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They also have been called “moderator bands” since they were believed to “moderate” or “checkrein” dilatation of the ventricle.
8
The main feature is that the musical
murmur is not present from birth, despite the congenital nature of the band, but
appears when the ventricle becomes dilated because of some unrelated strain such
as heart failure or systemic hypertension. To produce a murmur, the anomalous
band must be pulled taut and be located more or less perpendicular to the stream of
ow in the left ventricle. McKusick compared the murmur produced by the anomalous cords to the musical vibration produced by an Aeolian harp. Huchard’s rst
observation
6
of this lesion was in a 49year old man who had signs of severe systemic
arterial hypertension with cardiomegaly and congestive failure. Amurmur typical
of mitral regurgitation was audible in this patient over the cardiac apex and in the
left axilla. More medially, maximal in the area of the xiphoid and widely transmitted especially to the right of the sternum and to the cardiac base, was a purring or
snoring systolic murmur. McKusick pointed out that extracardiac musical murmurs
may occur in the same clinical setting and display the same characteristics, although
more variation with respiration usually can be demonstrated.
Anomalous cords are uniformly present in the left ventricular outow tract in
patients with persistent common atrioventricular canal, and occasionally in subjects
with isolated ventricular septal defect. McKusick has suggested that these anomalously inserted chordae tendineae may rarely produce unusual harmonics, which
have been demonstrated in the Roger murmur. Chiari’s network in the right atrium,
a common anatomic nding (2 to 3 per cent of necropsies),
cause of a musical precordial murmur, although 2 such cases have been described.
9
apparently is a very rare
10, 11
It is probable that, as with the ventricular bands and anomalous chordae tendineae,
not only does the Chiari network need to be properly oriented in relation to the
venae cavae, but also dilatation of the atrium with tensing of the network favors
development of such a murmur. Anomalous bands stretching from the septum to
the free wall also have been described in the left atrium,
12, 13
but murmurs resulting
from them have not been reported.
SUMMARY
The pertinent clinical and necropsy features are described in an elderly man who
had a loud precordial murmur when he was in severe cardiac decompensation. With
restoration of cardiac compensation the precordial murmur disappeared and the
heart became smaller. At necropsy, a brous cord was found in the left ventricle,
stretching from the septum to the free wall. It is suggested that the murmur was the
result of this band’s being stretched taut when the left ventricular cavity was dilated.
When the left ventricular chamber returned to normal size, the anomalous band
became lax and the murmur disappeared. Other reports describing anomalous left
ventricular bands are briey reviewed.
REFERENCES
1. TURNER, W. Heart with moderator band in left ventricle. J. Anat.& Physiol., 27
(n.s. 7):19, 1893.
2. TURNER, W. Heart with moderator band in left ventricle. J. Anat.& Physiol., 30
(n.s. 10):568, 1896.
3. TURNER, W. Moderator band in left ventricle. J. Anat.& Physiol., 32:373, 1898.
4. TURNER, W. Tricuspid left auriculo-ventricular valve. J. Anat.& Physiol., 32:374,
1898.
5. ROLLESTON, H. D. Heart showing a muscular band passing between the two
ventriculi papillares of the left ventricle and capable of acting as a moderator
band. J. Anat.& Physiol., 32:21, 1897.
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Case 92 anomalous left VentriCular Band
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6. HUCHARD, H. Traité Clinique des Maladies du Coeur et de L’aorte, ed. 3, Vol. III,
pp.640 and 641. Paris, O. Doin, 1905.
7. MCKUSICK, V. A. Cardiovascular Sound in Health and Disease, pp. 209–211.
Baltimore, Williams& Wilkins, 1958.
8. KING, T. W. An essay on the safety-valve function in the right ventricle of the
human heart, and the gradations of this function in the circulation of warmblooded animals. Guy’s Hosp. Rep., 2:104, 1837.
9. YATER, W. M. The paradox of Chiari’s network. Review and report of a case of
Chiari’s network ensnaring a large embolus. Am. Heart J., 11:542, 1936.
10
. ALVAREZ, J. A. and HERRMANN, G. Unusual signs from an expansive Chiari
network along with signs of a syphilitic aortic regurgitation, Am. J. Syph., 15:532,
1931.
11
. WILSON, R. Acase of Chiari’s network associated with a murmur resembling
the bruit de Roger. J.A.M.A., 111:917, 1938.
12
. ROLLESTON, H. D. Band in left auricle of heart. J. Anat. & Physiol., 30 (n.s. 10):5,
1896.
13
. TURNER, W. Moderator band in left auricle. J. Anat.& Physiol., 30:582, 1896.
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Case 93 Rocks in the Right Ventricle
A Complication of Congenital Right Ventricular
Infundibular Obstruction Associated With
Chronic Pulmonary Parenchymal Disease
David C. Dean, MD,† Thomas Pamukcoglu, MD,‡
and William C. Roberts, MD, F.A.C.C.
§
Buffalo, New York and Bethesda, Maryland
CALCIUM IN THE HEART is most frequently located in coronary arteries or in
aortic or mitral valve leaets or “rings.” Occasionally, however, it is found in left
atrial or left ventricular mural thrombi, thickened pericardia, intracardiac neoplasms and myocardium. The occurrence of calcic deposits in mural thrombi
indicates that thrombosis occurred in the distant past. In contrast to calcic deposits located in left atrial thrombi, left ventricular deposits are usually small and
rarely protrude into the cavity of the chamber. Calcic material in either of these
two chambers nearly always is attached to the endocardium over a broad base.
Localized protruding deposits of calcium in a cardiac ventricle is indeed rare, and
to our knowledge there are no reported instances of multiple focal intracardiac
masses of calcium in the right ventricle. Such was the case, however, in a patient
we recently studied.
CASE REPORT
A 56year old white man, who died on May15, 1967, had been well until age 33
when bronchial asthma developed during his service in the Army. Because of the
development of continuous wheezing, associated with exertional dyspnea, nonproductive cough, substernal chest pain, and frequent episodes of cough syncope, he
was examined at the Buffalo Veterans Administration Hospital in March1962 at
age 51. The blood pressure was 142/90mm. Hg, and rhonchi and wheezes were
audible over the chest. Agrade 1/6 precordial pansystolic murmur was present. The
chest roentgenogram revealed slight enlargement of the left ventricle and the major
pulmonary arterial branches. Numerous calcic densities were visible in the cardiac silhouette at this time. Arepeat roentgenogram three years later (Figure 1) was
unchanged. The electrocardiogram disclosed incomplete right bundle branch block
and an electrical axis of +90°.
The patient carried on his usual activities until four hours before death, when
severe, crushing pain in the chest developed, radiating down both arms. On admission to the hospital he was cyanotic and sweating profusely. The neck veins were
severely distended. Wheezes, rhonchi and rales were audible over both lungs.
From the Departments of Medicine and Pathology, State University of New York at Buffalo,
†
The Veterans Administration Hospital, Buffalo, New York
‡
and the Section of Pathology, National Heart Institute, National Institutes of Health,
Bethesda, Md.
§
Manuscript received January31, 1968.
Address for reprints: David C. Dean, M.D., Veterans Administration Hospital, Buffalo, N. Y.
14215, or William C. Roberts, M.D., Section of Pathology, National Heart Institute, National
Institutes of Health, Bethesda, Md. 20014.
104 DOI: 10.1201/9781003409342-13
Case 93 roCks in the right VentriCle
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Figure 1 Chest roentgenogram, taken in February1965. Numerous cardiac calcic densities (arrows) are present.
Aquadruple gallop rhythm was present. The blood pressure was 130/70mm. Hg on
admission, but was not recordable thereafter. The hematocrit was 52 per cent. The
electrocardiogram, which had shown incomplete right bundle branch block 19 days
earlier (Figure 2), now showed complete right bundle branch block. The patient failed
to respond to antihypotensive medication and died three hours after admission.
At necropsy, the heart weighed 480 gm. Eleven calcied nodules (rocks or stones),
ranging in diameter from 0.6 to 2.1cm., were present in the right ventricle (Figures 3
to 6). Each stone was attached to the right ventricular endocardium by a small (less
than 0.2cm.) brous stalk. The right ventricular stones consisted primarily of calcied material, but dense brous tissue surrounded the calcic deposits. Biochemical
examination of one of the right ventricular stones disclosed that it consisted of 32
per cent hydroxyapatite, 56 per cent tricalcium phosphate and 12 per cent protein.
The endocardium of the right ventricle, both beneath and between the stones, was
extensively but focally thickened. The entrance into the infundibulum was narrowed by thickened endocardial brous tissue, hypertrophied myocardium and
Figure 2 Electrocardiogram recorded 19 days before death.
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Figure 3 Diagrammatic representation of the right side of the heart. The 11 stones
(in black) in the right ventricle (R.V.) were of varying sizes and shapes, and each was
attached to the endocardium. At the entrance into the infundibulum there was an
area of discrete narrowing produced by thickened myocardium, endocardial brosis and a stone at this site. Distal to the area of right ventricular outow obstruction,
the endocardium was thickened, the result almost surely of turbulent ow. The ventricular aspects of the pulmonic valve cusps also were thickened by the same mechanism. The wall of the body of the right ventricle, that portion proximal to the area
of infundibular obstruction, was thicker than that portion distal to the obstruction.
The right atrium (R.A.) was dilated. (I.V.C.=inferior vena cava; P.T.= pulmonary
trunk; S.V.C.=superior vena cava; T.V.=tricuspid valve.)
Figure 4 Radiogram of the excised heart at necropsy demonstrating the right ventricular stones and the calcied plaques in the coronary arteries. The specimen had
been opened before the radiogram was taken.
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Figure 5 The opened right side of the heart. a, opened right atrium (R.A), tricuspid
valve and right ventricle. The stones in the right ventricle are apparent. The arrow
points to the stone upon which the biochemical analysis was performed. The septal
(S.L.), anterior (A.L.) and posterior tricuspid valve leaets and chordae tendineae
are thickened. b, close-up view of the inow portion of the right ventricle following
removal of the stone designated by the arrow in a. The endocardium is extensively
thickened. The arrow points to the previous site of attachment of the excised stone.
c, opened pulmonary trunk (P.T.), pulmonic valve and right ventricle again exposing the stones. The right (R.), left (L.) and posterior (P.) cusps of the pulmonic valve
are thickened. (T.V.=tricuspid valve orice.) d, pulmonic valve from above dem-
onstrating the area of narrowing (enclosed by the dashes) at the entrance into the
infundibulum.
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Figure 6 Photomicrographs of the right ventricular wall and of several stones.
a, section includes right atrial (R.A.) and right ventricular (R.V.) walls and tricuspid
valve (T.V.) leaet. The latter is considerably thickened by brous tissue. The stones
are designated. The inow portion of the right ventricular wall is thick. b, outow
portion of the right ventricle. The area of discrete narrowing is at the site of a large
stone. The endocardium beneath it is greatly thickened. The wall of the pulmonary
trunk (P.T.) and a pulmonic valve (P.V.) cusp are shown. (Both a and b are elastic tissue stains, each magnied × 2.5, reduced by 28 per cent.) c, hematoxylin and eosin
stain of one stone. (× 18, reduced by 28 per cent.)
stones located at this site. The pulmonic valve was wide open, although each of its
three cups was diffusely thickened by brous tissue. The wall of the right ventricle
in the inow tract measured up to 0.9cm. in thickness, and in the outow tract,
distal to the area of obstruction, up to 0.5cm. in thickness. The leaets of the tricuspid valve and most of its chordae tendineae were thickened by brous tissue.
The right atrial cavity was dilated and its walls thickened. The left atrial cavity was
mildly dilated. The left ventricular wall was of normal thickness and its cavity of
normal size. No scars or areas of softening were present in the left ventricular myocardium. The mitral and aortic valves were normal. The lumens of the right, left and
left circumex coronary arteries were narrowed between 25 and 75 per cent by focal,
brous and calcied plaques.
The lungs were congested and edematous and, focally, emphysematous and
brotic. Two laminated nodules were in the lung parenchyma, and on histologic examination each contained large numbers of organisms consistent with
Histoplasma capsulatum.
COMMENT
The origin of the stones in the right ventricle is uncertain, but at least two possibilities exist. First, they may represent the end stage of organized mural thrombi. The
patient presumably had mild right ventricular hypertension during his entire life,
the result of the infundibular narrowing. In later life this ventricular hypertension
was further aggravated by pulmonary parenchymal disease. The endocardial thickening in the right ventricle may represent organization of at thrombi. However,
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it may have resulted from trauma to this chamber by the right ventricular rocks
and by the turbulent ow produced by the infundibular obstruction. Asecond but
unlikely possibility is that the stones may represent calcied endocardial granulomas secondary to histoplasmosis. This possibility is suggested by the presence of
histoplasma granulomas in the lung, although no organisms were found in any of
the right ventricular stones or in other portions of the heart. Histoplasma organisms, however, have been known to cause pericarditis, myocarditis and valvular
endocarditis.
No reports describing deposits of calcium in the heart similar to those observed
in the patient described have appeared to our knowledge.
SUMMARY
Clinical and necropsy ndings are described in a 56year old man who was found
to have multiple rocks in the right ventricular cavity and a congenitally narrowed
infundibulum. The size, distribution and location of the rocks in the right ventricle
appear unique. Possible causes of the rocks in the heart are speculated upon.
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Case 94 The Angiographic Features of
a Case of Parachute Mitral Valve
Allan L. Simon, MD*, William F. Friedman, MD, William C. Roberts, MD
Baltimore, Md.
The parachute deformity is an uncommon variant of congenital mitral stenosis, consisting of the insertion of all of the chordae tendineae of the mitral valve into a single, large papillary muscle. In the initial description by Shone and associates,
other reports,
2, 3
the anomaly has been most often found as part of a developmental
complex consisting of aortic coarctation, subaortic stenosis, and supravalvular ring
in the left atrium. If diagnosed correctly, it is apparent that a corrective operation
may be accomplished.
4
In this regard, the angiographic appearance of the parachute
mitral valve per se has received scant attention, although the radiographic features
of the associated cardiovascular malformations have been discussed recently.
Adescription of the characteristic angiographic appearance of the left ventricular
cavity and mitral valve in a well-studied patient with the parachute deformity and a
discussion of the mechanism of obstruction to left ventricular outow in this disorder forms the basis of the present report.
CASE REPORT
Cyanosis and congestive heart failure were recognized shortly after the premature
birth of J. L. H. (N.I.H. 06-50-00), a 4-year-old Caucasian girl. She was treated with
oxygen and digitalis and was acyanotic when discharged from the hospital at 2
months of age. Her subsequent course was marked by retarded growth and frequent
respiratory infections, and at 2½ years of age she underwent cardiac catheterization
at another institution. The hemodynamic data (Table 1) were consistent with the
diagnosis of valvular pulmonic stenosis and subaortic stenosis. Severe mitral regurgitation was seen on a left ventricular angiocardiogram. The risk of operation was
considered prohibitive and the child was discharged from the hospital. The next 1½
years were characterized by chronic congestive heart failure and frequent episodes
of acute pulmonary edema which responded initially to increased digitalis, diuretics, salt restriction, and oxygen. She had become refractory to these measures and
was in pulmonary edema when rst referred and admitted to the National Heart
Institute.
Physical examination revealed a markedly cachectic, acyanotic girl (height
87cm., weight 9.7 kilograms). The chest was barrel shaped and the heart greatly
enlarged. A continuous thrill was prominent at the apex and a systolic thrill was
palpable in the suprasternal notch. The rst and second heart sounds were single;
third and fourth heart sounds were audible at the lower left sternal border. AGrade
1
and in
5
From the Radiology Department, Clinical Center, and the Cardiology Branch, Section of
Pathology, National Heart Institute, National Institutes of Health, Bethesda, Md.
Received for publication March25, 1968.
* Present address: Director, Cardiovascular Diagnostic Laboratory, CMSC-5–109, The Johns
Hopkins Hospital. Baltimore, Md. 21205.
110 DOI: 10.1201/9781003409342-14
Case 94 the angiograPhiC features of ParaChute mitral ValVe
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Table 1: Hemodynamic ndings; pressure in millimeters of mercury
Age 2½ yr. Age 4 yr. Intra-operative
*
RAm
RV 130/10 142/6 70/1
MPA 22/10 32/18 22/6
PCW a 25
LAm 20 7
LVb 160/– 168/15 95/1
LVo 90/– 86/15
Aorta 90/50 86/42 70/30
LA-LV 9
Post-PVC pulse pressure 5
Abbreviations: RAm, right atrial; RV, right ventricular; MPA, main pulmo-
*
nary arterial; PCW, pulmonary capillary wedge; a, a wave; v, v wave; m,
mean pressure; LAm, left atrial mean; LVb, body of left ventricle; LVo, outow tract of left ventricle; PVC, premature ventricular contractions.
3/6 systolic ejection murmur radiated from the parasternal area at the third interspace into the neck and back. There was a Grade 4/6 decrescendo, holosystolic murmur and a Grade 3/6 diastolic rumbling murmur at the apex. The patient had a
hypochromic, microcytic anemia (hemoglobin 8 grams per cent, hematocrit 26 per
cent). The electrocardiogram showed right axis deviation, left atrial enlargement,
and right ventricular hypertrophy. Chest roentgenograms demonstrated massive
biventricular and left atrial enlargement and pulmonary venous congestion and
edema.
The ndings at cardiac catheterization are summarized in Table 1and, on the
basis of the hemodynamic ndings, together with the left ventricular angiocardiogram discussed in detail below, the child was referred for operation, with a diagnosis of valvular pulmonic stenosis, subaortic stenosis, and parachute deformity of
the mitral valve.
At operation, the mitral valve was found to be funnel shaped. Instead of normal
leaets, there was an extremely thick cone of brous tissue with a 4mm. eccentric
orice. All of the mitral chordae tendineae inserted on a single large papillary muscle which occupied the apex of the left ventricle (Figures 1, A and 2). Endocardial
thickening was noted in the left ventricular outow tract, opposite the mitral annulus. The mitral valve and papillary muscle were excised and replaced with a lowprole Kay-Shiley prosthetic valve; a pulmonary valvotomy was also performed.
The hemodynamic measurements determined immediately thereafter are presented
in Table 1and reveal marked reductions in the gradients across both the pulmonary
valve and subaortic regions compared to the preoperative values. The absence of
ow measurements, however, precludes estimation of changes in orice size.
The postoperative period was characterized by marked respiratory distress and
signs of insufcient cardiac output. The patient died 42hours postoperatively, presumably of dysfunction of the prosthetic mitral valve.
At postmortem examination, the foramen ovale was patent. Both ventricles and
the interventricular septum were markedly enlarged. The commissures of a domeshaped pulmonic valve with a small central orice had been separated at operation
5 7
v 24
m 19
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