RHEUMATIC CARDITIS AND RHEUMATIC FEVER

The incidence of acute rhematic fever has fallen dramatically in this country over the last 20 years, a fortunate circumstance, since treatment remains unsatisfactory. Sulphonamides, penicillin and corticosteroids have all in turn been found in greater or less degree disappointing. Rest in bed and the administration of sodium salicylate or soluble aspirin remain, at least in domicil¬iary practice, the mainstays of treatment.

Penicillin.—While penicillin is without action on the course of rheumatic fever or carditis, it is used in treatment to eliminate persistent (tonsillar) streptococcal infection and to prevent re-infection. A week's intensive course of soluble penicillin is given at the outset followed throughout the patient's stay in hospital and after discharge with phenoxymethylpenicillin by mouth. Such treatment is given along with salicylate or other anti-rheumatic drug.

Corticosteroids.—In some patients with severe pancarditis in whom the administration of salicylates pushed to toxic limits have failed, corticosteroids have produced dramatic improvement, leading not only to disappearance of fever and arthritis but to rapid resolution of the cardiac affection, improvements which have been maintained. In others, however, the hormones have failed to influence the condition, and there seems to be no means of predicting which patients will respond and which will not. On occasion, however, corticosteroids produce effects of an altogether different order from those of other lines of treatment. Prednisolone is preferable to cortisone, since its salt-retaining action is less marked. The other recognized side-effects have not proved troublesome in the relatively short courses required in acute carditis. It is well to reserve its use for acute cases under institutional care.

Rest.—The child who develops acute or subacute rheumatism should be confined to bed till all the signs of rheumatic activity have subsided. Good nursing is essential, and during an. active carditis the patient should not be allowed to do anything for himself. The achievement of complete physical rest in young children without acute symptoms may present difficulty, but confine¬ment to bed should be maintained even although the child is moving about freely in bed, for to allow such a child up greatly increases the demands made on the heart and circulation. During the period of fever, with its accompanying profuse perspiration, careful toilet of the skin is necessary and will lessen the risk of sweat rashes. The diet should be fluid and light, as for any other febrile condition. The duration of bed rest is discussed below.

Salicylates.—Salicylate has generally been regarded as being without action on the progress of the carditis, though exerting a striking and specific effect on the fever and arthritis. Recent work, however, suggests that it may produce similar effects to cortisone and corticotrophin through the pituitary-adrenal system. There is evidence that when the blood salicylate level is kept steadily between 30 and 40 mg. per 100 ml., not only are the fever and arthritis abolished but the E.S.R. returns more quickly to normal and the duration of the disease is shortened.
If the condition is to be treated in domiciliary practice without biochemical control the drug must be given in doses sufficient to produce the symptoms of mild salicylate intoxication. So-called drug resistance is in many cases due to under-dosage. The daily dose required varies with the age and weight of the patient. The guide to the dose in any individual case is clinical: the abolition of joint pains and of pyrexia, or the development of mild symptoms of salicism (deafness, tinnitus, etc.). In general, for an adult, doses of 1-5 to 2 g. (20 to 30 gr.) two- or three-hourly will be required, a total of 12 g. (200 gr.) per day being commonly sufficient. In children the effective daily dose varies from 4 to 8 g. (60 to 120 gr.) per day, according to age. The old practice of giving sodium salicylate with bicarbonate simply enhances excretion, and necessitates giving about twice the amount of salicylate to produce a given effect. Such doses may produce gastric irritation, and this may be the limiting factor in dosage. When sodium salicylate is badly tolerated, aspirin, or, better, soluble aspirin, may be substituted. The latter is prescribed as tablets to be dissolved in cold water immediately before use. Dosage again has to be pushed so far as the tolerance of the patient permits. The intravenous administration of salicylate has not been shown to possess appreciable advantages over oral administration and the risk of toxic reactions is much increased.
Once fever and pain have subsided, or when symptoms of salicism have appeared, the dosage of salicylate should be reduced. This can generally be done within a few days from the start of treatment. The drug should not, however, be entirely discontinued, but should be administered in smaller doses so long as the rheumatic process remains active. The maintenance dose is that which will just suffice to keep pain and fever in abeyance, and for an adult is generally from 6 to 7 g. (100 to 120 gr.) per day. Recurrence of acute symptoms is common when the maintenance dose is reduced as low as 4 g. (60 gr.) per day. Should such recurrence occur, the dose must be temporarily increased.

Local Treatment for the affected joints should be simple. Wrapping in cottonwool and bandaging to secure rest generally suffice during the few days of acute pain. The application of liniments is valueless. Care is essential in patients in whom pain lasts more than a few days, since, contrary to general teaching, permanent joint affection may result from rheumatic fever. This rare complication is seen now and then in adolescent patients in whom unequivocal carditis has been associated with arthritis of small joints of hands and mandible, and in whom lasting deformity has resulted. Continuous immobilization of joints always carries the risk of later limitation of movement. Therefore passive movement through the maximum range, short of causing pain, should be performed daily as soon as the most acute symptoms subside, and immobilization during the rest of the day should be in the best orthopzedic position.
Focal Sepsis.—In many cases of rheumatic fever a focus of streptococcal infection in tonsils or upper respiratory tract remains active throughout the course of the disease. Penicillin, used as suggested above, has rendered it rare nowadays for the physician to be faced with the serious decision of advising tonsillectomy at the risk of provoking a severe and possibly fatal exacerbation. The risk is such that every effort should be made to eradicate streptococci by anti¬biotics rather than hazard tonsillectomy. Cover by antibiotics should be given before operations on teeth or tonsils in apparently quiescent rheumatic cases.
Routine tonsillectomy of healthy children does not protect them against subsequent attacks of rheumatic fever, nor after a first attack does it appreciably lessen the risk of recurrence of rheumatism. It is wise to advise tonsillectomy" only when the local condition is such as would demand operation in a non-rheumatic case, to choose the time for operation with great care and to secure antibiotic cover Duration of Rest.—The question of how long a patient with rheumatic: carditis is to be kept in bed is not easily answered. No hard-and-fast rule cani be laid down, though many advocate a minimum of three months' rest in bed;
after even a mild attack. Certainly one encounters with distressing frequency patients with established valvular disease who give a history of arthritis treated by a short period of rest followed by a quick return to normal activity. On the other hand, in fully one-third of all cases with symptoms of the effort syndrome admitted to an army centre for rehabilitation, the onset of the symptoms and of the disability could be traced to a doctor's warning to parents or patient that strenuous activity must be avoided. It is true that in all cases with clinical evidence of active carditis, rest in bed should be prolonged so long as the signs of activity persist, and this may extend over a period of many months. It is wrong, however, to prolong rest after signs of activity have disappeared. Not only is an established, static valvular lesion not benefited by such treatment, but there is evidence that moderate exercise is beneficial for the subjects of early valvular disease.
The criteria for determining quiescence of carditis must, therefore, be con¬sidered. A smouldering valvulitis of sufficient intensity to produce eventual gross fibrosis may go on for many months, and yet with very little clinical upset. The pulse rate and temperature may be normal and cardiac enlargement absent even though active infection persists in the valves. Sinus arrhythmia is not a safe indication that the heart has escaped damage, and the duration or degree of joint involvement bears no relation to the extent of the cardiac lesions. Sub¬cutaneous nodules when present indicate persistent rheumatic activity, but their absence does not exclude active carditis. Electrocardiographic changes (pro¬longed P-R interval, prolongation of the duration of QRST beyond the calculated normal for the heart rate and T-wave inversions) when present are indicative of active myocarditis. The return of the blood sedimentation rate (E.S.R.) to normal is generally a guide to the cessation of activity, but is not infallible. It may be normal in the presence of congestive failure, but such cases are easily recognized, and the question of allowing the patient up does not arise. Now and then a normal E.S.R. persists in a patient showing no signs of failure while other evidence of carditis is obvious. Conversely, a raised E.S.R. does not necessarily indicate carditis, for many conditions increase it. By careful clinical examination the true significance of the E.S.R. can usually be assessed. Other criteria of cessation of activity are gain in weight in children if not due to oedema, stabilization of the position of the apex beat and of physical signs in the heart, and a stable pulse rate, particularly during sleep.
Children who have suffered from repeated attacks of carditis may develop a severe and occasionally fatal acute pancarditis. The handling of such a case can be as sad a task as any that a doctor has to face; ordinary measures fail to arrest or retard the disease, and treatment is symptomatic. In such fulminating cases corticosteroids have on occasion proved valuable, but in a proportion these drugs, too, fail to stay the course of the disease.
Convalescence.—When the period of complete rest in bed is over, the return to activity should be gradual and carefully supervised. Any recrudescence •of the rheumatic process should be met by a prompt return to complete rest.
The child who has weathered rheumatic carditis is frequently sent for a :short period to a convalescent home, and then returns to school and to full routine. This is unsatisfactory, and is probably responsible for much later disability. Convalescence should be protracted, and after the period in hospital the child should be sent to a convalescent home. In some parts of the country special homes have been established where adequate medical attention and supervision are available in healthy surroundings.
After-Care.—On discharge from such an institution/or after convales¬cence at home, the child should return gradually to activity, the limitation of effort being determined by the extent of the cardiac damage. In cases with mild but quiescent valvular lesions full activity is allowable with a prohibition only on such strenuous exertions as competitive sports. A patient with gross myocardial damage and hypertrophy must, however, lead a quiet, sedentary life. Careful follow-up with periodic assessment of general health and cardiac condition is essential, and any sign of renewed rheumatic activity demands prompt measures—rest in bed, etc. It is understandable that such solicitous after-care may well engender a cardiac neurosis—a point always to be borne in mind.
The prevention of recurrent streptococcal infection is important, by the long-continued administration of penicillin either by the weekly injection of 1,000,000 units of a long-acting preparation (benzathine penicillin, B.P.) or the oral administration of 250 mg. of phenoxymethyl penicillin twice daily.
The choice of a future occupation should always be made under the guidance of the doctor. Instances of young adults with advanced cardiac lesions engaged in strenuous occupations are all too familiar, and would not occur if after care were efficient. It is vital that the education of a child should not be neglected during long periods of semi-invalidism. Many rheumatic children leave school at sixteen years with much less than average schooling and are driven on to the unskilled labour market. Better education means greater ability to secure a sedentary occupation which will not lead so soon to a cardiac breakdown. There is ample scope for the doctor, in village or city, to make himself acquainted with local industries, and by direct approach to employers to secure the right niche for the individual patient. Light, skilled crafts (wood- and leather-work; radio mechanics ; precision instrument making, watchmaking, etc.) are remunerative, make little call on physical strength and maintain the patient's interest and independence. Often the journey to and from work or school, and the exposure to inclement weather involved, impose greater strain and risk of relapse than the actual physical strain of employment,
It is implicit that diagnosis must be accurate. Far too many children are lightly diagnosed as suffering from valvular disease on the basis of a systolic or exocardial murmur unaccompanied by cardiac enlargement, and it is fair to say that for one young adult with a mitral stenosis that has been missed by his doctor one sees several with innocent murmurs labelled organic. When such important matters are involved as the whole future of a young patient, his choice of vocation or her fitness for marriage and child-bearing, it is surely incumbent on the doctor to exercise the greatest care. As diagnosis is admittedly often difficult, the doctor should seek specialist and radiological help before pro¬nouncing an opinion. A thoughtless remark after a superficial examination may have disastrous consequences.