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(Figure 1C). The patient received a St. Jude Medical mechanical prosthesis, and his
postoperative course was uncomplicated.
The esti mated incidence of unicu spid aortic valve is 0.02%.
1-3
Duri ng development,
the aortic valve is formed from three tubercles, which each develop a cusp and
sinus of Valsalva. Fusion of the cusps results in a unicuspid valve. Unicommissural
unicuspid valves, as in our case, have one lateral attachment and an eccentric orice.
Acommissural unicuspid valves have no lateral attachment to the aorta.
REFERENCES
1. Novaro GM, Mishra M, Grifn BP. Incidence and echocardiographic features
of congenital unicuspid aortic valve in an adult population. J Heart Valve Dis
2003;12(6):674–678.
2. Roberts WC, Ko JM. Frequency by decades of unicuspid, bicuspid, and tricuspid
aortic valves in adults having isolated aortic valve replacement for aortic stenosis,
with or without associated aortic regurgitation. Circulation 2005;111(7):920–925.
3. Roberts WC, Ko JM. Clinical and morphologic features of the congenitally
unicuspid acommissural stenotic and regurgitant aortic valve. Cardiology
2007;108(2):79–81.
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CASE 1502 COMPARISON OF THE QUANTITY OF CALCIFIC DEPOSITS IN BIOPROSTHESES
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Case 1502 Comparison of the Quantity of Calcific
Deposits in Bovine Pericardial Bioprostheses in
the Mitral and Aortic Valve Positions in the Same
Patient Late After Double-Valve Replacement
William Clifford Roberts, MD,
d
and Gregory John Matter, MD
BA,
a,b,d
Carlos Ernesto Velasco, MD,a Jong Mi Ko,
c
Dallas, Tex
Among patients undergoing cardiac valve replacement, the aortic valve is most
commonly replaced, the mitral valve next, and, infrequently, both the mitral and
aortic valves. When the latter situation occurs and when the substitute valves
inserted are both bioprostheses, it is possible to compare the rates of degenerative
change because one bioprosthesis serves as a control for the other. In 1983, Warnes
and associates
1
reported on 5 patients with porcine bioprostheses in both the mitral
and aortic valve positions from 18 to 107 months, and in each of the 4 patients in
which the bioprosthesis was in place for greater than 18 months, the quantity of
calcic deposits on the cusps of the bioprosthesis in the mitral valve position was
much greater than that on the prosthesis in the aortic valve position. The present
report was prompted by observing a patient who had a bovine parietal pericardial
bioprosthesis in both the mitral and aortic positions explanted after they had been
in place for 77 months; the quantity of calcium in the bioprosthesis in the aortic
valve position was massive, and that in the bioprosthesis in the mitral position was
minimal.
CLINICAL SUMMARY
A patient, who was born on March25, 1949, had acute rheumatic fever in childhood
and hypothyroidism since age 20 years. She was in her usual good health until
April2001 (age 52years), when exertional dyspnea appeared, and it soon progressed
to orthopnea, which prompted hospital admission. Echocardiographic and cardiac
catheterization data are shown in Table 1. Electrocardiographic analysis disclosed
sinus rhythm.
On May 22, 2001, both the mitral and aortic valves were replaced with
Carpentier Edwards pericardial bovine bioprostheses treated with the XenoLogix
From the Departments of Internal Medicine (Division of Cardiology),a Pat h olog y,b and
Cardiothoracic Surgery,
Medical Center, Dallas, Tex.
Received for publication Jan 29, 2009; accepted for publication Feb 8, 2009; available ahead of
print March27, 2009.
Address for reprints: William C. Roberts, MD, Baylor Heart and Vascular Institute, Baylor
University Medical Center, 3500 Gaston Ave, Dallas, TX 75246 (E-mail: wc.roberts@baylorhealth.edu).
J Thorac Cardiovasc Surg 2009;138:1448–50
Copyright © 2009 by The American Association for Thoracic Surgery
doi:10.1016/j.jtcvs.2009.02.022
DOI: 10.1201/9781003409281-49 231
c
and the Baylor Heart and Vascular Institute,d Baylor University
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Table 1: Cardiac catheterization data in the patient presented
October10, 2007
Variable
Pulmonary artery (mm Hg) 75/40 102/54
Right ventricle (mm Hg) 75/28 102/24
Right atrium (mm Hg)
A wave 30 28
V wave 23 25
Mean 22 20
Pulmonary artery wedge (mm Hg)
A wave 37 42
V wave 38 46
Mean 34 33
Left ventricle (mm Hg) 159/28
Aorta (mm Hg) 165/95 130/82
Cardiac index (L · min1 · m2) 1.8 2.0
Ejection fraction (%) 40
Body weight (lbs) 156 138
Height (inches) 62 62
–, No information available.
the rst operation)
May18, 2001 (before
(before the second
operation)
Figure 1 Native mitral and aortic valves (A and B) and bovine pericardial bioprostheses in the mitral and aortic valve positions (C and D) in the patient described. A,
Anterior mitral leaet from the atrial aspect and aortic valve from the ventricular
aspect. B, Anterior mitral leaet from the ventricular aspect and aortic valve from
the aortic aspect. Both native valves are devoid of calcic deposits. C, Bioprosthesis
in the mitral position from the atrial aspect and bioprosthesis in the aortic position
from the aortic aspect. D, Bioprosthesis in the mitral position from the ventricular
aspect and bioprosthesis in the aortic position from the ventricular aspect. Heavy
calcic deposits are present on both surfaces of the bioprosthesis in the aortic valve
position.
232
CASE 1502 COMPARISON OF THE QUANTITY OF CALCIFIC DEPOSITS IN BIOPROSTHESES
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Figure 2 Radiograph of the bioprostheses excised from the aortic valve position
(left) and the mitral valve position (right). The calcic deposits are huge on the left
and small on the right.
tissue treatment process (Edwards LifeSciences, Irvine, Calif), which removes
approximately 98% of phospholipids, which are calcium-binding sites. The excised
anterior mitral leaet weighed 0.77 g, and the 3-cuspid aortic valve weighed 0.74 g
(Figure1, A and B). Both valves were free of calcium.
The patient was thereafter well until April2002, when a febrile illness developed.
Streptococcus sanguis was cultured from the blood. She was treated with antibiotics,
and her usual health returned. Vegetations were never observed on either
bioprosthesis by means of echocardiographic analysis. Another echocardiogram in
March2003 disclosed stenosis of the bioprosthesis in the aortic valve position.
She continued to be well until August2007, when exertional dyspnea recurred,
and within 3 weeks, she was essentially bedridden. She was rehospitalized on
September24, 2007, and re peat cardiac catheteriz ation (Ta b le 1) and echoca rdiographic
analysis showed mild bioprosthetic mitral regurgitation and nearly nonmovable
bioprosthetic cusps in the aortic valve position. The bioprosthesis in the aortic valve
position could not be crossed at cardiac catheterization. The coronary arteries were
normal on angiographic analysis.
On October 12, 2007, both bioprostheses were replaced with mechanical
prostheses, and a tricuspid valve annuloplasty was performed. At the time of
the operation, a small paravalvular leak was seen in the mitral position. The
bioprosthesis in the mitral position (no. 25) weighed 4.04 g, and the bioprosthesis
in the aortic position (no. 21) weighed 3.86 g (Figure1, C and D). Radiographs of
the operatively excised bioprostheses showed huge calcic deposits in the aortic
prosthesis and small deposits in the mitral prosthesis (Figure2). Her postoperative
course was relatively uneventful. As of November 2008, she is active, and her
activities are not limited. By means of echocardiographic analysis, her pulmonary
arterial systolic pressure had decreased to 34mm Hg, and left ventricular ejection
fraction had increased to 55%. There was only trace tricuspid valve regurgitation.
DISCUSSION
The patient described had parieta l pericardial bovine bioprosthe ses in both the mitral
and aortic valve positions for 77 months and during that period developed huge
quantities of calcium on the cusps of the bioprosthesis in the aortic valve position
and only small quantities of calcium on the cusps of the bioprosthesis in the mitral
valve position. Because the closing pressure on the mitral bioprosthesis is usually
about a third higher than that on the aortic bioprosthesis (peak left ventricular
systolic pressure vs end-diastolic aortic pressure; normally approximately 120 vs
80 mm Hg), it might be expected that the degeneration of a bioprosthesis in the
233
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mitral position would be greater (more calcium and more tears) and more rapid than
that of a bioprosthesis in the aortic position, but the opposite was the case in the
patient described herein. Why might that be the case? Some possibilities include the
following:
1. Parietal pericardial bovine bioprostheses are not the same as porcine aortic
valve bioprostheses. The former are thicker and less exible and possibly withstand the left ventricular peak systolic pressure and the aortic end-diastolic
pressure more easily than the more delicate porcine aortic cusps.
2. The bovine bioprosthesis in the aortic position was defective and not properly
prepared, whereas the one in the mitral position was not.
3. The febrile illness the patient had beginning 11 months after the initial cardiac operation could have been active infective endocarditis that affected
the bioprosthesis in the aortic position but not the bioprosthesis in the mitral
position.
4. Smaller bovine parietal pericardial bioprostheses calcify more rapidly and
more extensively than do larger bovine pericardial bioprostheses.
5. The paravalvular leak in the mitral position and the absence of a leak in the
aortic position provided a ‘‘bypass shunt,’’ diminishing the effect of the full
force of the peak left ventricular systolic pressure on the bioprosthetic cusps
in the mitral position.
None of these 5 possibilities can be proved or disproved, but this report might
stimulate careful follow-up of similar patients to determine whether this distribution
of calcium in the 2 left-sided bioprostheses is a pattern or an exception.
REFERENCE
1. Warnes CA, Scott ML, Silver GM, Smith CW, Ferrans VJ, Roberts WC. Comparison
of late degenerative changes in porcine bioprostheses in the mitral and aortic
valve position in the same patient. Am J Cardiol 1983;51:965–968.
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CASE 1506 COMBINED MITRAL AND AORTIC STENOSIS OF RHEUMATIC ORIGIN
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Case 1506 Combined Mitral and Aortic
Stenosis of Rheumatic Origin with DoubleValve Replacement in an Octogenarian
William Clifford Roberts
Albert Carl Henry III
a,b,d,
c
*, Jong Mi Kod, John Ryan Schumachera,
Replacement of both mitral and aortic valves in octogenarians is infrequent
especially for combined mitral stenosis (MS) and aortic stenosis (AS) of rheumatic
etiology. Such was the case, however, in the patient to be described herein.
An 81-year-old woman, who was born on 23 September 1925, had increasing
dyspnea for several years because of what was believed to be “pulmonary brosis,”
worse in the lower lobes than the upper lobes. In December2004, she was placed
on home oxygen, and in January2005, on continuous oxygen by nasal cannula. She
had never smoked and had no history of acute rheumatic fever. Because of rather
dramatic worsening of her dyspnea, she was hospitalized at Baylor University
Medical Center on 1 May 2007. Her body mass index was 20 kg/m
2
. Precordial
examination disclosed an apical diastolic murmur and a basal ejection type systolic
murmur. Electrocardiogram disclosed prolonged P-R interval and increased voltage
compatible with left ventricular hypertrophy. Telemetric monitoring disclosed runs
of atrial brillation.
Cardiac catheterization disclosed the following pressures in mm Hg: pulmonary
artery wedge a-wave 21, v-wave 34, and mean 22; pulmonary artery, 68/26 (mean 39);
left ventricle, 129/21 and aorta, 113/46; pulmonary arterial wedge—left ventricular
mean diastolic gradient 8, and simultaneous left ventricular-aortic peak systolic
gradient, 16. Cardiac index was 2.2 L/min/m
0.8 and the aortic valve area, 0.7cm
2
. Angiography disclosed insignicant coronary
2
. The calculated mitral valve area was
arterial narrowing.
On 4 May2007, both left-sided cardiac valves (Figure1) were replaced with St.
Jude Medical prostheses: mitral #29, and aortic #19. The postoperative course was
relatively smooth. The pulmonary arterial pressure fell dramatically, and she was
discharged to the Baylor Specialty Hospital on 14 May2007 where she remained for
17 days.
Thereafter, she did well until about 1 January 2008, when she noted pedal
edema which progressed to anasarca and a weight gain from 112 to 148 lb. She
was rehospitalized on 4 February2008. The blood hemoglobin was 9.8 g/L and the
a
Department of Internal Medicine, Division of Cardiology, Baylor University Medical Center, Dallas,
TX, United States
b
Department of Pathology, Baylor University Medical Center, Dallas, TX, United States
c
Department of Cardiothoracic Surgery, Baylor University Medical Center, Dallas, TX, United States
d
Baylor Heart and Vascular Institute, Baylor University Medical Center, Dallas, TX, United States
Received 29 October2008; accepted 1 November2008
Available online 30 November2008
*
Corresponding author. Department of Internal Medicine, Division of Cardiology, Baylor
University Medical Center, Dallas, TX, United States.
E-mail address: wc.Roberts@baylorhealth.edu (W.C . Roberts).
DOI: 10.1201/9781003409281-50 235
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Figure 1 Photographs of the operatively excised aortic valve (left) and mitral
valve (right). The mitral valve is shown from the atrial aspect in a and from the ventricular aspect in b. Radiographs of each valve are shown in c.
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CASE 1506 COMBINED MITRAL AND AORTIC STENOSIS OF RHEUMATIC ORIGIN
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Table 1: Functional and anatomic classication of valvular heart disease in 1010
necropsy patients aged > =15years
Functional class Patients
1. Aortic stenosis
(AS) 292 (29%) 256 (88%) 0 35 (12%) 0 1 (0.3%)
2. Mitral stenosis
(MS) 189 (19%) 0 117 (62%) 40 (21%) 13 (7%) 19 (10%)
MS+AS 152 (15%) 0 0 120 (79%) 0 32 (21%)
3.
4. Aortic
regurgitation
b
(AR)
5. Mitral
regurgitation
(MR) 97 (10%) 0 85 (88%) 8 (8%) 1 (1%) 3 (3%)
6. MS+AR 65 (6%) 0 52 (80%) 0 0 13 (20%)
7. MR+AR 45 (4%) 0 0 39 (87%) 0 6 (13%)
8. AS + MR 23 (2%) 0 0 21 (91%) 0 2 (9%)
9. Tricuspid
stenosis+MS±AS 28 (3%) 0 0 0 4 (14%) 24 (86%)
Totals 1010 (100%)c363 (36%) 254 (25%) 273 (27%) 18 (2%) 102 (10%)
AV=aortic valve; MV=mitral valve; TV=tricuspid valve.
Reproduced with permission from Elsevier and the author (Roberts WC. Am J Cardiol
1983;51:1005–1028).
a
Excludes patients with mitral regurgitation secondary to coronary heart disease (papillary
muscle dysfunction), carcinoid heart disease, hypertrophic cardiomyopathy, and those with
infective endocarditis limited to 1 or both right-sided cardiac valves. Tricuspid valve regurgitation was present in many patients in most of the 9 functional groups. All patients were in
functional class III or IV (New York Heart Association), and more than half had 1 or more
cardiac operations.
b
In many patients, the aortic valve cusps were normal or nearly normal and the regurgitation
was the result of disease of the aorta (Marfan and Marfan-like syndrome, syphilis, systemic
hypertension, healed aortic dissection).
c
The hearts in all 1010 patients were examined and classied by WCR.
119 (12%) 107 (90%) 0 10 (8%) 0 2 (2%)
a
Anatomic class
AV MV MV-AV TV-MV TV-MV-AV
hematocrit, 30.3%. With diuretic therapy she lost 30 lb and returned home feeling
much better. She was now in persistent atrial brillation. As of October2008, she
was doing well.
Although now infrequent in the Western world, combined MS and AS of
rheumatic origin was relatively common when acute rheumatic fever was far more
prevalent. Roberts
1
collected 1010 cases at autopsy of valvular heart disease studied
from approximately 1955 to 1980: isolated AS (with or without regurgitation) was the
most frequent valve lesion (29%); isolated MS (with or without mitral regurgitation)
was next (19%), and combined MSand AS was in third place (15%) (Table1). None of
the patients with combined MSand AS, however, was > 80years of age. In the last 3
decades, combined MSand AS of rheumatic etiology was infrequent in the Western
world.
Combined MSand AS is particularly rare in octogenarians. Uricchio etal.
1959 reported 141 patients with combined MSand AS, all of whom had undergone
both mitral and aortic commissurotomy: their ages ranged from 23 to 67 years.
2
in
237
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Katznelson et al.
3
studied 22 patients ranging in age from 23 to 61years (mean
45); the average age of onset of symptoms of cardiac dysfunction was 40. Honey
studied 35 patients and their ages ranged from 24 to 54years (mean 40). Reid etal.
studied 15 patients aged 26 to 48. Morrow etal.
of whom had combined mitral and aortic valvulotomy. Zitnik etal.
patients who ranged in age from 34 to 55 (mean 42). Roberts and Sullivan
6
studied 8 patients aged 20 to 48, all
7
studied 10 such
8
studied
at necropsy 30 such patients who died within 60 days of double valve replacement
for combined MSand AS: one was an octogenarian, aged 83, but the others were
younger (mean age 57). Berman et al.
9
performed combined percutaneous mitral
and aortic valvulotomy in 6 patients, aged 60 to 83. Thus, only 2 of the 267 patients
in those studies with combined MSand AS were octogenarians.
Combined MS and AS is more common in women than in men. Of the 267
patients reported in the previously mentioned 8 studies,
Several studies have exam ined cardiac hemodynam ics in patients with combined
MSand AS.
2–9
Although there are exceptions, the degree of AS when combined with
MSis not as great (transvalvular peak systolic gradient) as in patients with isolated
AS. Furthermore the MS, as emphasized by Zitnik etal.,
2–9
171 (64%) were women.
7
can mask the presence of
AS. If the AS is missed in this circumstance and mitral valve commissurotomy or
replacement is performed and the downstream AS is neglected dire consequences
can ensue. Obviously, it is best to diagnose both MSand AS preoperatively, but if
one lesion is to be missed, it is far better to missthe upstream problem (MS) than the
downstream problem (AS).
And nally, combined MS and AS can produce an operative challenge. First,
the patients are most commonly women, some of whom, as in the present patient,
are of small stature and, consequently, have relatively small hearts.
8
In combined
MSand AS, neither the left ventricular cavity nor the ascending aorta is dilated, a
circumstance which provides less space for either the mitral or the aortic mechanical
prosthesis or bioprosthesis compared to the space provided in patients with isolated
AS who often have congenitally bicuspid aortic valves and dilated ascending aortas.
4
5
ACKNOWLEDGMENT
The authors of this manuscript have certied that they comply with the Principles of
Ethical Publishing in the International Journal of Cardiology.
REFERENCES
1. Roberts WC. Morphologic features of the normal and abnormal mitral valve. Am
J Cardiol 1983;51:1005–1028.
2. Uricchio JF, Goldberg H, Sinha KP, Likoff W. Combined mitral and aortic ste-
nosis: clinical and physiologic features and results of surgery. Am J Cardiol
1959;4:479–491.
3. Katznelson G, Jreissaty RM, Levinson GE, Stein SW, Abelmann WH. Combined
aortic and mitral stenosis. Am J Med 1960;29:242–256.
4. Honey M. Clinical and haemodynamic observations on combined mitral and
aortic stenosis. Brit Heart J 1961;23:545–555.
5. Reid JM, Stevenson JG, Barclay RS, Welsh TM. Combined aortic and mitral ste-
nosis. Brit Heart J 1962;24:509–515.
6. Morrow AG, Awe WC, Braunwald E. Combined mitral and aortic stenosis. Brit
Heart J 1962;24:606–612.
7. Zitnik RS, Piemme TE, Messer RJ, Reed DP, Haynes FW, Dexter L. The masking
of aortic stenosis by mitral stenosis. Am Heart J 1965;69:22–30.
238
10
CASE 1506 COMBINED MITRAL AND AORTIC STENOSIS OF RHEUMATIC ORIGIN
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8. Roberts WC, Sullivan MF. Clinical and necropsy observations early after simulta-
neous replacement of the mitral and aortic valves. Am J Cardiol 1986;58:1067–1084.
9. Berman AD, Weinstein JS, Saan RD, Diver DJ, Grossman W, McKay RG.
Combined aortic and mitral balloon valvuloplasty in patients with critical aortic
and mitral valve stenosis: results in six cases. J Am Coll Cardiol 1988;11:1213–1218.
10. Coats AJ. Ethical authorship and publishing. Int J Cardiol 2009;131:149–150.
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