Management Of Carcinoid Heart Disease Research Articles

Carcinoid Heart Disease.

Carcinoid heart disease (CHD) is a rare cardiac complication that occurs most commonly in patients with advanced neuroendocrine tumors and is a known sequela of carcinoid syndrome. Neuroendocrine tumors most widely associated with CHD include tumors in the small bowel, followed by lung, large bowel, pancreatic, appendiceal, and ovarian neoplasms. Carcinoid syndrome is a paraneoplastic syndrome caused by the release of serotonin and other substances from neuroendocrine tumors. It results in a spectrum of symptoms, including diarrhea, flushing, bronchospasm, and symptoms of congestive heart failure. Without treatment and for patients with advanced heart failure, the prognosis of CHD can be less than a year. Management of CHD is often challenging as patients typically present late, and the disease can progress rapidly. Therefore, optimal management of these patients requires close collaboration among various specialties to quantify disease burden, delay the progression of valvular disease, and determine the most effective surgical and medical management strategies depending on the cardiac manifestations to improve quality of life and reduce mortality. This involves a collaborative team, including cardiology and oncology, and often involves many other disciplines, including hepatobiliary and cardiovascular surgeons, endocrinologists, anesthesiologists, and gastroenterologists.

Management of carcinoid heart disease

Carcinoid Heart Disease (CaHD) is defined as the constellation of all cardiac manifestations that occur in patients with carcinoid tumors. Cardiac manifestations are generally due to the paraneoplastic effects of vasoactive substances secreted by carcinoid tumors. These primarily cause cardiac valve dysfunction and resultant heart failure. Successful management of patients with CaHD requires a multidisciplinary team to address both the classical manifestations of carcinoid syndrome, as well as the additional manifestations of cardiac dysfunction. While the cornerstone of medical management for carcinoid syndrome are somatostatin analogs (SSAs), there is no evidence to suggest that the usage of SSAs influences the development or progression of CaHD. Additionally, while liver-directed therapies provide a survival benefit to symptomatic carcinoid syndrome patients with resectable disease, there are conflicting data on the survival benefit of hepatic resection among patients with CaHD. Cardiac surgery in patients with CaHD is a complex undertaking, and is the only definitive treatment for symptom management in CaHD with significant survival benefit for patients in advanced disease states. Two crucial surgical decisions to be made are determining which valve(s) should be replaced, and what prosthetic should be utilized. While challenging in this often medically frail population, cardiac surgery confers a survival benefit and should be pursued in cases of symptomatic CaHD or progressive right ventricular dysfunction.

Management of carcinoid heart disease in neuroendocrine tumor patients: a retrospective study

Management of carcinoid heart disease in neuroendocrine tumor patients: a retrospective study

Carcinoid Heart Disease – A Review of Pathophysiology, Clinical Manifestations, Diagnosis and Management

Objective: Neuro-endocrine tumors (NET) resulting in syndromes of serotonin excess can lead to cardiac involvement, and substantial mortality and morbidity. This article is aimed at reviewing the pathophysiology, diagnosis and management of carcinoid heart disease (CHD). Results: The pathophysiology of CHD stems from chronic exposure to circulating vasoactive compounds. Frequent clinical evaluations, monitoring of biomarker levels and cardiac imaging play critical roles in screening and early recognition. The complexity of the disease necessitates a multidisciplinary approach, medical management and timely surgical intervention. Conclusion: Outcomes of CHD have improved, owing to advances in medical management and increased surgical expertise. Surgical valvular intervention is the only definitive therapy for the treatment of symptomatic CHD.

959 AN UNEXPECTED DIAGNOSIS BY ECHOCARDIOGRAPHY: APPLICATION IN CLINICAL PRACTICE

Abstract A carcinoid tumore is a rare neuroendocrinologic malignancy that commonly origins from enterochromaffin or Kulchitsky cells in the gastrointestinal tract and secretes bioactive molecules in blood. Up to 60% of patients with carcinoid tumors have cardiac involvement, often on the right-sided heart valves (typically the tricuspid valve and the pulmonic valve). A 66 year old woman was admitted to hospital with congestive heart failure, chronic diarrhea and weight loss. A contrast-enhanced computed tomography scan of the liver showed multiple masses (the largest 70 mm×45mm) compatible with metastases. A transthoracic echocardiogram showed dilatation of right sections of the heart with severe tricuspid regurgitation (RV 64 ml/beat, ERO 1 cmq) and mild pulmonary valve stenosis (Peak gradient 13 mmHg). Patient's aortic and mitral valves were normal in structure and function. The EKG revealed low-voltage QRS complexes of all leads, unspecific ST segment changes and sinusal rhythm. The blood tests showed a significant increase in chromogranin A (17670 ng/ml) and enolase; a 24-hour urine collection showed a total output of 5-hydroxyindoleacetic acid eleveted. A biopsy of the liver with immunostaining for chromogranin and synaptophysin were strongly positive and confirmed the diagnosis of metastatic carcinoid syndrome (grade 1, cells CDX2 positive). The echocardiographic findings of carcinoid heart disease is drumstick-like, rigid, and shortened leaflets with a visible regurgitant orifice, causing torrential tricuspid regurgitation. The pulmonic valve presents more frequently valvular stenosis. A combination of biomarkers and cardiac imaging is useful in screening and diagnosis of carcinoid heart involvement in patients with carcinoid syndrome. The management of carcinoid heart disease is hardful and requires a multidisciplinary approach, medical and surgical treatment, for optimized care.

Current Practice in Carcinoid Heart Disease and Burgeoning Opportunities.

Cardiac surgery with tricuspid valve and potentially pulmonic valve replacement at an experienced center is currently the most effective strategy available for the treatment of carcinoid heart disease. Cardiac surgery for carcinoid heart disease requires a multidisciplinary team including cardiology, medical oncology, cardiothoracic anesthesia, and cardiac surgery. Without cardiac surgery, morbidity and mortality from carcinoid heart disease is high. Aggressive management of carcinoid before and after cardiac surgery is critical. Over time, though, circulating carcinoid hormones can lead to destruction of prosthetic valves as well, resulting in recurrent right heart failure. Percutaneous options for valve repair may be on the horizon for management of carcinoid heart disease.

What do we know about carcinoid heart disease in the present era?

Carcinoid heart disease (CHD) is a severe complication of carcinoid syndrome (CS) found primarily in patients with small intestine neuroendocrine neoplasms (SI-NENs). Patients who develop CHD have significantly worse morbidity and mortality outcomes, highlighting the importance of clinical practice recommendations for CHD screening, diagnosis, and treatment that are both consistent and practical. CHD is characterized by white plaque-like deposits on the endocardial surface of heart structures, generally affecting the right heart valves, causing tricuspid and pulmonary regurgitation and, less commonly, valve stenosis. Cardiac imaging is essential for both the diagnosis and management of CHD. Previously, imaging for CHD was mostly achieved by echocardiography, but more recently, imaging has become multimodal. N-terminal pro-B-type natriuretic peptide (NT-proBNP) and 5-hydroxyindoleacetic acid in the urine (u5-HIAA) are currently the most effective markers used in screening CS patients and evaluating CHD severity. Managing patients with CHD is challenging since both systemic malignant disease and cardiac involvement must be treated concurrently. Early diagnosis and surgical intervention when required are critical to patient prognosis, especially in those without primary tumor resection. Valve replacement surgery is the most effective treatment for patients with advanced carcinoid heart disease for alleviating cardiac symptoms and contributing to survival outcomes. To deliver effective patient treatment, multidisciplinary team collaboration is needed. This review summarizes current research findings on CHD pathogenesis, clinical and epidemiological features, useful biomarkers and imaging modalities, and treatment strategies.

European Neuroendocrine Tumor Society (ENETS) 2022 Guidance Paper for Carcinoid Syndrome and Carcinoid Heart Disease

European Neuroendocrine Tumor Society (<scp>ENETS</scp>) 2022 Guidance Paper for Carcinoid Syndrome and Carcinoid Heart Disease

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.

Multidisciplinary team management of carcinoid heart disease.

Carcinoid heart disease (CHD) is a consequence of valvular fibrosis triggered by vasoactive substances released from neuroendocrine tumours, classically in those with metastatic disease and resulting in tricuspid and pulmonary valve failure. CHD affects one in five patients who have carcinoid syndrome (CS). Valve leaflets become thickened, retracted and immobile, resulting most often in regurgitation that causes right ventricular dilatation and ultimately, right heart failure. The development of CHD heralds a significantly worse prognosis than those patients with CS who do not develop valvular disease. Diagnosis requires a low threshold of suspicion in all patients with CS, since symptoms occur late in the disease process and clinical signs are difficult to elicit. As a result, routine screening is recommended using the biomarker, N-terminal pro-natriuretic peptide, and regular echocardiography is then required for diagnosis and follow-up. There is no direct medical therapy for CHD, but the focus of non-surgical care is to control CS symptoms, reduce tumour load and decrease hormone levels. Valve surgery improves long-term outcome for those with severe disease compared to medical management, although peri-operative mortality remains at between 10 and 20% in experienced centres. Therefore, care needs to be multidisciplinary at all stages, with clear discussion with the patient and between teams to ensure optimum outcome for these often-complex patients.

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: a Comprehensive Review

Carcinoid heart disease is a rare disorder that is associated with significant morbidity and mortality. In this review of the literature, we will present current concepts in diagnosis and management of carcinoid heart disease. Recent expert consensus guidelines highlight the role of echocardiography and screening with NT-proBNP for the evaluation of carcinoid heart disease. Advances in medical therapy along with better surgical outcomes highlight the experience and expertise that has been gained in the treatment of carcinoid heart disease. Carcinoid heart disease occurs in patients with neuroendocrine tumors who have carcinoid syndrome. Serotonin appears to play a central role in the development of carcinoid heart disease. Cardiac biomarkers and multimodality imaging can be used to aid in screening and diagnosis. The mainstay of treatment of carcinoid heart disease is surgery.

The 1, 2, 3, 4 of carcinoid heart disease: Comprehensive cardiovascular imaging is the mainstay of complex surgical treatment.

Carcinoid heart disease (CHD) is a rare complication of neuroendocrine tumors, most commonly involving the tricuspid and pulmonary valves. The mitral and aortic valves can also be affected, albeit rarely, in certain circumstances such as the presence of a patent foramen ovale. Transthoracic echocardiogram is generally considered the key imaging modality, but cardiac magnetic resonance can add valuable information, particularly in the assessment of pulmonary valve function or multivalvular disease. Previously, surgical management of CHD carried high mortality, as a result of less advanced surgical techniques and of late intervention, reserved for cases of severely symptomatic heart failure. Modern approaches are associated with significantly improved survival rates, even in multivalvular, complex cases. Valve replacement can provide survival benefits in patients with CHD, but the optimal timing for the intervention is uncertain, with data suggesting a trend of improved survival with earlier intervention. A comprehensive imaging assessment may contribute to establishing optimal surgical timing. This approach may shift the main driver of mortality from the cardiac involvement to the primary malignancy and lead to improved outcomes. We present a series of imaging findings in CHD patients who have successfully undergone simultaneous surgical replacement with bioprosthetic valves of 1 to 4 heart valves. The surgical decision in these patients was based on a multimodality cardiovascular approach, including transthoracic and transesophageal echocardiography and cardiac magnetic resonance. The patients had uncomplicated postoperative courses, significant symptomatic relief from heart failure symptoms, and there was no cardiovascular mortality. Early recognition of CHD with a multimodality approach may improve outcome, even in complex cases. Bioprosthetic valves are generally preferred in CHD due to decreased need for anticoagulation, despite concern for premature degeneration. A collaboration between the Oncology and Cardiology teams is essential for the long-term management of CHD patients.

Carcinoid-syndrome: recent advances, current status and controversies.

To review recent advances and controversies in all aspects of carcinoid-syndrome. Over the last few years there have been a number of advances in all aspects of carcinoid syndrome as well as new therapies. These include new studies on its epidemiology which demonstrate it is increasing in frequency; increasing insights into the pathogenesis of its various clinical manifestations and into its natural history: definition of prognostic factors; new methods to verify its presence; the development of new drugs to treat its various manifestations, both initially and in somatostatin-refractory cases; and an increased understanding of the pathogenesis, natural history and management of carcinoid heart disease. These advances have generated several controversies and these are also reviewed. There have been numerous advances in all aspects of the carcinoid-syndrome, which is the most common functional syndrome neuroendocrine tumors produce. These advances are leading to new approaches to the management of these patients and in some cases to new controversies.

Triple Valve Replacement in a Rare Case of Left-sided Carcinoid Heart Disease

Carcinoid heart disease (CHD) is characterised by plaque-like deposits of fibrous tissue on the endocardium as a result of production of vasoactive substances by carcinoid tumours. CHD typically affects the right-sided valves of the heart. Involvement of the left-sided valves rarely occurs, as it is thought that the pulmonary circulation inactivates the vasoactive substances. Left-sided CHD has been reported in patients with a patent foramen ovale and/or bronchial carcinoid lesions. We present a rare instance of left-sided CHD, occurring without an intracardiac shunt or bronchial carcinoid lesions. This 66-year-old male was diagnosed with a metastatic small bowel carcinoid tumour with liver metastases in 2014, and was managed with lanreotide injections. He was found to have CHD involving his tricuspid, pulmonic, and aortic valves. Initially, he was medically managed; however, following multiple presentations and worsening heart failure he was referred for surgical management. A successful elective triple valve replacement (and one coronary artery bypass grafting) was performed, with replacement of the tricuspid, pulmonary, and aortic valves with bioprosthetic valves. Histology of the resected valves was suggestive of chronic carcinoid valvulopathy. At a 2-month follow-up appointment after his surgical admission his heart failure symptoms had improved markedly. There are very few case reports in the literature documenting management of left-sided CHD and the pathophysiology is not well established. All valves should be assessed and patients with CHD should be undergo close surveillance, with consideration of valvular surgery in patients with severe valvular dysfunction to potentially improve prognosis and long-term functional status.

4-Valve Heart Disease and Right Heart Failure

Carcinoid heart disease is a rare form of heart disease due to secretion of vasoactive compounds, including serotonin, from gastrointestinal tumors. This E-challenge examines the case of a patient with advanced carcinoid heart disease who presented to the operating room (OR) for a tricuspid valve replacement. Once the patient was in the OR, intraoperative transesophageal echocardiography was used to discover a patent foramen ovale and involvement of all 4 valves with regurgitant lesions. The patient underwent tricuspid valve replacement, pulmonic valve replacement, right ventricular outflow tract reconstruction, and patent foramen closure in the OR and experienced subsequent fulminant right heart failure. Mechanical circulatory support was required to separate the patient from cardiopulmonary bypass, which was first attempted with an intra-aortic balloon pump and subsequently achieved with implantation of a right ventricular assist device. Multiple reports of acute right heart failure are available in the literature; however, this case helps illustrate several important considerations for the anesthesiologist. The effects of chronic circulating vasoactive compounds on the heart valves are well documented; however, it is likely that advanced carcinoid heart disease also will trigger pre-existing myocardial dysfunction, which may be underappreciated. Identifying patients who are at high risk for intraoperative right heart failure and considering what constitutes an adequate preoperative assessment of right heart function aid in preparing for OR management. In addition, reviewing the potential options for managing these patients when the traditional therapies are inadequate, including mechanical support and extracorporeal circulation, is a useful exercise in preparation. This case also highlights the contributions of intraoperative transesophageal echocardiography in the diagnosis and management of carcinoid heart disease, the need for additional preoperative optimization of these patients, and the management and potential complications of mechanical support.

Variation in Cardiac Screening and Management of Carcinoid Heart Disease in the UK and Republic of Ireland

AimsScreening for carcinoid heart disease is an important, yet frequently neglected aspect of the management of patients with neuroendocrine tumours (NETs). Screening is advocated in international guidelines, although recommendations on the modality and frequency are poorly defined. We mapped current practice for the screening and management of carcinoid heart disease in specialist NET centres throughout the UK and Republic of Ireland. Materials and methodsThirty-five NET centres were invited to complete an online questionnaire outlining the size of NET service, patient selection criteria for carcinoid heart disease screening and the modality and frequency of screening. ResultsTwenty-eight centres responded (80%), representing over 5500 patients. Eleven per cent of centres screen all patients with any NET, 14% screen only patients with midgut NETs, 32% screen all patients with liver metastases and/or carcinoid syndrome and 43% screen all patients with evidence of syndrome or raised urinary/serum/plasma 5-hydroxyindoleacetic acid (5HIAA). The mode of screening included clinical examination, echocardiography and biomarker measurement: 89% of centres carry out echocardiography, ranging from at initial presentation only (24%), periodically without clearly defined intervals (28%), annually (36%) or less than annually (12%); three centres use a scoring system to report their echocardiograms. Fifty per cent of centres utilise biomarkers for screening (chromogranins, plasma/urinary 5HIAA or most commonly N-terminal pro-brain natriuretic peptide) at varying time intervals. ConclusionThere is considerable heterogeneity across the UK and Ireland in multiple aspects of screening and management of carcinoid heart disease.

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.

Surgical management of carcinoid heart disease

Metastatic carcinoid tumor is often seen with flushing, diarrhea, and cardiac symptoms—the carcinoid syndrome. Cardiac failure is often associated with major morbidity and mortality in carcinoid disease. In this report, a case of successful cardiac valvar surgical intervention has resulted in prolonged alleviation of cardiac symptoms and survival.

Surgical Management of Carcinoid Heart Disease

Two female patients with carcinoid heart disease, ages 56 and 32 years, underwent pulmonic valve resection surgery and tricuspid valve replacement with a porcine bioprosthesis. Preoperatively, both patients were in function class 4 with severe right-side congestive failure and signs of tricuspid regurgitation and pulmonic stenosis. Both underwent surgery for porcine tricuspid valve replacement (33 and 31 mm valves) and pulmonic valve resection. Postoperatively, both patients had only minimal symptoms, including trace ankle edema and soft pulmonic murmurs, despite persistence of the systemic symptoms of carcinoid syndrome. Pre- and postoperative catheterization data documented hemodynamic improvements. One patient eventually died of hepatic failure due to metastatic disease. At autopsy, her bioprosthesis was free of carcinoid valvular changes.