is an important aspect of syphilitic infection. The involvement of the cardiovascular apparatus is often insidious in its onset and may be present for many years without giving rise to signs and symptoms. The best protection against this failure to recognise the early involvement of the cardiovascular mechanism is to maintain a high index of suspicion and to make frequent and careful searches for the evidence of its presence in all patients suffering from syphilis. Only by devotion to habitual suspiciousness and anticipatory watching are the early signs of cardiovascular syphilis detectable. Hence, an ounce of care in the early stages is better than a pound of treatment in the late stages. Inadequate treatment of early syphilis and late treatment are often at the root of cardiovascular syphilis.
Although Tr. pallida reach cardiovascular system, a short time after the initial infection, clinical evidence of syphilitic disease of the aorta which is invariably affected or its effect on the heart does not become apparent for a considerable period. The time at which clinical manifestations are noticeable varies widely from two to twenty years. Syphilitic aortitis, however, has been recognised as early as six months after the infection and there is little doubt that if in all cases of syphilis are examined scrupulously for its evidence at the commencement of, and during the treatment, many cases of aortitis will be diagnosed at an earlier stage. Frequency of cardiovascular involvement is greater in men than in women. It is 2 to 4 times common in men than in women (Stokes). Cardiovascular affections are more common in those patients who have had insufficient treatment in the early stages of infection, than in those who had late treatment. They appear to be infrequent in cases of inherited syphilis, of course in late survivors. In cardiovascular syphilis, the ascending part of the thoracic aorta, which is commonly affected, is the theatre of the most striking changes. The inflammation, which is by way of vasa vasorum and their terminations, is mainly confined to the adventitia and the media and leads to infiltration and replacement of the elastic and muscular tissue by fibrous tissue with the result that the wall of the aorta becomes weak and dilatable, Owing to the inflammation of the wall of the proximal aorta, the orifices of the coronary arteries becomes narrowed or blocked and the aortic valve becomes involved giving rise to aortic incompetence rather than aortic stenosis. True stenosis of the aortic orifice is very rare. The arch of the aorta is the favourite site for aneurismal dilatation. Syphilitic process though active usually at the aortic orifice may involve larger arteries of the body, cerebral vessels or myocardium. Instances of peripheral vascular syphilis causing obtiterative endarteritis as one of the fundamental changes are not unknown. Occasionally vascular syphilis, and its accompanying changes, underlie nervous or pulmonary syphilis and may not be easily recognizable as such.
It will be noted that the aortic incompetence and coronary stenosis are, in fact, the part of the mesaortitis of the proximal portion of the aorta, and that the various changes found in the syphilitic heart i.e. ischaemia, fibrosis, hypertrophy, dilatation, cardiac degeneration, angina pectoris etc., are the end results of aortitis, which increases the work and diminishes the blood supply of the organ. Syphilitic inflammation of the heart muscle or the coronary arteries beyond the aorta is scarcely observed. Isolated examples of localised gumma or diffuse luetic myocarditis, (a debatable point), have been recorded but they are as a rule rarities. Syphilitic involvement of the peripheral vessels producing arteriosclerosis or hypertension is not known. Thrombosis of the cerebral or spinal vessels occurs sometimes and results in paralysis.
Cases of cardiovascular syphilis may be divided into following groups. However, it is not uncommon to get combined lesions in many cases.
- Occult type or asymptomatic.
- Simple aortitis type.
- Aortic Regurgitant type
- Aneurysmal type.
- Coronary Occlusive type.
- Myocarditis type.
- Peripheral vascular type.
- Neurovascular type.
Barring neurosyphilis, there is probably no condition which is so vague and unreliable at its onset as early involvement of the cardiac system. AS stated above, cardiovascular syphilis, in its early stages, may not give rise to any definite or conspicuous signs and symptoms (occult type). Nevertheless, the clinical recognition of early and uncomplicated aortitis can be made correctly, ante mortem, in a large percentage of cases, by actual recording of symptoms and physical signs, importance of which is often likely to pass unheeded. Clinically syphilitic aortitis is by far the most important since fundamentally it is the cause of aortic insufficiency, aneurysm etc. It is, therefore, necessary to be familiar with early signs and symptoms of aortic disease:
- A change in the quality and quantity of the aortic second sound. A characteristic loud “tambour-like” second sound is the most significant sign of syphilitic aortitis. In hypertension, also, one encounters a loud second sound, but this sound lacks the “amphoric” or “echo-like” quality of the syphilitic aortitis and is either a “thudding” type or of a “clanging or bell-like” character rather than “tambour-like”.
- Dilatation of the aorta seen by Roentgenography, Fluoroscopy and increased retrosternal dullness especially between the 2 nd and the 4 th right costal cartilages (paramanubral area).
- Palpable and visible pulsation in the suprasternal notch.
- A rough systolic murmur over the aortic area.
- Slight hypertension in youths particularly of diastolic pressure.
- Substernal oppression or “burning”, pre-cordial anxiety, pain or distress. Pain lasts for a few minutes, does not radiate and is located behind the manubrium.
- Dyspnoea paroxysmal or nocturnal. Paroxysmal nocturnal dyspnoea is usually common.
- Abrupt onset of circulatory embarrassment in a young person.
These are important clues, which, when present in a syphilitic patient, need to be seriously considered and must not be dismissed lightly, if one desires to detect, cardiovascular syphilis at a treatable stage. Cardiac irregularities or disorders, especially with the previous history of venereal sore, should invariably be a signal for complete investigation. Critical scrutiny of the vascular and nervous systems in every syphilitic patient is obligatory. High grade nervous or pulmonary changes may be associated with vascular syphilis. In such cases, treatment is sought more often for some obvious painful lesion such as asthma, disturbances of sensation, osteomyelitis, iritis, than for real trouble and the clinician is often likely to be led astray. Hence is the need to regard this scrutiny as an integral part of the study of the syphilitic patient. The Wassermann reaction is often useful in varying one’s doubts. In untreated cases, it gives a large percentage of positive results. If necessary, provocative Wassermann may be considered; it, however, needs careful decision particularly in advanced cases. A negative Wassermann is not so valuable as positive reaction; for, the proportion of Wassermann negatively in the neurological phases of vascular disease of nervous system is fairly high. The result of the test, therefore, is to be considered in conjunction with the clinical data. Cardiac lesion coming on in the middle life is strongly suggestive of syphilitic infection. Tachycardia in a syphilitic patient may indicate myocardial degeneration. All avenues of investigation such as radiography, arthrodiagraphy, teleroentgenography, electrocardiography etc. should not be spared in arriving at a diagnosis.
Prognosis in these cases is an extremely difficult problem. It depends on a number of factors such as age sex, occupation of the patient., stage of the infection, site and extend of the lesion, nature of the lesion whether progressive or stationary, response of the patient to treatment, the cardiac efficiency, presence of complications etc. The prognosis is vastly better in patients with uncomplicated aortitis tan in those with aortic insufficiency, aneurismal sacculation or myocardial degeneration. Once the aorta is damaged or its end results have appeared in the heart, antisyphilitic treatment may at best arrest the advance of the disease, increase the expectancy of life and ease symptoms. There is no other disease on which reputations are wrecked not infrequently on the rock “Prognosis”. Physician cannot be a correct prophet at all times. Sudden death or fatal termination due to syncope or angina is not uncommon. It follows then that great prudence and reservation are essential in giving one’s opinion in any case.
Stronger emphasis cannot be laid on the fact that it is much easier too prevent the development of the cardiovascular syphilis than it is to cure it after it has appeared. This very fact should actuate medical men to adopt early and adequate treatment and to make periodic, careful and painstaking detailed examinations in every case of syphilis. The adoption of this procedure would save involvement of the vital organs in the body generally.
Granting that the general principles of treatment of cardiac disease hold good, the treatment of cardiovascular syphilis consists of an individualized specific therapy composed of careful administration of iodides, bismuth or mercury and arsenic. It is no longer justifiable to withhold specific therapy from patients with cardiovascular syphilis, even when the situation seems hopeless; for, treatment can be adjusted so as to do no harm to the patient and good results may be obtained. Iodides are of special value as they promote resolution and absorption of the syphilitic lesions and thus make the treponemicidal action of drugs more effective. When there is positive evidence of syphilitic infection in the presence of heart disease, it is immaterial whether that heart disease is secondary to syphilis or merely co-exists in the same individual; in either case, there is an imperative need for ante-luetic treatment. Even when the syphilitic disease is not the cause of the heart trouble, it invariably leads to an aggravation of the same and constitutes in some way or other to the ultimate circulatory break-down.
Exact details of treatment, which are bound to vary according to the individual requirements, can be worked out without much difficulty once the principles in the treatment of cardiovascular syphilis are thoroughly grasped. They are as follows:
- Effective modern treatment of primary and secondary syphilis can frequently avoid later cardiovascular complications.
- Early cardiovascular involvement can often be arrested completely; while in the late lesions treatment is worthwhile.
- The aim of the treatment is neither radical cure of the infection nor restitution of damaged structures but to promote healing of the active inflammatory process, to prevent further damage, and to compensate as far as possible; and thus to procure relief of symptoms and prolongation of life. This is possible by a judicial combination of medical care, specific treatment and attention to individual needs.
- It is important to find out the location and the extent of the lesion and to ascertain the condition of the heart; for the choice of the treatment will depend on these facts: In this connection, the following points should be found out.
- The state of the myocardium (by E.C.G.).
- The state of the coronaries (by history of angina and E.C.G.).
- The state of the aortic valves (by aortic regurgitation and its extent).
- The size of the aorta (by Fluoroscopy etc.).
- The co-existence of other factors (renal or hepatic, B.P., nervous, etc.).
- Rest and restriction of physical activity are required and may be especially important in cases of cardiac decompensation, where the administration of digitalis may have to be considered in addition.
- In planning a specific therapy it should be kept in mind that the treatment must not do any harm to the patient. Treatment procedure should be such as will avoid assiduously all possible reactions, early as well as late (therapeutic shock, therapeutic paradox, etc.); because it is undesirable to place any added strain on an already overburdened myocardium. Reactions may be prevented by initiating treatment with iodides and later on, with bismuth or mercury, and continuing this for a sufficient length of period before the commencement of arsenotherapy. Even when this preparatory treatment is ended, the danger is not yet entirely passed so that when arsenic is started, it should be in the form of sulpharsphenamine preferably of Neo-arsphenamine and should be used in a very small dose. Arsphenamine or old solvarsan (606) is absolutely contra-indicated in cardiac cases. At the same time it is wise to be prepared and ready to meet any emergency that may arise as the result of an infection.
Careful and discriminate judgment should be exercised in the introduction of arsenotherapy. As a general rule arsenic especially intravenously is contra-indicated at the beginning. The very first dose seems easily to upset the patient with cardiac syphilis; it is attended with grave dangers like coronary occlusion, aneurismal rupture, and heart failure. Occasionally it is followed by a sudden aggravation of symptoms attributed to focal reaction (Jarish-Herxheimer Reaction). Sometimes, it causes temporary improvement followed by the exaggeration of the condition (therapeutic paradox) due to the weakening effect of the rapid resolution of the lesion in the aorta or valve; a patient is, thus “pathogenically better but functionally worse”.
- Use of arsenic is of great value in arresting the progress of the disease and should not be delayed unnecessarily. Arsenic intra-muscularly or even intra-venously after a preliminary medication with iodides and bismuth for 3 or 4 weeks, may be permissible or even urgent under certain conditions where there is a perfectly efficient heart and at the same time where there ia an urgent need to check the progress of the disease i.e. in cases of early aortitis pure and simple. Mercury or bismuth is very slow in its action and does not constitute adequate treatment for early cardiovascular syphilis. Presence of late or extensive lesion or any complication precludes early use of arsenic. Under such circumstances, the heart being perfectly balanced, the treatment must be begun with a preliminary course of iodides and bismuth or mercury, extending over a period of 8 to 12 weeks, according to the gravity of the situation.
- Arsenic is absolutely contra-indicated in advanced aortic regurgitation, coronary involvement, aneurysm, cardiac decompensation, anginoid attacks, and myocardial degeneration. Where a high degree of circulatory disturbance is present or progressive cardiac failure is set in, it is far more urgent to remedy the cardiac defect and to re-establish satisfactory cardiac balance than to give arsenic, which is certainly out of question. So also is bismuth in such cases. Cardiovascular disease takes precedence over all other complications of syphilis in determining treatment procedure.
- Since treatment is given more for alleviation of symptoms and arrest of progress than rather than in the hope of procuring cure, it need not be intensive and continuous; rest periods on the contrary, are more helpful. Injections of arsenic and bismuth are given alternately rather than simultaneously.
- Treatment should be prolonged to an absolute minimum of 2 to 3 years to attain best results. The effect of the treatment is better measured in terms of arrest rather than serologic reversal particularly in late cases. The best results are obtained in syphilitic aortitis without dilatation or insufficiency. It, however, is not surprising to see occasionally aortic regurgitation being developed in spite of efficient and adequate treatment. “This may be a blessing in disguise rather than a disaster; for, a progressive disability is exchanged for a static one”.