Professional Documents
Culture Documents
HE STUDY which forms the basis of operation of the cardiac clinics and social serv-
this report was organized in 1928 (by ice departments of the general hospitals in the
T. D. J.) as a long-term project. It had Boston area has been remarkable, as has that
for its purpose three main objectives: (1) to of the family physicians. All have rendered
define within broad limits the course of rheu- wholehearted support, and in its over-all im-
matic fever, (2) to elucidate the evolution of plications this program has heeni truly a (om-
rheumatic heart disease during its formative mullity project.
years. and (3) to appraise certain features of A series of studies on special aspects of rheu-
the disease bearing on prognosis. With these matic fever and rheumatic heart disease based
ends in mind it has been carried forward with- upon this material was presented between 1 935
out interruption during the intervening years. and 1940. They emphasized some of the less
The experience recorded herewith is based well known features, including the clinical sig-
upon 3000 children and adolescents who have nificance of chorea,1 the disappearance of phys-
received protracted hospital care for rheumatic ical signs of rheumatic heart disease,2 the (de-
fever at the House of the Good Samaritan in layed appearance of valvular (lisease,3 the
Boston since 1921. In the present report we are development of pure mitral steniosis,4 and fatal
concerned only with the original 1000 patients rheumatic fever.' Finally, at the end of the
who entered the series between 1921 and 1931. first 10 years of the program an informal and
At least 20 years have now elapsed for this tentative report of pr ogress wsas made.6*
entire group. During this interval they have Since these earlier reports, another decade has
been followed in special clinics organized for passed and 20 years have now elapsed since the
this purpose at the above institution and at the initial observations. We have been privileged,
Massachusetts General Hospital. The majority with the aid of numerous associates, to follow
were seen at six to 12 month intervals, but this group from childhood through adolescence
more often when ailing. Readmission to the into early adult life. Their cooperation and
hospital when possible was the general policy confidence through the years have made
for recurrent rheumatic fever. The active co-
*
Since this earlier report a few of the original
From the House of the Good Samaritan and the group were re)lace(1 in sequence either because of
Massachusetts General Hospital, Boston, Mass. reasonable (loul)t as to the rheumatic nature of their
Presented in summarv at the First International original illness or because they were beyond the
Congress of Cardiology, Paris, September, 1950, arbitrary limit of 20 years at the time of onset estab-
and before the Twenty-fourth Annual Scientific lished for the series; hence the slight (liscrel)ancy
Session of the American Heart Association, Atlantic between the earlier and the l)resent figures l)earin(g
City, N. J., June, 1951. on the first 10 years.
836 Circulation, Volume I V, December, 19,51
E. F. BLAND AND T. D. JONES 837
possible this study and their contribution has fully followed patients for the 10 year inter-
been the basis of a better understanding of this val and eliminates the uncertainties of average
disease. figures and prediction tables.
At the end of this 20 year interval it now The studies of Wilson bearing upon prognosis
seems appropriate to render a second interim date back to 1916. She (in association with
report, to summarize the major events of the Lubschez) recently reported on her 30 years'
two decades, and to compare and contrast experience with 1042 children observed be-
these observations with the experience of tween 1916 and 1947.9 In contrast to Ash's
others elsewhere. and to our own approach, she reports in terms
of an "average" follow-up of 14.8 years and
COMPARABLE STUDIES employs the "life table technic" of Reed and
As a background for comparison, the early Merrill to predict the eventual outcome. In
observations of Cary Coombs in England, her series there were 226 deaths, of which 73.7
begun before World War I and completed in per cent were due to rheumatic heart disease
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1922, represent perhaps the first serious at- and 10.2 per cent to subacute bacterial en-
tempt by long-term observation to assess the docarditis (2.2 per cent for the entire group).
outcome of childhood rheumatism.7 The war The highest mortality occurred (as is to be
years cut directly across his studies and expected) in the first year, with an increased
rendered follow-up doubly difficult. However, risk at puberty. She estimated that an af-
of anl initial group of 253 cases with cardiac fected child has four chances out of five to
involvement, he was able to trace 218 for the survive 15 years after the onset of rheumatic
first year, 204 for five years, and 177 for 10 fever, three out of four to survive 20 years, and
years, with a mortality of 5.1, 11.2, and 21.4 two out of three to survive 30 years, with an
per cent respectively. He found 31 per cent over-all chance of one out of two to survive
recovered completely-a somewhat higher fig- 40 years.
ure than subsequent studies have revealed. The most satisfactory data on the progress
Later studies by Ash inl Philadelphia (1948) of rheumatic heart disease in the adult period
and Wilson inl New York (1948) are notable have been assembled by Grant from the
and serve as a basis for comparison with our earlier observations of Lewis.'0 The series
own experience inl Bostoni. There has been a consists of 1000 ex-soldiers with valvular
remarkably close agreement inl the three series heart disease, pensioned from the British
as to the outcome. Inl contrast to these care- Army after World War I, whose after-history
fully followed childhood groups, adequate for a 10 year period was followed by Grant, and
observationis on the course of rheumatic heart his associates (1933). This represents a slightly
disease inl the older decades have been meager, biased group, in that it concerned only mel,
and studies of fatalities rather than of sur- and other nonrheumatic types of -valvular
vivors have warped our perspective unfavoi- heart disease also were included. However, it
ably; the notable series of Grant (1933) is the serves as a well studied sampling of rheumatic
exception. heart disease in the third aiid fourth decades.
Reporting from the Children's Hospital in Pertinent data from Grant's study rearranged
Philadelphia, where a register had been kept for our purpose are summarized in table 1.
since 1922 of all patients treated for rheumatic It appears that in the decade from 30 to 40
fever, Ash assembled follow-up data for a full years of age the course of rheumatic heart
10 years on each of 537 children whose age at disease is characterized by three approximately
onset averaged 6.9 years.8 At the end of this equal groups: (1) a third remains unchanged,
10 year interval, 24.4 per cent died of rheu- (2) another third progresses, and (3) a final
matic infection, and 12 patients (2 per cent) third succumbs. From this study Grant coii-
died of bacterial endocarditis. This study by eluded that the outlook is not so bad as is
Ash is commendable, since it is restricted to generally thought.
838 RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE
instances, from family physicians; and in 29 37 per cent, and 27 per cent mortality in the
instances information concerning their earlier 20 year period. In contrast, chorea character-
illness was obtainable only from the family. istically was associated with a benign form of
TABLE 1.-The Course of Rheumatic Heart Disease in Adults
Ten Year Data-Grant, 1933
After History
Total Patients Number RHD Average Age Uneventful Auricular Congestive
and Fibrillation Failure Deaths
Unchanged Developed Developed
Although three out of four patients with this disappeared in 76 (11 per cent) instances, and
complication can now be saved, further ir- at the end of 20 years in 108 (16 per cent).
reparable injury to the heart is often evident. In the majority this improvement consisted in
In patients with free aortic regurgitation the the regression and ultimate disappearance of
outlook is less favorable. Prophylactic measures murmurs at the cardiac apex-a diastolic
during dental extractions and nose and throat rumble as well as a blowing systolic murmur
operations offer hope in preventing this uni- of grade 2 or greater intensity (classification of
formly serious complication. Levinel3) and a return to normal where
cardiac enlargement was originally present.
EVOLUTION OF RHEUMATIC HEART DISEASE In an occasional instance a blowing diastolic
Two decades of observation on this youthful murmur (slight aortic regurgitation) of grade
group have provided an opportunity to study 1 to 2 intensity has been observed to dis-
TABLE 6.-Attacks of Rheumatic Fever and Chorea by Years
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Patients with R.
F............ 979* 232 1216 175 1111 120 130 114 I 67 I 71 65 60 42 38 30 112 12 12 10
Per Cent of Liv-
ing ........... 98 23 23 18 12 13 15 13 8 8 8 7 6 5 4 1.55 1.5 1.6 1.4 1.1
Average. ... 19 11 6 1.4
* 21 patients had rheumatic heart disease without rheumatic fever when first seen.
PRHD MR MR+S MS
20 vears later 347 29 (33%t) 35 (40%) 16 (/9%) 7 (8%)
features it is designed to prevent, and hence for It is encouraging that three out of four of the
the present the considerable risk of surgery had survivors have little or no limitation; 76
best be reserved for those in actual need of relief. served in the armed forces in World War II (18
Aortic Valve Involvement. Involvement of with rheumatic heart disease and 58 with
the aortic valve manifested by a characteristic potential rheumatic heart disease). Four hun-
blowing diastolic murmur was present initially dred and twenty-one children have been born
in 194 patients and appeared subsequently in to the female patients of the original group,
179 others, so that during the 20 year period and the complications of pregnancy have been
373 patients showed signs of aortic regurgita- minimal. In the face of these favorable findings
tion (58 per cent of the 653 with rheumatic we are in complete agreement with the strong
heart disease). In 27 instances the aortic objections raised by Wilson and Lubschez9
diastolic murmur was the only evidence of to the discouraging estimate of Cohn and
cardiac involvement; in the remainder there Lingg14 that the mean duration from the onset
were coexisting signs of mitral valve disease. of rheumatic fever to death is only 13 years.
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In nine instances the diastolic murmur of The latter study, based on an analysis of
slight aortic regurgitation disappeared during deaths alone, ignores the experience of the
later years. Severe angina pectoris decubitus survivors-a biased type of sampling where
occurred in six patients; each had a high the prognosis depends on the poorest risks.
degree of aortic regurgitation with a diastolic Our patients are now well along in early
blood pressure approaching zero. adult life, and their progress is being followed
In 27 patients there occurred with the pas- with interest. The facts emerging from the
sage of years an increase in the intensity of the adolescent period justify a more hopeful at-
aortic systolic murmur accompanying the titude for the adults than generally prevails.
characteristic diastolic murmur to a degree Grant's series covering the fourth decade is in
that indicated the development of a consider- accord with this conclusion.10 Furthermore,
able element of stenosis. In only two instances evidence is already accumulating that there
did uncomplicated aortic stenosis become has been an amelioration in the frequency and
established. First, by the age of 27 pre-existing severity of rheumatic fever during the past 10
mitral systolic and diastolic murmurs and an years, and it is unlikely that this represents
aortic diastolic murmur had completely dis- merely a cyclic abatement in the natural course
appeared in a young man, leaving a loud of the disease.
aortic systolic murmur and thrill as unequivocal In conclusion, it is to be hoped that in the
evidence of pure aortic stenosis. Second, by the foreseeable future new and already promising
age of 25 in another male patient a slow measures for the prevention and arrest of
evolution in physical signs with slight residual rheumatic fever may render the data herewith
mitral regurgitation and a minimal degree of reported obsolete but useful nevertheless as
aortic stenosis has also occurred. It is evident, the record of a past and pitiful era in the evolu-
however, that pure aortic stenosis is infrequent tion of this devastating disease.
under the age of 30. Collateral observations
suggest that when such occurs it is often SUMMARY AND CONCLUSIONS
engrafted upon congenital bicuspid valves. From a 20 year study of 1000 patients with
rheumatic fever and/or chorea followed since
CONCLUDING REMARKS
childhood the major events of the two decades
At the end of 20 years 699 of the original have been summarized and compared with
1000 patients remain alive. Their average age the experience of others.
is now 28, but a considerable number are in On recovery from the initial illness, 653
the fourth decade. The majority are remark- patients had signs of rheumatic heart disease.
ably well. Their limitations are as follows: By the end of 20 years the signs of heart disease
none-slight moderate marked lost had disappeared in 108 (16 per cent).
555 133 6 5
PRIHD REID The remaining 347 patients recovered from
316 239 their initial illness without detectable heart
E. F. BLAND AND T. D. JONES 843
disease (potential rheumatic heart disease). Benedict F. MIassell, George P. Sturgis, Joseph E.
By the end of 20 years 154 (44 per cent) had Warren, and James WV. Dow.
The financial support of the Commonwealth Fund
acquired signs of valvular disease. during the earlier years, and of the Helen Hay
During the first 10 years 202 succumbed, Whitney Foundation during the last three years
and by the end of the second 10 years 301 have made this study possible.
had died. Rheumatic fever and congestive
heart failure accounted for 80 per cent of the REFERENCES
fatalities, and bacterial endocarditis for an 1JONES, T. D., AND BLAND, E. F.: Clinical sig-
additional 10 per cent. nificance of chorea as a manifestation of rheu-
A greatly enlarged heart or congestive failure matic fever. A study in prognosis. J. A. M. A.
early in the disease exacted the highest toll, 451: 837, 1935.
with an 80 per cent mortality in 20 years. 2BLAND, E. F., JONES, T. D., AND WHITE, P. D.:
Disappearance of the physical signs of rheu-
Pericarditis, subcutaneous nodules, and acute matic heart disease. J. A. AI. A. 107: 569,
arthritis occupied intermediate positions, with 1936.
3 , AND : The delayed appearance of heart
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Circulation. 1951;4:836-843
doi: 10.1161/01.CIR.4.6.836
Circulation is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231
Copyright 1951 American Heart Association, Inc. All rights reserved.
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