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Echocardiography
in Practice
A Case Oriented Approach
by Susan Wiegers, Ted Plappert, and Martin St. John Sutton
Carcinoid Heart Disease
Susan E. Wiegers, MD
Effort intolerance, pedal edema and increasing abdominal distension
led a 62-year-old woman to see her
physician. She had complained of diarrhea for several
months and had noticed frequent uncomfortable flushing
episodes. Her condition failed to improve with
vigorous diuresis. The classic echocardiographic
findings led to the search for carcinoid tumor and several small
liver metastases were discovered. Because
of intractable symptoms, she underwent tricuspid valve
replacement for treatment of her severe right heart
failure and tricuspid stenosis. However, postoperative
right ventricular dysfunction and severe bleeding due to
an uncontrollable coagulopathy led to her death 2 weeks
after surgery.
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Figure 17.1
(a) A parasternal right ventricular (RV) inflow view. In diastole
the tricuspid valve leaflets are thickened and shortened.
The tips are
rounded so that the leaflets resemble clubs. The valve is
moderately stenotic. The abnormal leaflets fail to open completely
but
do not dome as in rheumatic tricuspid stenosis. Both the right
ventricle (RV) and the right atrium (RA) are dilated.
(b) In the systolic view incomplete closure of the valve is
demonstrated. The leaflets are fixed, demonstrating little
movement during the cardiac cycle. |
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Figure 17.2
(a) Parasternal right ventricular inflow view with color
Doppler flow imaging. Right ventricular inflow during diastole
demonstrates
the proximal flow convergence-proximal isovelocity surface
area (PISA phenomenon)---characteristic of flow through stenotic
lesions. The inflow jet on the right ventricular side
is turbulent and aliases, also consistent with stenosis.
(b) In systole, torrential tricuspid regurgitation flows through
the leaflets, which are fixed. The turbulent jet fills the
right atrium.
The PISA phenomenon can now be seen on the ventricular side
of the valve. |
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Figure 17.3
Spectral display of continuous-wave Doppler across the
tricuspid valve from the apical position. The tricuspid
regurgitant velocity tracing is dagger-shaped with an early
peak pressure and rapid fall-off. This pattern is found
with right ventricular systolic failure. There is torrential
tricuspid regurgitation through the fixed tricuspid leaflets.
The peak systolic velocity cannot be used to estimate the
peak right ventricular systolic pressure because the right
ventricle and right atrium are essentially common chambers
during systole. The elevated diastolic velocity across the
tricuspid valve (above the baseline) and the prolonged pressure
half-time are evidence of the associated mild tricuspid
stenosis. |
Figure 17.4
Parasternal short-axis view at the base of the heart during
systole. The pulmonary valve (arrow) is thickened and
stenotic. The leaflets do not dome but are fixed in position.
The pulmonary artery is mildly dilated. |
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Figure 17.5
Parasternal short axis view at the base of the heart during
systole with color Doppler imaging. The turbulent jet across
the
pulmonary valve confirms the presence of important stenosis.
(b) Similar view in diastole. The color jet of pulmonary
regurgitation is broad at the level of the valves and appears
to fill the entire right ventricular outflow tract at its
origin. The fixed leaflets are both stenotic and regurgitant.
The severe pulmonary regurgitation demonstrated in this
view does not appear to extend far into the right ventricle.
This may be due to the right
ventricular diastolic pressures, or to the distal portion of
the jet being out of plane in this view. |
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Figure 17.6
Parasternal short-axis view of the heart in diastole. The right
ventricle (RV) is massively enlarged and is many times larger
than the left ventricle (LV) in this view. The flattening of
the
interventricular septum is due to right ventricular volume
overload. |
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Figure 17.7
(a) Apical four-chamber view in systole with color flow Doppler
imaging. The right ventricle (RV) is again demonstrated
to be
dilated compared to the left ventricle (LV). The jet of massive
tricuspid regurgitation arises more distally in the right ventricle
than
is normal. This is due to the fact that the tricuspid leaflets
are fixed open and the regurgitation commences at the leaflet
tips.
(b) Similar two-dimensional image in systole. The fixed position
of the tricuspid leaflets is well seen. The right atrium is
also
massively enlarged. |
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Figure 17.8
Subcostal view of the liver. The dilated hepatic vein is
demonstrated in the inferior portion of the image. Several
round nodules of metastatic carcinoid tumor are present
within the hepatic tissue. |
Discussion
Echocardiography is more sensitive than catheterization
for demonstrating cardiac involvement in carcinoid
disease.1 This patient had the classic findings of rightsided
involvement due to carcinoid syndrome. The tricuspid
valve is involved in 90% of patients with carcinoid
heart disease. The leaflets of the valve become fibrotic,
thickened, and fixed. The etiology of these pathological
changes are not clearly understood but are thought to be
related to the effects of serotonin and other vasoactive
substances secreted by the tumors. Interest in this entity
has increased with the recent finding that several anorectic agents
are possibly responsible for similar pathological changes in the
left-sided valves.
Differentiation from rheumatic disease is straightforward.
The entire tricuspid leaflet thickens and becomes immobile. In rheumatic
disease, commissural fusion leaves the body of the leaflet relatively
spared. The belly of the leaflet moves throughout the cardiac cycle
but domes in diastole, resulting in stenosis. In contrast, the
leaflets affected by carcinoid disease are thickened
and
completely immobile. Thus, severe regurgitation is always
associated with the lesion.
The spectral velocity pattern of the tricuspid regurgitation
has been previously described and is associated with
'wide open' regurgitation.2 On pulsed-wave Doppler, the
tricuspid regurgitation may appear laminar. The peak
right ventricular systolic pressure cannot be estimated
with torrential tricuspid regurgitation because the right
atrial systolic pressure approaches that of the right ventricle.
In this case, the presence of pulmonary stenosis
further precludes extrapolation of the tricuspid jet velocity
to peak pulmonary artery pressure. Pulmonary regurgitation
and stenosis were also seen in this patient and
have been reported to occur in 50% of the patients with
carcinoid heart disease. Right ventricular failure may
ensue from the volume and pressure load. Small pericardial
effusions may also occur. Intracardiac metastases and
left-sided valvular thickening3 as well as marked thickening
of the right atrial wall4 may also be seen and are best diagnosed
by transesophageal echocardiography. It is not uncommon to visualize
hepatic abnormalities during the subcostal images. However, consultation
with appropriate colleagues should be sought before a diagnosis is
tendered.
References
1. Robiolio PA, Rigolin W, Wilson JS, et
al. Carcinoid
heart disease.
Correlation of high serotonin with valvular abnormalities detected
by cardiac catheterization and echocardiography. Circulation
1995;92:790-5.
2. Pellikka PA, Tajik AJ, Khandheria BK, et
al. Carcinoid heart
disease.
Clinical and echocardiographic spectrum in 74 patients. Circulation
1993;87: 1 188-96.
3. Le Metayer P. Constans J, Bernard N, et
al. Carcinoid heart
disease: two cases of left heart involvement diagnosed by transthoracic
and transesophageal echocardiography Eur Heart J
1993;14:1721-3.
4. Lundin L, Landelius 1, Andren B, et al. Transesophageal echocardiography
improves the diagnostic value of cardiac ultrasound in
patients with carcinoid heart disease. Br Heart J 1990;61:1904.
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