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morphology of these particles were thought to be compatible with a virus, most likely a member of the Herpes
family (Herpes simplex or cytomegalovirus). They also
observed perichromatin granules of about 300A in diameter, but these did not envelope many nuclei of interstitial cells which resembled in shape the particles shown
here. They did not establish their identity, although
these particles were not inconsistent with those of viral
particles.
Titer of complement fixation test for adenovirus was
below 8X,which was regarded as normal. Since elevated
gammaglobulin and IgG concentrations were observed,
an immunologic-deficient status was not suggested. Discrepancy between adenovirus infection and failure of
elevation of complement fixation titer remains unexplained.
It is possible that adenovirus infection was unrelated
to the etiology of this relatively chronic case of interstitial fibrosing pneumonitis, but the histopathologic findings resembling viral infection and the data described
may support the hypothesis of Hamman and Rich that
persistent viral infection may be associated with some
cases of this disease.
ACKNOWLEDGMENT: We wish to express our appreciation to Dr. John Salvaggio of the LSU Medical School for
reviewing the manuscript. We also wish to thank Dr. Hiroto
Shimojo, Institute of Medical Science, Tokyo University, for
kindly supplying the antiserum.
1 Hamman L., Rich AR: Acute diffuse interstitial fibrosis of
the lungs. Bull Hopkins Hosp 74: 177-212, 1944
2 Liebow AA, Steer A, Billingsley JG: Desquamative interstitial pneumonia. Am J Med 39:369-404, 1965
3 Gaensler EA, Goff AM, Prowse CM: Desquamative interstitial pneumonia. N Engl J Med 274:113-126, 1966
4 Patchefsky AS, Banner M, Freundlich IM: Desquamative
interstitial pneumonia. Significance of intranuclear virallike inclusion bodies. Ann Int Med 743322-327, 1971
5 Mikami R, Nakayama A, Nobeji A, et al: Clinicopathological studies on diffuse interstitial pulmonary fibrosis of
unknown etiology. Nihonrinsho 27: 1829-1861, 1969
6 O'Shea PA, Yardley JH: The Hamman-Rich syndrome in
infancy: Report of a case with virus-like particles by
electron microscopy. Hopkins Med J 1263320-343, 1970
7 Peabody JW, Peabody JW Jr, Hayes EW, et al: Idiopathic
pulmonary fibrosis; its occurrence in identical twin sisters.
Dis Chest 18:330-343, 1950
8 Scadding JG: Diffuse pulmonary alveolar fibrosis. Therapeutische Umschau 30:191-198, 1973
9 McNary WF, Gaensler EA: Intranuclear inclusion bodies
in desquamative interstitial pneumonia. Electron microscopic observations. Ann Int Med 74:404-407, 1971
Systolic Anterior Motion of the
Mitral Valve without Asymmetric
Septal ~ ~ ~ e r t r o ~ h ~ *
Bemudine H. Bulkley, M.D., and Nicholas J . Fortuin, M.D.
This report describes a patient with echocardiographic
systolic anterior motion of the mitral valve causing the
anterior mitral leaflet to contact the septum in systole. At
necropsy a normal nonhypertrophied heart with normalsized ventricular cavities and a normal outflow tract and
mitral valve was found. Thus, asymmetric septal hypertrophy and abnormal mitrai valvular placement are not
reqoisites for systolic anterior motion of the mitral valve.
During systole, a marked forward movement of the anterior mitral leaflet developed in our patient in the setting
of hypovolemia and continuous intravenous administration of pressor drugs, suggesting, rather, that systolic anterior motion reflects a small, vigorously contracting ventricular cavity and that such dynamic subaortic obstruction is not pathognomonic of idiopathic hypertrophic
subaortic stenosis.
H
ypertrophic cardiomyopathy or idiopathic hypertrophic subaortic stenosis has certain characteristics
which are evident echocardiographically, including
asymmetric septal hypertrophy and systolic anterior motion of the mitral valve.'-3 Systolic anterior motion of
the mitral valve is usually present in asymmetric septal
hypertrophy with left ventricular outflow-tract obstruction, and "obstruction indices" have been determined
from the duration of this forward m o t i ~ n .This
~ ? ~report
describes echocardiographic systolic anterior motion of
the mitral valve in a patient with a normal heart and
without asymmetric septal hypertrophy and discusses a
possible explanation for the acquired abnormality of
rnitrd motion.
A 70-year-old white woman with myeloid metaplasia and
no history of precordial murmurs was admitted to the hospital
for hematologic evaluation. Three weeks before her death,
the patient underwent a laparotomy and suffered numerous
complications, including respiratory arrest and hypotension
due to sepsis and gastrointestinal bleeding. Hours after her
first hypotensive episode, for which intravenous therapy with
1-norepinephrine ( levarterenol) was begun, the patient developed a grade 3/6 systolic murmur at the left lower sternal
border for the first time. Pressor therapy was continued for
the remainder of her hospitalization because of sepsis and
intractable gastrointestinal bleeding. The systolic murmur
varied from grade 2/6 to grade 4/6 at various times and
"From the Cardiovascular Division, Department of Medicine,
the ohns Hopkins University School of Medicine and Hositd, Baltimore.
fupported in part by trainin grant 5T01 HL 05735 from
the National Institutes of ~ e a f t h .
Reprint requests: Dr. Bulkley, Division of Cardiology, ]ohm
Hopkins Hospid, Baltimore 21 21 8
694 BULKLEY, FORTUIN
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CHEST, 69: 5, MAY, 1976
FIGURE 1. Umetodmd photograph of echocardiogram showing anterior motion (SAM) of mitral
valve ( W )during systole ( awaws ) Note reduced left ventricular ( LV ) cavity size and active
motion of both LV free wall and septum. Because of study conditions, adequate ekxtmcadogram could not be recorded with this tracing;therefore, systole and diastole are indicated by
brackets.
.
appeared to increase while the patient was using the
respirator.
Because of the possibility of p e r i d t i s or infective enddcarditis, or both, an echocardiogram was obtained with a
portable echocardiograph while the patient was in the intensive care unit two weeks before death. The echocardiogram
showed no pericardial effusion. Systolic anterior motion of the
mitdvalve with the mibal leaflet contacting the septum
during syztole was observed and suggested the diagnosis of
idiopathic hypertrophic Subaortic stenosis ( Fig 1) ; however,
because of the electrical noise of the intensive care unit,
resolution of the echocardiogram was not sutficient to define
the size of the septum. Numerous blood cultures were negative, and obstructive idiopathic hypertrophic subaortic stenosis was considered as a likely cause of her changing munnur.
The patient died of respiratory failure and sepsis. At necropsy
the heart weighed 300 gm. Its epicardium was smooth and
glistening. All four cardiac valves, including the m i d valve,
were normal. All four cardiac chambers were of normal size.
The right ventricular wall measured 0.3 cm, and the left
ventricular free wall and septum each measured 12 cm ( Fig
2 ) . At no level was the interventricular septum thicker than
the left ventricular free wall. The left ventricular outllow
tract was normal and free of endocardial plaque. The coronary arteries were widely patent.
Systolic anterior motion of the mitral valve is an
important echocardiographic feature of idiopathic hypertrophic subaortic stenosis and is considered by some
to be virtually pathognomonic of this entity.'-' It has
FIGURE2 (right). Transverse sections through both right
( R V ) and left ( L V ) ventricular cavities near base of heart
( A , top) and towards apex (B, bottom). Heart is neither
hypertrophied nor dilated, and interventricular septum is
equal in thickness to LV free wall.
CHEST, 69: 5, MAY, 1976
SYSTOLIC ANTERIOR MOTION
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OF MITRM VALVE 895
been proposed that an abnormal relationship between
the septum and the mitral valve causes systolic anterior
motion of the mitral valve and is responsible for obstruction in hypertrophic cardiomyopathy; for some, this has
been a rationale for mitral valvular replacement as therapy for idiopathic hypertrophic subaortic stenosis.e.7
Findings in our patient indicate that systolic anterior
motion of the mitral valve is not pathognomonic of
idiopathic hypertrophic subaortic stenosis or asymmetric
septal hypertrophy, and that systolic anterior motion
does not imply anatomic abnormalities of the mitral
valve or interventricular septum.
Although systolic anterior motion of the mitral valve
does not necessarily mean asymmetric septal hypertrophy, whether or not such systolic mitral valvular
motion indicates some degree of outflow-tract obstruction or "subaortic" stenosis is uncertain. Echocardiographic evidence of a mitral valve that contacted the sep
tum in systole and clinical evidence of a grade 4/6 systolic ejection-typemurmur at the left lower sternal border
were highly suggestive of some degree of left ventricular
outflow-tract obstruction in our patient. Although this
patient had no left ventricular hypertrophy or endocardial plaques in the outflow tract at autopsy to indicate
chronic outflow-tract obstruction, the systolic murmur
developed while the patient was in the hospital, and the
outflow-tract obstruction, if it existed, would only have
been present for two to three weeks before death. Alternatively, the murmur and abnormal mitral valvular
movement may not be causally related but may merely
reflect the abnormal hemodynamic state of the left ventricle. Since intraventricular pressure recordings were
not obtained in this patient, we cannot determine which
of these possibilities is correct.
How can one explain the sudden development of
systolic anterior motion of the rnitral valve and what may
have been a subaortic stenosis in this patient? Hours
prior to the development of the murmur, the patient
became acutely hypotensive and was started on intravenous therapy with pressor drugs. Despite fluid-replacement therapy, a relative state of volume contraction
probably persisted because of subsequent recurrent
gastrointestinal bleeding and sepsis. At autopsy the heart
had a normal-sized ventricular cavity. It is surmised that
the combination of volume depletion, adrenergic stimulation, and a contracted ventricular cavity led to a
hyperdynamic state with systolic cavity obliteration
similar to that seen in hypertrophic cardiomyopathy, and
that this led to anterior motion of the mitral valve during
systole. In our view, systolic anterior motion is a nonspecific motion pattern of the rnitral valve which occurs
because of reduced left-ventricular ,cavity size and hyperkinetic left ventricular contraction. Although mitral
motion has not been examined specifically, it has been
shown experimentally in dogs that long-term administration of isoproterenols.9 norepinephrine infusion,1° or
acute hemorrhagic shock alone" can produce hemodynamic and angiographic changes strikingly similar to
those of idiopathic hypertrophic subaortic steno~is.~
Similarly, dynamic subaortic obstruction in man may not
be pathognomonic of idiopathic hypertrophic subaortic
stenosis.
Thus, although systolic anterior motion of the mitral
valve may not mean asymmetric septal hypertrophy or a
primary hypertrophic cardiomyopathy, it may mean a
hyperdynamic contractile state in which systolic cavity
obliteration produces marked systolic anterior motion of
the mitral leatlet. Whether systolic anterior motion of the
mitral valve actually produces outflow-tract obstruction
is uncertain, but findings in this patient seem to indicate
that it is neither pathognomonic of asymmetric septal
hypertrophy nor necessarily indicative of an abnormal
anatomic relationship between the interventricular s e p
tum and mitral valve.
1 Shah PM, Gramiak R, Kramer DH: Ultrasound localization of left ventricular outflow tract obstruction in hypertrophic obstructive cardiomyopathy. Circulation 40:3-11,
1969
2 Popp RL, Harrison DC: Ultrasound in the diagnosis and
evaluation of therapy of idiopathic hypertrophic subaortic
stenosis. Circulation 40:905-914, 1969
3 Pridie RB, Oakley CM: Mechanism of mitral regurgitation in hypertrophic obstructive cardiomyopathy. Br
Heart J 32:203-208, 1970
4 Shah PM, Gramiak R, Adelman AG, et al: Role of
echocardiography in diagnostic and hemodynamic assessment of hypertrophic subaortic stenosis. Circulation
44:891-898, 1971
5 Hemy WL, Clark CE, Glancy DL, et al: Echocardiographic measurement of the left ventricular outflow gradient in idiopathic hypertrophic subaortic stenosis. N
Engl J Med 288:989-993,1973
6 Ellis JG, Terneny OJ, Winters WL,, et al: Critical role of
the mitral valve leaflet in hyperh-ophic subaortic stenosis
and amelioration of the disease by mibal valve replacement. Chest 59:378-382, 1971
7 Cooley Dh, Leachman RD, Wukasch DC: Diffuse muscular subaortic stenosis: Surgical treatment. Am J Cardiol
31: 1-6, 1973
8 Criley JM, Lewis KB,White RI, et al: Pressure gradients
without obstruction: A new concept of "hypertrophic
subaortic stenosis." Circulation 32:881-887, 1965
9 Krasnow N, Rolett E, Hood WB, et al: Reversible obstruction of the ventricular outflow tract. Am J Cardiol
11:1-7, 1963
10 Blaufus AH, Laks MM, Garner D, et d: Production of
ventricular hypertrophy simulating "idiopathic hypertrophic subaortic stenosis" ( IHSS ) by subhypertensive
infusion of norepinephrine in the conscious dog. Clin Res
23:77A, 1975 (abstract)
11 Martin AM, Hackel DB, Spach MS, et al: Cineangiocardiography in hemorrhagic shock. Am Heart J 69:283-2&1,
1985
696 BULKLEY, FORTUIW
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CHEST, 69: 5, MAY, 1976