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Case rePorts in Cardiology
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Figure 1 a, Opened mitral valve showing only one papillary muscle (enclosed by
broken line). The leaets are attached to the papillary muscle by very short chordae
tendineae. The leaets are diffusely and irregularly thickened but free of calcium
deposits. b, Intact pulmonic (P.V.) and aortic valves (A.V.). The former is severely
stenotic. c, Opened left atrium showing thrombus occluding the Kay-Shiley mitral
orice. The left atrial wall is thickened and the chamber is dilated but no supramitral ring is present. d, Transverse section showing that the cavities of both ventricles are small. The arrow points to thickened endocardium in the left ventricular
outow tract.
(Figure 1, B). Athin membrane of clot over the atrial aspect of the prosthetic mitral
disc valve completely occluded its orice (Figure 1, C).
DISCUSSION
In the present patient, the hemodynamic determination of pressure gradients across
the left and right ventricular outow tracts and the mitral valve localized 3 sites of
obstruction within the heart (Table1). Pulmonic valvular stenosis was clearly demonstrated by the pullback pressure recording across the right ventricular outow
tract, and by a right ventricular angiocardiogram. Mitral and subaortic stenosis
were also demonstrated by catheter pullback. The decline in systemic arterial pulse
pressure following a premature ventricular contraction suggested that functional
rather than xed orice obstruction to left ventricular outow existed at the subvalvular level.
6
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Case 94 the angiograPhiC features of ParaChute mitral ValVe
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Figure 2 Diagrammatic representation of the cardiac lesions. The markedly thickened mitral leaets insert almost directly into the single, large papillary muscle at
the apex of the left ventricle. Obstruction to left ventricular ejection is caused by
restriction of posterior motion of the leaets which prevents their retraction from
the outow tract during systole, and contact between the mitral leaets and hypertrophied muscular interventricular septum. Mitral regurgitation results from
restricted leaet motion which prevents occlusion of the mitral orice.
Angiocardiography greatly facilitated the more precise assessment of the
lesions responsible for obstruction at both the subaortic and mitral valve levels.
Severe deformity of the mitral valve as well as marked thickening of its leaets was
observed in all phases of the cardiac cycle. The diastolic position of the mitral leaflets can be seen in lateral views of the opacied left ventricle (Figure 3, F); there
was restriction of forward motion of the leaets, leading to a funnel shape. During
systole, in addition to the demonstration of mitral regurgitation, there was marked
anterior concavity of the anterior mitral leaet, and the leading edges of both leaflets were seen to be projecting into the left ventricular outow tract well below the
aortic valve (Figure 3, D). It was apparent that the leading edge of the anterior mitral
leaet formed the posterior component of the obstruction to the left ventricular ejection. Amarkedly thickened interventricular septum formed the anterior and lateral components of the obstruction. Normally, during systole, the anterior mitral
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Figure 3 Left ventricular angiocardiograms and diagrams of the contracted ventricle, in the frontal projection after ejection (A and B), the hypertrophied muscular interventricular septum (S) can be seen bulging into the lateral aspect of the
left ventricular outow tract. The outow tract obstruction is visible as a V-shaped
radiolucent line, formed by the leading edges of the mitral leaets (ML) as they
come in contact with the area of the septal hypertrophy, about 2cm. below the
opened aortic valve (A). The single large papillary muscle (PAP. M ) is seen as a
radiolucent defect at the apex of the left ventricle. In diastole (C), the septal hypertrophy results in a deformity along the inferior surface of the outow tract. The
papillary muscle cannot be delineated, since it is surrounded by a large pool of contrast material. In the lateral projection, after ventricular contraction (D and E) the
hypertrophied interventricular septum (S) protrudes into the anterior portion of
the outow tract. The thickened anterior mitral leaet (AML) and posterior mitral
leaet (PML) are held forward in the outow tract, several centimeters below the
aortic valve, forming the posterior component of the subaortic obstruction. Mitral
regurgitation (MR) is also demonstrated. There is superimposition of the body and
apex of the left ventricle so that the papillary muscle is obscured. In diastole in the
lateral projection (F), septal hypertrophy is visible along the anterior aspect of the
left ventricular outow tract, immediately below the aortic valve, and the indentation along the inferior aspect of the ventricle is also caused by hypertrophy of the
muscular septum. The interface between the opacied left ventricular blood and
less-opacifìed left atrial blood is formed by the mitral leaets (arrows) and shows
their restricted opening and a funnel deformity.
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Case 94 the angiograPhiC features of ParaChute mitral ValVe
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leaet swings posteriorly out of the outow portion of the ventricle and meets the
posterior mitral leaet to occlude the mitral orice so that, in spite of contraction
of the muscular interventricular septum, the outow tract is widened.
7
In the frontal projection, during systole (Figure 3, A), contact between the leading edge of the
deformed mitral valve and the anterior bulge of the hypertrophied interventricular
septum was visible as a V-shaped, thick, radiolucent line several centimeters below
the aortic valve. This location corresponds to the point of pressure change within
the ventricle. The normal left ventricular outow tract shows no such radiolucent
defect in systole, since the mitral valve moves posteriorly away from the septum.
7
Although chordae tendineae may not be visualized on a normal left ventricular
angiogram, ordinarily two discrete papillary muscles are noted in both the frontal
and lateral projections. The anterior muscle may be seen along the anterosuperior surface and the posterior muscle along the posteroinferior surface of the ventricle.
7
Both
lling defects were not present in this patient. Rather, only a single, large lling defect
was seen in an unusual position, occupying the cardiac apex (Figures 2 and 3, A).
It was of particular interest that the dynamics and the appearance of the left ventricular outow obstruction in this patient resembled those shown to exist in idiopathic hypertrophic subaortic stenosis (IHSS). In the latter condition, the pressure
gradient within the body of the left ventricle is thought by some authors to be caused
by the abnormal systolic position of the leading edge of the mitral valve leaets as
it contacts the hypertrophied interventricular septum.
7
In IHSS, it is postulated that
the abnormal position of these leaets, which causes mitral regurgitation and subaortic obstruction, probably results from traction on the chordae tendineae due to
dislocation of the left ventricular papillary muscles by the hypertrophied septum.
In this patient, the parachute deformity of the mitral valve (with shortening and
fusion of the chordae tendineae as well as brosis of the mitral valve) prevents the
normal systolic excursion of the mitral leaets and is responsible for the subaortic
stenosis and mitral regurgitation. For this reason, the diagnosis of parachute deformity of the mitral valve should be entertained whenever the angiocardiographic
association of a single large papillary muscle at the apex coexists with abnormal
systolic position of the mitral valve.
REFERENCES
. Shone, J. D., Sellers, R. D., Anderson, R. C., Adams, P., Jr., Lillehei, C. W., and
1
Edwards, J. E.: The developmental complex of “parachute mitral valve,” supravalvular ring of left atrium, subaortic stenosis, and coarctation of aorta, Am. J.
Cardiol. 11:714, 1963.
2
. Mehrizi, A., Hutchins, G. M., Wilson, E. F., Breckenridge, J. C., and Rowe, R. D.:
Supravalvular mitral stenosis, J. Pediat. 67:1141, 1965.
3
. Swan, H., Trapnell, J. M., and Denst, J.: Congenital mitral stenosis and systemic
right ventricle with associated pulmonary vascular changes frustrating surgical
repair of patent ductus arteriosus and coarctation of the aorta, Am. Heart J. 38:914,
1949.
4
. Prado, S., Levy, M., and Varco, R. E.: Successful replacement of “parachute” mitral
valve in a child, Circulation 32:130, 1965.
5
. Carey, L. S., Sellers, R. D., and Shone, J. D.: Radiologic ndings in the development
complex of parachute mitral valve, supravalvular ring of left atrium, subaortic
stenosis, and coarctation of aorta, Radiology 82:1, 1964.
6
. Brockenbrough, E. C., Braunwald, E., and Morrow, A. G.: Ahemodynamic technic
for the detection of hypertrophic subaortic stenosis, Circulation 23:189, 1961.
7
. Simon, A. L., Ross, J., Jr., and Gault, J. H.: The angiographic anatomy of the left
ventricle and mitral valve in idiopathic hypertrophic subaortic stenosis, Circulation
36:852, 1967.
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Case 108 Chronic Intravascular Hemolysis
(Renal Hemosiderosis) After Incomplete
Prosthetic Closure of a Ventricular Septal
Defect and Noncalcific Aortic Regurgitation
Thomas J. Liddy, MD, and William C. Roberts, MD
Pathologic Anatomy Branch, National Cancer Institute and Section of Pathology, National
Heart and Lung Institute, National Institutes of Health, Bethesda, Maryland 20014
Clinical and pathologic features of the case of an 11-year-old boy who
underwent operative repair of tetralogy of Fallot nine months before death are
described. Aortic regurgitation was produced inadvertently at operation during
an unsuccessful patch closure of the ventricular septal defect. Postoperatively,
the child developed severe cardiac failure and mild anemia. During a second
operation seven days before death, aortic blood, which regurgitated through
the aortic valve, was observed to contact the nonendothelialized ventricular
septal patch, which only partially closed the septal defect. Erythrocytes were
traumatized by the jet of blood contacting the nonendothelialized patch,
liberating free hemoglobin, which was ltered by glomeruli and reabsorbed by
renal tubules (renal hemosiderosis). Intravascular hemolysis after patch closure
of ventricular septal defect has not been described previously.
CHRONIC INTRAVASCULAR HEMOLYSIS occurs occasionally in patients with
severe calcic aortic valvular stenosis and regurgitation.
valves with a caged-ball or Teon-leaet prosthesis, however, intravascular hemolysis invariably occurs,
2–4
but it usually is not clinically signicant. Chronic intra-
1
After replacement of aortic
vascular hemolysis also occurs occasionally in patients with partial atrioventricular
canals after prosthetic closure of the atrial septal defects, with or without repair of
the cleft anterior mitral leaets.
5–7
The hemolysis in these patients has been attributed to contact of erythrocytes regurgitated from the left ventricle at high velocity
and pressure against the prosthetic atrial septal patch. A review of the literature
disclosed that intravascular hemolysis after operative closure of ventricular septal defect with prosthetic material has not been described. However, intravascular
hemolysis did develop in a patient studied by us, in whom aortic valvular regurgitation was produced inadvertently while a ventricular septal defect was being closed
incompletely. Clinical and autopsy ndings in this patient are described.
REPORT OF A CASE
An 11-year-old boy had undergone repair of tetralogy of Fallot eight months before
admission to the National Heart Institute. The ventricular septal defect allegedly
had been closed by an Ivalon patch and the valvular and infundibular pulmonic
stenosis was thought to be alleviated. During closure of the septal defect, a suture
had caught an aortic valvular cusp and aortic regurgitation occurred. Severe rightsided congestive failure, evident immediately after operation, became progressively
Received April10, 1969; accepted for publication May27, 1969.
Dr. Liddy’s present address is: St. Barnabas Medical Center, Livingston, N. J. 07039.
Requests for reprints should be directed to Dr. Roberts.
116 DOI: 10.1201/9781003409342-15
Case 108 ChroniC intraVasCular hemolysis (renal hemosiderosis)
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Figure 1 Opened heart of the patient. a (upper), the aortic root, aortic valvular
cusps, Ivalon patch (arrow), anterior leaet of the mitral valve (M.V.), and the ventricular septum. The patch closing the ventricular septal defect is not covered by
endothelium. The perforations in the noncoronary cusp are not seen in this view.
b (lower), the right ventricle (R.V), tricuspid valvular orice (T.V. O.), ventricular septal patch (arrow), and site of the surgically removed pulmonic valvular cusps (P.V.).
more severe. Catheterization a month after operation disclosed a residual large leftto-right shunt at the ventricular level.
When admitted to the National Heart Institute for the rst time eight months later
(a month before death), the patient was severely ill (class IV). Agrade 5/6 ejection-type
systolic murmur was audible over the entire precordium and a grade 4/6 decrescendo
diastolic blowing murmur was heard along the left sternal border. The hematocrit was
37%, hemoglobin 11.3 Gm. per 100 ml., platelet count 59,000 per cu. mm., and leukocyte
count 6,000 per cu. mm. Serum iron was 86 µg. and the total iron-binding capacity, 330
µg. The direct Coombs’ test proved negative. Total serum bilirubin was 2.7 mg. per
100 ml. and blood urea nitrogen, 10 mg. per 100 ml. Urine was normal. Repeat cardiac
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Figure 2 Kidney. Upper left, cut section. Upper right, same kidney after soaking
in Prussian blue solution for 2 min. The cortex stained dark blue, indicating heavy
deposits of iron, whereas the medulla did not stain. Lower left, photomicrograph of
a section of kidney stained by the Prussian blue method. The dark-stained tubules
indicate deposits of iron. The medulla (bottom) is free of iron deposits. × 20. Lower
right, close-up showing that the iron deposits are situated predominantly in the
cytoplasm of the proximal convoluted tubules, although some iron-positive material
is present in Bowman’s space and in the lumen of the proximal tubules. × 230.
catheterization disclosed a left-to-right shunt (1.5 to 1) at the ventricular level and elevated right ventricular (60/20mm. Hg) and right atrial pressures (mean, 17; a wave,
23; v wave, 23mm. Hg). The femoral arterial pressure was 110/48mm. Hg. At reoperation (seven days before death), two perforations, each about 0.5cm. in diameter, were
found in the noncoronary aortic valvular cusp. The aortic valvular cusps were brotic,
smooth, and free of calcic deposits; the two perforations, which were responsible
for the severe aortic regurgitation, were closed by sutures. It was apparent that the
regurgitant stream was in direct line with the ventricular septal patch, which was not
covered by endothelium. The residual shunt resulted from partial detachment of the
ventricular septal patch; the detached portion was reapproximated to the margin of
the defect by sutures. Postoperatively, the patient had prolonged periods of hypotension, grand mal seizures, and hyperbilirubinemia (17 mg. per 100 ml.), and died.
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Case 108 ChroniC intraVasCular hemolysis (renal hemosiderosis)
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At autopsy, the ventricular septal defect and the aortic valvular perforations were
well closed (Figure 1). The erythroid elements in the bone marrow were hyperplastic
and large deposits of iron were present in the cytoplasm of the proximal convoluted
tubules of the kidney (Figure 2). No stainable iron was present in the liver or spleen.
COMMENTS
The intravascular hemolysis almost certainly resulted from damage to erythrocytes
which contacted the nonendothelialized ventricular septal patch. Blood which regurgitated from the aorta through the perforated aortic valvular cusp and ejected from
the left ventricle through the residual ventricular septal defect had direct contact with
the prosthetic patch. Although few clinical tests for hemolysis were performed, there
is unequivocal anatomic evidence, i.e., renal hemosiderosis, that chronic intravascular
hemolysis had occurred. Chronic intravascular hemolysis is the only condition which
causes severe renal hemosiderosis without associated deposits of iron in the liver or
8
Acute hemolysis (resulting from cardiopulmonary bypass, for example) may
spleen.
cause glomerular ltration of hemoglobin, but hemosiderin in these patients is present only in the tubular lumens and in Bowman’s spaces.
3
Prolonged periods of intravascular hemolysis are necessary before stainable iron can be detected in the cells of
the proximal convoluted tubules. The amount of intravascular hemolysis required to
produce severe renal hemosiderosis is not precisely known, but the extracorpuscular
hemoglobin, at least initially, must exceed 100 to 140 mg. per 100 ml. plasma for hemoglobin to lter through renal glomeruli.
depletes the serum haptoglobin, and this threshold falls accordingly.
9
Prolonged intravascular hemolysis, however,
10
The hemolysis
in the patient described was well compensated, since he was only mildly anemic. The
pronounced erythroid hyperplasia of the bone marrow in the presence of normal arterial oxygen saturation, however, indicates an active stimulus to erythropoiesis.
REFERENCES
1. Roberts, W. C.: Renal hemosiderosis (blue kidney) in patients with valvular heart
disease. Amer. J. Path. 48: 409–419, 1966.
2. Pirofsky, B., Sutherland, D. W., Starr, A., and Griswold, H. E.: Hemolytic anemia complicat-
ing aortic-valve surgery. An autoimmune syndrome. New Eng. J. Med. 272: 235–239, 1965.
3. Roberts, W. C., and Morrow, A. G.: Renal hemosiderosis in patients with pros-
thetic aortic valves. Circulation 33: 390–398, 1966.
4. Sears, D. A., and Crosby, W. H.: Intravascular hemolysis due to intracardiac pros-
thetic devices. Diurnal variations related to activity. Amer. J. Med. 39: 341–354, 1965.
5. Sayed, H. M., Dacie, J. V., Handley, D. A., Lewis, S. M., and Cleland, W. P.: Haemolytic
anaemia of mechanical origin after open heart surgery. Thorax 16: 356–360, 1961.
6. Sigler, A. T., Forman, E. N., Zinkham, W. H., and Neill, C. A.: Severe intravascular hemoly-
sis following surgical repair of endocardial cushion defects. Amer. J. Med. 35: 467–480, 1963.
7. Verdon, T. A., Jr., Forrester, R. H., and Crosby, W. H.: Hemolytic anemia after
open-heart repair of ostium-primum defects. New Eng. J. Med. 269: 444–446, 1963.
8. Leonardi, P., and Ruol, A.: Renal hemosiderosis in the hemolytic anemias:
Diagnosis by means of renal biopsy. Blood 16: 1029–1038, 1960.
9. Lathem, W.: The renal excretion of hemoglobin: Regulatory mechanisms and the differ-
ential excretion of free and protein-bound hemoglobin. J. Clin. Invest. 38: 652–658, 1959.
10
. Veneziale, C. M., McGuckin, W. F., Hermans, P. E., and Mankin, H. T.:
Hypohaptoglobinemia and valvular heart disease: Association with hemolysis
after insertion of valvular prostheses and in cases in which operation had not
been performed. Mayo Clin. Proc. 41: 657–662, 1966.
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Case 122 Congenital Atresia of the
Left Main Coronary Artery
Nicholas J. Fortuin, MD,* and William C. Roberts, MD
Bethesda, Maryland
Clinical and pathologic features are described in a sixty-one year old man with
long-standing clinical manifestations of ischemic heart disease. In addition to
severe coronary atherosclerosis, congenital atresia of the left main coronary
artery was present. Sudden death probably resulted from acute narrowing of
the conus artery, the major collateral supplying the left ventricle.
The ndings of angina pectoris, left bundle branch block, atrial brillation, systemic
hypertension, hyperglycemia and hypercholesterolemia in an adult strongly suggest ischemic heart disease secondary to coronary atherosclerosis. Apatient recently
studied with these features died suddenly and at necropsy was found to have a congenitally atretic left main coronary artery in addition to extensive coronary atherosclerosis. Adescription of the coronary anomaly found in him and a discussion of
its meaning and signicance are presented.
CASE REPORT
A sixty year old physician (J.M.) died suddenly at home on March8, 1969. He had
been in good health until 1949 (age forty) when atrial brillation appeared. He was
given digitalis for control of ventricular rate, and he continued to be asymptomatic
until 1958 (age forty-nine) when he noted the onset of exertional, substernal chest
pain which was typical of angina pectoris. Evaluation at this time disclosed moderate obesity and mild hypertension (blood pressure 150/100mm Hg). An electrocardiogram (Figure 1) showed atrial brillation and nonspecic ST-T wave changes.
Achest roentgenogram (Figure2) showed the heart to be at the upper limits of normal in size. During the next ten years the angina did not worsen and occurred only
with heavy exertion. Routine yearly examinations documented the persistence of
mild systemic hypertension, but no changes in chest roentgenogram or electrocardiogram were noted. When seen on March12, 1968, (one year before death) the patient
had no new complaints. Blood pressure was 160/90mm Hg. The heart was enlarged
by palpation, and a grade 2/6 mid-systolic ejection murmur heard maximally at the
base but well transmitted to neck and apex was described for the rst time. An electrocardiogram (Figure1) showed atrial brillation and left bundle branch block. On
chest roentgenogram (Figure2) the heart was enlarged. The two-hour postprandial
blood glucose level was 258 mg per cent, serum cholesterol 317 mg per cent and
serum uric acid 8.6 mg per cent. In January1969 the patient consulted a physician
complaining of a clear-cut change in the pattern of his angina. Chest pain occurred
more frequently, with less provocation, occasionally after heavy meals and at rest.
From the Section of Pathology, National Heart and Lung Institute, National Institutes of
Health, Bethesda, Maryland 20014. Requests for reprints should be addressed to Dr. William C.
Roberts, National Heart and Lung Institute, National Institutes of Health, Bethesda, Maryland
20014. Manuscript received February16, 1970.
* Present address: 1004 Columbia Street, Chapel Hill, North Carolina 27514.
120 DOI: 10.1201/9781003409342-16
Case 122 Congenital atresia of the left main Coronary artery
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Figure 1 Electrocardiograms recorded nearly ten years apart.
Figure 2 Chest roentgenograms taken nearly ten years apart.
121
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