Rheumatic heart disease: IV. The life history of the severe form of the disease

RHEUXATIC IIEART DISEASE

IV. THE LIFE HISTORY OF THE SEVERE FORM OF THE DISEASE”

DAVID DAVIS, M.D., AND SOMA WEISS, M.D. BOSTON, MASS.

CLINICAL DURATION OF RHEUMATIC INFECTION

K NOWLEDGE of the time of onset of rheumat.ic infection and the age at death is essential in a study of the life history of rhen-

matic heart disease. In previous communications’ the age distribution at death in a group of patients with rheumatic heart disease was presented.l, ’ Corresponding statistical data showing the onset of the rheumatic infection leading to heart disease are difficult to obtain because of the well-recognized fact that advanced cardiac damage not infrequently occurs without any recognized clinical manifestation of the rheumatic infection, and because at times cardiac lesions precede the first recorded attack of rheumatic fever. Some idea of the duration of the disease can, nevertheless, be obtained by assuming that the first recorded attack of rheumatic fever represents the time of onset of the infection. The error in such an assumption would make the average duration of the disease longer than that indicated by the clinical data to be presented. This error is counteracted, on the other hand, by the fact that in some instances the onset of cardiac damage may have been independent of the first attack of rheumatic fever.

The duration of rheumatic heart, disease was studied in a group of patients with neeropsies, in whom rheumatic heart disease was even- tually the cause of death. An earlier analysis showed that of 369 patients with unquestionable rheumatic heart disease, 44 per cent died of this disease; in 11 per cent rheumatic heart disease was a contrib- uting cause ; in 17 per cent death was due essentially to subacute or acute bacterial endocarditis on a rheumatic basis; and in 27 per cent it was due to causes not associated with rheumatic heart disease. The data presented in this communication bear only on cases in which death was caused by rheumatic heart disease.

Aye of Onset. The clinical data in 108 cases wit,h necropsies clearly indicated whether or not there had been a previous rheumatic infection. Eighty-t.hree of these cases, 40 males’ and 43 females, gave a definite history and 9 a suggestive history of either rheumatic fever

*From the Thorndike Memorial Laboratory, Secon,d and Fourth Medical Services (Harvard), Boston City Hospital, and the Department of Medicine, Harvard Medical

School.

486

UP chorea. In 16 cases there was no rec,ollection of any previoas c~h~im-~ matic manifestation. Thirty additional cases were included with a, definite history of rheumatic infection but withont necropsg studies., making a total of 113 cases with positive rheumatic. histories.

TABLE I

- -__ AGE GROUP

Pears O-10

IS-20 91-30 31:40 41-50 31-60 61-70 71-50

----- ~- ___.---- CASES -.--.--

Per Cent ‘7.4 40.8 14.1 i2.P

3.5 0.9 0.9 0.0 -__

Table I shows the distribution according to a.ge of onset of ~,heu- maric fever or chorea. The earliest age of onset was three years; in 3 cases infection was acquired at five years. The majority of instances in the first decade, however, occurred nearer the ape of ten years. In accordance with general knowledge, a high percentage was noted in the first and second decades, 77 patients having had their first a,ttack before the twentieth year. -4 smaller but appreciable group appzr- ently acquired the infection in the third and fourth decades, a!rd 3 in the fifth decade. In one instance t,he first. and fata.1 attack oceur~ed in the sixty-first year, and gross and histological evidence of a. typical acute rheumatic pancarditis was found on post-mortem examination.

Rdatio~z of .First Attack fo Chset of Cardiac Damage. Some oppor- tunity to answer the qnestion as to whether the first recorded attack of rheumatic fever was coincident with the earliest cardiac damage was presented in 10 cases in which a first attack of rheumatic fever rrsnited in a. fatal attack of heart disease. In 5 of these 10 cases, valxlar and myocardial lesions mere entirely acute; there was no yross or microscopic evidence of a past infection as indicated. by c!:ronie inflammatory reactions. In the group showing evidence or” old rheumatic heart disease the degree of involvement was slight in 3 patients and marked in 2. The ages of the 5 patients with acute iesions were 13, 22, 35, 35, and 61 years; file ages of those showing fresh as well as old lesions were 11: 15, 19, 22, and 35 years. The duration of illness in 8 of these 10 patients was one month or less; in

2, six and eight months. ln this group of 10 cases, then. the onset of tile first recorded rhea-

mattic infection corresponded with the onset of demonstrable rheamatic ileart disease in but 50 per cent. Deductions as to the significance of the first recorded attack cannot be drawn from so small a group. it

488 THE AMERICAK HEART JOURNAL

is interesting, however, that onset of rheumatic carditis should so often-in 4 out of 5 cases-be associated with an attack of rheumatic fever occurring for the first time in adult life.

Duration of Life After Pimt Attack. The duratioli of life after the first recorded attack of rheumatic fever in 83 fatal cases is shown in Table II. Twenty-five patients lived five years or less, 6 having died from the initial attack and 8 in less than two years from the time of onset. The remainder showed a dnration of life ranging from six to

TABLE II

DURATION OF LIFE AFTER FIRST ATTACK IN 83 CASES WITH NEC~OPSY RECORDS -

DURATION OF LIFE CASES - Pears NO.

o- 5 25 6-10 10

11-15 10 76-20 13 21-25 10 26-30 5 31-35 36-40 :

forty years after the earliest manifestation. As would be expected, rheumatic cardiac activity at death is more frequent in patients dying a short time after the first attack of rheumatic infection. In an unselected group of 21 patients in whom death occurred within three years a.fter onset of infection, activity was noted in 18; in a second unselected group comprising 19 paCents, who had lived twenty-five or more years after the first infection, activity was noted in 9.

TABLE III

DURATION OFLIFE IN 36 CASES ~+RHEU&IATIC FEVER ACCORDING TO AGE AT OGXET OF FIRST ATTACK

18 CASES WITII ONSET BEFORE 18 CASES WITH ONSET AFTER AGE OF lo AGE OF 26 -

Pean-s Pea,m 2 0 2 0 ; 0

10 Y 12 4 14 5 15 7 15 8 18 8 20 10 20 10 21 .' 12 27 20 32 20 32 20

34 35 2":

DAVIS A?r’D WEISS : R.HEUM;i’Tli\ YEAI:T DISEASE -sd3

There is a general belief that rheumatic infection is more &Uil&&li~

to the myocardium in the early years of life than when it ocwvs for t,he first time after the a.ge of twenty-five years. Table III gives the duration of life in two groups: one, in which the first attack occurred at or before the age of ten; the ot.her, in which the first attaolr occurred at or after the age of twenty-six fears. In the latter group the duration of life was appreciably shorter, and death followed soou after the first attack of rheumatic fever in 5 cases, mhereas in the first group no immediate fatality occurred. The duration of life was a,p- proximately 30 per cent longer in Group 1. This finding suggests that the rheumatic infection is more damaging to the heart when the first attaek occurs in an adult. Another possible explanation of the difference, however, may be that after adult yea= the first recorded attack of the rheumatic infection is less likely to represent the arue onset.

DURATIOK OF HEART FAILURE

An analysis was made of the duration of the cardiac symptoms, as indicated by the duration of the “present illness” in 134 patients even- tuaXy dying as a result. of rheumatic heart disease. The “present illness, ” as employed in this study, covers the period from the ocsef, of signifimnt and proyressive symptoms to the time of death. In some instances in which the patients gave a. history of having had symptoms for many years, the exact time of onset was not clear, either because of the insidious character of the progression, or because, as often happens, patients who are informed in ea,rly youth that they have heart disease go through life with a mild nearosis or feay of over- exertion, and date the onset of symptoms from the time of the medical diagnosis. In other and rather frequent instances there is a, t,rue btrt slight limitation of the cardiac reserve which remains stztionary for many years, after which a definite downhill progression of symptoms c~ecurs. Eleven of the 134 cases studied for the duration of sympr,oms showed an initial period of several years in which there was either such mild limitation of circulatory efficiency or a neurosis rna~i~e~~~~~ itself as such. The prelfminary period in these 11 ca,ses was not re- garded as a part of the present illness,

The character of onset and the course of cardiac manifestations shojved wide variation. The following are characteristic clinical groaps :

1. Acute Hea.& Failwe and Rapid Death. The illness is of s&den onset with severe pulmonary symptoms in a patient previously in good health. The patient. is forced to bed with marked dyspnea, orthopnea, cyanosis, pulmonary congestion and edema, and dies within from two da,ys to six weeks.

2. Se&e or Subaczcte Head Failure With Recove~By. There is a relatively sudden onset of weeks or months in &ration, as in Group 1: but

490 THE BNERICAK HEART JOURNAT,

recovery occurs. The recovery is either complete xjthin a few weeks, with freedom from all symptoms, or partial, with persisting dyspnea on exert.ion and limitation of cardiac reserve. Prognosis is difficult, for death may occur at any time within from three months to ten years, and after one or more attacks of failure. It may occur as a climax to increasing limitation of reserve, or precipitately as acnte failure.

3. Praogmssive Heart Failure. In this group the onset is gradual, with slowly progressive failure over a period of from one to three years. Recovery is slight or ins,ignificallt in spite of prolonged bed rest. The cardiac reserve steadily diminishes and death ensues.

4. Crccd~ual Onset of Heart Failure With Recovery of Xhort Duration.

The onset is as in Group 3 but with some recovery after prolonged rest and digitalis. There is relative comfort for a period of several months, but rarely longer; failure then occurs and death ensues.

TABLE IV DURATION OF HE~~RT FAILURE IN 131 FATAL CASES

DURATION OF FAILURE

Moritlas 1 or less 6 or less

12 or less PeaYs

1 or more 3 or more 5 or more

10 or more 15 or mole

CASES

No. Per Cent 36 27 62 46 78 58

56 42 44 33 10 14

5 4 3 2

Table IV presents the distribution of the 134 cases according to the duration of “ present illness. ’ ’ Twenty-two paCents died within two weeks from the time of onset of definite cardiac symptoms; 36 within one month ; 62 within six months; and 78, or more than 50 per cent, within one year. Forty-four cases showed a cluration of illness of three or more years, 19 cases of five or more years, and 5 cases of ten or more years.

TABLE V

DURATTOK OF LIFE ~PTER ONSET OF COKGESTIVE FAILURE IN 56 CASES WITH To.rar, ILLNESS EXCEEDIKG 1.5 YEARS

DURATIOX OF LIFE

Months 1 or less 2- 6 7-12

Pears 1.5-3 3.5-5 G- 10

.- CASES

-! NO. 11 14

4

16 6 5

urutio7b of Congestive Pa/&cm. It is of prognostic imi.‘ortaw.X? ‘0 obtain information as to the expectancy of life after the onset of definite congestive failure necessitating rest in bed. Accordingly. the duration of life in this stage of failure was analyzed in a group of 56 cases with a total duration of illness, including the period preceding the conges- rive failure, of one and one-half years or longer (Tab1.e V). In some of these ca,ses there were one or more intervals of relative improve- nrent after the onset of severe failure. In 27 eases, or approximately one-half, congestive failure persisted fey more than one and one-half years. In 11 cases, death occurred aft,er fa,ilare had been present one month or less

Facto,rs Deteuniwing an Acute Fulntinnt~i~~g COWSC. Onr of the striking findings among these paGents with rheumatic heart disease was rhe high frequency of acute cardiac deaths. Some of the factors which may be responsible for such a fulminating eours~ are: active rheumatic infection? infections other than rheumatic, embolism, and pu!- monary complications, including sudden general or left-sided failwe with pulmonary edema. Xex apparently is not a significant factor, 5inee in a group with an illness lastin, 0- six mont,hs or less there were 33 males and 31 females, and in a gro~1p with an illness of two years or more, 23 males and 32 females. ,4ge, likewise, does not appear to be a related factor. A correlation of the clinical course with the posi- nlortem findings, on the other hand, suggests that the presence of ccctivity, and particularly the site and degree of the activity, is of im-. pcrta,nce. Thus, in a group of 36 patients with an illness of one month or less: activity was present in 26. In a proup of 29 patients with an iilness of four years or longer, there was activity in 5, questionable aetirit,y in 5, and absence of active infection in 19. A comparison of these groups suggests that active carditis: if sufficiently- severe, may precipitate a. rapid downhill course.

Tn. some patients enzbobis~~~ obviously resulted in a rapid course. Em- holism usually occurs in the presence of auricular fibrillation: early

TABLE VI

CAGSE OF DEATH IN 36 CASES WITH a CARDIAC ILLNESS OF CSE ?vIONTH On Less IK DURATION

~_____ --.-- - -- OATlSE OF DEATH x0. OF CASlm

Embolism 8 F’ailure with active carditis 8 Failure without active oarditis 5 Failure with active carditis and ternkal infection 4 Failure with active car&is and pulmonary ateiectasis 3 Failure without active carditis and with terminal infection 2 Failure with active carditis and acute nephritis 2 Failure with pregnancy I Failure with embolism and infection 1 Purulent pericarditis Unknown : _____- -..--

492 THE AMERICAN HEART JOURNAL

or late in the course of congestive failure.3 Thus in the group of 36 pat,ients with an illness of one month or less, significant. embolism oe- curred eight times, as compared with seven times in a, group of the same size with an illness of four years or longer.

Table VI shows the clinical state just before death in 36 patients with a fulminating course. Embolism was responsible for death in 8 patients. General failure was’ present in 26, and in 17 of this number active carditis was found. In ‘7 of the 17 with active carditis, terminal infection, nsually bronchopneumonia, occurred. In 3 patients with general failure and carditis, significant pulnionary atelectasis was found at the post-mortem examination. In 5 patients the final failure was unaccompanied by infection. In 1 patient general failure was apparently precipitated by the terminal months of pregnancy. In 2 patients general failure with active carditis and acute nephritis coexisted. The exact r8le of the acute nephritis in the causation of death was difficult to evaluate.

An analysis of the degree of vn,lvular danbage in the same 36 patients showed moderate valvulitis with stenosis of either mitral or aortic valves in 14 patients and marked changes in 2. The remaining patients showed only minor l.esions of the valves, stenosis being only slight or absent in most instances. The death of paCents not dying of such complications as embolism was, therefore, in all probability due to myocardial damage. The condition of the myocardium in 17 patients may have been significantly aggravated by. t&e active rheumatic infect.ion. In the 2 patients with marked valvular lesions there was no evidence of active infection, and it is probable t,hat death was due chiefly to mechanical failure.

DISCUSSION

This study, as well as the data presented in previous communications,ll 2 confirms the common clinical observation that the course of rheumatic heart disease shows great variation. In the entire group of 474 cases there were 269 in which death was due to causes other than rheumatic heart disease.l In these cases, as well as in those in which cardiac damage was only partially responsible for death, it was not feasible to study the life history of the disease. The clinical course of rheumatic heart disease, as observed in the cases here analyzed, is that characteristic of cases in which cardiac damage even- tually leads to death; hence the data presented are not comparable with those obtained from the study of patients irrespective of the degree of cardiac involvement.4a 5

The distribution of cases in the present group according to the age at onset of rheumatic infection corresponded closely with that in the group observed clinically by Willius.F Analysis according to duration of life after onset of rheumatic fever showed an approximately equal

distribution in the group less than two years, the gronp t\~o tt: i-it’ years, and in the five-year periods up to twenty-five pears. A smaiiIe~ number of patients lived as long as forty years after the first infee- tion, ultimately succumbing to the hea,rt disease. Since duration of life varies so greatly, one cannot speak with justification of the average duration of life after t,he first infection. Prognostication as to expectancy of life after the first infection is, therefore, not feaeiblr. In accordance with the findings of Willius,6 there is some tendency toward a shorter duration of life wheu the first infection deorlops after the age of twenty-five years than when this occurs before the age of ten years. This difference cannot he esplained by the shortea~ expectancy of life after the age of twenty-five years. A probable in- terpretation is that adults are less tolerant to rheumatic cardiac ilk--

feetions.

The fact that in many eases the duration of life is short follo\rillg the appearance of cardiac symptoms, and particuiarly following the onset of congestive failure, is significant. Thus, of the patients pre- senting these symptoms, 58 per cent were dead within a yea.r after the onset of cardiac symptoms. As high as 27 per cent, exhibited oartliac symptoms. or signs of less than one month’s duration. Age and sex, as well as the distribution and the degree of valvalar damage, played no significant r61e in the precipitation of this rather acute type of cardiac death. Acute rheumatic myocarditisl embolism, and acute infections other than rheumatic fever were the most frequent precipi- tating factors. Lams and Levine’ have also emphasized the freqtrenc) of death resulting from causes other than chrovie failure of the IU~:I- cardium.

SUMMARY

1. Data are presented bearing on the course of fatal rheumatic heart disease as obtained from the records on 113 cases and 83 necropsp examinations.

2. The age at onset of the first rheumatic infection varied frctrxr three to sixty-one years, the majority of cases occurring in the first and seeond decades.

3. The duration of life after the onset of clinica! evidence of rh~u- rnatic infection varied from a fen- weeks to forty years. About 50

per cent of the patients lived from six to twenty-five years, the nnm- ber of cases showing fairly even distribution according to five-year intervals.

4. More than 50 per cent of the patients died within one year after xhe onset, of definite cardiac failure.

5. The clinical types of heart failure enconntered a,re described, and the high frequency of relatively short duration of life followi~~g the onset of significant cardiac symptoms, and particularly of congestive

494 THE AMBXICAK HEART JOURNAL

failure, is analyzed. Almost one-third of the patients succumbed as a result of a fulminating course precipitated by factors other than chronic myocardial failure.

6. In the fulminating fatal course acute carditis, embolism, and infections other than rheumatic fever played the most significant r8les.

REEXRENCES

1. Davis, D., and Weiss, S.: Rheumatic Heart Disease: I. Incidence and R81e in the Causation of Death. A Studv of 5.215 Consecutive Neeronsies. AM. HEART . , A I J. 7: 146, 1931.

2. Davis, D, and Weiss, S.: R,heumatic Heart Disease: II. Incidence and Distribu- tion of the Age of Death, Ax H&ART J. 8: 152, 1932.

3. Weiss, S., and Davis, D.: Rheumatic Heart Disease: III. Embolic Manifesta- tions, AK HFART J. 9: 45, 1933.

4. Grant, R,. T.: After Histories for Ten Years of s, Thousand Men Suffering from Heart Disease. A Study in Prognosis, Heart 16: 275, 1933.

5. Coombs, C. F,: Rheumatic Heart Disease, Bristol, 1924, John Wright & Sons, Ltd.

6. Willius, F. A.: A Study of the Course of Rheumatic Heart Disease, AN. HEART J. 3: 139, 1927.

7. Laws, C. L., and Levine, S. A.: Clinical Notes on Rheumatic Heart Disease With Special Reference to the Cause of Death, Am. J. M. SC. 186: 833, 1933.

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Rheumatic heart disease: IV. The life history of the severe form of the disease