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CASE REPORTS IN CARDIOLOGY
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Case 1531 Carcinoid Heart Disease Without the
Carcinoid Syndrome but with Quadrivalvular
Regurgitation and Unsuccessful Operative Intervention
William Clifford Roberts, MD
Paul A. Grayburn, MD
Burton, MD
a
b,d
a,b,d,
*, Cyril Abie Varughese, DOe, Jong Mi Ko, BAd,
, Robert Frederick Hebeler, Jr., MDc, and Elizabeth C.
A 53-year-old woman is described who underwent mitral and aortic valve
replacement and tricuspid valve annuloplasty for pure regurgitation at all
3 valve sites for unrecognized carcinoid heart disease without the carcinoid
syndrome 22 days before death. Metastatic carcinoid was not recognized until
necropsy, which disclosed a probable ovarian primary but with large hepatic
metastases and left-sided cardiac involvement either greater than or equal
to the right-sided involvement. Pulmonary hypertension, very unusual in
carcinoid heart disease, persisted postoperatively and probably played a role
in the patient’s early death. Hepatic metastasis with ovarian primary is most
unusual in this circumstance. © 2011 Elsevier Inc. All rights reserved.
(Am J Cardiol 2011;107:788–792)
It was in 1930, 80 years ago, when the rst patient with a metastasizing
carcinoid neoplasm associated with brous lesions on the right side of the heart was
described.
1
In 1931, the rst patient with metastasizing carcinoid syndrome (head
and upper chest ushes and diarrhea) associated with brous lesions not only on
the tricuspid and pulmonic valves but also on the anterior mitral leaet and left
ventricular mural endocardium was described.
reports have appeared describing clinical and morphologic features of the carcinoid
syndrome and carcinoid heart disease.
in the small intestine, widespread metastases, and specic carcinoid plaques limited
to the right side of the heart.
2, 3
The present report was prompted by study of a patient
2, 3
2
Subsequently, of course, numerous
Most patients have the primary carcinoid
with severe mitral and aortic regurgitation leading to double valve replacement but
without symptoms of the carcinoid syndrome but with metastasizing carcinoid.
CASE DESCRIPTION
A 53-year-old mother of 6, who was born May5, 1955, and died February18, 2009, had
been well until November2008, when she noted exertional dyspnea, subcutaneous
peripheral edema, and recurring palpitations, which proved to be runs of atrial
brillation. The symptoms gradually worsened, with episodes of rapid heart rate,
each lasting several minutes. On January26, 2009, cardiac catheterization disclosed
the following pressures in mm Hg: pulmonary arterial wedge mean 22, a wave
30, v wave 28; pulmonary trunk 56/10; right ventricle 56/18; right atrial mean 9, a
wave 15, v wave 10; left ventricle 130/26; and aorta 127/58. Left ventriculography
a
Departments of Pathology, bInternal Medicine (Cardiology), and cCardiothoracic Surgery;
d
Baylor Heart and Vascular Institute, Baylor University Medical Center; and eDepartment of
Internal Medicine, Methodist Dallas Medical Center, Dallas, Texas, USA. Manuscript received
September1, 2010; revised manuscript received and accepted October11, 2010.
*
Corresponding author: Tel: 214-820-7911; fax: 214-820-7533.
E-mail address: wc.roberts@baylorhealth.edu (W.C. Roberts).
240 DOI: 10.1201/9781003409281-51
CASE 1531 CARCINOID HEART DISEASE WITHOUT THE CARCINOID SYNDROME
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disclosed a normal-sized left ventricular cavity, with an ejection fraction of 60%
and 4+/4+ mitral regurgitation. Aortography disclosed 4+/4+ aortic regurgitation.
The coronary arteries were angiographically normal. Echocardiography showed
thickened mitral and aortic valve cusps and a normal-sized left ventricle (Figure1).
On January27, 2009, the mitral and aortic valves were replaced with mechanical
prostheses (#20 ATS Medical [Minneapolis, Minnesota] in the aortic position and
#29 St. Jude Medical [St. Paul, Minnesota] in the mitral position). AMaze procedure
was also performed as well as tricuspid valve annuloplasty. The operatively excised
valves are shown in Figures2 and 3. The 6-day postoperative hospital course was
characterized by a weight gain of 6.6 kg (from 76.7 to 83.3 kg), sinus rhythm, and
gradual ambulation.
On February 13, 2009, the patient was rehospitalized because of increasing
weakness and dyspnea, evidence of gastrointestinal bleeding (on warfarin with an
international normalized ratio of 4.7), and large pleural and pericardial effusions.
The blood hemoglobin level was 11.1 g/dl, and the hematocrit was 35%. The
serum bilirubin level was 1.4 mg/dl; alkaline phosphatase, 295 U/L; aspartate
aminotransferase, 210 U/L; and alanine aminotransferase, 147 U/L. The blood urea
nitrogen level was 17 mg/dl and glucose 96 mg/dl. The brain natriuretic peptide
level was 430 pg/ml (normal range <100). Echocardiography now showed a normalsized, normally functioning left ventricle, a very dilated and dysfunctional right
ventricle, and large pericardial and right pleural effusions (Figure4). Approximately
1,800 ml of serous uid was drained from the right pleural space. Shortly thereafter,
the patient had a cardiac arrest and died.
At necropsy, classic carcinoid neoplasms were present in the right ovary (2.3 ×
2.1 × 1.9cm), the liver (2 hemorrhagic and necrotic carcinoid masses, 10 × 9 × 9 and
3 × 3 × 3cm; liver weight 2,100 g); the serosa of the bowel (1 nodule) and uterus (1
nodule); the adrenal glands (multiple small nodules); the pancreas (1 nodule, 0.3cm);
and the heart (multiple microscopic-sized nodules). Sections of the tumors in the
liver, ovary, and adrenal glands were positive for neuron-specic enolase.
The heart (Figures 5 to 8) weighed 370 g. The coronary arteries were free of
atherosclerotic plaque. The left ventricular cavity was small, and the right ventricular
cavity was very dilated. No myocardial foci of brosis or necrosis were present,
Figure 1 Preoperative (1 day) echocardiograms in the patient are described. (A)
Four-chamber view showing the thickened mitral and tricuspid leaets and the
normal-sized right ventricular and left ventricular cavities. (B) Long-axis view
again showing the thickened mitral leaets and also a thickened aortic valve cusp.
AO=aorta; LA=left atrium; LV=left ventricle; RA=right atrium; RV=right ventricle.
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Figure 2 Photographs of the operatively excised mitral (A,B) and aortic (C,D)
valves. (A) View of the atrial aspect of the anterior mitral leaet. (B) View of the ventricular aspect of the anterior mitral leaet with marked brous thickening of the
chordae tendineae. Afragment of posterior leaet with attached papillary muscle
is also visible. (C) Focally thickened ventricular aspect of the tricuspid aortic valve.
(D) Aortic aspect.
Figure 3 Photomicrograph of anterior mitral leaet (A), chordae tendineae (B),
and aortic valve cusp (C). The underlying leaet and chordae are normal, but both
are quite thickened by superimposed cellular brous tissue devoid of elastic brils.
The sinus portion of the aortic valve is lled with cellular brous tissue devoid of
elastic bers (C). Elastic van Gieson’s stains (100×).
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CASE 1531 CARCINOID HEART DISEASE WITHOUT THE CARCINOID SYNDROME
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Figure 4 Four-chamber echocardiogram shortly before death in the patient
described. The right ventricular cavity is severely dilated, whereas the left ventricle
(LV) is not dilated. LA=left atrium; RA=right atrium; RV=right ventricle.
Figure 5 X-ray of the heart at necropsy. Prostheses are present in the mitral and
aortic valve positions, and a ring is in the tricuspid valve annular position. The left
ventricular cavity is not dilated, whereas the right ventricular cavity is considerably
dilated.
except in the posteromedial left ventricular papillary muscle, which was brotic.
Both atria were dilated, the right more than the left. The pulmonic valve cusps were
thickened, and the anterior cusp was rigid and immobile. The foramen ovale was
closed.
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Figure 6 View of heart after removing the atrial walls and most of the ascending
aorta and pulmonary trunk. The prosthesis in the mitral position lls the entire
“oor” of the left atrium. The cloth-covered ring is visible in the tricuspid valve
annulus.
COMMENTS
Our patient underwent mitral and aortic valve replacement and tricuspid valve
annuloplasty for pure mitral, aortic, and tricuspid valve regurgitation. Examination
of the operatively excised valves suggested an appearance similar to that described
in patients having taken fenuramine-phentermine for weight reduction. The
patient, however, denied having ever taken that medication. Postoperatively, the
patient’s condition worsened, and she died 22 days after the operation. Necropsy
disclosed carcinoid tumors in 1 ovary, both adrenal glands, the liver, the pancreas,
the serosal surfaces of the uterus and bowel, and the heart (intramyocardial). There
was never evidence of the carcinoid syndrome (ushing, diarrhea), and the presence
of the carcinoid neoplasm was not diagnosed until necropsy, which also disclosed
evidence of carcinoid heart disease involving both right-sided cardiac valves as well
as both left-sided valves.
The primary in the patient described was not certain, but the ovary appeared
to be the most logical site. Only 1 of the 2 ovaries was involved, and the cancerous
nodule was >2cm in diameter. Hepatic metastasis of carcinoid with ovarian primary,
however, is quite unusual.
4, 5
Chatterjee and Heather6 found hepatic carcinoid
metastases in only 1 of 35 reported cases with primary carcinoid in the ovary.
Although our patient had only 2 carcinoid metastases in the liver, both were large.
In our patient, carcinoid heart disease involved all 4 cardiac valves: the pulmonic
valve to a worse extent than the aortic valve, but the mitral valve to a worse extent
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CASE 1531 CARCINOID HEART DISEASE WITHOUT THE CARCINOID SYNDROME
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Figure 7 View of various “slices” of the cardiac ventricles showing the very small
left ventricular cavity and the very dilated right ventricular cavity.
Figure 8 View of the pulmonic valve from above. The anterior cusp is very thick
and immobile. The other 2 cusps are only mildly thickened by brous tissue.
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than the tricuspid valve. Although the pulmonic valve was heavily involved by the
carcinoid process, there was no pressure gradient across the valve, although the
end-diastolic pressures in both the pulmonary trunk and the right ventricle were
similar, indicating pure pulmonic regurgitation.
Is the electrocardiogram helpful in patients with malignant carcinoid
in diagnosing carcinoid heart disease? No. Ross and Roberts
electrocardiograms in 34 patients with the carcinoid syndrome: the total 12-lead
QRS voltages
7
in the 19 patients with carcinoid heart disease ranged from 58 to
3
examined
227mm (mean 105; standardization 10mm) and in the 15 patients without carcinoid
heart disease from 89 to 192mm (mean 132). Twelve-lead QRS voltages in 16 men
aged 44 to 74years (mean 55) without cardiovascular disease ranged from 84 to
159mm (mean 124) and heart weight from 288 to 392 g (mean 351).
8
Thus, in groups
of patients with the carcinoid syndrome, those with carcinoid heart disease have
lower total 12-lead QRS voltages, but much overlap occurred between the 2 groups.
Our patient had a total 12-lead QRS voltage of 127mm. The duration of the P-R,
QRS, and Q-T intervals and heart rates at rest were similar in the 2 groups with and
without carcinoid heart disease.
The presence of pulmonary hypertension in our patient is most unusual in
carcinoid heart disease and reasonably can be attributed to the left-sided mitral
disease.
9
The reason for its persistence postoperatively is unclear. The patient’s
symptoms worsened considerably postoperatively. The discs of the prostheses in
both mitral and aortic positions moved without interference.
Cardiac valve replacement and/or “repair” for carcinoid heart disease is
becoming more accepted, but nevertheless, outcomes are not always favorable.
9
In
an early report from the Mayo Clinic, 9 of 26 patients died in the early perioperative
period and 9 others a mean of 19 months postoperatively. Only 4 of their 26 patients
had mitral or aortic valve replacement, and none had both valves replaced, as did
our patient. Although the mortality was higher, late operative survival (8 of 26
patients) resulted in considerable decrease (2 patients) or elimination (6 patients)
of symptoms.
patients with carcinoid heart disease who underwent operation for left- and rightsided valve disease:
9
Alater report from the same institution summarized results in 11
10
the tricuspid valve was replaced in all 11, the pulmonic in 3
(valvectomy in 7), the mitral valve in 6 (repair in 1), and the aortic valve in 4 (repair
in 2). There were 2 perioperative deaths and 4 additional deaths in a mean follow-up
period of 41 months. All but 1 operative survivor improved by >1 functional class.
In retrospect, had the presence of carcinoid been recognized preoperatively or at
operation in our patient, both tricuspid and pulmonic valves probably also would
have been replaced (quadruple valve replacement).
11
ACKNOWLEDGMENT
We thank Brad J. Roberts, BS, RCS, RDCS, for his help in preparing the echocardiogram.
REFERENCES
1. Cassidy MA. Abdominal carcinomatosis with probable adrenal involvement.
Proc Roy Soc Med 1930;24:139 –141.
2. Roberts WC, Sjoerdsma A. The cardiac disease associated with the carcinoid
syndrome (carcinoid heart disease). Am J Med 1964;36:5–34.
3. Ross EM, Roberts WC. The carcinoid syndrome: comparison of 21 necropsy
subjects with carcinoid heart disease to 15 necropsy subjects without carcinoid
heart disease. Am J Med 1985;79:339–353.
4. Chaoqalit N, Connolly HM, Schaff HV, Webb MJ, Pellikka PA. Carcinoid
heart disease associated with primary ovarian carcinoid tumor. Am J Cardiol
2004;93:1314–1315.
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CASE 1531 CARCINOID HEART DISEASE WITHOUT THE CARCINOID SYNDROME
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5. Rabban JT, Lerwill MF, McCluggage WG, Grenert JP, Zaloudek CJ. Primary ovar-
ian carcinoid tumors may express CDX-2: a potential pitfall in distinction from
metastatic intestinal carcinoid tumors involving the ovary. Int J Gynecol Pathol
2009;28:41–48.
6. Chatterjee K, Heather JC. Carcinoid heart disease from primary ovarian carci-
noid tumors. Am J Med 1968;45:643–648.
7. Siegel RJ, Roberts WC. Electrocardiographic observations in severe aortic valve
stenosis: correlative necropsy study to clinical, hemodynamic, and ECG variables demonstrating relation of 12-lead QRS amplitude to peak systolic transaortic pressure gradient. Am Heart J 1982;103:210–221.
8. Odom H II, Davis L, Dinh HA, Baker BJ, Roberts WC, Murphy ML. QRS voltage
measurements in autopsied men free of cardiopulmonary disease: a basis for
evaluating total QRS voltage as an index of left ventricular hypertrophy. Am J
Cardiol 1986;58:801–804.
9. Connolly HM, Nishimura RA, Smith HC, Pellikka PA, Mullany CJ, Kvols
LK. Outcome of cardiac surgery for carcinoid heart disease. J Am Coll Cardiol
1995;25:410–416.
10. Connolly HM, Schaff HV, Mullany CJ, Rubin J, Abel MD, Pellikka PA. Surgical
management of left-sided carcinoid heart disease. Circulation 2001;104:I36–I40.
11. Arghami A, Connolly HM, Abel MD, Schaff HV. Quadruple valve replacement in
patients with carcinoid heart disease. J Thorac Cardiovasc Surg 2010;140:1432–14 34.
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Case 1559 43.3-Year Durability of a SmeloffCutter Ball-Caged Mitral Valve
Stuart J. Head, BS, Jamie Ko, Rajeev Singh, MD, William C. Roberts, MD, and
Michael J. Mack, MD
Extended durability of mechanical heart valves has been documented for many
years. We describe a case of a ball-caged mechanical valve implanted 43.3years
previous to developing valve dysfunction. The patient presented with both
prosthetic valve stenosis and insufciency. This Smeloff-Cutter valve (Cutter
Laboratories, Berkeley, CA) in the mitral position was dysfunctional due to lipid
absorption, which resulted in ball variance and concomitant pannus growth
prevented optimal seating of the ball in its cage. This is the longest length of
time in which a Smeloff-Cutter mechanical valve has been originally implanted.
(Ann Thorac Surg 2011;91:606–8) © 2011 by The Society of
Thoracic Surgeons
In the early 1960s, heart valves were introduced for hear t valve replacement, which
resulted in the rst mechanical valve prosthesis implantation of a Starr-Edwards
valve (Edwards Lifesciences, Irvine, CA) in the mitral position in 1961.
newly developed ball-caged valves followed in quick succession, manufactured by
a number of companies producing aortic and mitral valve prostheses. The SmeloffCutter valve (Cutter Laboratories, Berkeley, CA) was introduced in 1964, being the
rst “full-ow” valve, which was achieved by including an additional smaller cage
on which the ball could rest during valve closure.
2
“Ball variance” is a previously documented cause of valve dysfunction,3 in which
lipid absorption in the ball causes it to grow and form surface irregularities, increasing
the risk of thromboembolic events and valve dysfunction. We report a case in which
ball variance and pannus overgrowth caused a 43.3-year implanted Smeloff-Cutter
ball-caged valve to fail. This case is the longest implantation time of this type of valve.
1
After this,
At age 13, mitral valve stenosis developed in the patient, due to rheumatic fever.
Therefore, the patient underwent a valve replacement. Recently, the 56-year-old
woman presented with New York Heart Association functional class IV symptoms of
heart failure and palpitations as a result of long-standing persistent atrial brillation.
Transthoracic and transesophageal echocardiography (Figure1) revealed moderate
to severe mitral valve regurgitation and severe mitral stenosis with a valve area of
2
and a mean gradient of 15mm Hg. There was decreased left ventricular
0.7 cm
function with an ejection fraction of 40%, systemic pulmonary hypertension, and
severe tricuspid regurgitation.
Cardiopulmonary Research Science and Technology Institute, Dallas, Texas, Department of
Cardio-Thoracic Surgery, Erasmus University Medical Center, Rotterdam, the Netherlands,
Department of Pathology, The Baylor Heart and Vascular Institute, Dallas, Department of
Cardiology, The Diagnostic Clinic of Longview, Longview, and Heart Hospital Baylor Plano,
Plano, Texas
Accepted for publication June29, 2010.
Address correspondence to DrMack, Heart Hospital Baylor Plano, 1100 Allied Dr, Plano, TX
75093; e-mail: mmack@csant.com.
248 DOI: 10.1201/9781003409281-52
CASE 1559 43.3-YEAR DURABILITY OF A SMELOFF-CUTTER BALL-CAGED MITRAL VALVE
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Figure 1 Preoperative echocardiogram showing both mitral stenosis and
insufciency.
The patient had been noncomplaint with warfarin therapy until suffering a
stroke in 1988 after discontinuing therapy, which left her with a signicant residual
right hemipharesis. However, this event subsequently made her extremely diligent
in taking the anticoagulant with good control and no further clinical events. For this
reason, she opted for another mechanical valve.
At the time of the redo mitral valve replacement, a pre-cardiopulmonary bypass
transesophageal echocardiographic nding consisted of the mitral valve prosthesis
in a good position with severe mitral valve regurgitation. A 2.4 m/s maximum
velocity was measured across the valve, calculating to a peak gradient of 23mm Hg
and a mean gradient of 9mm Hg. The valve area was 2.3cm
and 0.7cm
2
measured by continuity equation. Furthermore, a left ventricular ejection
2
by pressure halftime
fraction of 40% was obtained. All measurements are consistent with preoperative
ndings.
The limited access procedure was performed exposing only the aorta and right
atrium. Asuperior septal approach to the mitral valve was performed. Inspection
of the valve revealed some erosion of the ball (Figure2). Pannus formation built
up around the valve prevented the ball to seat completely in its cage. There was
an extensive amount of calcium built up both in the annulus and the ventricular
muscle below the annulus. The 43.3-year-old valve was replaced with a 25-mm On-X
bi-leaet mechanical valve (On-X Life Technologies Inc, Austin, TX). After the valve
was implanted, transesophageal echocardiography measured a maximum velocity
across the valve of 1.3m/s, with a peak-to-peak gradient of 7mm Hg and a mean
gradient of 3mm Hg. By pressure half-time the valve area had improved to 3.5cm
2
.
Concomitant tricuspid valve annuloplasty with a 26-mm tricuspid annulus ring
and a full left-sided and right-sided Cryo maze procedure were performed. Her
postoperative course was uneventful. She was discharged home on postoperative
day 6. The patient is in New York Heart Association functional class Iat her most
recent follow-up at 6 weeks postoperatively.
COMMENT
Valve dysfunction after extended durability can be the result of a wide variety
of causes, including lipid absorption into the ball and pannus formation,
causing tissue impingement as the two most common. Absorption increases the
249
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