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Figure 3 Radiograph of the heart at necropsy. Ahuge amount of calcium is located
beneath the posterior mitral leaet and extends across the anterior (A) mitral leaet
forming a circle “O.” The left circumex coronary artery (LCCA) is heavily and diffusely calcied (about 50% of patients with a congenitally bicuspid aortic valve have
a dominant left circumex rather than a dominant right coronary artery [RCA], the
situation in about 90% of patients with a tricuspid aortic valve). Calcic deposits also
are present in the left main and left anterior descending coronary arteries.
of the posterior mitral valve leaet and the mural endocardium of the left
ventricle. When the calcium deposits are extensive, a “C”-shaped or “J”-shaped
conguration can appear on radiograph. In rare cases, the calcium extends
across the anterior mitral leaet forming an “O”-shaped conguration.
annular calcium is often associated with mild or moderate mitral regurgitation,
but severe regurgitation due to the calcium deposits alone probably does not
2
Signicant mitral stenosis due to annular calcium has been reported only
occur.
in the setting of left ventricular outow obstruction, as occurred in the present
patient.
3
REFERENCES
1. Roberts WC. The senile cardi ac calcication syndrome. Am J Cardiol 1986;58: 572–574.
2. Roberts WC, Waller BF. Mitral valve “annular” calcium forming a complete
circle or “O” conguration: clinical and necropsy observations. Am Heart J
1981;101:619–621.
3. Hammer WJ, Roberts WC, deLeon AC. “Mitral stenosis” secondary to combined
“massive” mitral annular calcic deposits and small, hypertrophied left ventricles. Hemodynamic documentation in four patients. Am J Med 1978;64:371–376.
210
2
Mitral
CASE 1357 MITRAL “ANNULAR” CALCIUM FORMING A COMPLETE CIRCLE “O”
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Figure 4 Photographs of the heart at necropsy. Upper left: view of left (LV) and
right (RV) ventricles showing normal-sized cavities and a small area of myocardial
necrosis in the posterior wall; lower left: transverse sections of the cardiac ventricles
showing small cavities and no myocardial lesions; upper right: view of mitral (MV)
and tricuspid valve (TV) orices from the atrial aspects, with pulmonary trunk (PT);
lower right: close-up view of the MV from the left atrium showing the anterior (A)
and posterior (P) leaets.
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Case 1359 The Heaviest Known
Operatively-Excised Aortic Valve
John B. Sims, MD
a,b
, Brad J. Roberts, BS, RDCS
Robert F. Hebeler, Jr., MD
a,c
, and Paul A. Grayburn, MD
a,b
, William C. Roberts, MD
a,b
*
a,b
,
A 60-year-old man whose operatively excised stenotic and regurgitant aortic
valve weighed nearly 15 g, approximately 30 times the normal weight in an adult,
is described. To the investigators’ knowledge, this is the heaviest aortic valve
ever encountered in a human being. © 2006 Elsevier Inc. All rights reserved.
(Am J Cardiol 2006;97:588–589)
1
The normal tricuspid aortic valve in adults weighs on average 0.5 g.
stenotic operatively excised aortic valves weigh from 1 to 6 g, and the heaviest, until
the present case, seen by 1 of us (WCR) over a 40-year period was just >11 g.
Most
2
In
this report, we describe a patient whose operatively excised stenotic aortic valve
weighed nearly 15 g.
• • •
A 60-year-old weightlifter born in August1944 was referred due to a thoracic aortic
aneurysm. At age 55, he was told that his heart was “too severely damaged to undergo
a cardiac operation.” In recent months, he had developed exertional and nocturnal
dyspnea, lower leg edema, and a 20-pound increase in weight. His body mass index
was 26 kg/m
2
. He was well developed and muscular. Agrade 4/6 diastolic blowing
murmur was present and best heard over the cardiac apex, and a grade 3/6 systolic
ejection murmur was present and best heard over the right upper sternal border. The
lungs were clear, and there was no subcutaneous edema. The blood hematocrit was
36%. An electrocardiogram showed sinus bradycardia with left bundle branch block
and a QRS width of 160 ms. By transthoracic echocardiography, the diameter of the
aorta at or near the sinotubular junction was 6.5cm, the left ventricular peak systolic
diameter was 7.7cm, and the left ventricular peak diastolic diameter was 8.3cm. The
left ventricu lar ejection fract ion was about 25%. The aortic valve was bicuspid, severely
regurgitant, moderately stenotic, and heavily calcied. Calcic deposits were also
present in the mitral annular region, and moderate mitral regurgitation was present.
During evaluation, the patient developed atrial brillation, with a ventricular rate of
120 beats/min, his blood pressure decreased to 90/60mm Hg, and severe dyspnea
ensued. Rapid cardioversion was successfully performed, and the dyspnea lessened.
His serum creatinine decreased from 1.8 to 1.4 mg/dl with diuresis.
Cardiac catheterization disclosed normal epicardial coronary arteries and the
following pressures: pulmonary artery wedge A wave 35, V wave 42, and mean
a
Department of Internal Medicine, Division of Cardiology, bHeart and Vascular Institute,
c
Department of Cardiothoracic Surgery, Baylor University Medical Center, Dallas, Texas.
and
Manuscript received August31, 2005; revised manuscript received and accepted September2,
2005.
*
Corresponding author: Tel: 214-820-7712; fax: 214-820-7533.
E-mail address: paulgr@baylorhealth.edu (P.A. Grayburn).
212 DOI: 10.1201/9781003409281-43
CASE 1359 THE HEAVIEST KNOWN OPERATIVELY-EXCISED AORTIC VALVE
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Figure 1 The patient’s aortic valve is shown in a photograph (left) and an x-ray
(right). The valve consists of 2 cusps. One cusp has a raphe, and the length of the
raphe from the free margin to its extension of the aorta measures about 3.8cm. The
raphe cusp is heavily calcied and weighs 8.78 g. The nonraphe cusp weighs 5.53 g.
The 2 cusps together weigh 14.31 g.
33mm Hg; pulmonary artery 65/30mm Hg; right ventricle 63/17mm Hg; and aorta
90/52mm Hg (mean 69). The left ventricular cavity was not entered by the catheter.
The cardiac index was 2.54 L/min/m
2
.
On December 14, 2004, the ascending aorta was replaced with a 30-mm
Hemashield graft (Boston Scientic, Natick, Massachusetts), which included a 29
free style porcine xenograft with implantation of the coronary arteries into the
graft. Mitral valvuloplasty was also performed. The aortic valve weighed 14.31 g
(Figure 1). Because of early bleeding postoperatively, the patient was taken back
to the operating room for reexploration of the mediastinum, where a hematoma
was found and evacuated. Because of complete heart block produced at operation,
a biventricular pacemaker was inserted 7 days postoperatively. When contacted
7 months after the operation, the patient had no symptoms attributable to heart
disease.
• • •
What might be the explanation for the huge mass of this patient’s aortic valve? It
is now well established that congenitally unicuspid and bicuspid stenotic aortic
valves are on average much heavier than stenotic tricuspid aortic valves.
2
The
present patient had a congenitally bicuspid aortic valve. Furthermore, it is now well
established that men with stenotic aortic valves on average have heavier valves than
women with stenotic aortic valves, and the patient described herein was a man.
It is also known that patients with signicantly elevated serum cholesterol levels
have more calcic deposits in their aortic valve cusps, mitral annuli, and epicardial
coronary arteries than other subjects of similar ages and the same gender with much
213
2
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lower levels.
have serum total cholesterol levels >800 mg/dl at birth and by age 10years may
develop considerable calcic aortic stenosis.
3
Patients with homozygous familial hypercholesterolemia, for example,
4
Unfortunately, the cholesterol numbers
in the present patient were uncertain. Larger adult patients with stenotic aortic
valves tend to have larger valves than smaller patients of similar ages and the same
gender. The present patient, however, weighed only 165 pounds (75 kg) and was 68
inches (173cm) in height.
The weight of a stenotic aortic valve is determined principally by the quantity
of calcium in the valve. Of nearly 2,000 operatively excised stenotic aortic valves in
patients >20years of age examined by 1 of us (WCR), only 2 did not have calcic
deposits. The quantity of deposits in the aortic valve in this patient was by far the
greatest any of us had seen previously, but the reason for such a large quantity of
calcium in the present patient was unclear.
REFERENCES
1. Silver MA, Roberts WC. Detailed anatomy of the normally functioning aortic
valve in hearts of normal and increased weight. Am J Cardiol 1985;55:454 – 461.
2. Roberts WC, Ko JM. Weights of operatively-excised stenotic unicuspid, bicuspid,
and tricuspid aortic valves and their relation to age, sex, body mass index, and
presence or absence of concomitant coronary artery bypass grafting. Am J Cardiol
2003;92:1057–1065.
3. Boon A, Cheriex E, Lodder J, Kessels F. Cardiac valve calcication: character-
istics of patients with calcication of the mitral annulus or aortic valve. Heart
1997;78:472–474.
4. Sprecher DL, Schaefer EJ, Kent KM, Gregg RE, Zech LA, Hoeg JM, McManus
B, Roberts WC, Brewer HB Jr. Cardiovascular features of homozygous familial
hypercholesterolemia: analysis of 16 patients. Am J Cardiol 1984;54:20–30.
214
CASE 1361 A STARR-EDWARDS PROSTHESIS IN THE MITRAL VALVE POSITION
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Case 1361 A Starr-Edwards Model 6120 Mechanical
Prosthesis in the Mitral Valve Position for 38Years
Mark A. Peterman, MD, Michael S. Donsky, MD, Gregory J. Matter, MD, and
William Clifford Roberts, MD*
The Starr-Edwards caged-ball prosthesis for cardiac valve replacement was rst
successfully used in the mitral position in September1960. Because of swelling of
the silicone rubber poppet due to lipid inltration, the processing of the silicone
rubber was altered in 1965 in hopes of preventing poppet degeneration. We describe
ndings in a woman who had mitral valve replacement in 1965 utilizing the revised
silicone rubber poppet; the prosthesis remained in place for 38years.
• • •
A 78-year-old woman was admitted to Baylor University Medical Center in
August2003 in functional class IV heart failure. At age 38 (1963), she had had aortic
valve replacement with a Starr-Edwards mechanical prosthesis and mitral valve
commissurotomy. At age 40 (1965), she had repeat aortic valve replacement and
mitral valve replacement, both with Starr-Edwards mechanical prostheses. She did
well until age 63 (1988), when a third aortic valve replacement, this time with a St.
Jude Medical prosthesis, and a tricuspid valve annuloplasty were performed. She
also had chronic anemia, for which she had received several transfusions.
On admission, blood pressure was 110/63mm Hg, and heart rate was 82 beats/
min. Her body mass index was 23 kg/m
the left sternal border and a grade 2/6 diastolic murmur at the cardiac apex. Rales
were heard at the bases of both lungs. The liver was large, and the lower legs were
severely edematous. The hemoglobin was 9.5 g/dl, and the hemotocrit was 29.5%.
An echocardiogram disclosed that her mean transmitral diastolic gradient was
18mm Hg; the left ventricular ejection fraction was about 50%. The aortic pressure
was 150/59mm Hg, and the pulmonary artery pressure, 75/20mm Hg. Coronary
arteriography showed no signicant coronary narrowings.
The Starr-Edwards model 6120/30 mm (3M) mechanical valve, which had
been in the mitral position for 38 years, was excised and a porcine bioprosthesis
was implanted via a right mini-thoracotomy approach. The patient recovered
uneventfully and was discharged on the fth postoperative day after reinitiation of
warfarin therapy.
After photographi ng it, the excised Sta rr-Edwards prosthesis was sent to Edwards
Lifesciences LLC (Irving, California), the laboratory that had originally produced
the prosthesis. The prosthesis was disassembled to acquire the serial number of the
valve, which conrmed it to be a model 6120. The poppet was deeply orange, swollen,
2
. Agrade 3/6 systolic murmur was heard at
Departments of Internal Medicine (Cardiology Division), Cardiothoracic Surgery and
Pathology, and the Baylor Heart & Vascular Institute, Baylor University Medical Center,
Dallas, Texas. Manuscript received September 6, 2005; revised manuscript received and
accepted October11, 2005.
*
Corresponding author: Tel: 214-820-7911; fax: 214-820-7533.
E-mail address: wc.roberts@BaylorHealth.edu (W.C. Roberts).
DOI: 10.1201/9781003409281-44 215
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Figure 1 Views of the operatively excised model 6120 Starr-Edwards prosthesis
that had been in place for 38years.
Figure 2 View of the poppet and age after incising the poppet in its mid-portion.
The arrow (left) shows a region of elongated wear marks. The ball shows a demarcation of color probably corresponding to penetration of blood components, primarily
lipids, by absorption (right).
216
CASE 1361 A STARR-EDWARDS PROSTHESIS IN THE MITRAL VALVE POSITION
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Figure 3 Scanning electron micrograph showing micropores on the poppet’s surface (each ×500).
and moved only when signicant pressure was applied (Figure1). Asenior scientist
at the manufacturer’s laboratory determined that the prosthetic poppet weighed 7.4
g, a gain of 0.9 g from its original weight (14% increase). After cutting the poppet in
half, the maximal diameter was 21.8mm, an increase of 1.7mm (8% increase) from
its original diameter (Figure2). Several cuts were present on the poppet’s surface,
each about 1mm long, and light wear groovings, due to restricted motion/rotation,
also were present on the poppet’s surface. Additionally, micropores were evident
on the poppet’s surface on scanning electron microscopy (Figure3). The maximal
excursion of the poppet was about 0.5cm. There was also host tissue overgrowth
covering about 50% of the aortic aspect of the prosthetic ring (Figure1). (This tissue
overgrowth combined with the poppet swelling restricted poppet motion leading
to both prosthetic stenosis and erythrocyte hemolysis. The latter resulted from high
shear jetting backow from incomplete seating of the poppets.) Other than a local
burnish region on 1 of the 4 metallic struts, corresponding to the sliding contact
of the poppet as opposed to the normal rolling contact, the cage and the other 3
metallic struts showed no evidence of wear.
• • •
The rst successful mechanical prosthesis in the mitral valve position in humans
was the Starr-Edwards Model 6000.
systemic embolization as well as ball variance
1
This model had a signicant incidence of
1
(occasionally lethal2). The model
6120 Starr-Edwards mitral valve prosthesis was introduced in 1965 with a reduction
in the surface area of metallic components, and an improved barium-impregnated
1
poppet.
This model had a far lower incidence of systemic embolization (3% vs 38%)
217
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compared with model 6000.
1
The next models (6300) were introduced in 1968 and
added cloth covering to the stents. The modied cloth-covered series (model 6400
or track valve) was introduced in 1974 and discontinued in 1980, because results
showed no advantage over the original 6120 model.
Although ball variance is usually discovered within 8 years of implantation,
late ball variance has been described in early models in patients up to 20 years
after implantation.
reoperation of a Starr-Edwards mitral prosthesis was 31years in a cloth-covered
model with a metal poppet.
3
The longest interval previously reported from implantation to
4
To our knowledge, ball variance has not been reported
in the model 6120 Starr-Edwards prosthesis. The present case is remarkable for the
extremely late onset of prosthetic mitral valve stenosis and the occurrence of severe
ball variance in a model 6120 Starr-Edwards mitral valve prosthesis.
REFERENCES
1. Freimanis I, Starr A. The unnatural history of valvular heart disease. Late results
with silastic ball valve prostheses. J Cardiovasc Surg 1984;25:191–198.
2. Roberts WC, Levinson GE, Morrow AG. Lethal ball variance in the Starr-Edwards
prosthetic mitral valve. Arch Intern Med 1970;126:517–521.
3. Grunkemeier GL and Starr A. Late ball variance with model 1000 Starr-Edwards
aortic valve prosthesis. Risk analysis and strategy of operative management. J
Thorac Cardiovasc Surg 1986;91:918–923.
4. Goshima M, Shiono M, Yamamoto T, Inoue T, Hata M, Sezai A, Niino T, Nakamura
T, Ye Z, Negishi N, Sezai Y. Reoperation for a Starr-Edwards ball valve prosthesis
implanted in mitral position 31years ago. Jpn J Thorac Surg 2003;56:535–540.
218
CASE 1374 SUDDEN ONSET OF “CARDIAC” SYMPTOMS
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Case 1374 Sudden Onset of “Cardiac” Symptoms,
(?) Mild or Severe Aortic Valve Stenosis Involving
a Congenitally Bicuspid Aortic Valve, and Nearly
Normal Coronary Arteries in an Octogenarian
William C. Roberts, MD,
1-3
Paul A. Grayburn, MD
1-3
An 86-year-old woman who lived alone was in good health until one evening, just
after cleaning the kitchen, she suddenly noted palpitations, dizziness, weakness, and
cool clammy skin while sitting. She called an ambulance service and was brought
to a hospital. The symptoms resolved spontaneously after a few minutes. She was
61 inches tall and weighed 158 lb (body mass index, 29.8 kg/m
2
). Agrade 2–3/6
systolic ejection murmur was heard over the precordium and best at the cardiac
base and into both carotid arteries. An ECG showed sinus rhythm, atrial premature
complexes, and no ST-segment or T-wave abnormalities. An echocardiogram
(Figure 1) disclosed the following dimensions in centimeters: left ventricle end
diastole was 4.3 and peak systole was 3.7, left ventricular and ventricular septal walls
were each 1.3, and the left atrial cavity was 3.1. The calculated peak transvalvular
aortic gradient was 47 mm Hg; blood hematocrit was 33% and hemoglobin was
10.9 g/dL. S erum total cholesterol was 260mm Hg, low-density lipoprotei n cholesterol
177mm Hg, high-density lipoprotein cholesterol 88 mm Hg, and triglycerides
62 mg/dL. Troponins and creatine kinase levels were normal.
Cardiac catheterization disclosed the following pressures: pulmonary artery
wedge Awave 29, V wave 36, mean 25; pulmonary artery, 54/26; right ventricle,
62/23; left ventricle, 206/33; and ascending aorta, 183/80m m Hg. The peak transaortic
valve gradient was 23 and the mean gradient was 29mm Hg, the calculated aortic
valve area was 0.61cm
was 60%. The cardiac index was 2.2 L/min/m
2
(index, 0.36cm2/m2), and the left ventricular ejection fraction
2
. Coronary angiogram disclosed no
signicant narrowings.
The aortic valve at operation was found to be congenitally bicuspid (Figure2). It
was excised virtually intact. It weighed 2.51 g: the raphe cusp weighed 1.74 g and the
nonraphe cusp, 0.69 g
12
. Her postoperative course was uneventful.
COMMENTS
The above-described elderly woman who was known to have a precordial murmur
for many years was asymptomatic until a single near-syncopal episode prompted
hospitalization and cardiac evaluation. The transvalvular peak systolic pressure
gradient across her aortic valve was only 23mm Hg (mean gradient, 29mm Hg) in
From the Departments of Internal Medicine, Division of Cardiology;1 and Pathology;2 and
the Baylor Heart and Vascular Institute,
Address for correspondence: William C. Roberts, MD, Baylor Heart and Vascular Institute, Baylor
University Medical Center, 621 North Hall Street, Suite H-030, Dallas, TX 75226
E-mail: wc.roberts@baylorhealth.edu
Copyright of American Journal of Geriatric Cardiology is the property of LeJacq
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DOI: 10.1201/9781003409281-45 219
3
Baylor University Medical Center, Dallas, TX
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