Circulation, Vol 87, 1188-1196, Copyright © 1993 by American Heart Association
PA Pellikka, AJ Tajik, BK Khandheria, JB Seward, JA Callahan, HC Pitot and LK Kvols
BACKGROUND. The carcinoid syndrome is a rare cause of acquired valvular
heart disease. Although the typical echocardiographic features of carcinoid
heart disease are well recognized, this large series provides new
information about unusual manifestations of the disease as well as the role
of Doppler echocardiography. METHODS AND RESULTS. Between 1980 and 1989,
132 patients with carcinoid syndrome underwent echocardiographic study. The
echocardiographic, Doppler, and clinical features of the 74 patients (56%)
with echocardiographic evidence of carcinoid heart disease are described.
Among these patients, 97% had shortened, thickened tricuspid leaflets.
Tricuspid regurgitation was present in all 69 patients with carcinoid heart
disease who underwent Doppler examination, and it was of moderate or severe
degree in 62 patients (90%). Severe tricuspid regurgitation was
characterized by a dagger-shaped Doppler spectral profile with an early
peak pressure and rapid decline. The pressure half-time was prolonged
(mean, 116 msec), which is consistent with associated tricuspid stenosis.
The pulmonary valve appeared thickened, retracted, and immobile in 36
patients (49%) and was diminutive to the extent of not being visualized in
an additional 29 patients (39%). Among the 47 patients who underwent
Doppler evaluation of the pulmonary valve, regurgitation was present in
81%, and stenosis was present in 53%. Left-sided valvular involvement was
present in five patients (7%), four of whom had patent foramen ovale or
carcinoid tumor involving the lung. Previously undescribed myocardial
metastases were present in three patients (4%) and were confirmed by biopsy
in each case. Small pericardial effusions were present in 10 patients
(14%). Patients with and without echocardiographic evidence of carcinoid
heart disease did not differ with regard to sex, age, location of the
primary tumor, duration of diagnosis, or duration of symptoms of carcinoid
syndrome. However, the mean pretreatment level of urinary
5-hydroxyindoleacetic acid was higher in patients with carcinoid heart
disease than in patients without carcinoid heart disease (270 versus 131
mg/24 hrs, p < 0.001). The symptom of dyspnea was more prevalent among
patients with carcinoid heart disease than in patients without the disease
(54% versus 27%, p = 0.003); as expected, heart murmurs were also noted
more frequently in patients with disease (92% versus 43%, p < 0.0001).
Treatment regimens and response to therapy were similar in the two groups.
Survival of patients with echocardiographic evidence of carcinoid heart
disease was reduced compared with those without cardiac involvement (p =
0.0003). ECG and chest roentgenographic findings in patients with carcinoid
heart disease were nonspecific. CONCLUSIONS. The broad spectrum of
carcinoid heart disease is detailed in this large series. This includes not
only right-sided valvular lesions but also left-sided involvement,
pericardial effusion, and myocardial metastases.
ARTICLES
Carcinoid heart disease. Clinical and echocardiographic spectrum in 74 patients
Division of Cardiovascular Diseases and Internal Medicine, Mayo Clinic, Rochester, Minn. 55905.
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|
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