Unusual cardiac involvement in carcinoid syndrome

1322

Brief

Communications

American

Our data suggestthat in a selected group of patients, prior RVMI is consistent with preserved long-term functional aerobic capacity over a 3 to 10 year follow-up period. Despite markedly depressed resting RVEF (mean = 30 & 7%), resting RV hemodynamics were surprisingly normal and all patients were able to increase their cardiac outputs with stress and achieve workloads normal for their age and sex. Furthermore, all patients were clinically well with NYHA functional class I to II status. This dissociation between a good exercise capacity and considerably diminished RV function, also reported by Haines et al., may in part reflect the well-preserved left ventricular function seen in this series. All patients had a resting left ventricular EF of at least 48%) and none fell below 42% during exercise. Thus the evidence suggeststhat significant degreesof right ventricular dysfunction in patients with RVMI are well tolerated as long as left ventricular function is simultaneously well preserved. REFERENCES

1. Lore11 B, Leinbach RC, Pohost GM, Gold HK, Dinsmore RE, Hutter AM, Pastore JO, DeSanctis RW: Right ventricular infarction: Clinical diagnosis and differentiation from cardiac tamponade and pericardial constriction. Am J Cardiol 1979;43:465. 2. Lopez-Sendon J, Coma-Canella I, Gamallo C: Sensitivity and specificity of hemodynamic criteria in the diagnosis of acute rieht ventricular infarction. Circulation 1981:64:515. 3. Cintron GB, Hernandez E, Linares E, Aranda JM: Bedside recognition, incidence and clinical course of right ventricular infarction. Am J Cardiol 1981;47:224. 4. Baigrie RS, Haq A, Morgan CD, Rakowski H, Drobac M, McLaughlin P: The spectrum of right ventricular involvement in inferior wall myocardial infarction. A clinical, hemodynamic and non-invasive study. J Am Co11 Cardiol 1983; 1:1396. 5. Haines DE, Beller GA, Curling M, Craddock GB, Mygaard TW, Gibson RS: Right ventricular dysfunction does not limit exercise tolerance after acute inferior myocardial infarction (ah&r). Circulation 1984;7O(suppl II):II-409.

Unusual cardiac syndrome

involvement

in carcinoid

Vicki L. Schiller, M.D., Michael C. Fishbein, M.D.,* and Robert J. Siegel, M.D. Los Angeles, Calif.

The unique feature of this caseis the echocardiographic visualization of direct involvement of the heart by a metastatic carcinoid tumor. J.P. was a 73-year-old man with a 21/z-year history of carcinoid syndrome prior to his death from hepatic failure. Carcinoid tumor was identified in gallbladder and prostate specimens

From the Divisions Medical Center. Reprint requests: 5314, Cedars-Sinai 90048.

of Anatomic Robert Medical

Pathology*

J. Siegel, Center,

and Cardiology,

M.D., Division 8700 Beverly

of Blvd.,

Cardiology, Room Los

Angeles,

CA

when the patient

first presented

distribution

more often

on the right

side of the

heart than on the left, commonly seenon the endocardial surface of the right atrium and ventricle as well as on the pulmonic and tricuspid valvular leaflets.‘,“,” The plaques appear to be preferentially located in areas of excessive turbulence or in the paths of regurgitant streams and therefore are seenmost often on the atria1 septum immediately

Cedars-Sinai

obtained

with clinical symptoms of biliary tract diseaseas well as urinary obstruction. The patient was admitted to the hospital 6 months prior to his death for the initiation of chemotherapy. On examination, the patient was flushed and diaphoretic, yet in no acute distress. Blood pressure was 120/70 mm Hg, heart rate was 84, and there was no jugular venous distention. The cardiac point of maximal impulse was nondisplaced in the fifth intercostal space. Heart sounds were distant. There was a grade II/VI systolic ejection murmur at the base.A firm, nodular liver without pulsations was palpable 16 cm below the right costal margin. ECG findings revealed changesconsistent with old inferior and anteroseptal myocardial infarctions. Chestx-ray films showedborderline cardiomegaly without pulmonary vascular redistribution, and evidence of three nodules in the right lower lung zone. An echocardiogram was performed to assesspossiblecardiac involvement 3 months prior to death. M-mode echocardiography revealed concentric left ventricular hypertrophy with 12 mm thickness of both the ventricular septum and posterior wall. Two-dimensional echocardiography demonstrated a prominent 20 x 5 mm echo density seenprojecting into the right atrium from the interatrial septum (Fig. 1). This echo was seen to move independently of the tricuspid valve, clearly delineating it from a valvular process,At necropsy, the heart weighed 320 gm. A well-defined 20 X 15 mm X 5 mm nodule was seen‘in the interatrial septum, 30 mm above the tricuspid valve anterior to the coronary sinus ostium. There was no chamber dilation, but the left ventricle measuredup to 1.9 cm in thickness and had scarring in the anteroseptal and posterior walls. There was severe coronary atherosclerosis.None of the cardiac valves or mural endocardium had the fibrous thickening of carcinoid plaques.Microscopically, the nodule in the interatrial septum had the histologic and histochemical features of carcinoid tumor (Fig. 1). In addition, a microscopic focus of metastatic carcinoid was present in the fibrous scar tissue in the left ventricular wall. The primary lesion in carcinoid heart diseaseis the carcinoid plaque-a smooth, glistening, whitish-gray elevation

The pathologic and echocardiographic features of carcinoid plaques in the heart and their sequelaehave been well defined.1-fiThe fibrous lesionsusually result in tricuspid and pulmonic stenosis and regurgitation and, as a consequence,produce right-sided congestiveheart failure.

December 1986 Heart Journal

above the junction

of the septal

and

anterior

tricuspid valvular leaflets and in the right atria1 appendage.5,6 Ultrastructural studieshave shownthat the plaques are composed of smooth muscle cells embedded in a stroma of reticulin fibers, acid mucopolysaccharides,and

volume

112

Number

6

B Fig. 1. A, Two-dimensional echocardiographic apical echo density (arrow), approximately 30 min cephalad interatrial septum into the atria1 cavity. B, Low-power nodule (arrow) shown in A. (Hematoxylin and eosin monotonous population of cells in an acinar arrangement and argyrophil stains were positive. (Hematoxylin and

collagen, but devoid of elastic fibers.6 In our patient, a prominent echo density projected into the right atrium from the interatrial septum. Because of the echocardiographic features, the lesion was not typical of carcinoid heart disease but rather was thought initially to represent a eustachian valve. A persistent eustachian valve is not uncommon in adults, and may appear as a linear echo density at the junction of the inferior vena cava and the right atrium.; It has been well appreciated that two-dimensional echocardiography is valuable in diagnosing the characteristic valvular lesions of carcinoid heart disease. Fibrous plaques involving the tricuspid and pulmonary valves and restrict leaflet motion, causing valvular stenosis and regurgitation.]-” Doppler echocardiography can be used to assess more quantitatively the degree of valvular dysfunction.4 In addition, right ventricular enlargement, paradoxical motion of the interventricular septum, and pericardial effusions have been reported in carcinoid heart disease.‘-’ This report documents that in patients with carcinoid syndrome, cardiac involvement may not manifest itself only by the usual valvular lesions due to endocardial plaque formation, but also by direct metastatic involvement. Awareness of the occurrence of this lesion and its echocardiographic features should allow premortem diagnosis of this rare manifestation of carcinoid syndrome.

four-chamber view demonstrating a 20 K ,? mm to the tricuspid valve (TV), projecting prom the photomicrograph demonstrating the metastatic stain; original magnification X16.1 Insl-t shows characteristic of carcinoid tumors. Ar;
3.

4.

5.

6. 7.

Bissett K: Tricuspid insufficiency in carcirlc)id heart disease: Echocardiographic description. AM HEAHY J 1981:IO1:107. Forman MB, Byrd BF, Oates JA. Rokcrtson RM: Twodimensional echocardiography in t,he diqnosis of carcinoid heart disease. AM HEART J 1984;107:452. Marin-Huerta E, Navascues I, Palomeqlir CF. Nunez A, Cobos MA, Asin E: Carcinoid heart disease: A Doppler echocardiographic report. Eur Heart J I9&5;6806 Roberts WC, Sjoerdsma A: The cardiac &ease associated with the carcinoid syndrome (carcinoid hc!rt disease). Am J Med 1964;36:5. Ferrans V, Roberts WC: The carcinoid rndocardial plaque: An ultrastructural study. Hum Pat.hol 1!~~~$7::187. Panidis IP, Kotler MN, Mintz GS. Ros:i .I: Clinical and echocardiographic features of right atria! ~:ASSPS. AM HEART ,I 1984;107:746.

Clinically coronary

successful long-term recanalization

laser

Garrett Lee, M.D., Jorge M. Garcia, M.D. Ming C. Chan, M.D., Paul J. Corso, M.D., James Bacos, M.D., Marshall H. Lee, M.D., August0 Pichard, M.D., Robert L. Reis, M.D. and Dean T. Mason, M.D. Concord and San Francisco, Calif., and Washington, D.C.

REFERENCES

1. Howard

RJ, Drobac M, Rider MD, Keane TJ, Finlayson J, Silver M, Wigle ED, Rakowski H: Carcinoid heart disease: Diagnosis by two-dimensional echocardiography. Circulation 1982;66:1059. 2. Baker BJ, McNee VD. Scovil JA, Bass KM, Watson JW,

From Northern California Heart & Lung Institute. Hospital Center, Washington, D.C.: and Weslrrn Francisco.

( onwrd: i+mrt

Washington Institute. San