Circulation. 1996;93:187-188
(Circulation. 1996;93:187-188.)
© 1996 American Heart Association, Inc.
Carcinoid Heart Disease
Allen S. Anderson, MD;
Daniel Krauss, MD;
Claudia Korcarz, DVM;
Roberto M. Lang, MD
From the Noninvasive Cardiac Imaging Laboratory, Section of Cardiology,
Department of Medicine, University of Chicago (Ill).
Correspondence to Roberto M. Lang, MD, Director Cardiac Noninvasive
Imaging Laboratory, University of Chicago, 5841 South Maryland Ave, MC 5084,
Chicago, IL 60637. E-mail: rlang@medicine.bsd.uchicago.edu.
Key Words: Cardiovascular Images • heart disease • valves
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Introduction
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A53-year-old Indian man developed
watery diarrhea. He was treated
for inflammatory bowel disease without
improvement. Three months
before admission, he developed lower
extremity edema, which
progressed to include massive scrotal edema as
well as dyspnea
on exertion, orthopnea, and paroxysmal nocturnal
dyspnea. An
ECG (Fig 1
) and chest radiograph (Fig
2
) were performed. An
upper gastrointestinal series
and small bowel series were unrevealing.
An abdominal computed
tomography scan demonstrated the presence
of multiple liver masses (Fig
3
). A percutaneous fine-needle
liver biopsy was
performed and yielded the cells seen in Fig
4
. A
24-hour urine collection for 5-hydroxyindoleacetic acid
was markedly
elevated at 871 µmol/d (normal, <200 µmol/d).
Echocardiographic
findings are summarized in Figs 5
and 6
.
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Figure 1. ECG revealing sinus tachycardia at 100 beats per
minute, right bundle-branch block, right-axis deviation, and poor
R-wave progression. In carcinoid heart disease, the low voltage also
noted here has been associated with increased short-term mortality
after valve replacement.1
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Figure 2. Posteroanterior chest radiogram depicting an
enlarged cardiac silhouette with increased pulmonary vascular markings
and cephalization of flow, bibasilar pulmonary edema, and a left
pleural effusion. The azygous vein and superior vena cava are
distended.
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Figure 3. Left. Computed tomography scan of the abdomen after
the administration of oral and intravenous contrast media. Multiple
low-attenuation, contrast-enhancing masses in the liver are noted, many
of which are fluid filled. The largest mass measures 5x7 cm. There is
no evidence of abdominal or retroperitoneal adenopathy. The spleen,
pancreas, and kidneys are normal.
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Figure 4. Right. Small, round, monotonous lymphocytic type
cells typical of carcinoid tumor (see arrow).
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Figure 5. Left. Carcinoid involvement of the tricuspid valve.
A, Two-dimensional echocardiogram showing thickened and retracted
tricuspid valve leaflets that exhibited reduced excursion. B, Tricuspid
leaflets fail to completely coapt in systole. C, Color flow image
depicting severe tricuspid regurgitation with the regurgitant jet
filling the dilated right atrium. D, Continuous-wave Doppler
examination of the tricuspid valve demonstrating elevated early peak
systolic pressure (velocity, 3 m/s) with rapid pressure decline
suggestive of severe tricuspid regurgitation. Note the "dagger"
appearance of the systolic regurgitant flow signal due to the large v
wave. The mean transtricuspidal gradient was elevated at 9 mm Hg, which
is consistent with the combination of stenosis and regurgitation
secondary to a fixed tricuspid orifice. The presence of tricuspid
stenosis is also corroborated by the presence of a prolonged pressure
half-time (158 milliseconds).
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Figure 6. Right. Carcinoid involvement of the pulmonic valve.
A, Two-dimensional echocardiogram showing a thickened, retracted, and
stenotic pulmonic valve. B, Continuous-wave Doppler examination of the
pulmonary artery at the pulmonic valve level showing pulmonary valve
stenosis (peak velocity, 2.8 m/s) and regurgitation with a relatively
short deceleration time, which is consistent with severe regurgitation.
C, Color flow image showing a systolic jet of pulmonary stenosis. D,
Color flow examination showing a diastolic jet of pulmonary
regurgitation.
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Based on our findings (Figs 1 through
6
),
the suspected diagnosis of
carcinoid syndrome with cardiac involvement was confirmed. Therapy with
the long-acting somatostatin analogue octreotide resulted in marked
improvement in the patient's diarrhea. Heart failure improved but did
not resolve with medical therapy. A recent report1
suggests that in the current era of improved therapy for metastatic
carcinoid disease, progressive right heart failure has become a major
cause of morbidity and mortality. Despite high perioperative mortality,
valve surgery has been proposed as definitive therapy, resulting in a
significant improvement in functional class and prolonged
survival.1 Valve replacement surgery was therefore
recommended.
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Acknowledgments
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We gratefully acknowledge the contribution of Louis M. Cohen,
MD.
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Footnotes
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The editor of Images in Cardiovascular Medicine is Hugh A. McAllister,
Jr,
MD, Chief, Department of Pathology, St Luke's Hospital and
Texas Heart
Institute, and Clinical Professor of Pathology,
University of Texas Medical
School and Baylor College of Medicine
Circulation encourages readers to submit cardiovascular images to
Dr Hugh A. McAllister, Jr, St Luke's Episcopal Hospital and Texas Heart
Institute, 6720 Bertner, MC 4-265, Houston, TX 77030.
(Circulation. 1996;93:187-188.)
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References
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1. 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;2:410-416.
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