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Rheumatic Fever, Diagnostic Criteria, and Rheumatic Heart Disease
RHEUMATIC FEVER, DIAGNOSTIC CRITERIA, AND - RHEUMATIC HEART DISEASE H.
WILLIAM ELGHAMMER,
M.D., F.A.C.P."
IN presenting these patients I wish to bring out the nature of rheumatic fever in childhood, to consider the findings necessary for the establishment of a definite diagnosis and to discuss rheumatic heart disease. CASE I
_The first patient is a boy, six years of age, who was brought to the Clinic ten days ago because of tiredness, lack of appetite, low grade fever, recurrent nosebleeds, arid _frequent pains in the arms, legs and abdomen. The mother -. stated that the boy had been in good health until six months ago. During the past winter he had had recurrent colds and severe tonsillitis with swelling of the lymph glands of the neck on two occasions.
Fig. 1.-Electrocardiogram in Case I, showing prolonged P-R interval, 032 seconds, and diphasic T wave in Lead Ill. Physical examination revealed a fretful, blond, blue-eyed boy, not acutely ill.· There was a distinctive pallor of his face. General nutrition was below par -and the flesh was soft and flabby. The tonsils were hypertrophic and injected. Several strands of lymphoid tissue were seen in the posterior pharynx. The pos• Professor of Pediatrics and Chairman of the Department of Pediatrics, Loyola University Medical School; Senior Pediatrician, Mercy-University Hospital; Attending Pediatrician, La Rabida-Jackson Park Sanitarium, Chicago.
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H. WILLIAM ELGHAMMER
terior cervical chain of glands were numerous and enlarged. The anterior cervical glands were only moderately enlarged. Tenderness along the sternocleidomastoid muscle was present. The mucous membrane of the anterior nasal septum was congested and on the right side a bloody crust was present, giving evidence of recent hemorrhage. Heart action was regular but rapid, there was no enlargement and no murmurs were heard. This boy is the third child in a family of five children; an older sister had chorea three years ago. The father has rheumatic heart disease; the mother is well. An aunt on the mother's side died of "heart trouble." The patient was admitted five days ago to the hospital for further study and observation. His temperature on entrance was 101 F. (rectal). Subjective symptoms similar to those I have already mentioned were present, with the additional complaint of localized pain in the right knee, left ankle and precordial distress. Laboratory findings obtained in the last few days show red cells 4,200,003, white cells 12,600, hemoglobin 13 gm., 72 per cent polymorphonuclears, 26 per cent lymphocytes, 1 per cent eosinophiIs, 1 per cent monocytes. The urine was clear, specific gravity 1.022; there was no albumin or sugar present and the microscopic examination was negative. Erythrocyte sedimentation rate was 40 mm. in one hour (Landau). Electrocardiogram (Fig. 1) showed prolonged P-R interval, 0.32 seconds, and diphasic T wave in Lead Ill. The tuberculin skin test was negative. Agglutination tests for the typhoid group and undulant fever were negative. 0
Diagnostic Criteria.-As you will notice, the right knee joint and left ankle are tender but not swollen nor red or hot to touch. The apex beat is in the fourth interspace mid clavicular line. There is no cardiac enlargement. The rhythm is regular, the first heart tone at the apex is loud and rumbling but no murmurs are heard. The clinical diagnosis upon admittance to the hospital was focal infection and suspected rheumatic fever. The diagnosis today is active rheumatic fever, mild polyarthritis, and rheumatic carditis. Are we justified in making this diagnosis? We notice in the history the presence of rheumatic infection in the father and the older sister, and that one aunt died of "heart trouble." Rheumatic fev~!J,:a~_~_IJl.~r!.e~f!'mili~lJe'!lde.nfY and iti.s J}eld by som~ tha_~_!.li1s_tre.naency jsdefinitely a hereditary phenomenon following the mendelian 1!1:W. This hereditary predisposition to the disease, although modified by other factors, such as geographical location, nutritional state, and changes in environment, may make it possible to predict the occurrence of rheumatic fever. The presence of rheumatic fever in the family certainly should put us on our guard and as we watch these children we should constantly be on the lookout for the early signs and characteristic manifestations of this disease. Rk~Jf,'matic fever is essentially a childhood disease, and it is in the child t';!!..~~~he dise.ase presents its greatest variety of symptoms and manifestatW1J$. T..h~. highest incidence of ~nset is found between the fifth and qjp.s.hsear. Occasionally in the older child as well as in the adult the onset is sudden, preceded by an attac;k_Qf~9g1ii]liti~, or respiratory inf~ followed bx: seyerJ!_p.:tin, s}Y_elli.!YL~n.d t~t1<.lerness in one or ITIg.re_ioims, aS~Qciated . with high temperature and marked constitutional sYI?2torns. In many of these cases, however, what is regarded
RHEUMATIC :FEVER, RHEUMATIC HEART DISEASE
27
as the onset of the disease is in reality an exacerbation occurring in a previously infected rheumatic individual.
The. vast .rnajori~y ofcasesofJlJis d,iseasf/have an irzsidious. onset.
The-rypicaf sequence of events is well demonstrated in the history and findings of our patient, i.e. tiredness, loss of appetite, irritability, distinctive pallor of the face, vague mild aching pains in the limbs and joints, accompanied by low grade fever and increased pulse rate. These findings are all due to toxemia and may either singly or combined be present in any toxic state, and do not permit us to go any further than the diagnosis of focal infection, toxic state, and suspected rheumatic fever. The additional symptoms of epistaxis, t~nderll~~~.jn s~veral joints, pr~E<2£~!~l ~istress, together with increased sedimentation rate, modenite leukocytosis, and myocardial involvement, as evideiiced by the electrocardiogram, definitely establish the diagnosis of active rheumatic fever, mild polyarthritis, and rheumatic carditis. Many of the symptoms and findings may be produced, as previously pointed out, by other conditions. Childhood tuberculosis may be eliminated by tuberculin tests and x-ray studies of the chest, leukemias, severe anemia and mononucleosis differentiated by morphological blood studies, and other subacute and chronic infections ruled out by the employment of specific agglutination tests. The vague fleeting pains of rheumatic fever, so-called "growing pains," usually occur after rest and on attempt at motion. Th~.<.!l!e r~Heyed by. heat and the administration of salicylates. However, one should keep in mind that similar pain may be associated with other infections or postural defects. It often becomes necessary to differentiate the abdominal pain from acute appendicitis. This is a rather difficult task at times. A careful history revealing some rheumatic manifestations, the occurrence of the pain over a long period of time, together with an increased sedimentation rate enables one to make a diagnosis of "r~eumatic abdomen," and save the patient the risk of surgery. Polyarthritis is not a common manifestation of rheumatic fever in chiIdt:.~n. When it occurs the most common sites are the knees, ankles and wrists, and it is usually rather mild in comparison to that seen in adults. The differentiation from acute poliomyelitis may be difficult, particularly during epidemics of the latter disease. In its severe form one should rule out the presence of osteomyelitis, septic arthritis, leukemia, purpura rheumatica and undulant fever. Chorea is generally accepted as a definite manifestation of rheumatic fever. The onset of this disease is very insidious and mild types may often be overlooked. We should realize that even mild attacks of chorea may be associated with severe rheumatic heart disease. Subcu'tf1:..nef'!!:L?1QduJe.l,lUOSt commonly fQ.und.alollg tendons of ..tl)e ~.C)I~tl1~.l1aD9s., eLl>()\Ys, knees, spine and occipital regloli"·d~tc actjve rheumatic process. These nodules occur in groups and arc US\}a!!Y.-.!mLt.clJ~leLQL.'p;l!nful. . -. . . . . . . . . . .. .
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H. WILLIAM ELGHAMMER
The major manifestations of rheumatic fever are polyarthritis, chore.!h. sg~C:l:ltan~ol:ls. !1Qd_l:ll~s and ~~!?i~i~: Among the minoL m:otllifestations we include fever, "growinKpains," abdominal pains, epistaxis, erythem~l!!:J:fS:~I!:a!ll~__ and increased se~imentation rate. The presence of rheumatic fever in the family strongly suggests the possibility ot this disease in the progeny. The presence of minor rheumatic manifestations in the child, together with one or more major manifestations, makes the diagnosis of rheumatic fever quite certain. The disease in its inactive state may be recognized by the finding of valvular damage and associated cardiac murmurs or the presence of manifest chorea. CASE 11
Our second patient is a girl ten years of age, who was admitted to the La Rabida-Jackson Park Sanitarium five months ago. Her past history reveals the onset of rheumatic fever at the age of eight years, evidenced by minor manifestations and mild polyarthritis. After three months' rest in bed she apparently had recovered from her active rheumatic fever and was allowed to attend school. In the spring of 1944 she developed chorea of moderate severity necessitating bed rest for six weeks. During the summer she was apparently well, although rather nervous and failed to gain in weight. At the time of returning to school in the fall she was found to have a systolic murmur at the apex transmitted to the scapular region, increased second pulmonic sound, and slight cardiac enlargement. The work in school soon became too difficult, the child, appearing listless and tired, would often refuse to attend school and preferred to rest in bed. She was admitted to the Sanitarium in January 1945 because of weakness, poor appetite, loss of weight, rapid pulse, and afternoon temperature of 101 F. (rectal) (Fig. 2). Physical examination revealed a pale, tired girl. The flesh was soft, the skin moist and clammy. The throat appeared normal, cervical glands not enlarged, lungs resonant and clear throughout. The apex beat, diffuse and rapid, was located in the fifth interspace outside the midclavicular line and there was a slight cardiac enlargement to the right. A prolonged, high pitched, loud systolic murmur was heard at the apex, masking the first heart sound, and an increased second pulmonic sound. Orthodiagraphic tracing confirmed the cardiac enla.\"gement, and showed a prominent left auricle. The electrocardiogram, blood findings, and urinalysis were essentially normal. Erythrocyte sedimentation rate was 32 mm. in one hour (Landau). The patient was given the usual sanitarium care, i.e., absolute bed rest, acetylsalicylic acid, grains 15, with phenobarbital, grain lh three times daily, and offered a standard high protein, high vitamin diet. After three weeks her appetite improved and she began to gain in weight. Color returned to her face, she became cheerful and happy. The sedimentation rate decreased and reached normal values in about six weeks after her admittance. The heart decreased in size as shown by repeated orthodiagraphic tracings. The apex beat became more limited in area, the systolic murmur at the apex less loud and shorter, the first heart sound more audible and distinct. Her progress up to six weeks ago was very good and encouraging. However, in the middle of April she developed a pharyngitis with a temperature of 102 F. lasting three days, followed by loss of appetite. The sedimentation rate, which had remained normal since the latter part of February and throughout her acute illness, became elevated three weeks following the acute attack of pharyngitis, and has ranged between 24 and 40 mm. until four days ago when it dropped sharply to a low point of 5 mm. The sleeping pulse rate, previously 10 to 15 beats below the waking rate, began at the onset of the increased 0
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RHEUMATIC FEVER, RHEUMATIC HEART DISEASE
sedimentation rate to approximate the waking rate. In the last few days the sleeping pulse rate has exceeded the pulse rate present while the patient is awake.
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Fig. 2.-Clinical findings and course in Case H.
Diagnostic Criteria.-As you see this patient here this morning you will notice that she is pale and has a tired, worried look on her face. Her voice is weak and she seems short of breath. The main subjective symptoms are weakness, precordial distress and nausea. Her heart findings are the same as previously observed. However, we find at this time tenderness in the epigastrium and enlargement of the liver to two fingerbreadths below the costal margin. From these findings and the sudden marked drop in the sedimentation rate, we are able to diagnose beginning cardiac failure. May I again point out that we are unable to elicit any further findings in the heart or any sign of passive congestion in the lungs. Considering this case in retrospect, it becomes evident that this patient's heart was irivolved from the very beginning of her rheumatic fever, that the insult to her heart is primarily that of myocardial damage, and only secondarily that of endocardial changes. The myoc:trdial changes which are by far the more serious are directly asso. ciated with the length, severity and recurrences of the rheumatic process. It is evident that in the rheumatic child we are dealing with an active systemic infection. We are concerned with the effect of this infection upon the connective collagenous tissues throughout the body and especially within the myocardium. Valvular changes may take
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H. WILLIAM ELGHAMMER
place in due time, produced by proliferative and scarring lesions of the endocardium covering the leaflets, but they are only of secondary importance. The presence or absence of murmurs per se is of little diagnostic or prognostic significance. The correct determination of the activity of the infection is of paramount importance in order to treat these patients adequately, to know when they may be given ambulatory activities and to evaluate their progress and their prognosis. Aside from clinical improvement, the subsidence of fever, the return of the blood picture to normal, and normal relation between waking pulse rate and sleeping pulse rate, we have found the erythrocyte sedimentation rate to be in most cases a reliable test for the determination of the activity of the rheumatic infection. In all cases of rheumatic fever the sedimentation test should be employed routinely, repeated at weekly or biweekly intervals. By this we may follow the progress of the disease, detect exacerbations early, even before clinical findings are evident, and as in this case determine beginning cardiac failure. It has been our experience that cardiac failure almost always occurs at the height of the infection, rarely if ever, as in adults, from myocardial strain imposed upon the heart from valvular lesions or general bodily exertion. The paradoxical drop in the sedimentation rate at the inception of cardiac failure has not as yet been satisfactorily explained. It is of interest that the enlargement of the liver occurs simultaneously with this drop in the sedimentation rate and prior to any other signs of increased venous pressure or cardiac changes. Clinically the enlargement of the liver and the presence of tenderness in the epigastrium are of greatest iiiJportance and serve admirably as a guide in estimating the functional . state of the heart. TREATMENT
The treatment and successful management of the rheumatic child depends on a thorough understanding of the nature of the disease on the part of the physician and continued education of the public. The recognition of the insidious onset of rheumatic fever and its variable clinical manifestations will insure early attention and diagnosis. A general understanding of the chronicity and tendency to recurrence of the disease will promote careful medical supervision and thorough periodical physical examinations over a long period of time which is most essential in order to achieve success in the fight against this common crippling disease of childhood. Prophylactic Treatment.-The various predisposing factors met with in the etiology of rheumatic fever suggest many measures to be considered in the prophylaxis of this infection. Adequate clothing, avoidance of chilling and exposure to dampness and cold are of great importance. A well-balanced daily routine in regard to play, work and rest, and regularity of meals is very essential. Unfortunately children of preschool age are often allowed to dispense with the afternoon rest, many
RHEUJ\IATIC FEVER, RHEUMATIC HEART DISEASE
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are sent to the kindergarten in the afternoon and are occupied with additional work, such as music lessons and dancing. Recreation of a negative form, such as movies and radio programs, are allowed to occupy an excessive portion of the child's daily life. Children with history of rheumatic fever in the family should be given particular attention. Common colds, pharyngeal infections, tonsillitis and childhood diseases, as we well know, often initiate rheumatic infection. Convalescence from these diseases should be prolonged beyond the time usually allowed and we should be on the alert for the appearance of early signs and manifestations of acute rheumatic fever. lJ;_~_2~~!:aE!iv~_ n.~~V()':ls child, a~'!th~J:_.C()lllIl}OIl c!ini<:.a,!. en~ity, whQ d~1!()~.!:(!S2011g.to_the'ordiilary management of rest ana routine, is often found to have a chronic infection of the upper respiratory trac~. Such conditions as repeated colds, otitis media and tonsillitis, I think should be given particular attention. The removal of chronically diseased tonsils and adenoids is not in itself a means of preventing rheumatic infection. I think that there are times when the removal of tonsils and adenoids mav be harmful and even disastrous to the child. However, if the child presents no systemic infection, during the favorable season of the year-th~t is, @lysummer-definitely diseased tonsils and adenoids should be removed from standpoint of general health and of relieving the child of this burden. . We are accustomed to regard a normal gain in weight as being an indication of optimum health, therefore we should give particular attention to the child who is not making the usual increase in growth and weight. Being undernourished increases the hazards of the child in regard to acute rheumatic infection. . Since a cold and damp climate frequently plays a p!:ominent role in tIte_predisposing etiology, it may be advisable to remove certain types oL£hild..r~rf to a warmer climate in order to save them from acquiring t~:disease. . Treatment During the Active Stage.-Treatment of rheumatic fever during the active stage consists mainly of bed rest, attention to nutrition and relief of symptoms. ~s.~ a.nej.. relax'!.tion are necessary to rcduce metabolic expenditure and to promote the development of the natural defense mechanism. Merely keeping the child in bed, oftcn under duress, is not inducive of rest and relaxation. The child as well as. the family should be informed of what we are trying to aCCOlllplish, the program well explained and accepted both hy the patient and those who are to attend and care for the child. A general diet, moderately low in carbohydrate, with the additioll of vitamin n in the form of cod liver oil, will suffice. Of late a great number of vitamins have been suggested but I think we can truthfully say that a well balanced diet has proved quite satisfactory. A
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H. WILLIAM ELGHAMMER
high carbohydrate diet tends to induce sudden increase in weight but not the type of growth that is indicative of resistance. Salicylates in some form or other are most commonly employed. They have a very definite beneficial effect on fever, muscle and joint pains and seem to have a direct action upon the exudative phase of the pathological process. However, we cannot ascribe any specific action to this drug. Moderate dosage, such as 10 to 20 grains three times daily, depending upon the severity of the disease and the age of the child, is to be preferred. Salicylate poisoning although rather rare should be watched for. Chemotherapy has given universally disappointing results and we may state that. sulfonamides are distinctly GQJ:ltraindicated during the active stage orthe disease. .. The treatment of chorea has undergone many variations. Fowler's solution so popular years ago has been entirely discarded. Nirvanol with its toxic reactions likewise has been discontinued as well as fever therapy, due to the fact that the treatment in most instances is more severe to the patient than the disease itself. Bodily as well as mental rest with the use of sedatives, such as phenobarbital, bromides and chloral hydrate, has proved to give the best results. W ((pave found. warm bat.~~_or.'Ya!mp3:<::ks given twice daily to be very beneficial. lIieTnducement of purposeful movements, such as attempts by the patient to feed himself, to handle objects and to walk around the bed, has shortened the period of incoordination and muscular weakness. Treatment of Acute Rheumatic Heart Disease (Carditis).-The treatment of acute rheumatic carditis is essentially the same as that of the acute stage of rheumatic fever. As previously pointed out, heart failure in children differs from that in adults in that the infection is the principal cause rather than mechanical stress and strain. In other words, cardiac failure or decompensation always occurs at the height of the infection. Rls:.~!sid~diajlure withdyspnea is the common type. We rarely see generalized edema. Absolute bed rest is imperative but we should allow the patient to assume whatever position he finds most comfortable. He should be handled cheerfully, allaying his fears with reassurance. Salh;ylates should be administered if tolerated, together with sedatives, even codeine and morphine, if deemed necessary. In cases. without demonstrab}_e_.~c!ema the use of diuretics, such as salyrgan,6:S to i cc. intravenously eveii· tnri£diiys, or theocalcin, 7Yz grains twice or three t!mes. daily, often prove to be very effective. We have had very disappointing results with the use of digitalis and feel that in some instances the administration of this drug has shortened the patient's life. The i!l~~venousinjection of a 20 per cent glucose solution is an ex~I1tsuPpOriiye-tr~atment. . .. ... Convalescent Care.-When the patient is free of pain and has recovered from the acute stage of the disease, and begins his convalescence we are confronted with the most difficult part of the manage-
RHEUMATIC FEVER, RHEUMATIC HEART DISEASE
33
ment. Although the convalescent care may take months, we should assure the patient that the restrictions necessary are only temporary. With gradual increase in activities and provision for school work at home or in institutions, we are able to enlist the cooperation on the part of the child and to prevent the development of psychological problems. We have found the sanatorium care for convalescent rheumatic children to be of greatest value and in some cases indispensable. When the infection has become latent or inactive, as judged by clin~ ic-al improvement and return of the sedimentation rate to normal, tJ.1.(! child should be given graded activities, SUcl~U1S sjtting up in a chair f~lLhQll.Ltwic(! daily, t~king his meals at the table, bathroom p.!iY.ik~s., O!l~J'!Ql1J:'up and. around in his room. During this increase in activity, the child should be carefully watched for any signs of rheumatic activity which if it occurs necessitates return to complete best rest. Unnecessary prolonged bed rest imposed on account of the presence of cardiac murmurs or of "heart trouble" is to be criticized. The prevention of recurrences of rheumatic infection is of the greatest importance. Our aim is to keep the rheumatic fever in an inactive state. By so doing we are able to save the child from any further damage, materially prolong its life and return the growing individual to normal life and activity. Resistance to disease may be said to bean indicator of optimal health. To insure health we must carefully supervise the daily routine in regard to the quality and quantity of the diet, determine the safe "carrying load" of work, i.e. studies, bodily exercise and to provide adequate rest and sleep. Reactivation of the disease is usually associated with hemolytic streptococcal infections, therefore these children should be guarded against exposure to infections of this kind. Whenever the rheumatic child acquires an infection it should receive vigorous antistreptococcal treatment in the form of adequate chemotherapy. The sulfonamides should be continued until the streptococcal infection is definitely conquered and then followed by the administration of salicylates. These children should be carefully observed for four to five weeks following streptococcal infections. Continued salicylate medication may mask the presence of an exacerbation. It is, therefore, advisable to discontinue the medication for a period of time and then evaluate the clinical and laboratory findings before deciding if the child escaped an exacerbation and if the rheumatic fever is latent or in an inactive state. . The prolonged prophylactic use of sulfonamides has of late been advocated and used to a great extent. In patients with low resistance and marked susceptibility to repeated streptococcal infections, where control management and isolation has failed to bring about immunity response, the protective use of chemotherapy may be employed. However, we should keep constantly in mind that the use of sulfonamides does not improve the host, their action is directed entirely against
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the attacking streptococcal organisms. '1 'here is also danger that the streptococcal organisms may adjust themselves to the drug, i.e., become sulfonamide-fast. If this occurs and our patients become infected with such strains of streptococci, a very serious situation results. SUMMARY
Rheumatic fever is essentially a ~!~hood disease and it is in the child that the disease presents its greatest variety of symptoms and manifestations. The rheumatic infection produces a chronic inflammatory process of th~~-collagenous connective tissues throughout the body involving first arid foremost the heart, often the joints, the subcutaneous tissue, tli~~'b~ain and 'other-organs. ~,,-, .' The disease has a marked familial tendency and the susceptibility seems to be definitely a heredItary' 'phenomenon following the mendelian law. The onset of rheumatic fever in children is usually insidious and during the active phase of the infection some cardiac involvement is always present. In over half of the cases such involvement is evidenced by clinical findings, in practically all cases it can be demonstrated by cardiographic and fluoroscopic studies. The severity and persistency of the rheumatic infection determines the degree of cardiac damage and the course of the disease. g.!1t:hrocyte sedimentation rate is a reliable test for xtw determination of activity 6f the infection. I,L~hQuldbe routinely employed in aJI rheumatic patients, serving as a guide to treatment, management !!nd prognosis. 'The paradoxical drop. in the sedimentation rate together with enlaJ:g(:lnent of the liver furnishes an early and reliable indication of b_~ginning cardiac failure. The treatment and successful management of the rheumatic child depends on a thorough understanding of the nature of the disease on the part of the physician and continued education of the public. The recognition of the insidious onset of rheumatic fever and its variable clinical manifestations will insure early attention and diagnosis. Treatment during the active stage of the disease consists mainly of lled··..rest, atteptiQn. to .' nutrition and relief of symptoms. S~licylates h!l.Y,e a very beneficial effect on fever, Illllscle and joint pains and s~elJl to have a direct action upon the exudative phase of the pathoIQg!c process. The treatment of acute rheumatic heart disease is essentially the same as that of the acute stage of rheumatic fever. Cardiac failure occurs at the height of the infection and differs from failure commonly seen in adults. G~nvalescent Care should be prolonged over a period of many ~ont~s_aI1d is most successfully carried out in a sanatorium for rheuJBit1~. children,
WILLIAM ELGHAMMER,
M.D., F.A.C.P."
IN presenting these patients I wish to bring out the nature of rheumatic fever in childhood, to consider the findings necessary for the establishment of a definite diagnosis and to discuss rheumatic heart disease. CASE I
_The first patient is a boy, six years of age, who was brought to the Clinic ten days ago because of tiredness, lack of appetite, low grade fever, recurrent nosebleeds, arid _frequent pains in the arms, legs and abdomen. The mother -. stated that the boy had been in good health until six months ago. During the past winter he had had recurrent colds and severe tonsillitis with swelling of the lymph glands of the neck on two occasions.
Fig. 1.-Electrocardiogram in Case I, showing prolonged P-R interval, 032 seconds, and diphasic T wave in Lead Ill. Physical examination revealed a fretful, blond, blue-eyed boy, not acutely ill.· There was a distinctive pallor of his face. General nutrition was below par -and the flesh was soft and flabby. The tonsils were hypertrophic and injected. Several strands of lymphoid tissue were seen in the posterior pharynx. The pos• Professor of Pediatrics and Chairman of the Department of Pediatrics, Loyola University Medical School; Senior Pediatrician, Mercy-University Hospital; Attending Pediatrician, La Rabida-Jackson Park Sanitarium, Chicago.
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H. WILLIAM ELGHAMMER
terior cervical chain of glands were numerous and enlarged. The anterior cervical glands were only moderately enlarged. Tenderness along the sternocleidomastoid muscle was present. The mucous membrane of the anterior nasal septum was congested and on the right side a bloody crust was present, giving evidence of recent hemorrhage. Heart action was regular but rapid, there was no enlargement and no murmurs were heard. This boy is the third child in a family of five children; an older sister had chorea three years ago. The father has rheumatic heart disease; the mother is well. An aunt on the mother's side died of "heart trouble." The patient was admitted five days ago to the hospital for further study and observation. His temperature on entrance was 101 F. (rectal). Subjective symptoms similar to those I have already mentioned were present, with the additional complaint of localized pain in the right knee, left ankle and precordial distress. Laboratory findings obtained in the last few days show red cells 4,200,003, white cells 12,600, hemoglobin 13 gm., 72 per cent polymorphonuclears, 26 per cent lymphocytes, 1 per cent eosinophiIs, 1 per cent monocytes. The urine was clear, specific gravity 1.022; there was no albumin or sugar present and the microscopic examination was negative. Erythrocyte sedimentation rate was 40 mm. in one hour (Landau). Electrocardiogram (Fig. 1) showed prolonged P-R interval, 0.32 seconds, and diphasic T wave in Lead Ill. The tuberculin skin test was negative. Agglutination tests for the typhoid group and undulant fever were negative. 0
Diagnostic Criteria.-As you will notice, the right knee joint and left ankle are tender but not swollen nor red or hot to touch. The apex beat is in the fourth interspace mid clavicular line. There is no cardiac enlargement. The rhythm is regular, the first heart tone at the apex is loud and rumbling but no murmurs are heard. The clinical diagnosis upon admittance to the hospital was focal infection and suspected rheumatic fever. The diagnosis today is active rheumatic fever, mild polyarthritis, and rheumatic carditis. Are we justified in making this diagnosis? We notice in the history the presence of rheumatic infection in the father and the older sister, and that one aunt died of "heart trouble." Rheumatic fev~!J,:a~_~_IJl.~r!.e~f!'mili~lJe'!lde.nfY and iti.s J}eld by som~ tha_~_!.li1s_tre.naency jsdefinitely a hereditary phenomenon following the mendelian 1!1:W. This hereditary predisposition to the disease, although modified by other factors, such as geographical location, nutritional state, and changes in environment, may make it possible to predict the occurrence of rheumatic fever. The presence of rheumatic fever in the family certainly should put us on our guard and as we watch these children we should constantly be on the lookout for the early signs and characteristic manifestations of this disease. Rk~Jf,'matic fever is essentially a childhood disease, and it is in the child t';!!..~~~he dise.ase presents its greatest variety of symptoms and manifestatW1J$. T..h~. highest incidence of ~nset is found between the fifth and qjp.s.hsear. Occasionally in the older child as well as in the adult the onset is sudden, preceded by an attac;k_Qf~9g1ii]liti~, or respiratory inf~ followed bx: seyerJ!_p.:tin, s}Y_elli.!YL~n.d t~t1<.lerness in one or ITIg.re_ioims, aS~Qciated . with high temperature and marked constitutional sYI?2torns. In many of these cases, however, what is regarded
RHEUMATIC :FEVER, RHEUMATIC HEART DISEASE
27
as the onset of the disease is in reality an exacerbation occurring in a previously infected rheumatic individual.
The. vast .rnajori~y ofcasesofJlJis d,iseasf/have an irzsidious. onset.
The-rypicaf sequence of events is well demonstrated in the history and findings of our patient, i.e. tiredness, loss of appetite, irritability, distinctive pallor of the face, vague mild aching pains in the limbs and joints, accompanied by low grade fever and increased pulse rate. These findings are all due to toxemia and may either singly or combined be present in any toxic state, and do not permit us to go any further than the diagnosis of focal infection, toxic state, and suspected rheumatic fever. The additional symptoms of epistaxis, t~nderll~~~.jn s~veral joints, pr~E<2£~!~l ~istress, together with increased sedimentation rate, modenite leukocytosis, and myocardial involvement, as evideiiced by the electrocardiogram, definitely establish the diagnosis of active rheumatic fever, mild polyarthritis, and rheumatic carditis. Many of the symptoms and findings may be produced, as previously pointed out, by other conditions. Childhood tuberculosis may be eliminated by tuberculin tests and x-ray studies of the chest, leukemias, severe anemia and mononucleosis differentiated by morphological blood studies, and other subacute and chronic infections ruled out by the employment of specific agglutination tests. The vague fleeting pains of rheumatic fever, so-called "growing pains," usually occur after rest and on attempt at motion. Th~.<.!l!e r~Heyed by. heat and the administration of salicylates. However, one should keep in mind that similar pain may be associated with other infections or postural defects. It often becomes necessary to differentiate the abdominal pain from acute appendicitis. This is a rather difficult task at times. A careful history revealing some rheumatic manifestations, the occurrence of the pain over a long period of time, together with an increased sedimentation rate enables one to make a diagnosis of "r~eumatic abdomen," and save the patient the risk of surgery. Polyarthritis is not a common manifestation of rheumatic fever in chiIdt:.~n. When it occurs the most common sites are the knees, ankles and wrists, and it is usually rather mild in comparison to that seen in adults. The differentiation from acute poliomyelitis may be difficult, particularly during epidemics of the latter disease. In its severe form one should rule out the presence of osteomyelitis, septic arthritis, leukemia, purpura rheumatica and undulant fever. Chorea is generally accepted as a definite manifestation of rheumatic fever. The onset of this disease is very insidious and mild types may often be overlooked. We should realize that even mild attacks of chorea may be associated with severe rheumatic heart disease. Subcu'tf1:..nef'!!:L?1QduJe.l,lUOSt commonly fQ.und.alollg tendons of ..tl)e ~.C)I~tl1~.l1aD9s., eLl>()\Ys, knees, spine and occipital regloli"·d~tc actjve rheumatic process. These nodules occur in groups and arc US\}a!!Y.-.!mLt.clJ~leLQL.'p;l!nful. . -. . . . . . . . . . .. .
28
H. WILLIAM ELGHAMMER
The major manifestations of rheumatic fever are polyarthritis, chore.!h. sg~C:l:ltan~ol:ls. !1Qd_l:ll~s and ~~!?i~i~: Among the minoL m:otllifestations we include fever, "growinKpains," abdominal pains, epistaxis, erythem~l!!:J:fS:~I!:a!ll~__ and increased se~imentation rate. The presence of rheumatic fever in the family strongly suggests the possibility ot this disease in the progeny. The presence of minor rheumatic manifestations in the child, together with one or more major manifestations, makes the diagnosis of rheumatic fever quite certain. The disease in its inactive state may be recognized by the finding of valvular damage and associated cardiac murmurs or the presence of manifest chorea. CASE 11
Our second patient is a girl ten years of age, who was admitted to the La Rabida-Jackson Park Sanitarium five months ago. Her past history reveals the onset of rheumatic fever at the age of eight years, evidenced by minor manifestations and mild polyarthritis. After three months' rest in bed she apparently had recovered from her active rheumatic fever and was allowed to attend school. In the spring of 1944 she developed chorea of moderate severity necessitating bed rest for six weeks. During the summer she was apparently well, although rather nervous and failed to gain in weight. At the time of returning to school in the fall she was found to have a systolic murmur at the apex transmitted to the scapular region, increased second pulmonic sound, and slight cardiac enlargement. The work in school soon became too difficult, the child, appearing listless and tired, would often refuse to attend school and preferred to rest in bed. She was admitted to the Sanitarium in January 1945 because of weakness, poor appetite, loss of weight, rapid pulse, and afternoon temperature of 101 F. (rectal) (Fig. 2). Physical examination revealed a pale, tired girl. The flesh was soft, the skin moist and clammy. The throat appeared normal, cervical glands not enlarged, lungs resonant and clear throughout. The apex beat, diffuse and rapid, was located in the fifth interspace outside the midclavicular line and there was a slight cardiac enlargement to the right. A prolonged, high pitched, loud systolic murmur was heard at the apex, masking the first heart sound, and an increased second pulmonic sound. Orthodiagraphic tracing confirmed the cardiac enla.\"gement, and showed a prominent left auricle. The electrocardiogram, blood findings, and urinalysis were essentially normal. Erythrocyte sedimentation rate was 32 mm. in one hour (Landau). The patient was given the usual sanitarium care, i.e., absolute bed rest, acetylsalicylic acid, grains 15, with phenobarbital, grain lh three times daily, and offered a standard high protein, high vitamin diet. After three weeks her appetite improved and she began to gain in weight. Color returned to her face, she became cheerful and happy. The sedimentation rate decreased and reached normal values in about six weeks after her admittance. The heart decreased in size as shown by repeated orthodiagraphic tracings. The apex beat became more limited in area, the systolic murmur at the apex less loud and shorter, the first heart sound more audible and distinct. Her progress up to six weeks ago was very good and encouraging. However, in the middle of April she developed a pharyngitis with a temperature of 102 F. lasting three days, followed by loss of appetite. The sedimentation rate, which had remained normal since the latter part of February and throughout her acute illness, became elevated three weeks following the acute attack of pharyngitis, and has ranged between 24 and 40 mm. until four days ago when it dropped sharply to a low point of 5 mm. The sleeping pulse rate, previously 10 to 15 beats below the waking rate, began at the onset of the increased 0
0
29
RHEUMATIC FEVER, RHEUMATIC HEART DISEASE
sedimentation rate to approximate the waking rate. In the last few days the sleeping pulse rate has exceeded the pulse rate present while the patient is awake.
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Fig. 2.-Clinical findings and course in Case H.
Diagnostic Criteria.-As you see this patient here this morning you will notice that she is pale and has a tired, worried look on her face. Her voice is weak and she seems short of breath. The main subjective symptoms are weakness, precordial distress and nausea. Her heart findings are the same as previously observed. However, we find at this time tenderness in the epigastrium and enlargement of the liver to two fingerbreadths below the costal margin. From these findings and the sudden marked drop in the sedimentation rate, we are able to diagnose beginning cardiac failure. May I again point out that we are unable to elicit any further findings in the heart or any sign of passive congestion in the lungs. Considering this case in retrospect, it becomes evident that this patient's heart was irivolved from the very beginning of her rheumatic fever, that the insult to her heart is primarily that of myocardial damage, and only secondarily that of endocardial changes. The myoc:trdial changes which are by far the more serious are directly asso. ciated with the length, severity and recurrences of the rheumatic process. It is evident that in the rheumatic child we are dealing with an active systemic infection. We are concerned with the effect of this infection upon the connective collagenous tissues throughout the body and especially within the myocardium. Valvular changes may take
30
H. WILLIAM ELGHAMMER
place in due time, produced by proliferative and scarring lesions of the endocardium covering the leaflets, but they are only of secondary importance. The presence or absence of murmurs per se is of little diagnostic or prognostic significance. The correct determination of the activity of the infection is of paramount importance in order to treat these patients adequately, to know when they may be given ambulatory activities and to evaluate their progress and their prognosis. Aside from clinical improvement, the subsidence of fever, the return of the blood picture to normal, and normal relation between waking pulse rate and sleeping pulse rate, we have found the erythrocyte sedimentation rate to be in most cases a reliable test for the determination of the activity of the rheumatic infection. In all cases of rheumatic fever the sedimentation test should be employed routinely, repeated at weekly or biweekly intervals. By this we may follow the progress of the disease, detect exacerbations early, even before clinical findings are evident, and as in this case determine beginning cardiac failure. It has been our experience that cardiac failure almost always occurs at the height of the infection, rarely if ever, as in adults, from myocardial strain imposed upon the heart from valvular lesions or general bodily exertion. The paradoxical drop in the sedimentation rate at the inception of cardiac failure has not as yet been satisfactorily explained. It is of interest that the enlargement of the liver occurs simultaneously with this drop in the sedimentation rate and prior to any other signs of increased venous pressure or cardiac changes. Clinically the enlargement of the liver and the presence of tenderness in the epigastrium are of greatest iiiJportance and serve admirably as a guide in estimating the functional . state of the heart. TREATMENT
The treatment and successful management of the rheumatic child depends on a thorough understanding of the nature of the disease on the part of the physician and continued education of the public. The recognition of the insidious onset of rheumatic fever and its variable clinical manifestations will insure early attention and diagnosis. A general understanding of the chronicity and tendency to recurrence of the disease will promote careful medical supervision and thorough periodical physical examinations over a long period of time which is most essential in order to achieve success in the fight against this common crippling disease of childhood. Prophylactic Treatment.-The various predisposing factors met with in the etiology of rheumatic fever suggest many measures to be considered in the prophylaxis of this infection. Adequate clothing, avoidance of chilling and exposure to dampness and cold are of great importance. A well-balanced daily routine in regard to play, work and rest, and regularity of meals is very essential. Unfortunately children of preschool age are often allowed to dispense with the afternoon rest, many
RHEUJ\IATIC FEVER, RHEUMATIC HEART DISEASE
31
are sent to the kindergarten in the afternoon and are occupied with additional work, such as music lessons and dancing. Recreation of a negative form, such as movies and radio programs, are allowed to occupy an excessive portion of the child's daily life. Children with history of rheumatic fever in the family should be given particular attention. Common colds, pharyngeal infections, tonsillitis and childhood diseases, as we well know, often initiate rheumatic infection. Convalescence from these diseases should be prolonged beyond the time usually allowed and we should be on the alert for the appearance of early signs and manifestations of acute rheumatic fever. lJ;_~_2~~!:aE!iv~_ n.~~V()':ls child, a~'!th~J:_.C()lllIl}OIl c!ini<:.a,!. en~ity, whQ d~1!()~.!:(!S2011g.to_the'ordiilary management of rest ana routine, is often found to have a chronic infection of the upper respiratory trac~. Such conditions as repeated colds, otitis media and tonsillitis, I think should be given particular attention. The removal of chronically diseased tonsils and adenoids is not in itself a means of preventing rheumatic infection. I think that there are times when the removal of tonsils and adenoids mav be harmful and even disastrous to the child. However, if the child presents no systemic infection, during the favorable season of the year-th~t is, @lysummer-definitely diseased tonsils and adenoids should be removed from standpoint of general health and of relieving the child of this burden. . We are accustomed to regard a normal gain in weight as being an indication of optimum health, therefore we should give particular attention to the child who is not making the usual increase in growth and weight. Being undernourished increases the hazards of the child in regard to acute rheumatic infection. . Since a cold and damp climate frequently plays a p!:ominent role in tIte_predisposing etiology, it may be advisable to remove certain types oL£hild..r~rf to a warmer climate in order to save them from acquiring t~:disease. . Treatment During the Active Stage.-Treatment of rheumatic fever during the active stage consists mainly of bed rest, attention to nutrition and relief of symptoms. ~s.~ a.nej.. relax'!.tion are necessary to rcduce metabolic expenditure and to promote the development of the natural defense mechanism. Merely keeping the child in bed, oftcn under duress, is not inducive of rest and relaxation. The child as well as. the family should be informed of what we are trying to aCCOlllplish, the program well explained and accepted both hy the patient and those who are to attend and care for the child. A general diet, moderately low in carbohydrate, with the additioll of vitamin n in the form of cod liver oil, will suffice. Of late a great number of vitamins have been suggested but I think we can truthfully say that a well balanced diet has proved quite satisfactory. A
32
H. WILLIAM ELGHAMMER
high carbohydrate diet tends to induce sudden increase in weight but not the type of growth that is indicative of resistance. Salicylates in some form or other are most commonly employed. They have a very definite beneficial effect on fever, muscle and joint pains and seem to have a direct action upon the exudative phase of the pathological process. However, we cannot ascribe any specific action to this drug. Moderate dosage, such as 10 to 20 grains three times daily, depending upon the severity of the disease and the age of the child, is to be preferred. Salicylate poisoning although rather rare should be watched for. Chemotherapy has given universally disappointing results and we may state that. sulfonamides are distinctly GQJ:ltraindicated during the active stage orthe disease. .. The treatment of chorea has undergone many variations. Fowler's solution so popular years ago has been entirely discarded. Nirvanol with its toxic reactions likewise has been discontinued as well as fever therapy, due to the fact that the treatment in most instances is more severe to the patient than the disease itself. Bodily as well as mental rest with the use of sedatives, such as phenobarbital, bromides and chloral hydrate, has proved to give the best results. W ((pave found. warm bat.~~_or.'Ya!mp3:<::ks given twice daily to be very beneficial. lIieTnducement of purposeful movements, such as attempts by the patient to feed himself, to handle objects and to walk around the bed, has shortened the period of incoordination and muscular weakness. Treatment of Acute Rheumatic Heart Disease (Carditis).-The treatment of acute rheumatic carditis is essentially the same as that of the acute stage of rheumatic fever. As previously pointed out, heart failure in children differs from that in adults in that the infection is the principal cause rather than mechanical stress and strain. In other words, cardiac failure or decompensation always occurs at the height of the infection. Rls:.~!sid~diajlure withdyspnea is the common type. We rarely see generalized edema. Absolute bed rest is imperative but we should allow the patient to assume whatever position he finds most comfortable. He should be handled cheerfully, allaying his fears with reassurance. Salh;ylates should be administered if tolerated, together with sedatives, even codeine and morphine, if deemed necessary. In cases. without demonstrab}_e_.~c!ema the use of diuretics, such as salyrgan,6:S to i cc. intravenously eveii· tnri£diiys, or theocalcin, 7Yz grains twice or three t!mes. daily, often prove to be very effective. We have had very disappointing results with the use of digitalis and feel that in some instances the administration of this drug has shortened the patient's life. The i!l~~venousinjection of a 20 per cent glucose solution is an ex~I1tsuPpOriiye-tr~atment. . .. ... Convalescent Care.-When the patient is free of pain and has recovered from the acute stage of the disease, and begins his convalescence we are confronted with the most difficult part of the manage-
RHEUMATIC FEVER, RHEUMATIC HEART DISEASE
33
ment. Although the convalescent care may take months, we should assure the patient that the restrictions necessary are only temporary. With gradual increase in activities and provision for school work at home or in institutions, we are able to enlist the cooperation on the part of the child and to prevent the development of psychological problems. We have found the sanatorium care for convalescent rheumatic children to be of greatest value and in some cases indispensable. When the infection has become latent or inactive, as judged by clin~ ic-al improvement and return of the sedimentation rate to normal, tJ.1.(! child should be given graded activities, SUcl~U1S sjtting up in a chair f~lLhQll.Ltwic(! daily, t~king his meals at the table, bathroom p.!iY.ik~s., O!l~J'!Ql1J:'up and. around in his room. During this increase in activity, the child should be carefully watched for any signs of rheumatic activity which if it occurs necessitates return to complete best rest. Unnecessary prolonged bed rest imposed on account of the presence of cardiac murmurs or of "heart trouble" is to be criticized. The prevention of recurrences of rheumatic infection is of the greatest importance. Our aim is to keep the rheumatic fever in an inactive state. By so doing we are able to save the child from any further damage, materially prolong its life and return the growing individual to normal life and activity. Resistance to disease may be said to bean indicator of optimal health. To insure health we must carefully supervise the daily routine in regard to the quality and quantity of the diet, determine the safe "carrying load" of work, i.e. studies, bodily exercise and to provide adequate rest and sleep. Reactivation of the disease is usually associated with hemolytic streptococcal infections, therefore these children should be guarded against exposure to infections of this kind. Whenever the rheumatic child acquires an infection it should receive vigorous antistreptococcal treatment in the form of adequate chemotherapy. The sulfonamides should be continued until the streptococcal infection is definitely conquered and then followed by the administration of salicylates. These children should be carefully observed for four to five weeks following streptococcal infections. Continued salicylate medication may mask the presence of an exacerbation. It is, therefore, advisable to discontinue the medication for a period of time and then evaluate the clinical and laboratory findings before deciding if the child escaped an exacerbation and if the rheumatic fever is latent or in an inactive state. . The prolonged prophylactic use of sulfonamides has of late been advocated and used to a great extent. In patients with low resistance and marked susceptibility to repeated streptococcal infections, where control management and isolation has failed to bring about immunity response, the protective use of chemotherapy may be employed. However, we should keep constantly in mind that the use of sulfonamides does not improve the host, their action is directed entirely against
34
the attacking streptococcal organisms. '1 'here is also danger that the streptococcal organisms may adjust themselves to the drug, i.e., become sulfonamide-fast. If this occurs and our patients become infected with such strains of streptococci, a very serious situation results. SUMMARY
Rheumatic fever is essentially a ~!~hood disease and it is in the child that the disease presents its greatest variety of symptoms and manifestations. The rheumatic infection produces a chronic inflammatory process of th~~-collagenous connective tissues throughout the body involving first arid foremost the heart, often the joints, the subcutaneous tissue, tli~~'b~ain and 'other-organs. ~,,-, .' The disease has a marked familial tendency and the susceptibility seems to be definitely a heredItary' 'phenomenon following the mendelian law. The onset of rheumatic fever in children is usually insidious and during the active phase of the infection some cardiac involvement is always present. In over half of the cases such involvement is evidenced by clinical findings, in practically all cases it can be demonstrated by cardiographic and fluoroscopic studies. The severity and persistency of the rheumatic infection determines the degree of cardiac damage and the course of the disease. g.!1t:hrocyte sedimentation rate is a reliable test for xtw determination of activity 6f the infection. I,L~hQuldbe routinely employed in aJI rheumatic patients, serving as a guide to treatment, management !!nd prognosis. 'The paradoxical drop. in the sedimentation rate together with enlaJ:g(:lnent of the liver furnishes an early and reliable indication of b_~ginning cardiac failure. The treatment and successful management of the rheumatic child depends on a thorough understanding of the nature of the disease on the part of the physician and continued education of the public. The recognition of the insidious onset of rheumatic fever and its variable clinical manifestations will insure early attention and diagnosis. Treatment during the active stage of the disease consists mainly of lled··..rest, atteptiQn. to .' nutrition and relief of symptoms. S~licylates h!l.Y,e a very beneficial effect on fever, Illllscle and joint pains and s~elJl to have a direct action upon the exudative phase of the pathoIQg!c process. The treatment of acute rheumatic heart disease is essentially the same as that of the acute stage of rheumatic fever. Cardiac failure occurs at the height of the infection and differs from failure commonly seen in adults. G~nvalescent Care should be prolonged over a period of many ~ont~s_aI1d is most successfully carried out in a sanatorium for rheuJBit1~. children,