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Carcinoid heart disease: a rare cause of right ventricular dysfunction
RV Pruijsten, A Lorsheyd, JH Kirkels, MJM Cramer
Drs RV Pruijsten, arts-assistent cardiologie
Drs A Lorsheyd, arts-assistent cardiologie
Dr JH Kirkels, cardioloog
Dr MJM Cramer, cardioloog
Department of Cardiology, Heart Lung Centre Utrecht; University Medical Centre Utrecht
Address for correspondence: Drs RV Pruijsten
Afdeling Cardiologie, UMC Utrecht, Hart Long Centrum Utrecht, Heidelberglaan 100, 3584 CX Utrecht.
E-mail: pruijsten@yahoo.com
A 70-year-old man with a ten-year history of ileal carcinoid disease, including mesenterial and liver metastases, was referred to our department because of dyspnoea and severe peripheral edema. Despite ileocecal resection, chemotherapy, laser-induced thermotherapy of liver metastases and treatment with somatostatin, the disease was progressive. On physical examination, pulse frequency was 60/minute (regular) and blood pressure 100/60 mmHg. Auscultation of the heart revealed no significant murmers. Monophasic internal jugular pulsations were elevated at pre-auricular level and severe pitting edema was seen at both lower extremities. An electrocardiogram showed sinus rhytm with normal AV- conduction, QRS-width of 110 ms, right axis deviation and low voltage of all leads.
Transthoracic echocardiography revealed a dilated right ventricle (enddiastolic diameter 4.7 cm) with poor systolic function. The tricuspid valve leaflets were thickened, retracted and immobile, leading to incomplete coaptation, severe regurgitation and mild stenosis. The right ventricular wall showed diffuse plaque-like thickening (see Figure 1). Carcinoid heart disease was diagnosed. A possible explanation for the absence of a significant tricuspid regurgitation murmer might be equilibration of right atrial and ventricular pressure. Because of the poor right ventricular function and progressive metastatic carcinoid disease, valvular surgery was not performed. Instead, a conservative treatment with diuretics and coumarines was chosen.
| Movie 1. Deposits of fibrous tissue at the right ventricular wall (apical 4-chamber view, ‘mirror image’: right ventricle at the right upper quadrant). |
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 Click the image to view the movie |
| Movie 2. Thickened, retracted and immobile tricuspid valve leaflets, leading to incomplete coaptation (parasternal short-axis view). |
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| Click the image to view the movie |
| Figure 1. Thickened, retracted and immobile tricuspid valve leaflets, leading to incomplete coaptation. The right ventricular shows deposits of fibrous tissue (parasternal short-axis view). |
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With an incidence of 1.2 to 2.1 per 100,000, carcinoid tumors are rare.1 20 to 30 percent of patients initially present with symptoms of a carcinoid syndrome, which is caused by the tumor’s release of vasoactive substances, like serotonin.2 More than half of these patients develop carcinoid heart disease, which is a major cause of morbidity and mortality.3 The disease is characterized by deposits of fibrous tissue, most commonly on the endocardium of the right ventricle and right sided valves. In cases of important tricuspid or pulmonary valve regurgitation or stenosis, cardiac valve replacement should be considered. However, it is associated with a significant mortality risk.4
References
- Modlin IM, Sandor A. An analysis of 8305 cases of carcinoid tumors. Cancer 1997;79:813-29.
- Moller JE, Connolly HM, Rubin J, Seward JB, Modesto K, Pellikka PA. Factors associated with progression of carcinoid heart disease. N Engl J Med 2003;348:1005-15.
- Westberg G, Wangberg B, Ahlman H, Bergh CH, Beckman-Suurkula M, Caidahl K. Prediction of prognosis by echocardiography in patients with midgut carcinoid syndrome. Br J Surg 2001;88:865-72.
- Roberts, WC. A unique heart disease associated with a unique cancer: carcinoid heart disease. Am J Cardiol 1997;80:251-6.
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