Carcinoid heart disease: diagnosis by two-dimensional echocardiography.

Howard, Robert; Drobac, Milutin; Rider, W.D.; Keane, Thomas J.; Finlayson, James K.; Silver, Marcy; Wigle, E. Douglas; Rakowski, Harry

doi:10.1161/01.cir.66.5.1059

Cited by 67 publications

(32 citation statements)

References 14 publications

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“…The classic features of advanced carcinoid heart disease typically involving the tricuspid valve and pulmonary valve have been well described. 3,10,11 However, the spectrum of disease is wide.…”

Section: Discussionmentioning

confidence: 99%

Features of Carcinoid Heart Disease Identified by 2- and 3-Dimensional Echocardiography and Cardiac MRI

Bhattacharyya

Toumpanakis

Burke

et al. 2010

Circ: Cardiovascular Imaging

131

143

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Background-Carcinoid heart disease is a rare form of valvular heart disease. We sought describe the spectrum of carcinoid heart disease identified by echocardiography and cardiac MRI. Method and Results-Two hundred fifty-two patients with carcinoid syndrome underwent a range of investigations including 2D transthoracic echocardiography, 3D transthoracic echocardiography and transesophageal echocardiography, and cardiac MRI. Fifty-two patients had evidence of carcinoid heart disease. Involvement of the tricuspid, pulmonary, mitral, and aortic valves were found in 47 (90%), 36 (69%), 15 (29%), and 14 (27%), respectively. Myocardial metastases were found in 2 (3.8%) patients. Several patterns of disease were identified depending on the extent and severity to which each leaflet and its associated subvavlular apparatus was affected. Thirteen of 15 (87%) patients with left-sided carcinoid involvement had a patent foramen ovale. Three patients with severe degree of shunting had severe valvular regurgitation. Patients with mild/moderate degree of shunting had mild or moderate valvular regurgitation. Three-dimensional transthoracic echocardiography/transesophageal echocardiography provided detailed anatomic information particularly for the tricuspid and pulmonary valves. Cardiac MRI allowed complementary assessment of valvular heart disease and delineation of myocardial metastases. Gallium-68 octreotide positron emission tomography identified neuroendocrine metastases. Conclusions-Carcinoid heart disease is a heterogeneous disease with a wide spectrum of echocardiographic findings. A multimodality approach is needed in patients with this complex pathology. (Circ Cardiovasc Imaging. 2010;3:103-111.)

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Section: Discussionmentioning

confidence: 99%

Features of Carcinoid Heart Disease Identified by 2- and 3-Dimensional Echocardiography and Cardiac MRI

Bhattacharyya

Toumpanakis

Burke

et al. 2010

Circ: Cardiovascular Imaging

131

143

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“…The development of more sensitive investigational methods has led to the realization that cardiac lesions are probably TABLE 3 present more often in patients with malignant carcinoid tumors than previously believed. [1][2][3][4][5][6] Malignant midgut (jejunum, ileum and proximal colon) carcinoid tumors release vasoactive substances such as serotonin, histamine, bradykinin, kallikrein, prostaglandins, and tachykinins.30-34 Some of these have been suggested as possible etiologic agents for the carcinoid heart disease. This study of 68 well-characterized patients with malignant midgut carcinoid tumors reveals a significantly higher urinary 5-HIAA excretion in patients with severe carcinoid heart disease than in those without or with a lesser degree of right heart abnormalities.…”

Section: Discussionmentioning

confidence: 99%

Carcinoid heart disease: relationship of circulating vasoactive substances to ultrasound-detectable cardiac abnormalities.

et al. 1988

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Cardiac ultrasound investigation of 68 prospectively studied patients with histologically proven midgut carcinoid tumors showed right heart disease in 66%. The abnormal findings included morphologic and functional aberrations of the tricuspid valve in 52% and 83%, respectively, right atrial and ventricular enlargement in 53% and 30%, and paradoxical systolic septal contractions in 19%. The patients with the most pronounced right heart disease had significantly higher (p<.01) plasma levels of the tachykinins neuropeptide K and substance P as well as higher (p<.001) urinary excretion of the serotonin metabolite 5-hydroxyindoleacetic acid. These patients also had the most extensive tumor disease. The occurrence of echocardiographic abnormalities of the left heart was similar to that in healthy individuals of the same age, but abnormalities were less frequent among the patients with severe right heart disease. Electrocardiographic changes were nonspecific. Right heart disease thus seems to be present more often than previously reported in patients with malignant midgut carcinoid tumors. The severity of cardiac involvement does not seem to be related to the duration of carcinoid disease but more to the extent of the disease, i.e., higher plasma levels of serotonin and tachykinins. Circulation 77, No. 2, 264-269 1988. PATIENTS with carcinoid syndrome often develop a characteristic heart disease previously reported to occur in 19% to 53% of cases. l-6 In a majority of the patients the endocardial fibrous lesions considered to be pathognomonic of carcinoid heart disease2' 7-10 involve the cavities and valves of the right heart. Only a small number of cases with typical changes of the left heart have been reported. l -13 In many patients with carcinoid heart disease, the predominant terminal symptoms and death may be attributed directly to the cardiac disease.1' [14][15][16][17] ever, none of these substances has been found responsible for the characteristic cardiac lesions.The present study was designed to investigate prospectively the extent of cardiac disease in patients with carcinoid tumors by means of ultrasound investigation. In an effort to study possible etiologic agents the urinary excretion of 5-hydroxyindoleacetic acid (5-HIAA) and the plasma levels of the tachykinins neuropeptide K (NPK) and substance P (SP) were compared in patients with midgut carcinoid tumors and different degrees of carcinoid heart disease. MethodsStudy population. Cardiac ultrasound studies were performed in 68 consecutive patients (36 women and 32 men) with a midgut type of malignant carcinoid tumor admitted to the Department of Internal Medicine, University Hospital, Uppsala, Sweden, over a 6 year period (1979)(1980)(1981)(1982)(1983)(1984)(1985). The diagnosis of a carcinoid tumor was histologically proven by the Grimelius and Mason silver-staining techniques18' 19 on tumor tissue obtained at biopsy or operation. The patients median age was 64 years (range 38 to 83) and at the time of the cardiac investigation, carcinoid...

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“…The excess of hormones such as 5-HT, substance P, and bradykinins released into the circulation (Mirtensson rt ul., 1985;Nobin et al, 1982Nobin et al, , 1983 cause premature death from cardiac and nutritional failure. In fact, cardiac complications have been claimed to be one of the most important lethal factors in carcinoid syndrome (Costello, 1975;Robertson and Sjoerdsma, 1964 (Howard et al, 1982). They investigated and followed 14 patients with carcinoid syn- drome and, although 8 patients had definite abnormalities of the right-sided cardiac valves, no patient died as a direct result of carcinoid heart disease.…”

Section: Discussionmentioning

confidence: 99%

Heart involvement in metastatic carcinoid disease

Törnebrandt

Eskilsson

Nobin

1986

Clinical Cardiology

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Summary: Sixteen patients with metastatic carcinoid tumors of ileal or cecal origin were studied in order to evaluate the frequency and degree of cardiac involvement in a nonselected patient group. We have also studied the correlation between plasma hormone levels (e.g., 5-hydroxytryptamine (5-HT) and substance P) and the degree of cardiac involvement. The patients underwent physical examinations, electrocardiograms, chest x-rays, cardiac catheterization, and echocardiography , Plasma levels of 5-HT and substance P were analyzed. Carcinoid heart involvement was found in 3 of 16 patients (19%) but no patient had subjective symptoms associated with heart disease. Four patients (25%) had slight pulmonary hypertension. No left-sided heart lesions were seen. No correlation between blood levels of 5-HT or substance P and heart involvement was found. Eight patients died during the follow-up period, but in none of these was the cause of death cardiac failure. Carcinoid heart disease is not as common in our patients as in patients selected on a car- diological basis described in earlier studies. Echocardiography appears to be the most efficient technique for detection of even subclinical heart involvement and a useful tool for following its progress.

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Carcinoid heart disease: diagnosis by two-dimensional echocardiography.

Cited by 67 publications

References 14 publications

Features of Carcinoid Heart Disease Identified by 2- and 3-Dimensional Echocardiography and Cardiac MRI

Features of Carcinoid Heart Disease Identified by 2- and 3-Dimensional Echocardiography and Cardiac MRI

Carcinoid heart disease: relationship of circulating vasoactive substances to ultrasound-detectable cardiac abnormalities.

Heart involvement in metastatic carcinoid disease

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