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Purpose: The aim of this study was to evaluate signs of right-sided heart dysfunction on staging computed tomography (CT) as indirect indicators of carcinoid heart disease., Patients and Methods: Patients with functionally active neuroendocrine neoplasm and different grades of tricuspid valve regurgitation (TR) were identified. Two readers independently reviewed contrast-enhanced staging CT performed within 90 days before or after echocardiography. Logistic regression and receiver operating analyses were used to asses the predictive value of right ventricle-left ventricle ratio (RV-LV ratio), ventricular septal bowing, retrograde contrast filling of the hepatic veins during contrast injection, and time to aortal enhancement greater than 100 Hounsfield units during bolus tracking for TR., Results: Forty-four examinations were evaluated (11 with TR = 0, 16 with TR = 1, 9 with TR = 2, and 8 with TR = 3). Right ventricle-LV ratio was found to predict TR less than or equal to 1 versus TR greater than 1 (P = 0.0188) and TR less than or equal to 1 versus TR equals 2 (P = 0.0082). A prolonged time to aortal enhancement greater than 100 Hounsfield units during bolus tracking predicted TR less than or equal to 1 versus TR greater than 1 (P = 0.0077). Area under the curve for RV-LV ratio was 0.86 when differentiating TR less than or equal to 1 versus TR equals 2. With a cutoff of 1.07, sensitivity was 0.89, and specificity was 0.72., Conclusions: In patients with functionally active neuroendocrine neoplasm, an RV-LV ratio of more than 1.07 predicted TR with a relatively high sensitivity and moderate specificity and thus could serve as an indicator of subclinical carcinoid heart disease on routine staging CT.

Purpose: Carcinoid heart disease (CHD) with severe valve destruction represents the major cause of high morbidity and mortality in patients with carcinoid syndrome. In this paper, we present a novel interventional treatment approach and report the first clinical result achieved in a patient with extensive CHD., Methods and Results: A woman with an ileal neuroendocrine tumour (G2, Ki67: 5%) presented with severe CHD (NYHA IV) affecting both the pulmonary and the tricuspid valve. First, a balloon-expandable 23-mm Edwards SAPIEN™ was successfully implanted percutaneously into the pulmonary valve. Since no catheter-based techniques were available for the replacement of the native tricuspid valve, we implanted an Edwards SAPIEN 26-mm valve into the vena cava inferior between the right atrium and the ostium of the hepatic veins to reduce abdominal congestion. The implantation was technically successful and completely prevented regurgitation into the vena cava inferior and abdominal veins. After this procedure, the patient's clinical condition improved significantly, and she achieved near-normal exercise tolerance (VO2 max: 24.4 ml O2/kg/min, NYHA II)., Conclusion: We demonstrated that percutaneous valve implantation may offer a novel, minimally invasive option in high-risk patients with severe CHD., (© 2015 S. Karger AG, Basel.)

Carcinoid heart disease (CHD) commonly occurs in association with primary gastrointestinal tract carcinoid tumors with hepatic metastases. Unlike primary gastrointestinal tract carcinoid tumors, primary ovarian carcinoid tumors may cause CHD without hepatic metastases, accounting for only 0.3 % of all carcinoid tumors. Only 37 cases of CHD from primary ovarian carcinoid tumors have been reported. We present a case of CHD in which tricuspid valve thickening and shortening led to reduced valve mobility with the resulting severe tricuspid regurgitation. Considering these characteristics of an abnormal tricuspid valve, we suspected CHD, but prosthetic valve replacement was performed without sufficient systemic examination before surgery. Two years after valve replacement, the patient underwent excision of a mass in the lower abdomen, which was diagnosed as an ovarian carcinoid tumor by histopathological examination. The patient has been observed for more than 3 years after tricuspid valve replacement. She has not experienced bioprosthetic valve leaflet degeneration or dysfunction, although it has been reported that bioprosthetic valves may degenerate in patients with carcinoid tumors. Sufficient systemic examinations should be performed to explore the cause of disease.

Background: Carcinoid tumor with consecutive endocardial fibroelastosis of the right heart, known as carcinoid heart valve disease (CHVD) or Hedinger's syndrome, is accompanied by combined right-sided valvular dysfunction with regurgitation and stenosis of the affected valves. Cardiac surgery with replacement of the tricuspid and/or pulmonary valve is an established therapeutic option for patients with Hedinger's syndrome. Little is known about the long term outcome and the choice of prosthesis for the pulmonal position is still a matter of debate., Methods: The authors report three cases of pulmonary valve replacement with stentless bioprostheses (Medtronic Freestyle, Medtronic PLC, Minneapolis, MN, USA) due to severe pulmonary valve degeneration in consequence of Hedinger's syndrome., Results: All patients presented with re-stenosis of the pulmonal valve conduit at the height of the anastomoses in a premature fashion. Due to the increased risk for repeat surgical valve replacement, two patients were treated by transcatheter heart valves., Conclusion: We do not recommend the replacement of the pulmonary valve with stentless bioprostheses in patients with CHVD. These valves presented with an extreme premature degeneration and consecutive re-stenosis and heart failure.

Described herein is a 67-year-old woman who underwent replacement of both tricuspid and pulmonic valves because of severe isolated right-sided systolic heart failure. The cause of the heart failure preoperatively was believed to be the result of left breast radiation a year earlier. At operation, however, the pulmonic valve was excised and a biopsy of the stiff-walled right atrium was performed, and histologic examination of each was classic of carcinoid heart disease. She never awoke postoperatively. Postoperatively, computed tomography disclosed numerous masses in the liver. Retrospectively, clues to the presence of carcinoid heart disease include thickening of both the tricuspid and pulmonic valve leaflets by echocardiogram, a pressure gradient, albeit small, across the pulmonic valve, the plastering of the septal tricuspid-valve leaflet to the ventricular septum, the total absence of left-sided heart disease, and the presence of extremely low 12-lead QRS electrocardiographic voltage., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Purpose: This study evaluates the prevalence of cardiac metastases in patients with serotonin producing neuroendocrine tumours (NET), examined with 18F-FDOPA PET/CT, and the relationship of these metastases to the presence of carcinoid heart disease (CHD) based on echocardiography., Background: CHD occurs in patients with serotonin producing NET. The diagnostic method of choice remains echocardiography. The precise prevalence of cardiac metastases is unknown given the limitations of standard technologies. Nuclear medicine modalities have the potential to visualize metastases of NET., Methods: All patients who underwent 18F-FDOPA PET/CT because of serotonin producing NET between November 2009 and May 2012 were retrospectively analyzed. The presence of cardiac metastasis was defined as myocardial tracer accumulation higher than the surrounding physiological myocardial uptake. Laboratory tests and transthoracic echocardiography (TTE) results were digitally collected., Results: 116 patients (62 male) underwent 18F-FDOPA PET/CT, mean age was 61±13 years. TTE was performed in 79 patients. Cardiac metastases were present in 15 patients, of which 10 patients also underwent TTE. One patient had both cardiac metastasis (only on 18F-FDOPA PET/CT) and echocardiographic signs of CHD. There were no differences in echocardiographic parameters for CHD between patients with and without cardiac metastases. TTE in none of the 79 patients showed cardiac metastases., Conclusion: The prevalence of cardiac metastases detected with 18F-FDOPA PET/CT in this study is 13%. 18F-FDOPA PET/CT can visualize cardiac metastases in serotonin producing NET patients. There appears to be no relationship between the presence of cardiac metastases and TTE parameters of CHD.

The patient, C.P., is a 59-year-old woman who was diagnosed with metastatic carcinoid of the terminal ileum in May 2003. In June 2003, she underwent an extensive resection including hemicolectomy, cholecystectomy, distal pancreatectomy, and splenectomy with metastatic disease in her pancreas, mesentery, and liver. She had been treated with octreotide, everolimus, oxaliplatin, and multiple hepatic artery embolizations in the past eight years and, most recently, capecitabine and bevacizumab with monthly octreotide. She has had intermittent pleural effusions not requiring intervention and a trace pericardial effusion. Her tumor is functional, meaning it demonstrates hormonal hypersecretion which causes flushing, diarrhea, bronchospasm, and abdominal pain.

Background: Coronary spasm has been described in the context of carcinoid heart disease, but few information are available over perioperative coronary spasm., Methods: We describe the case of a patient developing severe spasm of the entire coronary tree immediately after tricuspid valve surgery for carcinoid heart disease, leading to severe myocardial failure., Results: The spasm was diagnosed by coronary angiography and resolved by intracoronary injection of trinitrine. We present the angiographic features of simultaneous spasm of all coronary arteries and of its evolution., Conclusion: The present case illustrates the likelihood of perioperative spasm of the entire coronary tree in carcinoid heart disease, and the usefulness of coronary angiography for both diagnosis and treatment., (© 2013 Wiley Periodicals, Inc.)

Carcinoid heart disease typically presents with pulmonary stenosis and tricuspid regurgitation. Management is intended for symptomatic relief, and valvular intervention is indicated in refractory heart failure. Balloon valvuloplasty is an option for patients not suitable for surgery. We report the case of a patient with a carcinoid tumour, who developed postoperative refractory hypoxemia. Transthoracic echocardiogram revealed carcinoid pulmonary and tricuspid valve disease, with severe pulmonary stenosis. Balloon valvuloplasty was performed with major clinical improvement., (Copyright © 2013 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Carcinoids are rare tumors originating from neuroendocrine cells. A large proportion of these tumors produce serotonin and other biologically active hormones which may produce carcinoid syndrome characterized by flushing, diarrhoea and bronchospasm. Carcinoid heart disease, a rare complication of carcinoid syndrome, may itself have a great impact on life expectancy of patients with carcinoid syndrome. The authors present a case history of a patients with carcinoid heart disease and they review the symptoms, diagnosis and therapeutic options of this rare complication of carcinoid syndrome.

We present an adult with metastatic carcinoid disease affecting the heart, in whom live/real time three-dimensional transthoracic echocardiography (3DTTE) provided incremental value over two-dimensional transthoracic echocardiography (2DTTE). Initial 2DTTE was able to demonstrate severe pulmonic and tricuspid regurgitation, but was unable to visualize the posterior leaflet of the tricuspid valve or the right (right anterior) leaflet of the pulmonic valve. Further analysis with 3DTTE demonstrated thickening, restricted mobility, and noncoaptation of all three leaflets of both the tricuspid and the pulmonary valves. En face viewing of tricuspid and pulmonary regurgitation vena contractas permitted more reliable quantification of regurgitation severity. In addition, localized, linear, echogenic areas consistent with carcinoid deposits were noted along the inner walls of the right atrium, atrial septum, and inferior vena cava. To the best of our knowledge, endocardial carcinoid deposits have never been reported by 2D or 3D echocardiography. En face viewing of these deposits by 3DTTE enabled measurement of their dimensions and areas. Subcostal examination also identified large circumscribed hepatic lesions consistent with metastatic disease. Neither the carcinoid deposits nor the metastatic lesions were detected by 2DTTE. This case demonstrates the usefulness of 3DTTE as a supplement to 2DTTE in more comprehensively assessing carcinoid involvement of the heart.

We present an adult with metastatic carcinoid disease affecting the heart, in whom live/real time three-dimensional transthoracic echocardiography (3DTTE) provided incremental value over two-dimensional transthoracic echocardiography (2DTTE). Initial 2DTTE was able to demonstrate severe pulmonic and tricuspid regurgitation, but was unable to visualize the posterior leaflet of the tricuspid valve or the right (right anterior) leaflet of the pulmonic valve. Further analysis with 3DTTE demonstrated thickening, restricted mobility, and noncoaptation of all three leaflets of both the tricuspid and the pulmonary valves. En face viewing of tricuspid and pulmonary regurgitation vena contractas permitted more reliable quantification of regurgitation severity. In addition, localized, linear, echogenic areas consistent with carcinoid deposits were noted along the inner walls of the right atrium, atrial septum, and inferior vena cava. To the best of our knowledge, endocardial carcinoid deposits have never been reported by 2D or 3D echocardiography. En face viewing of these deposits by 3DTTE enabled measurement of their dimensions and areas. Subcostal examination also identified large circumscribed hepatic lesions consistent with metastatic disease. Neither the carcinoid deposits nor the metastatic lesions were detected by 2DTTE. This case demonstrates the usefulness of 3DTTE as a supplement to 2DTTE in more comprehensively assessing carcinoid involvement of the heart., (© 2012, Wiley Periodicals, Inc.)

A 53-year-old woman is described who underwent mitral and aortic valve replacement and tricuspid valve annuloplasty for pure regurgitation at all 3 valve sites for unrecognized carcinoid heart disease without the carcinoid syndrome 22 days before death. Metastatic carcinoid was not recognized until necropsy, which disclosed a probable ovarian primary but with large hepatic metastases and left-sided cardiac involvement either greater than or equal to the right-sided involvement. Pulmonary hypertension, very unusual in carcinoid heart disease, persisted postoperatively and probably played a role in the patient's early death. Hepatic metastasis with ovarian primary is most unusual in this circumstance., (Copyright © 2011 Elsevier Inc. All rights reserved.)

We present the case of a 69-year-old woman with a metastatic neuroendocrine tumour of the ileum and severe tricuspid valve regurgitation due to carcinoid valvulopathy. Carcinoid heart disease arises in the context of metastatic serotonin-producing neuroendocrine tumours and typically presents as structural and functional abnormalities of tricuspid (TV) or pulmonary valve (PV). Carcinoid heart disease arises from a low-grade neuroendocrine tumour derived from serotonin-producing enterochromaffin cells that reduces the mobility of the leaflets. Following the development of this type of heart failure, the prognosis is unfavourable and patients usually die as a result of heart failure and not because of the metastatic disease. Our patient was not considered a candidate for valvular surgery because of the progressive nature of the malignancy. However, surgical valve replacement is a therapeutic option that, although it has a significant mortality, needs to be considered in selected patients and leads to improvement in functional capacity and survival.

Background: Carcinoid heart disease is a rare form of valvular heart disease. We sought describe the spectrum of carcinoid heart disease identified by echocardiography and cardiac MRI., Method and Results: Two hundred fifty-two patients with carcinoid syndrome underwent a range of investigations including 2D transthoracic echocardiography, 3D transthoracic echocardiography and transesophageal echocardiography, and cardiac MRI. Fifty-two patients had evidence of carcinoid heart disease. Involvement of the tricuspid, pulmonary, mitral, and aortic valves were found in 47 (90%), 36 (69%), 15 (29%), and 14 (27%), respectively. Myocardial metastases were found in 2 (3.8%) patients. Several patterns of disease were identified depending on the extent and severity to which each leaflet and its associated subvalvular apparatus was affected. Thirteen of 15 (87%) patients with left-sided carcinoid involvement had a patent foramen ovale. Three patients with severe degree of shunting had severe valvular regurgitation. Patients with mild/moderate degree of shunting had mild or moderate valvular regurgitation. Three-dimensional transthoracic echocardiography/transesophageal echocardiography provided detailed anatomic information particularly for the tricuspid and pulmonary valves. Cardiac MRI allowed complementary assessment of valvular heart disease and delineation of myocardial metastases. Gallium-68 octreotide positron emission tomography identified neuroendocrine metastases., Conclusions: Carcinoid heart disease is a heterogeneous disease with a wide spectrum of echocardiographic findings. A multimodality approach is needed in patients with this complex pathology.

A diminutive pulmonary artery and right ventricular outflow tract in a 46-year-old woman with a 10-year history of carcinoid syndrome required transannular pulmonary patch enlargement to allow replacement of the pulmonary and tricuspid valves with bioprostheses. The avoidance of anticoagulation permitted further hepatic arterial embolization without an increased risk of bleeding.

Carcinoid disease arises from a low-grade neuroendocrine tumour derived from serotonin-producing enterochromaffin cells. It is the most common tumour affecting the small bowel. The majority of patients who progress to carcinoid syndrome develop cardiac disease selectively involving the right side of the heart, whereas left heart disease is unusual. The most common cause of death is dilatation and dysfunction of the right ventricle. Right ventricular dysfunction is largely secondary to pathological endocardial fibrosis of the tricuspid and pulmonary valves, presenting with regurgitation and stenosis. Average survival falls to only 11 months with the onset of symptoms, but recent evidence suggests that survival can be improved by early surgery in selected individuals. This article reviews the particular role that cardiovascular magnetic resonance imaging has in the management of carcinoid heart disease.

In a 56-year-old lady, a carcinoid tumor of the terminal ileum metastasized to regional lymph nodes, and the liver was removed by hemicolectomy in 2002. Following a history of cutaneous flushing, diarrhea, and bronchoconstriction 3 years later, a somatostatin therapy was instituted. As flushing and diarrhea resolved and levels of urinary excretion of 5-hydoxyindoleacetic acid decreased, shortness of breath was progressive and prompted a cardiac exam. Despite poor resolution, echocardiography revealed a thickening of the tricuspid valves (TK) with reduced mobility along with right atrial (RA) and right ventricular (RV) dilatation. The pulmonary valve was unobtrusive. Magnetic resonance (MR) imaging revealed extensive fibrous tissue extending from the valvular base to the tip of the tricuspid leaflets. Retraction and immobilization of the TK caused a mild stenosis and a large regurgitant flow. Because medical treatment of tricuspid regurgitation was ineffective, the TK was excised and a Hancock 25-mm bioprosthetic valve was implanted. The postoperative course was uncomplicated, and the patient recuperated and resumed normal daily activities., (2009 Wiley Periodicals, Inc.)

Cardiac surgery for symptomatic carcinoid heart disease in conjunction with adjunctive therapy is being increasingly shown to improve the long-term outlook of patients with carcinoid heart disease. Herein is reported the case of a female patient with heart failure, secondary to carcinoid heart disease affecting all four cardiac valves, who successfully underwent quadruple valve replacement in the same sitting. The patient made an uneventful postoperative recovery, and had an excellent quality of life at the one-year follow up examination.

Background: The management of patients with carcinoid heart disease poses two major challenges for the anaesthetist: carcinoid crisis and low cardiac output secondary to right ventricular (RV) failure. Carcinoid crises may be precipitated by the administration of catecholamines and histamine-releasing drugs., Methods: We analysed a series of 11 patients [six males, median (range) age 60 (42-73) yr] with severe symptomatic carcinoid heart disease who underwent multivalve surgery (right-sided valves, n=8; right- and left-sided valves, n=3) between 2001 and 2007., Results: All patients received octreotide intraoperatively [650 (300-1050) microg] to prevent carcinoid symptoms and vasoplegia. Those patients on a greater preoperative octreotide regime required additional intraoperative octreotide [median (range) dose 320 (300-850) vs 750 (650-1050) mug]. Similarly, the use of greater doses of aprotinin (> 5 KIU) was associated with greater requirements for octreotide [475 (300-700) vs 750 (320-1050) microg] and higher glucose levels (> or =8.5 mmol litre(-1)). Catecholamines were generally required in those patients who presented with a worse New York Heart Association functional class. Overall mortality was 18% (n=2) and only one episode of mild intraoperative carcinoid crisis was observed., Conclusions: Carcinoid crisis and RV failure still remain the primary challenges for the anaesthesiologist while managing patients with carcinoid heart disease. Our study supports the administration of catecholamines to wean patients off cardiopulmonary bypass, particularly in the presence of myocardial dysfunction. Those patients on higher octreotide dosages may require close intraoperative glucose monitoring. Despite high operative mortality, surgical outcome has been improved potentially due to earlier patient referral and better perioperative management.

The presence of a patent foramen ovale (PFO) is associated with morbidity and mortality in patients with carcinoid heart disease (CHD). We report a 66-year-old male patient with tricuspid and pulmonary valve regurgitation secondary to CHD, who developed severe hypoxia due to a right-to-left shunt through a PFO. A 35 mm Amplatzer septal occluder was deployed to reduce the right-to-left shunt as an urgent procedure. Tricuspid and pulmonary valve replacements were electively performed using ON-X mechanical prostheses (31/33 mm and 19 mm, respectively) 70 days after the percutaneous procedure. Transcatheter closure of a PFO prior to definitive right-sided valve surgery can be a useful treatment for CHD patients with acute haemodynamic derangement.

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.

A 55-year-old woman with carcinoid valvular heart disease was referred for consideration of right-sided valve replacement. By transthoracic 2-dimensional and Doppler echocardiography, there was severe tricuspid regurgitation and mild right ventricular dysfunction. The pulmonary valve was thickened with annular constriction and severe regurgitation. Real-time 3-dimensional transthoracic echocardiography provided an en face view of pulmonary valve from the right ventricular outflow perspective, not obtainable by routine 2-dimensional echocardiography. Three-dimensional echocardiography may play an incremental role for the preoperative assessment of the pulmonary valve in patients with carcinoid heart disease.

A 66-year-old man with known metastatic carcinoid tumor presented with increasing dyspnoea, right heart failure and marked hypoxaemia which did not correct with oxygen. Echocardiography demonstrated severe tricuspid regurgitation, moderate pulmonary regurgitation and marked right heart dilatation. The inter-atrial septum was aneurysmal, with a large patent foramen ovale (PFO) with continuous right to left shunting. Cardiac catheterization demonstrated oxygen saturations of 96% in the pulmonary veins and 74% in the left atrium with a significant right to left shunt. During percutaneous closure of the PFO, anaesthetic induction resulted in marked systemic hypotension and worsening hypoxia related to systemic vasodilatation and increased shunting. PFO flow was temporarily obstructed with a sizing balloon resulting in a rapid increase in arterial oxygen saturation from 60% to >90%, but marked systemic hypotension due to acute left ventricular preload reduction, requiring volume replacement and adrenaline. Following deployment of a PFO occluder device, prominent pulsatile splaying of the right and left discs was noted due to the severe tricuspid regurgitation, resulting in some residual inter-atrial shunting. Arterial oxygen saturation was 83%, increasing to 92% at day 4 post-procedure as tissue organization occurred within the device, and the patient reported improvement in dyspnoea.

The case is presented of a fungal-origin endocarditis affecting the eustachian valve. During surgery for pulmonary and tricuspid valve replacement, a 54-year-old male with carcinoid disease was found to have a 3-cm vegetation attached to the eustachian valve. Histopathological assessment of the vegetation revealed the presence of Candida species. The patient made a good postoperative recovery and was continued on a three-month course of antifungal therapy.

Primary ovarian carcinoid disease with cardiac involvement is a very rare syndrome, which requires interdisciplinary management. We report on a patient with primary ovarian carcinoid tumor with severe tricuspid valve insufficiency who was curatively treated after successful tumor resection and tricuspid valve replacement.