Prevalence, one-year-incidence and predictors of carcinoid heart disease.

BACKGROUND: Carcinoid heart disease (CHD) caused by neuroendocrine tumours (NET) is associated with an increased morbidity and mortality due to valvular dysfunction and right sided heart failure. The present study aimed to assess the prevalence and one-year-incidence of CHD in NET patients. Tumour characteristics, laboratory measurements, and echocardiographic findings were evaluated to identify predictors of CHD manifestation. METHODS: The study was an investigator-initiated, monocentric, prospective trial. Patients with NET without previously diagnosed CHD were included and underwent comprehensive gastroenterological and oncological diagnostics. Echocardiographic examinations were performed at baseline and after one year. RESULTS: Forty-seven NET patients were enrolled into the study, 64% of them showed clinical features of a carcinoid syndrome (CS). Three patients presented with CHD at baseline and three patients developed cardiac involvement during the follow-up period corresponding to a prevalence of 6% at baseline and an incidence of 6.8% within one year. Hydroxyindoleacetic acid (5-HIAA) was identified to predict the occurrence of CHD (OR, 1.004; 95% CI, 1.001-1.006 for increase of 5-HIAA), while chromogranin A (CgA), and Kiel antigen 67 (Ki 67%) had no predictive value. Six patients with CHD at twelve-month follow-up revealed a tendency for larger right heart diameters and increased values of myocardial performance index (MPEI) at baseline compared to NET patients. CONCLUSION: The prevalence at baseline and one-year-incidence of CHD was 6-7%. 5-HIAA was identified as the only marker which predict the development of CHD.

Patent foramen ovale in carcinoid heart disease: The potential role for and risks of percutaneous closure prior to cardiothoracic surgery.

Neuroendocrine tumours (NETs) are rare but once metastasised, can lead to the release of vasoactive substances into the systemic circulation, and the classical features of carcinoid syndrome (CS) such as flushing and diarrhoea. A consequence of CS is carcinoid heart disease (CHD) which primarily affects the right-sided heart valves and can eventually lead to right heart failure. In this cohort, tricuspid and/or pulmonary valve replacement provides symptomatic relief. A patent foramen ovale (PFO) in patients with CHD can lead to the shunting of oxygen deficient blood to the systemic circulation causing hypoxaemia and reduced exercise tolerance. Additionally, the haemodynamic changes caused by regurgitant right-sided heart valves can increase the patency of a PFO allowing the passage of vasoactive substances to the systemic circulation thereby affecting the left-sided heart valves. We present data on the incidence of PFO in patients referred for surgery at our centre, in which the standard approach is to close the defect at time of cardiothoracic surgery. In addition, we present a series of four cases that highlight how the option of percutaneous PFO closure prior to open valve surgery may reduce haemodynamic instability and open a window of opportunity to enhance preoperative status. Percutaneous PFO closure then acts as a bridge to definitive cardiothoracic surgery, although there are risks in such an approach.

Outcome of carcinoid heart syndrome in patients enrolled in the SwissNet cohort.

BACKGROUND: Carcinoid heart disease is a rare disease which develops in patients with functional neuroendocrine tumors in an advanced tumor state. Patients diagnosed with carcinoid heart disease have a poor longtime prognosis with respect to morbidity and mortality and long-term data on patient outcomes are lacking. METHODS AND RESULTS: In this retrospective study, we analyzed outcomes of 23 patients with carcinoid heart disease enrolled into the SwissNet database. We observed that early diagnosis with echocardiographic surveillance of carcinoid heart disease during the course of the neuroendocrine tumor disease was beneficial to overall survival of patients. CONCLUSION: Through nationwide patient enrollment, the SwissNet registry is a powerful data tool to identify, follow-up and evaluate long-term patient outcomes in patients with rare neuroendocrine tumor driven pathologies including carcinoid heart syndrome with observational methods enabling better therapy optimization to improve patient`s long-term perspectives and survival. In line with the current ESMO recommendations, our data proposes that heart echocardiography should be included as part of the general physical assessment in patients with newly diagnosed NET.

The prevalence, operations, and outcomes of carcinoid heart disease.

BACKGROUND: Carcinoid heart disease (CHD) is a sequela of carcinoid liver metastases (LM). The true prevalence of CHD is unknown due to infrequent screening by transthoracic echocardiography (TTE). Octreotide is believed to protect against new and recurrent CHD, but supporting data are scant. This study determined CHD prevalence and outcomes in patients screened by TTE and treated with octreotide. METHODS: Records of carcinoid patients from 2001 to 2021 were reviewed. Survival was estimated by Kaplan-Meyer curves and compared by log-rank. RESULTS: Among 282 patients screened by TTE, overall survival was lower in CHD (n = 40) versus non-CHD (n = 242) patients (p < 0.001). Despite octreotide therapy, 21 patients developed CHD. Among patients with inoperable LM, survival was lower in CHD patients without valve replacement (VR) (p < 0.001), but similar between CHD patients with VR and non-CHD patients. CHD patients with VR and hepatic cytoreduction had survival similar to CHD patients without VR. CONCLUSION: VR improves survival in CHD patients with inoperable LM. Hepatic cytoreduction after VR should be reserved for carefully selected cases. Our data do not support a protective effect of octreotide.

Managing carcinoid heart disease in patients with neuroendocrine tumors.

Carcinoid heart disease is a complex clinical entity frequently complicating the course of neuroendocrine tumors and carcinoid syndrome and is associated with significant morbidity and mortality. Although the pathogenesis of carcinoid heart disease remains poorly understood, it appears that the exposure to excessive circulating levels of serotonin contribute a key role, triggering a cascade of events that ultimately results in the development of plaque-like material on the endocardial surfaces of the valve leaflets. The occurrence of carcinoid heart disease may initially run an asymptomatic period, followed by the development of symptoms of congestive cardiac failure. The diagnosis of carcinoid heart disease is suspected by raised biomarkers, such as serum NT-pro-BNP and confirmed by imaging modalities, with echocardiogram being the gold standard to date. Carcinoid heart disease treatment remains challenging as in addition to cardiac dysfunction, tumor burden needs to be tackled with, hence requiring a multidisciplinary approach. Therapy comprises watchful waiting during the first initial stages of the disease; medications for heart failure; optimal control of serotonin secretion from the NET with pharmacotherapy, interventional means or even surgical techniques; and, in selected patients, cardiac valve replacement. The current review summarizes the literature on the diagnosis and management of carcinoid heart disease.

Carcinoid Heart Disease: A Guide for Clinicians.

Carcinoid heart disease is the collective term for all cardiac manifestations that develop in patients with carcinoid. The cardiac manifestations of carcinoid tumors are attributed to the paraneoplastic effects of vasoactive substances released by the malignant cells. The clinical manifestations of carcinoid heart disease include valvular destruction leading to valvular regurgitation and stenosis, right-sided heart failure, and metastatic carcinoid disease. A combination of biomarkers and cardiac imaging is used in screening and diagnosis of carcinoid heart disease in patients with carcinoid syndrome. The management of carcinoid heart disease involves medical and surgical treatment and requires a multidisciplinary approach for optimized care.

Carcinoid heart disease.

Rare neuroendocrine tumours (NETs) that most commonly arise in the gastrointestinal tract can lead to carcinoid syndrome and carcinoid heart disease. Patients with carcinoid syndrome present with vasomotor changes, hypermotility of the gastrointestinal system, hypotension and bronchospasm. Medical therapy for carcinoid syndrome, typically with somatostatin analogues, can help control symptoms, inhibit tumour progression and prolong survival. Carcinoid heart disease occurs in more than 50% of these patients and is the initial presentation of carcinoid syndrome in up to 20% of patients. Carcinoid heart disease has characteristic findings of plaque-like deposits composed of smooth muscle cells, myofibroblasts, extracellular matrix and an overlying endothelial layer which can lead to valve dysfunction. Valvular dysfunction can lead to oedema, ascites and right-sided heart failure. Medical therapy of carcinoid heart disease is limited to symptom control and palliation. Valve surgery for carcinoid heart disease should be considered for symptomatic patients with controlled metastatic carcinoid syndrome. A multidisciplinary approach is needed to guide optimal management.

Cardiopatía carcinoide como presentación inicial de síndrome carcinoide / Carcinoid cardiopathy as the first manifestation of carcinoid syndrome

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Medial hypertrophy of the ovarian vein: a novel type of vascular pathology associated with a primary ovarian carcinoid tumor.

Primary carcinoid tumors of the ovary are rare accounting for only 1% of neoplasms that are associated with the carcinoid syndrome. However, the carcinoid syndrome can occur in the absence of hepatic metastases due to the release of vasoactive peptides directly into the systemic circulation via the ovarian vein. We present a 69-yr-old woman presenting with carcinoid valvular disease and congestive cardiac failure who was found to have a primary left ovarian carcinoid tumor. At operation it was noted that the left ovarian vein had an unusually firm and thickened appearance, and histologic examination revealed marked fibromuscular medial hypertrophy with luminal compression. There was no associated vascular elastosis. This ovarian venous alteration appears to represent a novel addition to the spectrum of cardiovascular injuries associated with carcinoid tumors.

Current concepts in diagnosis and perioperative management of carcinoid heart disease.

Carcinoid tumors are neuroendocrine tumors with a very unpredictable clinical behavior. In the setting of hepatic metastases, the tumor's release of bioactive substances into the systemic circulation results in carcinoid syndrome: a constellation of symptoms among which cutaneous flushing, gastrointestinal hypermotility, and cardiac involvement are the most prominent. Cardiac manifestations, also known as carcinoid heart disease, are secondary to a severe fibrotic reaction which frequently involves the right-sided valves and may extend towards the subvalvular apparatus leading to valve thickening and retraction. Left-sided involvement is rare and mostly observed in the presence of an interatrial shunt, endobronchial tumor localization, and high tumor activity. Echocardiographic techniques often reveal noncoaptation of the valves, which are fixed in a semiopen position. In patients with advanced lesions and severe valvular dysfunction, surgery is currently the only definitive treatment to potentially improve quality of life and provide survival benefit. Although cardiac surgery has been traditionally reserved for those patients with symptomatic right ventricular failure, a significant trend towards improved surgical outcomes has triggered a more liberal referral for valve replacement. Carcinoid heart disease poses two distinct challenges for the anesthesiologist: carcinoid crisis and low cardiac output syndrome secondary to right ventricular failure. Carcinoid crisis, characterized by flushing, hypotension, and bronchospasm, may be precipitated by catecholamines and histamine releasing drugs used routinely in patients undergoing valve surgery. Although a broader utilization of octreotide have significantly simplified the anesthetic and perioperative management of these patients, a very balanced anesthetic technique is required to identify and manage low cardiac output syndrome.

Risk factors for the development and progression of carcinoid heart disease.

The development of valvular heart disease in patients with carcinoid syndrome is thought to be related to the secretion of vasoactive substances by a tumor. We sought to identify modifiable risk factors for the development of carcinoid heart disease because this may help define strategies to attenuate the disease process. Two hundred fifty-two patients with carcinoid syndrome were prospectively followed with serial echocardiograms at 6-month intervals. Clinical characteristics, biochemical markers, and radiologic markers were measured at set intervals. An echocardiographic scoring system was applied. Patients were defined as having progression of carcinoid heart disease if the echocardiographic score increased by ≥25%. After a median follow-up of 29 months, 44 patients developed carcinoid heart disease or had progression of existing valvular dysfunction. At time of progression of carcinoid heart disease compared to the previous 6 months, there was a significant increase in median levels of 5-hydroxyindoleacetic acid (5-HIAA; 791 vs 460.5 µmol/24 hours) and flushing episodes (4.5 vs 2 episodes per day). Independent predictors of the development or progression of carcinoid heart disease were a 5-HIAA level ≥ 300 µmol/24 hours and ≥ 3 episodes of flushing per day. 5-HIAA levels of ≥ 300 to 599, 600 to 899, and > 900 µmol/24 hours conferred 2.74, 3.16, and 3.40 times the risk of progression of carcinoid heart disease, respectively. In conclusion, a 5-HIAA level ≥ 300 µmol/24 hours and ≥ 3 flushing episodes per day are predictors of the development or progression of carcinoid heart disease.

Echocardiographic follow-up of treated patients with carcinoid syndrome.

Carcinoid heart disease is a rare disease, and its prevalence is uncertain. The aim of the present prospective study was to assess the prevalence of carcinoid heart disease using annual echocardiographic follow-up. We studied 80 consecutive patients presenting with histologically proven digestive endocrine tumor and carcinoid syndrome. All patients underwent annual conventional transthoracic echocardiographic studies and measurement of biologic carcinoid markers. Right- and left-sided carcinoid heart disease and the score of the severity of carcinoid heart disease were systematically assessed. At baseline, the prevalence of right- and left-sided carcinoid heart disease was 33% and 8%, respectively. At the end of follow-up, the corresponding prevalence was 53% and 21%. The correlations were strong between urinary 5-hydroxyindoleacetic acid (5-HIAA) and the right-sided carcinoid heart disease score (r = 0.75, p <0.0001), between urinary 5-HIAA and the left-sided carcinoid heart disease score (r = 0.83, p = 0.001), and between urinary 5-HIAA and the overall carcinoid heart disease score (r = 0.84, p <0.0001). All patients with > or =3 years of carcinoid syndrome and increased level of urinary 5-HIAA presented with echocardiographic evidence of valve disease on routine monitoring. In conclusion, the prevalence of carcinoid heart disease remained high and increased during follow-up. Carcinoid heart disease progresses over time, highlighting the need for echocardiographic follow-up once carcinoid syndrome has been diagnosed.

Prevalence of patent foramen ovale and usefulness of percutaneous closure device in carcinoid heart disease.

The aim of this study was to assess (1) the incidence of patent foramen ovale (PFO) in carcinoid syndrome (CS) and (2) the feasibility of percutaneous closure procedure in selected patients with CS. One hundred eight patients were prospectively studied: 54 with CS and an age- and gender-matched control group. All patients underwent conventional and contrast echocardiography. Patients with clinical signs of dyspnea (New York Heart Association class > or =III), cyanosis, carcinoid heart disease (CHD), and severe PFO were referred for the percutaneous closure of PFO. The prevalence of PFO was 41% in patients with CS and 22% in the control group (p = 0.03) and was significantly higher in patients with CHD (59%, p = 0.009). Four patients (14% of those with CHD) were referred for the percutaneous closure of PFO, and 3 patients ultimately underwent PFO closure (using Amplatzer septal occluders). At 6-month follow-up, New York Heart Association class was improved in all patients, as well as arterial blood gas results (p = 0.04) and 6-minute walking distance (p = 0.03), but all patients presented residual right-to-left shunts. In conclusion, this prospective study demonstrates that in patients with CHD, the prevalence of PFO is high and that percutaneous closure of PFO is feasible, with a reduction in symptoms but with residual shunting.

Analysis of 150 patients with carcinoid syndrome seen in a single year at one institution in the first decade of the twenty-first century.

Carcinoid heart disease (CHD), reported in 50% to 70% of patients with carcinoid syndrome, is thought to be related to the production of 5-hydroxytryptamine by the tumor. The development of new therapeutic modalities designed to reduce tumor hormone production may have altered the development of CHD. Currently, echocardiography is performed when clinical suspicion of CHD exists. The aim of this study was to establish the prevalence of CHD in the setting of modern treatment regimens and delineate whether a screening program for CHD is needed. One hundred fifty patients with carcinoid syndrome were screened for CHD by transthoracic echocardiography. The functional status of patients was classified according to New York Heart Association class. Thirty patients (20%) were found to have CHD. Of those with CHD, 14 (47%) had left- and right-sided valvular lesions. Patent foramen ovale was present in all patients with left-sided CHD. Forty-three percent of patients were in New York Heart Association class I, 40% in class II, 13% in class III and 3% in class IV. Eight patients (27%) with moderate or severe valvular lesions were in class I. Thirty-seven percent of patients with CHD had no physical signs. In conclusion, the presence of symptoms or abnormalities on clinical examination has a low sensitivity for the presence of CHD. Therefore, screening with echocardiography, even in patients who are asymptomatic, should be advocated.

Assessment of patent foramen ovale in carcinoid heart disease.

BACKGROUND AND OBJECTIVE: Carcinoid syndrome may involve right carcinoid heart disease (CHD), secondary to the release of vasoactive substances. Left CHD is rare, as the inactivation of serotonin by the lung protects the left heart. We attempted to evaluate the prevalence of CHD and patent foramen ovale (PFO) with serial contrast transthoracic echocardiographic studies and to determine the markers of right and left CHD progression. METHODS: Forty-one consecutive patients with proved digestive endocrine tumor and carcinoid syndrome were prospectively enrolled. All patients underwent serial conventional and contrast transthoracic echocardiographic studies. Right and left CHD, PFO, radiological examinations, and biological carcinoid markers were systematically assessed. RESULTS: Left CHD was present in 5 patients at baseline and in 13 patients (32%) during follow-up (P = .03). The 13 patients with left CHD also had PFO, and no left CHD occurred without PFO (P < .0001). Right CHD was present in 16 patients (39%) at baseline and in 25 patients (61%) at the end of follow-up (P = .04). The prevalence of right and left CHD was significantly higher in patients with PFO (88% and 76%, respectively; P < .04). A progression of right and left CHD was present, respectively, in 19 and 9 patients but was mainly found in patients with PFO (15 and 9 patients; P < .0001). The main marker of CHD progression was the presence of PFO (odds ratio 44.2, 95% confidence interval 4.4-447.7; P = .001). CONCLUSIONS: PFO is a new marker of CHD progression and should be systematically assessed with routine contrast transthoracic echocardiography in patients with carcinoid syndrome to determine patients at high risk of CHD progression.

Carcinoid tumors and fibrosis: an association with no explanation.

Carcinoid tumors are slow-growing neuroendocrine neoplasms most commonly associated with the gut and broncho-pulmonary system. In many instances, they are identified at surgery for unexplained bowel obstruction or during exploration of the small bowel in search of a primary tumor once distant metastases have been detected. Carcinoid tumors of the small bowel often present with pronounced fibrosis in the peri-tumoral tissues, distant in the heart or lungs, and locally in the peritoneal cavity. Despite medical and therapeutic advances that have alleviated symptoms and prolonged life, a substantial subset of patients develops mesenteric and small bowel carcinoid fibrosis and/or carcinoid heart disease. Fibrosis, and increasingly cardiac heart disease, are important components of intestinal carcinoid disease and are of considerable clinical concern, as both of these conditions reflect a connective tissue disorder whose etiology, biology, and therapy are unknown. In the past, individuals with carcinoid disease died of metastasis and uncontrollable symptomatology. Currently, there exists no clinical method to determine the development of fibrosis and little is understood about the biological basis of fibrosis. The elucidation of the biology and management of fibrosis is thus an issue of paramount clinical and scientific importance in determining appropriate diagnostic and therapeutic strategy. Therefore, the unraveling of the molecular events indicative of fibrosis in these cells and the identification of appropriate therapeutic targets is of considerable patient-care relevance. We have surveyed the world literature over the past 40 yr to evaluate both the incidence of carcinoid processes and track the evolving understanding of this process. In addition, we have provided more current mechanistic information in regard to the biological basis of fibrosis associated with small bowel carcinoid tumors.

Carcinoid heart disease: an update.

BACKGROUND: Carcinoid tumours are a poorly defined collection of lesions, histopathologically indistinguishable from gastroentero-pancreatic neuroendocrine tumours. In this report, we discuss epidemiology and survival, clinical presentation, carcinoid valvular heart disease (CVHD), histopathological considerations and treatment options. METHODS: Review and update of the literature. RESULTS: The term carcinoid suggests a disease entity, but with increasing knowledge it becomes progressively confusing. To avoid further confusion, it is advisable to define these tumours using differentiation, stage, primary site, known tumour products and an associated clinical syndrome. Incidence varies between 0.8 and 1.9/100,000 population. About 20% present with metastases, with a 5-year survival varying between 15% and 35%. Metastatic disease frequently accompanies the carcinoid syndrome (flushing, diarrhoea, wheezing and CVHD). CVHD incidence is about 50%, and seems unrelated to disease duration and tumour mass. An aetiological relation of CVHD with urinary 5-HIAA remains to be confirmed. Resection is the only curative option. Surgery can also offer prolonged palliation and is needed to restore bowel transit in obstructive/ischaemic bowel problems. Adequate palliation of hormone-related symptoms can also be achieved by somatostatin analogues, meta-iodo-benzyl-guanidine preparations and interferon-alpha formulations, all with a 70% response rate. Embolization of liver metastases has led to objective responses in about 50% of patients, but is accompanied by significant side effects. CONCLUSIONS: Most patients are cured by surgery. Symptom relief is the main target in metastatic disease and can be achieved by a range of equally potent biologically active medications, debulking surgery and hepatic embolization.

Incidence and evolution of carcinoid syndrome in the heart.

BACKGROUND AND AIMS OF THE STUDY: Carcinoid syndrome originates from carcinoid tumors localized in the gastrointestinal system, pancreas, biliary vessels, bronchi, ovaries and testes. The condition is characterized by flushing, telangiectasias, diarrhea, bronchoconstriction and cardiac involvement, while cardiac lesions may occur in 50% of patients. This study aimed to investigate-non-invasively-the frequency, severity and evolution of carcinoid heart involvement. METHODS AND RESULTS: Among 87 patients with carcinoid syndrome undergoing routine echocardiography, 39 (45%) had cardiac involvement; 36 of these had thickened, shortened, immobile tricuspid leaflets. Tricuspid regurgitation was present in all 36, it was moderate or severe in 22. Pressure half-time rose to 117 +/- 35 ms, implying mild tricuspid stenosis. The pulmonary valve was also thickened, retracted and immobile with stenotic appearance in 19 patients; regurgitation was present in 16, and mild in 11. Nine patients had pericardial effusion. Of 39 patients with cardiac lesions, 18 had at least one follow up study within 16 +/- 7 months; in 15 cases the valve lesions deteriorated. Tricuspid regurgitation became more severe in six and pressure half-time rose from 107 +/- 46 to 132 +/- 50 ms. Pulmonary regurgitation deteriorated in two, while three patients had new pulmonary valve involvement. The peak gradient across the pulmonary valve increased from 18 +/- 7 to 25 +/- 9 mmHg. Twenty-two patients without heart involvement in the first examination had a follow up study (19 +/- 5 months) during which seven developed valvular lesions, with four moderate and three mild tricuspid regurgitation; mild stenosis was present in all cases. Two patients with mild tricuspid regurgitation had mild mixed pulmonary valve involvement, while of those with moderate tricuspid regurgitation, one also had moderate pulmonary stenosis with mild regurgitation. Five patients with both tricuspid and pulmonary valve diseases had interventional treatment: three underwent balloon valvuloplasty on both valves simultaneously due to stenotic lesions but later relapsed; the other two underwent double valve replacement with biological prostheses but in both cases the pulmonary valve developed severe destruction leading to one death. The other four patients survived for 36, 41, 25 and 16 months, respectively. CONCLUSIONS: Right valvular heart disease occurs frequently in patients with carcinoid syndrome. Evolution of lesions is rapid, leading to right heart failure, though death usually occurs from progressive systemic disease and rarely from pulmonary stenosis. Surgery is the most effective treatment and balloon valvulotomy is only palliative. However, therapy of the systemic condition is predominantly the treatment of choice.

Carcinoid heart disease. Clinical and echocardiographic spectrum in 74 patients.

BACKGROUND: The carcinoid syndrome is a rare cause of acquired valvular heart disease. Although the typical echocardiographic features of carcinoid heart disease are well recognized, this large series provides new information about unusual manifestations of the disease as well as the role of Doppler echocardiography. METHODS AND RESULTS: Between 1980 and 1989, 132 patients with carcinoid syndrome underwent echocardiographic study. The echocardiographic, Doppler, and clinical features of the 74 patients (56%) with echocardiographic evidence of carcinoid heart disease are described. Among these patients, 97% had shortened, thickened tricuspid leaflets. Tricuspid regurgitation was present in all 69 patients with carcinoid heart disease who underwent Doppler examination, and it was of moderate or severe degree in 62 patients (90%). Severe tricuspid regurgitation was characterized by a dagger-shaped Doppler spectral profile with an early peak pressure and rapid decline. The pressure half-time was prolonged (mean, 116 msec), which is consistent with associated tricuspid stenosis. The pulmonary valve appeared thickened, retracted, and immobile in 36 patients (49%) and was diminutive to the extent of not being visualized in an additional 29 patients (39%). Among the 47 patients who underwent Doppler evaluation of the pulmonary valve, regurgitation was present in 81%, and stenosis was present in 53%. Left-sided valvular involvement was present in five patients (7%), four of whom had patent foramen ovale or carcinoid tumor involving the lung. Previously undescribed myocardial metastases were present in three patients (4%) and were confirmed by biopsy in each case. Small pericardial effusions were present in 10 patients (14%). Patients with and without echocardiographic evidence of carcinoid heart disease did not differ with regard to sex, age, location of the primary tumor, duration of diagnosis, or duration of symptoms of carcinoid syndrome. However, the mean pretreatment level of urinary 5-hydroxyindoleacetic acid was higher in patients with carcinoid heart disease than in patients without carcinoid heart disease (270 versus 131 mg/24 hrs, p < 0.001). The symptom of dyspnea was more prevalent among patients with carcinoid heart disease than in patients without the disease (54% versus 27%, p = 0.003); as expected, heart murmurs were also noted more frequently in patients with disease (92% versus 43%, p < 0.0001). Treatment regimens and response to therapy were similar in the two groups. Survival of patients with echocardiographic evidence of carcinoid heart disease was reduced compared with those without cardiac involvement (p = 0.0003). ECG and chest roentgenographic findings in patients with carcinoid heart disease were nonspecific. CONCLUSIONS: The broad spectrum of carcinoid heart disease is detailed in this large series. This includes not only right-sided valvular lesions but also left-sided involvement, pericardial effusion, and myocardial metastases.