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CASE REPORTS IN CARDIOLOGY
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of normal; there was evidence of right pleural thickening. The blood hematocrit
level was 39%; white blood cell count (WBC), 8,000/cu mm; and the differential
blood smear, normal. The latex test for rheumatoid arthritis was positive (4+).
The electrocardiogram showed left axis deviation and enlarged notched P waves
in leads II and V
consistent with left atrial enlargement. He received nitrogen
1
mustard, adrenal steroids, and weekly intramuscular gold injections; for several
months he showed improvement.
At age 71 (March1965) he was rehospitalized because of left-sided pleuritic
chest pain, cough, slight orthopnea and exertional dyspnea, fever, and lower
leg edema. Aleft-sided empyema was found and drained. The cardiac physical
ndings, ECG, and size of the heart on chest roentgenogram were unchanged.
Fibrotic changes, congestion and hyperination were seen in the lung elds as
well as a left pleural effusion. Because of ndings suggesting cardiac failure, he
was digitalized.
He was rehospitalized in May 1965 because of a fall which fractured the
medial meniscus of the right knee and because of rapid worsening of the
arthritis. During his 83 days in the hospital, the previously described cardiac
ndings persisted and, in addition, occasional premature atrial and ventricular
contractions were recorded. The patient’s hematocrit level was now 31% and
he weighed 46.7 kg (103 lb); three months earlier he had weighed 62.1 kg (137
lb). He was discharged in August, and thereafter received weekly gold and
corticosteroids intramuscularly. He was totally bedridden during his last year
which was cha racterized by repeated episo des of pneumonia, periods of confu sion
and disorientation, fracture of one femur, Cushing’s syndrome secondary to the
corticosteroid therapy, episcleritis which progressed to scleromalacia perforans,
and multiple decubiti. His severe weakness gradually progressed, and 12hours
before death he became comatose. At no time during his last year were there
signs of cardiac decompensation.
At autopsy (GMC, 66A-599), severe deforming rheumatoid arthritis involved
numerous joints. There was severe ulnar deviation of both hands and subluxation
of the metacarpophalangeal joints and enlargement of the proximal and distal
interphalangeal joints. Subcutaneous nodules were present in the areas of many
joints. The pericardial and pleural spaces were obliterated by adhesions. An abscess
(5 × 5 × 2cm) was present in the lower left pleural space, and it communicated
with the left lower lobe of the lung via a bronchopleural stula. The bronchi
were dilated and many were lled with purulent material. Typical rheumatoid
granulomas were observed grossly and histologically in the skin and subcutaneous
tissue, lungs, larynx, pleural and pericardial surfaces, splenic capsule, dura, and
adventitia of the bowel at the esophagogastric junction. In addition, numerous
focal yellow (rheumatoid) nodules, identical histologically to those observed in the
tissues mentioned, were present in the endocardium of the right and left atria, all
four cardiac valves, membraneous ventricular septum, and in the myocardium of
both atria and ventricles. The cardiac lesions are described in detail in Figure1
through 6. The heart weighed 350 gm. Both mitral and aortic valvular leaets were
considerably thickened, moderately rigid, and their orices probably incompetent.
In contrast, the focal nodules in the tricuspid and pulmonic valves almost certainly
did not interfere with proper functioning of these structures. Both atria were mildly
dilated. The yellow nodules located in the membraneous and upper muscular
septum caused these structures to be thick and rm. Several yellow plaques
were noted in the walls of the major coronary arteries, but there was no luminal
narrowing.
Special stains for pyogenic and acid-fast bacteria and fungi on selected sections
of heart and lung disclosed no organisms.
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CASE 78 CARDIAC VALVULAR LESIONS IN RHEUMATOID ARTHRITIS
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Figure 1 Atrioventricular valves. Top, Opened left atrium (LA), mitral valve, and
left ventricle (LV). Between arrows is row of nodules at line of attachment of posterior (P) leaet. Both anterior (AML) and posterior leaets are diffusely thickened,
but chordae tendineae are normal. Bottom, Opened right atrium (RA), tricuspid valve
and right ventricle (RV). Leaets have been reected back into right atrium exposing
small focal rheumatoid nodules (arrows) at line of attachment of anterior (ATL) and
septal (STL) tricuspid leaets as well as on leaets themselves.
COMMENT
Although nonspecic forms of pericarditis, myocarditis, and coronary arteritis have
been observed at necropsy in patients with rheumatoid arthritis (RA),
nodules (granulomas) similar to subcutaneous nodules are pathognomonic and
occur in the heart in 1% of 3% of autopsied patients with this condition.
22 patients with rheumatoid granulomas involving cardiac valves or valvular rings
have been reported.
1
In ten subjects the mitral valve alone was involved; in four,
the aortic alone; in three, both mitral and aortic; in one, the tricuspid alone; in one,
both mitral and tricuspid; in three, the mitral, aortic, and pulmonic; and in two, all
four valves. The order of incidence of involvement of the cardiac valves—mitral,
aortic, triscuspid, and pulmonary—is as in rheumatic fever, and as in the Hurler
syndrome. Seven of the 22 patients had auscultatory evidence of mitral or aortic
regurgitation or both, and ten of them had hearts weighing more than 300 gm. Most
2, 3
rheumatoid
2, 4
At least
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frequently, the valvular lesions were focal and did not appear to have interfered
with valvular function. In the present patient the diffuse nature of the involvement
of the mitral and aortic valvular leaets probably rendered them incompetent. The
cardiomegaly observed at necropsy and the clinical signs of cardiac dysfunction
can most readily be explained on the basis of valvular cardiac disease, since there
was no signicant coronary arterial narrowing, no systemic hypertension, and the
myocardial rheumatoid nodules do not appear to have been extensive enough to
have caused these ndings.
Figure 2 Semilunar valves. Upper left, Aortic valve as seen from above. Right (R), left
(L) and noncoronary (N) cusps are diffusely thickened, and cusps are partially fused
at two commissures. Upper right, Opened aortic valve. Diffuse thickening of anterior
mitral leaet (AML) also is apparent. LV indicates left ventricle. Lower left, Longitudinal
section through one pulmonic valvular cusp. Nodule farrow) is present at basal attachment of this cusp, but remainder of it is normal. Lower right, Histologic section of pulmonic valve (PV), pulmonary trunk (PT), and right ventricle (RV). Large nodule at base
of cusp is a typical rheumatoid granuloma (hematoxylin and eosin, × 20).
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CASE 78 CARDIAC VALVULAR LESIONS IN RHEUMATOID ARTHRITIS
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Figure 3 Atrioventricular valves. Left, Tricuspid valve (TV) leaet. Typical rheu-
matoid granuloma is located within substance of leaet. RV indicates right ventricle
(hematoxylin and eosin, × 12). Right, Mitral valve. Section includes posterior mitral
leaet (PML), and adjacent portions of left atrial (LA) and left ventricular (LV) walls.
Large rheumatoid granuloma is located within proximal three-fourths of posterior
leaet. Smaller nodule involves endocardium of left atrium as well. Two rheumatoid
granulomas are located within left ventricular wall (hematoxylin and eosin, × 6.5).
Although rheumatoid nodules in the hearts of patients with RA have been well
described, little attention has been given to the exact location of these lesions in the
valve leaets and ring s. In the present patient, and in the one previously stud ied by us
with rheumatoid heart disease, the location and size of the lesions were identical in
each of the respective cardiac valves. The lesions grossly were yellow, rm, smooth,
and focal or diffuse. On the tricuspid valve the lesions were focal and located at the
basal attachment of the septal and anterior leaets. The posterior tricuspid leaets
were uninvolved. Asingle nodule was present at the basal attachment of a pulmonic
valvular cusp. These right-sided cardiac lesions did not interfere with valvular
function since most of the valve tissue was uninvolved. In contrast to these rightsided lesions, those on the left-sided cardiac valves were diffuse; nearly all areas of
both mitral and aortic leaets were thickened.
Histologically, the lesions were located within the valve leaets leaving a
thin border of brous tissue to enclose the necrotic material (Figure 6). This thin
brous capsule probably represents the original and uninvolved valve tissue, and
prevents the necrotic debris within the valve leaets from being extruded into the
circulation. It appears from the study of the cardiac valves in these two patients that
the process begins as a reaction within the core or central portion of the valve leaet
preserving the peripheral portions. The valvular involvement in rheumatoid heart
disease thus is in direct contrast to that in carcinoid heart disease, in which the valve
itself remains normal and atypical brous tissue is deposited on the surface of the
valvular cusp (Figure7). Also, it differs from rheumatic valvular disease in which
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Figure 4 Myocardial rheumatoid granulomas. Left, Typical nodule replaces portion
of left atrial (LA) wall. Parietal pericardium was adherent to epicardium diffusely
in this patient, obliterating pericardial sac. Enclosed by dashed lines is cholesterol
granuloma which lies beneath rheumatoid granuloma (hematoxylin and eosin, × 22).
Right, Close-up of a left ventricular rheumatoid granuloma. Central portion consists
of necrotic debris which is surrounded by pleomorphic collection of cells, mainly
histiocytes, which are arranged perpendicular to border of necrotic material (hematoxylin and eosin, × 37).
Figure 5 Aortic and mitral valves. Left, Section includes aortic valve (AV) cusp,
anterior mitral leaet (AML) adjacent aorta (A), and left atrial (LA) wall (hematoxylin and eosin, × 2.5). Middle and right photographs are close-up views of areas
shown in brackets. Center, Necrotic debris is within core of valvular cusp, and is
surrounded by border of thin brous tissue, which probably represents remaining
original valvular tissue. Rheumatoid granulomas also are present between media
of ascending aorta (Ao) and the left atrial (La) wall (hematoxylin and eosin, × 11).
Right, Distal margin of anterior mitral leaet contains several rheumatoid granulomas (hematoxylin and eosin, × 11).
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CASE 78 CARDIAC VALVULAR LESIONS IN RHEUMATOID ARTHRITIS
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Figure 6 Portions of rheumatoid nodules in an aortic valve cusp (left) and in
membraneous ventricular septum (right). Langhans and foreign-body type giant
cells as demonstrated here were observed in many of the cardiac rheumatoid nodules. Necrotic central portion is on right of each photomicrograph (hematoxylin and
eosin, × 160).
the entire leaet, both its central and peripheral portions, is involved, often leaving no
residual normal or near-normal valvular tissue.
The reason the cardiac valves are involved at all in HA is unclear. If vasculitis is
considered to be an intrinsic part of the development of the subcutaneous nodules,
as proposed by Sokoloff et al,
occur. The cardiac valves, however, contain few blood vessels. Certainly, if vasculitis
predisposes to the development of rheumatoid granuloma, these lesions might be
expected to be much more frequent in the myocardium than in the endocardium
(valves), although this does not appear to be the situation.
SUMMARY
The clinical and necropsy ndings are described in a 72-year-old man with severe
deforming rheumatoid arthritis and rheumatoid cardiac nodules. The nodules were
located in all four cardiac valves, as well as in the pericardium and myocardium.
Rheumatoid nodules occur infrequently (3%) in the hearts of subjects with rheumatoid
arthritis, but the type of involvement is pathognomonic. The nodules, when present in a
cardiac valve, are located in the “core” or central portions of the leaets, and may cause
them to be incompetent. Involvement of the left-sided cardiac valves in this condition is
more frequent and more extensive than involvement of the right-sided valves.
ACKNOWLEDGMENTS
Mr. Larry D. Ent prepared the histologic sections and Mr. Gebhard Gsell took the
photomicrographs.
5
then involvement of valvular blood vessels must
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Figure 7 Similunar valvular cusp in various cardiac diseases. A normal cusp
is on top left for comparison. Normal components of rheumatically diseased cusp
are replaced by uniform dense brous tissue. In carcinoid heart disease, valve cusp
remains normal, and atypical brous tissue is simply superimposed on it. In rheumatoid heart disease, peripheral portions of original cusp remain, but central portions are replaced by rheumatoid granulomas.
GENERIC AND TRADE NAME OF DRUG
Phenylbutazone—Butazolidin.
REFERENCES
1. Carpenter, D.F.; Golden, A.; Roberts, W.C.: Quadrivalvular Rheumatoid Heart
Disease Associated with Left Bundle Branch Block, Amer J Med 43:922–929 (Dec)
1967.
2. Sokoloff, L.: Cardiac Involvement in Rheumatoid Arthritis and Allied Disorders:
Current Concepts, Mod Conc Cardiov Dis 33:847–850 (April) 1964.
3. Lebowitz, W.B.: The Heart in Rheumatoid Arthritis (Rheumatoid Disease), Ann
Intern Med 58:102–123 (Jan) 1963.
4. Baggenstoss, A.H.; Rosenberg, E.F.: Cardiac Lesions Associated with Chronic
Infectious Arthritis, Arch Intern Med 67:241–258 (Feb) 1941.
5. Sokoloff, L.; McCluskey, R.T.; Bunim, J.J.: Vascularity of the Early Subcutaneous
Nodule of Rheumatoid Arthritis, Arch Path 55:475–495 (June) 1953.
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CASE 89 FATAL ACUTE RHEUMATIC FEVER IN CHILDHOOD
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Case 89 Fatal Acute Rheumatic Fever in
Childhood Despite Corticosteroid Therapy
A Note on the Spectrum of Childhood Rheumatic Fever
D. Luke Glancy, M.D*, Rashid A. Massumi, M.D.**, William C. Roberts, M.D.***
Bethesda, MD., and Washington, D.C.
The incidence of death in childhood from rheumatic heart disease has been declining
in the United States for 3 decades.
1
The role of adrenocorticotrophic hormone and
adrenal corticosteroids in the continuing decline in the mortality rate during the
past 2 decades has been debated widely and remains uncertain. Nevertheless, death
from rheumatic carditis is now uncommon in children treated with these agents
and is rare during an initial attack of rheumatic fever.
2
Such occurred, however, in
2 children recently studied by us. Each died of different causes, which represent
widely separate bands in the spectrum of rheumatic heart disease in children.
REPORT OF PATIENTS
Patient 1. A3-year-old girl was hospitalized following an upper respiratory infection
because of a skin rash, which had features of both erythema marginatum and
urticaria, and arthritis of both ankles. Fever (101° F), pharyngitis, and a systolic,
ejection-type murmur at the left sternal edge were present. A leukocyte count
was 22,000 per cubic millimeter; anti-streptolysin 0 titer, 625 Todd units; corrected
sedimentation rate, 32 mm. per hour; and C-reactive protein, reactive. In the
hospital, the temperature rose to 103° F., arthritis of both knees developed while
that of the ankles subsided, and the systolic murmur became loudest at the cardiac
apex and radiated to the left axilla and back. The fever and arthritis responded
favorably to aspirin and prednisolone (50 mg. per day), but on her eighteenth day
of hospitalization, the twentieth day of illness, she developed dyspnea, tachypnea,
tachycardia (170 per minute), a ventricular gallop, hepatomegaly, and died.
Necropsy (A67–286) disclosed generalized cardiomegaly (weight, 105 grams;
expected weight, 60 gra ms), slight thickeni ng of all 4 cardiac valves, and histological ly
pancarditis with numerous Aschoff bodies (Figures1 and 2).
Patient 2. A 9-year-old girl was well until 1/2 years before death when she
developed typical signs and symptoms of acute rheumatic fever. She remained in
the hospital during the entire 1½-year period. A loud murmur typical of mitral
regurgitation was heard when she was rst examined and remained thereafter.
Congestive cardiac failure was present throughout the illness, but it appeared to be
Received for publication Feb. 12, 1968.
*
Staff Associate, Section of Pathology, National Heart Institute, National Institutes of Health,
Bethesda, Md. 20014.
**
Cardiologist, District of Columbia General Hospital, and Chief, Cardiopulmonary
Laboratory, George Washington University Division of Medicine, District of Columbia
General Hospital, Washington, D. C.
***
Chief, Section of Pathology, National Heart Institute, National Institutes of Health,
Bethesda, Md.
DOI: 10.1201/9781003409281-10 67
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Figure 1 The heart of Case 1. a, Opened right atrium (R.A), right ventricle (R.V.),
and slightly but diffusely thickened tricuspid valve. b, Opened left atrium (L.A.), left
ventricle (L .V.), and slightly but diffusely thickened aortic (A.V.) and mitral valves.
Above the posterior mitral leaet (P.) the left atrial endocardium is thickened and
irregular (MacCallum’s plaque). c, Photomicrograph showing pleomorphic inltrate
with large mononuclear cells, similar to those in Aschoff bodies, in a pulmonic valve
cusp. (Hematoxylin and eosin stain, × 208.)
less severe when the child was receiving prednisone (up to 40 mg., daily) and more
severe when this medication was discontinued. First degree heart block was present
until the last weeks of life when atrial brillation developed. She died unexpectedly.
Necropsy (A67–185) disclosed generalized cardiomegaly (weight, 320 grams;
expected weight, 115 grams); severe brosis of the mitral valve with a xed, severely
incompetent orice; a left atrial jet lesion; normal aortic, tricuspid and pulmonic
valves; and normal pericardium. Histologically, most myocardial bers of each
cardiac chamber were hypertrophied, but no inammatory cells or Aschoff bodies
were found (Figure3).
COMMENT
3
Although acute rheumatic fever occurs in patients under 4years of age,
so infrequently, and we are aware of only 14 children
4–13
who, like Patient 1, died
it does
of acute rheumatic fever before 4years of age and who were found at necropsy to
have Aschoff bodies. Photomicrographic demonstration of the Aschoff bodies was
presented in 5 of them.
weeks of acute rheumatic fever,
to decrease markedly the cellular reaction in rheumatic myocarditis.
4–7
Aschoff bodies are said to be absent during the rst few
14
and adrenal corticosteroids have been reported
15
The heart of
Patient 1, however, contained numerous, typical Aschoff bodies and an exuberant
cellular reaction despite a total symptomatic illness of only 20 days and treatment
with large doses of prednisolone.
In contrast to Patient 1, whose death was primarily due to acute rheumatic
myocarditis, Patient 2 died of severe mitral regurgitation. Although it is reasonable
to believe that acute rheumatic carditis was present at the beginning of her illness,
no inammatory lesions were present at necropsy 1½ years later, and except
for hypertrophy, the myocardium was normal. Cardiac failure in children with
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CASE 89 FATAL ACUTE RHEUMATIC FEVER IN CHILDHOOD
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Figure 2 Photomicrographs in Case 1. A, Inammatory nodule in the left atrial
endocardium, consisting of a necrotic center surrounded by large mononuclear cells
and a peripheral zone of polymorphonuclear neutrophils. B, Posterior mitral leaflet containing brinoid material, polymorphonuclear neutrophils, and large mononuclear cells. C and D, Aschoff bodies consisting of altered collagen surrounded
by large, mononuclear (Anitschkow) cells, and giant cells. (Hematoxylin and eosin
stains; A, ×160, B and C, ×400, and D, ×628; each reduced by 25 per cent.)
rheumatic heart disease suggests that the rheumatic carditis is still active,
Patient 2 this suggestion was supported by the transient, but apparently benecial
effects of prednisone. Her cardiac failure, however, was due to an operatively
correctable mechanical defect, mitral incompetence, and mitral valve replacement
might have been lifesaving. Rheumatic mitral regurgitation has been considered a
benign lesion in certain selected groups of patients,
1½ years after the initial episode of acute rheumatic fever.
16
and in
17
but in Patient 2 it caused death
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