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CASE REPORTS IN CARDIOLOGY
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Case 531 Cholesteryl Ester Crystals in a Porcine Aortic
Valvular Bioprosthesis Implanted for Eight Years
Victor J. Ferrans, MD, PhD; Bruce McManus, MD, PhD;
and William C. Roberts, MD, F.C.C.P.
Masses of crystals, which were largely composed of cholesteryl esters, were
found in a porcine aortic valvular bioprosthesis removed eight years after
implantation in the mitral position in a patient with rheumatic mitral valvular
stenosis. Histologic sections of grossly raised and nonraised yellow lesions
in the three cusps of this bioprosthesis revealed large clefts, which on frozen
section contained lipid-positive, birefringent crystals. These crystals gave a
positive reaction with the Schultz test for cholesterol. Biochemical analyses of
isolated nodules revealed a cholesterol content of 40 nmole/mg of wet tissue.
Of this cholesterol, 88percent was esteried, and the remaining 12percent was
free cholesterol. These cholesterol deposits are most likely derived from blood
lipids; however, they were not related to hyperlipidemia, since the patient had
normal blood levels of cholesterol and triglycerides.
Small lipid droplets are found relatively frequently in connective tissue of the leaets
of implanted cardiac valvular bioprostheses, as documented by previous electron
microscopic observations from this laboratory;
cholesterol have not been reported previously in implanted bioprostheses. Such a
nding forms the basis of the present report.
1
however, crystalline deposits of
CASE REPORT
A 76-year-old woman with a history of rheumatic mitral valvular stenosis, previous
closed mitral commissurotomy (1966), and replacement of the mitral valve with
a Hancock porcine aortic valvular bioprosthesis (1973) died in December 1981 in
intractable chronic congestive heart failure. She had marked pulmonary arterial
hypertension (100/10mm Hg before mitral replacement and 75/15mm Hg six months
after operation) and elevation of left ventricular end-diastolic pressure (20mm Hg in
1974); her systemic arteria l blood pressu re ranged up to 170/90m m Hg. The bioprosthetic
heart valve was considered to have functioned normally until the patient’s death.
Blood lipids had been within normal limits; cholesterol was 189 ± 36 mg/dl (mean ± SD
of nine values), and triglycerides were 107 mg/dl (mean of three values).
Anatomic Findings
At autopsy, the heart weighed 820 g. The coronary arteries were free of atherosclerotic
plaques. Atherosclerotic changes in aorta, cerebral vessels, and peripheral systematic
arteries were mild. The left atrium was markedly dilated and contained a small
mural thrombus. Both ventricles were hypertrophied, and the left was dilated. Focal
brous intimal thickening was present in the main left and right pulmonary arteries.
The mitral prosthesis was well-secured, without perivalvular leaks. The atrial and
ventricular aspects of the prosthetic valve ring were covered by brous tissue; this
From the Pathology Branch, National Heart, Lung, and Blood Institute, National Institutes of
Health, Bethesda, Maryland.
Reprint requests: Dr. Ferrans, National Institutes of Health, Bldg 10, Rm 7N208, Bethesda 20205
160 DOI: 10.1201/9781003409281-27
CASE 531 CHOLESTERYL ESTER CRYSTALS IN A PORCINE AORTIC VALVULAR BIOPROSTHESIS
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tissue, however, did not impinge on the bioprosthetic cusps. No tears or perforations
were present in the cusps. Tiny, elevated, yellow nodules, up to 2mm in diameter,
were evident grossly in each of the cusps, particularly on their outow surfaces
(Fig ur e s1A and B). Calcic deposits were not found on roentgenographic exam ination
of the bioprosthesis (Figure1C). Study of histologic sections revealed that the yellow
nodules on the leaets were composed of masses of crystals embedded in a coarsely
granular, eosinophilic matrix (Figure2 to 5). In sections of plastic-embedded tissues
these crystals appeared as empty, rectangular clefts that measured up to several
hundred μ in length. No cellular reaction (macrophages, foam cells, giant cells, or
Figure 1 Views of the porcine bioprosthesis after removal from the mitral position
and detachment from the stents. A, left: Inow surface, showing light areas (arrow-
heads) corresponding to large crystalline deposits. The cusps appear otherwise normal. B, center: Outow (left ventricular) surface, showing raised crystalline deposits
(arrowheads). C, right: Radiograph of the bioprosthesis, conrming the absence of
calcic deposits in the cusps.
Figure 2 Section of cusp, showing numerous, large clefts within the connective
tissue matrix of the cusp and nodular excrescence on the outow surface. Areas
of delamination of cuspal connective tissue are also noted. Alkaline toluidine blue
stain, ×50.
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Figure 3 High-power view of lesion shown in Figure2. Tlie supporting stroma
is predominantly collagenous; however, a rather deeply staining, granular material
is associated with the clefts remaining after lipid removal. Alkaline toluidine blue
stain, × 160.
Figure 4 Polarized light micrograph of portion of a cusp, showing diffuse inltration of the cuspal tissue by birefringent crystals, which tend to be localized on the
outow side of the cusp. Additional clefts without birefringent material represent
areas of extensive delamination of collagen. (Sudan black B stain, × 40).
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CASE 531 CHOLESTERYL ESTER CRYSTALS IN A PORCINE AORTIC VALVULAR BIOPROSTHESIS
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other inammatory cells) was associated with these nodules of crystals. Smaller,
nonraised masses of crystals were also present within the leaet connective tissue.
Sections stained by the von Kossa method did not show calcic deposits either
in association with the crystals or elsewhere in the bioprosthetic tissue. Frozen
sections stained with oil red O or with Sudan black B revealed multiple, small,
nonbirefringent, darkly stained lipid droplets; the crystals described above were
much less intensely stained than the lipid droplets. The crystals were markedly
birefringent (Figures4 and 5) and gave a positive reaction with the Schultz method
for the histochemical demonstration of cholesterol.
In addition to the lipid inltration described above, the connective tissue in the
bioprosthetic leaets showed moderate degrees of delamination (separation of the
connective tissue layers) and degeneration, with the formation of many empty spaces
within the substance of the leaets. Fibrous sheaths or thrombi were not present over
the cusps, and few cells (mostly macrophages) were present on the cuspal surfaces.
In order to further investigate the composition of the crystalline material, two
nodules were excised from the leaets with the aid of a dissecting microscope and
submitted for biochemical analysis. The results, expressed in nanomoles per mg of
wet tissue weight (mean± SE of quadruplicate determinations), were as follows: total
cholesterol, 40.25 ± 2.72, of which 5.18 ± 0.75 was in the form of free cholesterol and
34.07 ±2.82 in the form of cholesteryl esters.
Figure 5 High-power polarized light micrograph showing the birefringent crystals in cuspal tissue. Sudan black B stain, × 400.
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DISCUSSION
The observations in the present study document the presence of crystals, composed
mainly of cholesteryl esters, in a bioprosthesis which had been implanted in the
mitral position for eight years, during which time it had functioned well. Deposits of
lipid droplets, presumed to be composed of t riglycerides, have been observed in other
bioprostheses,
1
but such large, crystalline masses have not been reported previously,
to our knowledge, in implanted bioprostheses. Various types of lipid deposits
are known to occur in native cardiac valves in a number of metabolic disorders.
Most notable among the latter is homozygous type 2 hyperlipoproteinemia, in
which the aortic valve is the site of formation of large intracellular (foam cells) and
extracellular deposits of cholesteryl esters; other valves are involved to a much lesser
2–6
extent.
include Fabry’s disease,
Sandhoff’s disease,
cells, and cholesteryl ester storage disease,
have been detected in aortic valve. Lipid deposits also occur in association with
the lesions of calcic aortic stenosis and mitral annular calcication.
rubber balls in certain mechanical prosthetic valves have been reported
Other diseases in which lipid deposits have been found in cardiac valves
7,8
Farber’s disease,9 generalized GM1 gangliosidosis,
12
in which glycolipids are stored in valvular connective tissue
13
in which cholesteryl ester deposits
14, 15
10, 11
and
The silicone
16
to undergo
inltration with lipids derived from plasma lipoproteins; consequent swelling and
deformation of the ball may cause mechanical dysfunction of the prosthesis.
The reason for the accumulation of cholesterol in the bioprosthesis from our
patient remain s unclear. This patient did not have hyperlipidemia, as demonst rated by
multiple measurements of serum cholesterol and triglycerides. Thus, these deposits
cannot be regarded as consequences of hypercholesterolemia. The deposits in the
bioprosthesis from our patient were localized mainly on the outow (ventricular)
surface of the bioprosthesis, the site receiving the highest ventricular pressure. It
was surprising to nd that these deposits were not in association with a foreign body
reaction or any other type of inammatory inltrate, such as occurs with cholesterol
deposits in other areas of the body. This lack of inammatory reaction probably was
due to the fact that the cholesterol crystals were localized within the glutaraldehydexed porcine valvular tissue, which usually is not invaded by inammatory cells.
These deposits probably are derived from circulating plasma lipoproteins, since
there were no cells identied in adjacent areas of valvular tissue which could have
synthesized these large amounts of cholesterol in situ. Morphologic observations
on valvular bioprostheses implanted for eight years or longer have been few, and
it remains to be determined how prevalent this change is in these bioprostheses.
This is difcult to judge from review of gross anatomic and histologic observations.
On gross anatomic study, these deposits can resemble calcic deposits, which also
appear as pale nodules. It is possible that mild degrees of this alteration are relatively
frequent, but are not detected by study of routine histologic sections, from which
the cholesterol is removed during tissue processing. Polarized light microscopy of
frozen sections is the method of choice for the demonstration of these cholesterol
deposits.
ACKNOWLEDGMENT: We are grateful to Dr. Jeffrey M. Hoeg of the Molecular
Disease Branch, National Heart, Lung, and Blood Institute, for performing the
cholesterol analyses.
1
REFERENCES
1. Ferrans VJ, Spray TL, Billingham ME, Roberts WC. Structural changes in glu-
taraldehyde-treated porcine heterografts used as substitute cardiac valves:
Transmission and scanning electron microscopic observations in 12 patients.
Am J Cardiol 1978; 41:1159–1184
164
CASE 531 CHOLESTERYL ESTER CRYSTALS IN A PORCINE AORTIC VALVULAR BIOPROSTHESIS
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2. Stanley P, Chartrand C, Davignon A. Acquired aortic stenosis in a twelve-year-
old girl with xanthomatosis. N Engl J Med 1965; 273:1378–1380
3. Rothbard S, Hagstrom JWC, Smith JP. Aortic stenosis and myocardial infarction
in hypercholesterolemic xanthomatosis. Am Heart J 1967; 73:687–692
4. Wennevold A, Jacobsen JG. Acquired supravalvular aortic stenosis in familial
hypercholesterolemia: a hemodynamic and angiographic study. Am J Med 1971;
50:823–827
5. Barr DP, Rothbard S, Eder HA. Atherosclerosis and aortic stenosis in hypercho-
lesteremic xanthomatosis. JAMA 1954; 156:943–947
6. McCleary JE, Brunsting LA, Kennedy RLJ. Primary xanthoma tuberosum in
children; with classication of xanthomas. Pediatrics 1959; 23:67–75
7. Desnick RJ, Blieden LC, Sharp HL, Hofschire PJ, Moller JH. Cardiac valvular
anomalies in Fabry disease: clinical, morphologic, and biochemical studies.
Circulation 1976; 54:818–825
8. Ferrans VJ, Hibbs RG, Burda CD. The heart in Fabry’s disease: a histochemical
and electron microscopic study. Am J Cardiol 1969; 24:95–110
9. Farber S, Cohen J, Uzman LL. Lipogranulomatosis. A new lipoglycoprotein
“storage” disease. Mt Sinai J Med (NY) 1957; 24:816–837
10. Gonatas NK, Gonatas J. Ultrastructural and biochemical observations on a case
of systemic late infantile lipidosis and its relationship to Tay-Sachs disease and
gargoylism. J Neuropathol Exp Neurol 1965; 24:318–340
11. Hadley RN, Hagstrom JWC. Cardiac lesions in a patient with familial neurovis-
ceral lipidosis (generalized gangliosidosis). Am J Clin Pathol 1971; 55:237–240
12. Blieden LC, Desnick RJ, Carter JB, Krivit W, Moller JH, Sharp HL. Cardiac
involvement in Sandhoffs disease: inborn error of glycosphingolipid metabolism. Am J Cardiol 1974; 34:83–88
13. Fredrickson DS, Ferrans VJ. Acid cholesteryl ester hydrolase deciency.
(Wolmans disease and cholesteryl ester storage disease). In: Stanbury JB, et al,
eds. The metabolic basis of inherited disease, 4th ed. New York: McGraw-Hill,
1978:670–687
14. Kim KM, Valigorsky JM, Mergner WJ, etal. Aging changes in the human aortic
valve in relation to dystrophic calcication. Hum Pathol 1976; 7:47–60
15. Roberts WC, Perloff JK. Mitral valvular disease: a clinicopathologic survey of
the conditions causing the mitral valve to function abnormally. Ann Intern Med
1972; 77:939–975
16. Carmen R, Mutha SC. Lipid absorption by silicone rubber heart valve poppets—
in vivo and in vitro results. J Biomed Material Res 1972; 6:327–346
165
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Case 543 Severe Mitral Regurgitation Immediately
After Mitral Valve Replacement with a
Parietal Pericardial Bovine Bioprosthesis
Marc A. Silver, MD, Philip R. Orenburg, MD and William C. Roberts, MD
The occurrence of severe mitral regurgitation (MR) immediately after mitral valve
replacement (MVR) is exceedingly rare. One cause is interference with closure of a
prosthetic occluder by the left ventricular wall, calcic deposits, or residual unexcised
mitral leaet or chordae tendineae.
suture of the space between the prosthetic or bioprosthetic ring and the native anulus,
resulting in a paraanular communication.
severe MRimmediately after MVR by a bioprosthesis is described in the patient below.
A 64-year-old man who had had a precordial systolic murmur for many years had
been asymptomatic until 25 days before MVR, when signs of active infective endocarditis
appeared; severe congestive heart failure followed shortly thereafter. Group B beta hemolytic
streptococcus was grown on blood cultures. Agrade 3/6 precordial apical systolic blowing
murmur consistent with MR was audible. The pulmonary arterial wedge mean pressure
was 35, V-wave 50, and pulmonary arterial pressure 57/20mm Hg. At MVR, a vegetation
was present on the posterior mitral leaet to which several chordae had ruptured; the
leaet appeared to prolapse toward left atrium. The purely regurgitant valve was replaced
with a 25-mm lonescu-Shiley bioprosthesis. The patient’s early postoperative course was
characterized by systemic hypotension and coma. Amurmur consistent with MRwas heard
on precordial examination 2 days after operation. He died 8 days after operation, never
having regained consciousness. At necropsy, the heart weighed 690 g. Sutures surrounded
2 of the 3 stents of the mitral bioprosthesis, causing a straight-line stretching of the margins
of the bioprosthetic cusps between the 3 commissures. The result was a triangular-shaped
severely incompetent bioprosthetic orice (Figure1).
1
Another cause is incomplete obliteration by
1
Athird and hitherto unreported cause of
Figure 1 Mitral bioprosthesis in the patient described. a, view from left atrium
showing a triangular orice. b and c, 2 views from the left ventricular aspect. The
arrows point to the 2 sutures which encircled the stents, causing tautness of the
cusps and preventing them from coapting during ventricular systole.
From the Pathology Branch, National Heart, Lung, and Blood Institute, National Institutes of
Health, Bethesda, Maryland. Manuscript received and accepted April5, 1983.
166 DOI: 10.1201/9781003409281-28
CASE 543 SEVERE MITRAL REGURGITATION IMMEDIATELY AFTER MITRAL VALVE REPLACEMENT
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Thus, sutures encircling 2 of the 3 bioprosthetic stents tautened the cusps,
producing severe MR. The left atrium should not be closed after MVR until it is
clear that the cusps of a tissue valve or the occluder of a mechanical valve
2
are freely
mobile.
REFERENCES
1. Rob ert s WC. Complications of card iac valve replacement: character istic abnormal-
ities of prostheses pertaining to any or specic site. Am Heart J 1982;103:113–122.
2. Jones AA, Oils JB, Fletcher GF, Roberts WC. Ahitherto undescribed cause of
prosthetic mitral valve obstruction. J Thorac Cardiovasc Surg 1977;74:116–117.
167
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Case 633 Fatal Bioprosthetic Regurgitation
Immediately After Mitral and Tricuspid Valve
Replacements with lonescu-Shiley Bioprostheses
Wanda M. Lester, MD and William C. Roberts, MD
Signicant cardiac valve regurgitation immediately after cardiac valve replacement
is rare. Silver et al
1
described a patient who had severe bioprosthetic regurgitation
immediately after replacement of the mitral valve with an Ionescu-Shiley
bioprosthesis. The regurgitation resulted from inadvertent encircling of 2 of the 3
stents of the bioprosthesis by sutures, which prevented mobility of the cusps and
closure of the bioprosthetic orice. In this report we describe another patient in
whom a similar mechanism caused severe bioprosthetic regurgitation in both mitral
and tricuspid valve positions. Because this mechanism of development of severe
bioprosthetic regurgitation is apparently not well appreciated, we describe this
second patient in hopes of preventing this fatal complication in others.
E.R., a 55-year-old woman, died shortly after her fth cardiac operation. She had
had mitral commissurotomies when 26 and 35 years old, aortic (Björk-Shiley) and mitral
(Hancock) valve replacements when 46years old, and tricuspid valve replacement (Hancock)
1 month later. Apart from an intracranial hemorrhage later in the same year (1976), which
led to cessation of warfarin therapy and the use öf aspirin and dipyridamole, she did well
until about 4 months before death, when exertional dyspnea, easy fatigability, abdominal
swelling and leg edema developed. Because of worsening symptoms and signs, she was
hospitalized 2 months later. The neck veins were distended while she was sitting up and
v waves were visible. Rales were present over both lower lung elds. The liver was large
and pulsatile. Electrocardiogram showed atrial brillation and right bundle branch block.
At cardiac catheterization, the pressures (in mm Hg) were: right atrial mean 14, v wave 18;
right ventricle, 70/16; pulmonary artery, 70/25 (mean 35); pulmonary arterial wedge mean
13, v wave 16; left ventricle, 110/12, and aorta, 110/60. The mean diastolic gradient between
pulmonary artery wedge position and left ventricle was 5mm Hg and the mean gradient
between right atrium and right ventricle was 2 mm Hg. On left ventricular angiogram,
the bioprosthesis in the mitral position was competent. The ejection fraction was 44%.
Selective coronary angiograms showed no abnormalities. Aortogram disclosed 1+/4+ aortic
regurgitation. The cardiac index was 2.1 liters/min/m
2
. Pulmonary function tests were
consistent with moderate to severe pulmonary obstructive disease.
The Hancock bioprosthesis in the mitral position was replaced with a size 27 and the
Hancock bioprosthesis in the tricuspid position was replaced with a size 29 Ionescu-Shiley
bioprosthesis. Poor cardiac output with some excessive bleeding was evident immediately
after discontinuing bypass. The patient died about 8hours after beginning the operation.
Each cusp of the excised Hancock bioprostheses was intact and both valve orices were
competent. At necropsy, 1 stent of the bioprosthesis in the tricuspid valve position had
burrowed into the right ventricular myocardium, and 1 stent of this bioprosthesis was
surrounded by a suture (Figures1 and 2). The consequence of these 2 misadventures was a
straightening of the free margins of 2 of the 3 cusps so that this bioprosthetic orice was xed in
From the Pathology Branch, National Heart, Lung, and Blood Institute, National Institutes
of Health, Bethesda, Maryland 20205. Dr. Lester had a fellowship from the Canadian Heart
Foundation. Manuscript received and accepted November5, 1984.
168 DOI: 10.1201/9781003409281-29
CASE 633 FATAL BIOPROSTHETIC REGURGITATION IMMEDIATELY AFTER VALVE REPLACEMENTS
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Figure 1 a, heart after removal of the atrial walls showing the bioprostheses in the
tricuspid (T) and mitral (M) valve positions. The orices of both bioprostheses are
open and triangular in shape. The Björk-Shiley mechanical prosthesis in the aortic
(A) valve position appeared to have functioned well. b, view from the ventricular
aspect.
an open triangular-shaped position. One suture used to insert the bioprosthesis in the mitral
position crossed 2 of the 3 bioprosthetic cusps, making each immobile and preventing closure
of the bioprosthetic orices (Figures1 and 2). The occluder of the Björk-Shiley prosthesis in the
aortic valve position moved normally.
The fth and last operation in the patient was done because of distended neck
veins, subcutaneous edema and ascites, ndings believed to have resulted from
regurgitation of the bioprosthetic valve in the tricuspid valve position. The presence
or absence of regurgitation was not conrmed by right ventricular angiography.
At operation, no peribasilar bioprosthetic communications were present, each of
the 3 cusps of both bioprostheses was intact, and both orices appeared to close
satisfactorily. After replacement of the bioprostheses in both tricuspid and mitral
valve positions with “new”—and larger—bioprostheses, the patient had evidence of
severe low cardiac output. Necropsy disclosed that at least 2 of the 3 cusps of each of
the 2 newly inserted bioprostheses were immobile because either inadvertent suture
on the ventricular aspects of the bioprosthetic orices or burrowing of a stent into the
myocardial wall had caused straightening of the distal margins of the bioprosthetic
cusps and prevented cuspal movements so that the bioprosthetic orices could
not close during ventricular systole. Had smaller bioprostheses been used in this
81-pound, 61-inch-tall woman, possibly inadvertent anchoring of the sutures over
the margins of the cusps and the burrowing of 1 stent into a myocardial wall would
have been prevented. It is, of course, unwise to close an atriotomy incision until the
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