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Ann Thorac Surg 1999;67:537-539
© 1999 The Society of Thoracic Surgeons

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Case Reports

Carcinoid heart disease and carcinoid syndrome: successful surgical treatment

^a Division of Gastroenterologic and General Surgery, Mayo Clinic and Mayo Foundation, Rochester, Minnesota, USA
^b Division of Cardiovascular Diseases and Internal Medicine, Mayo Clinic and Mayo Foundation, Rochester, Minnesota, USA
^c Division of Thoracic and Cardiovascular Surgery, Mayo Clinic and Mayo Foundation, Rochester, Minnesota, USA

Accepted for publication July 15, 1998.

Address reprint requests to Dr Nagorney, Department of Surgery, Mayo Clinic, 200 First St SW, Rochester, MN 55905

	Abstract

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Tumor debulking can greatly improve quality of life for patients with malignant carcinoid syndrome, but hepatic cytoreduction is confounded by carcinoid heart disease, which can cause postsinusoidal portal hypertension, thereby increasing the risk of death from hemorrhage during hepatic resection. We describe a patient with metastatic carcinoid syndrome and carcinoid heart disease who had repair of his carcinoid heart disease and, after improvement of right-sided heart function, had successful hepatic debulking of carcinoid metastases.

	Introduction

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Carcinoid tumors are uncommon. The malignant carcinoid syndrome usually results from metastases to the liver or, rarely, from primary carcinoids alone. Vasoactive secretory products from the tumor are responsible for the clinical syndrome. Despite metastatic carcinoid disease, the prognosis is remarkable. The 5-year survival rate is nearly 50%, and median survival approaches 3 years, even with extensive metastases [1]. When incapacitating endocrinopathies develop, treatment by cytoreductive operation may provide complete and durable relief of symptoms [2].

Carcinoid heart disease occurs in approximately 50% of patients with the carcinoid syndrome and is characterized by tissue fibrosis of the tricuspid and pulmonary valves and the right ventricular endocardium [3, 4]. Pulmonary stenosis (90%), tricuspid insufficiency (47%), and tricuspid stenosis (42%) are encountered most commonly. Because right-sided heart failure is a major cause of death in patients with the carcinoid syndrome [5, 6], surgical treatment of carcinoid heart disease may afford palliation. Demonstrable valvular dysfunction associated with clinical progression of right heart failure remains the primary indication for operation. Optimally, symptomatic patients with carcinoid heart disease whose metastatic malignant disease is not life-threatening should be offered valve replacement before decompensation of right ventricular function [7, 8].

A 39-year-old man presented with complaints of diarrhea and flushing for more than 10 years. Five years before presentation, a cardiac murmur was auscultated. During the preceding year, progressive dyspnea on exertion, fatigue, and sweating had developed. Examination revealed an obese man with a plethoric face. Abdominal examination results were normal. His pulse was regular at 80 beats/min, and blood pressure was 100/70 mm Hg. He had a grade 3/6 holosystolic murmur with a diastolic rumble. He had jugular venous distention. His past history was significant for hypertension of 1 year’s duration, which was controlled with captopril and furosemide. His clinical performance status was Eastern Cooperative Oncology Group level 2.

Complete blood count, serum electrolyte levels, and liver function test results were normal. The urinary 5-hydroxyindoleacetic acid (5-HIAA) level was 214 mg/24 h (normal, 0 to 6.0 mg/24 h). The clinical carcinoid syndrome was controlled completely with the long-acting somatostatin analogue octreotide (Sandoz, East Hanover, NJ). Abdominal computerized tomography showed hepatic but no extrahepatic intraabdominal metastases. Hepatic magnetic resonance imaging revealed a 9-cm dominant hepatic metastasis in segment 4B and two ( 1 cm) lesions in segments 6 and 7.

Two-dimensional and Doppler echocardiography showed normal left ventricular size and function, with an ejection fraction of 63%. There was marked enlargement of the right heart chambers. The tricuspid valve had thickened, immobile leaflets. Tricuspid regurgitation was severe, and tricuspid stenosis was moderate. There was pulmonary stenosis with a gradient of 27 mm Hg. At cardiac catheterization, right atrial pressure was 20 mm Hg, with a right ventricular systolic pressure of 64 mm Hg.

Because of prohibitive risk of bleeding from the transection plane during hepatic resection caused by markedly elevated right-sided cardiac pressure and progressive cardiac deterioration, the carcinoid heart disease was addressed initially (March 1993). The tricuspid valve was excised and replaced with a 29-mm Carpentier-Edwards bioprosthesis. A pulmonary valvectomy was performed and was augmented with a pericardial patch of the pulmonary valve annulus. Pathologic analysis revealed that the tricuspid valve had diffuse leaflet thickening, chordal thickening and fusion, and commissural fusion. The pulmonary valve cusps were also diffusely thickened. His postoperative course was unremarkable. A postoperative echocardiogram at 2 months showed no tricuspid regurgitation and a mean tricuspid valve gradient of 6 mm Hg. Central venous pressure on physical examination decreased to 10 mm Hg at 2 months postoperatively.

Although his cardiac performance status improved, flushing, hot flashes, and diarrhea increased despite escalating doses of long-acting somatostatin. Consequently, resection of the abdominal carcinoid was undertaken. Intraoperatively, a desmoplastic reaction surrounding the primary carcinoid in the terminal ileum was found with two metastases in the liver. A segmental ileal resection, a hepatic polysegmentectomy-2, 3, and 4, and subsegmentectomy-6 was performed. Pathologic studies showed a primary carcinoid tumor of the ileum with transmural invasion forming a 2 x 2 x 1.5-cm mass. The two hepatic metastases measured 0.6 cm and 10 x 7.5 x 5.5 cm, respectively. His hospital course was uneventful. The urinary 5-HIAA level was 2.5 mg/24 h 5 months postoperatively, and results of magnetic resonance imaging of the liver were normal, except for postoperative changes.

Although the carcinoid syndrome resolved postoperatively, the patient experienced progressive chest discomfort and exercise limitations. In February 1997, he underwent tricuspid and pulmonary valve replacements (both St. Jude mechanical prostheses) for right heart failure from tricuspid stenosis and regurgitation and pulmonary regurgitation. Currently (December 1997), his cardiac functional status is estimated at 75% normal. He has experienced no carcinoid syndrome during low-dose octreotide therapy, with a urinary 5-HIAA level of 10 mg/24 h and hepatic magnetic resonance imaging showing several metastases (1 cm).

	Comment

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The clinical outcome of this patient suggests that improvement of both functional cardiac status and clinical manifestations of the carcinoid syndrome can be corrected surgically. Improved performance status after cardiac operation and complete resolution of the symptoms of flushing and diarrhea after abdominal operation clinically support the hypothesis.

We suggest that selected patients with carcinoid heart disease may be an exception to the dictum that metastatic malignancy is a contraindication to open cardiac operation. Several observations support this postulate. First, the natural history of clinical carcinoid disease is prolonged, with a median survival of 38 months and a range extending to 15 years [1]. In fact, median survival from the first recognized flush is 8.5 years. The quality of life poses a continual clinical challenge. Second, resection of all gross carcinoid tumor may be curative, and near-total (90% to 95%) debulking results in durable symptomatic relief [2]. Thus, other comorbidity often becomes the focus of clinical management. Third, medical treatment for metastatic carcinoid tumors has become increasingly effective [1, 9, 10]. Both quality and duration of life may be improved oncologically but limited cardiovascularly. Finally, surgical treatment of carcinoid heart disease has proved beneficial in selected patients, even with unresectable metastases [8–11]. Clearly, if cardiac surgical intervention is effective in this setting, surgical therapy for hepatic metastases should be reconsidered. Although hepatic metastases have regressed with successful cardiac operation, such responses are incomplete and temporary [12].

Our recommendation for initial cardiac repair is not without risk [8, 11]. Coexistent metastatic disease predisposes to arrhythmias, fluid and electrolyte abnormalities, and perioperative carcinoid crisis. Preoperative management requires careful fluid and electrolyte management. Endocrine blockade with a long-acting somatostatin analogue and avoidance of any antiarrhythmic drugs that trigger histamine release are essential. Optimal choice of valve prosthesis is unknown. Both tissue and mechanical valves carry inherent risks. Although tissue valves risk recurrent carcinoid plaque, they provide easier management of coagulation in patients undergoing hepatic resection or embolization and in patients with impaired hepatic function. Symptomatic patients refractory to endocrine blockade should undergo hepatic artery embolization before cardiac repair. Hepatic resection is not precluded by previous embolization.

Symptomatic metastatic carcinoid disease can be successfully palliated by hepatic operation. For patients with right-sided heart failure, the risks of surgical intervention are increased greatly. Selected patients with carcinoid heart disease may benefit from tricuspid or pulmonary valve operations. With the postoperative improvement of cardiac functional status, hepatic debulking of carcinoid tumor can be undertaken. Both the resolution of the debilitating symptoms of carcinoid disease and a greatly improved quality of life may result.

	References

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4. Ferrans V.J., Roberts W.C. The carcinoid endocardial plaque: an ultrastructural study. Hum Pathol 1976;7:387-409.[Medline]
5. Norheim I., Oberg K., Theodorsson-Norheim E., et al. Malignant carcinoid tumors: an analysis of 103 patients with regard to tumor localization, hormone production, and survival. Ann Surg 1987;206:115-125.[Medline]
6. Trell E., Rausing A., Ripa J., Torp A., Waldenstrom J. Carcinoid heart disease: clinicopathologic findings and follow-up in 11 cases. Am J Med 1973;54:433-444.[Medline]
7. Knott-Craig C.J., Schaff H.V., Mullany C.J., et al. Carcinoid disease of the heart: surgical management of ten patients. J Thorac Cardiovasc Surg 1992;104:475-481.[Abstract]
8. Connolly H.M., Schaff H.V., Nishimura R.A., et al. Cardiac surgery for carcinoid heart disease. Circulation 1997;96(Suppl I):I-507.
9. Coupe M., Levi S., Ellis M., et al. Therapy for symptoms in the carcinoid syndrome. Q J Med 1989;73:1021-1036.[Abstract/Free Full Text]
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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Monica L. McDonald David M. Nagorney Hartzell V. Schaff Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by McDonald, M. L. Articles by Schaff, H. V. Search for Related Content PubMed PubMed Citation Articles by McDonald, M. L. Articles by Schaff, H. V.