| A woman aged 46 was found to have severe tricuspid valve disease 14 years after the diagnosis of a malignant carcinoid tumour of the ileum and 33 years after the onset of symptoms attributable to the disease. Increasing ascites requiring repeated paracentesis had not responded to chemotherapy including infusion of 5-fluorouracil and vincristine into the coeliac artery. The tricuspid valve was replaced by a Bjork-Shiley prosthesis. After operation there was no recurrence of ascites or oedema. She remains well one year and 11 months later. It is suggested that valve replacement surgery should be considered more often in patients with carcinoid heart disease. hepatomegaly and ascites sould not be attributed too readily to advancing malignant disease without careful consideration of the role of right-sided valvar lesions in the production of these signs. ( info) |
| We report a patient who presented for elective exploratory laparotomy, and resection of a pelvic mass, which was thought to be ovarian carcinoma. Intraoperative transesophageal echocardiography demonstrated right-sided valvular heart lesions, which suggested the diagnosis of carcinoid syndrome before a pathologic confirmation was obtained. This article discusses the classical presentation and anesthetic management of patients with carcinoid syndrome and emphasizes the importance of proper preoperative diagnosis and careful planning if the incidence and severity of the symptoms that this condition can provoke are to be reduced. ( info) |
13/64. Triple valve replacement in a patient with severe carcinoid heart disease. We report on the case of a 34-year-old male patient suffering from end-stage carcinoid heart disease with severe tricuspid, pulmonary and mitral valve regurgitation. In addition, a persisting foramen ovale was present. The primary carcinoid tumor was never discovered. However, urine 5-hydroxy-indole-acetic-acid (5-HIAA) were consistently elevated after the first diagnosis of carcinoid disease and after eight years of medication with octreotide and Interferon alpha-2b our patient developed significant cardiac insufficiency mainly due to severe valvular dysfunction. Ultimately, mechanical tricuspid, mitral and pulmonary valve replacement was performed. Twelve hours following the operation the patient had to be returned to the operating room for persisting intrathoracic hemorrhage. He recovered uneventfully and was discharged from hospital on day 37. Twelve months following triple valve replacement the cardiac status recovered from preoperative NYHA-IV to NYHA-I. ( info) |
| In this case report the typical echocardiographic features of carcinoid heart disease are presented. Newer treatments such as the use of a somatostatin analogue, interferon and hepatic de-arterialisation have improved the prognosis in patients with carcinoid syndrome. Nevertheless this syndrome portends a poor prognosis in patients with cardiac involvement. Cardiac lesions are mainly located in the right side of the heart. Regurgitation and stenosis of the tricuspid and pulmonary valve, leading to right heart failure, are the most common cardiac manifestations of the disease. Elevated levels of serotonin are probably responsible for the development of these cardiac lesions. Despite treatment resulting in significant reductions of urinary levels of 5-HIAA, regression of the cardiac manifestations in carcinoid syndrome has not been observed. Two-dimensional and Doppler echocardiography are the main tools to establish the diagnosis and severity of carcinoid heart disease. Cardiac surgery for carcinoid heart disease might improve symptoms and longevity, but the scarce data report on early mortality of over 35%. ( info) |
15/64. Metastatic carcinoid to the conducting system-is it a rare or merely unrecognized manifestation of carcinoid cardiopathy? We report a unique case of carcinoid cardiopathy. The patient, a 59-year-old white woman with a known carcinoid tumor of the terminal ileum, presented with complete heart block and subsequently died. Because of her clinical presentation, we examined the conducting system at autopsy. The atrioventricular node was heavily infiltrated by metastatic carcinoid. The tumor was also seen in the surrounding atrial myocardium. No other metastatic lesions were seen in the heart. Carcinoid cardiopathy in the form of right-sided endocardial and valvular thickening has been well documented in the literature, and we found these features in our case as well. To our knowledge, carcinoid has never been documented in the cardiac conducting system until now. We found 2 cases of possible involvement of the conducting system reported in the literature. Unfortunately, the conducting system was not examined postmortem in either of these cases. If conducting system involvement is not unique to the present case, patients with metastatic carcinoid and cardiac problems may require different management. We hope that this case will stimulate further postmortem study of the conducting system in patients with metastatic carcinoid tumor. ( info) |
| A 65-yr-old woman with atypical complaints and a tricuspid insufficiency murmur underwent transthoracic echocardiography, which showed right-sided abnormalities, but did not allow clear visualization of the valves. Subsequent transoesophageal imaging, however, raised the suspicion of carcinoid heart disease, because of the typical lesions. The diagnosis was thereafter confirmed by biochemical investigations, scintigraphy and abdominal ultrasound. This case suggests that transoesophageal echocardiography may have additive value in the detection of carcinoid heart disease. ( info) |
| A rare clinical occurrence, heart failure in the setting of tricuspid stenosis should immediately trigger a search for underlying systemic carcinoid disease. carcinoid tumor cells can secrete a variety of vasoactive substances that result in skin erythema, excretory diarrhea, bronchospasm, and hemodynamic instability, but these manifestations are noted only in a few patients. Right heart valvular disease is common since the vasoactive noxious substances pass through the right heart unaffected and undergo metabolism in the pulmonary circulation, thereby decreasing involvement of the left-sided valves. Localization of the carcinoid tumor followed by surgically directed valvular treatment is mandatory for relief of symptoms. In nonoperative candidates, cytotoxic chemotherapy or long-term symptomatic drug treatment with somatostatin is indicated. ( info) |
18/64. Carcinoid case presentation and discussion: the American perspective. The rationale underlying an aggressive approach in the management of some carcinoid patients is explained and illustrated by the presented case of a middle-aged man with advanced classic typical midgut carcinoid. The patient exhibited somatostatin receptor scintigraphy-positive massive liver metastases, carcinoid syndrome, severe tricuspid and pulmonic cardiac valve disease with congestive heart failure, ascites and malnutrition. He had been treated for several years with supportive medications and biotherapy including octreotide and alpha interferon but his tumor eventually progressed and his overall condition was markedly deteriorated when he first sought more aggressive treatment. This consisted of prompt replacement of both tricuspid and pulmonic valves, followed by hepatic artery chemoembolus (HACE) injection and then surgical tumor debulking including excision of the primary tumor in the small intestine. In addition, radiofrequency ablation was utilized to reduce the volume of metastases in the liver. Prophylactic cholecystectomy was also performed and a biopsy of tumor was submitted for cell culture drug resistance testing. This was followed by systemic chemotherapy utilizing the drug (docetaxel) which the in vitro studies suggested as most likely to be effective. His excellent response to this succession of treatments exemplifies the successful application of aggressive sequential multi-modality therapy. ( info) |
19/64. Carcinoid disease of the heart. Surgical management of ten patients. Between 1982 and 1989, 10 patients with carcinoid heart disease underwent tricuspid valve replacement with a mechanical prosthesis at our institution. Pulmonary valvectomy was performed in nine patients and pulmonary valve replacement with a pulmonary homograft was performed in one. Two patients had carcinoid tumor metastatic to the heart, involving the right atrium in one case and both ventricles in the other. One patient had concomitant coronary artery bypass with the saphenous vein, and one patient had a quadruple valve replacement for histologically proved carcinoid disease of all four valves. The 30-day mortality was 10% and the late mortality was 30%. The remaining six patients were alive 4, 4, 4, 7, 24, and 46 months postoperatively. A review of the English literature identified 28 additional patients who underwent tricuspid valve replacement for carcinoid heart disease. There was no significant difference in the survival of patients with a bioprosthesis versus a mechanical valve in the tricuspid position. The 4-year survival for the 38 patients undergoing tricuspid valve replacement for carcinoid heart disease was 48% /- 13%. Symptomatic patients who have carcinoid heart disease and whose metastatic malignant disease is not an imminent threat to life should be offered valve replacement. Operating soon after the onset of increasing cardiac symptoms, before the often rapid deterioration in right ventricular failure, optimizes the benefits. ( info) |
| carcinoid heart disease is a frequent complication of the carcinoid syndrome, usually presenting as right-sided valvular heart disease. Although the diagnosis is most often made with cardiac ultrasound, MRI and CT are valuable techniques for diagnosing carcinoid heart disease. ( info) |