Surgery For Carcinoid Heart Disease Research Articles

The impact of peptide receptor radionucleotide therapy with Lutetium-177 DOTATATE on cardiac metastases in neuroendocrine tumors

Abstract Background Cardiac metastasis from neuroendocrine tumors (NET) is rare and has been thought to potentially result in life threatening sequalae. Peptide receptor radionucleotide therapy (PRRT), such as Lutetium-177 (Lu-177) DOTATATE, has been validated as effective treatment for patients with NET who have progressed on somatostatin analogue (SSA) therapy and more recently suggested for use in SAA-naïve higher risk grade 2 and grade 3 NETs. However, Lu-177 DOTATATE therapy has been found to have potentially serious adverse effects both locally and systemically. Overall, the clinical significance of cardiac metastasis of NET, as well as the safety and efficacy of Lu-177 DOTATATE in this cohort, has not been thoroughly evaluated. Purpose This study assessed the outcomes and clinical consequences of cardiac metastatic involvement in patients with NET. Furthermore, this study investigated the safety of Lu-177 DOTATATE therapy in patients with cardiac metastases from NET. Methods All patients who underwent 68Ga-DOTATATE positron emission tomography for evaluation of NET across three tertiary centers between 2016 and 2022 were retrospectively evaluated. Patients with evidence of cardiac metastasis were further evaluated. Baseline characteristics, treatment modality, and clinical outcomes including mortality, arrhythmia or conduction disease, carcinoid heart disease, and heart failure admissions were recorded. Patients with cardiac metastases were grouped and compared based on therapeutic modality (conservative therapy with surveillance or SSA, Lu-177 DOTATATE therapy, or surgical resection of metastases). Results From 3253 patients with NET, 51 patients were identified to have cardiac metastases (mean age 66.7 ± 12.2 years, 54% male). Three patients underwent surgical resection of cardiac metastases, and in two of whom this was concomitant while undergoing valve surgery for carcinoid heart disease. Patients with cardiac metastasis had significantly higher mortality than patients without cardiac metastasis (HR 2.12, 95%CI 1.00 – 4.52, p = 0.049, Figure 1A). Median follow-up from diagnosis of cardiac metastasis was 3.64 years [IQR 1.59 – 5.64]. There was no significant difference in clinical outcomes between patients that received Lu-177 DOTATATE therapy (n = 27) and conservative therapy (n = 21). Specifically, there was no significant difference in mortality risk at five years post the diagnosis of cardiac metastasis between these groups (p = 0.47, Figure 1B). Conclusion Patients with cardiac metastasis from NETs have worse clinical outcomes than NET patients without cardiac metastatic involvement. Lu-177 DOTATATE therapy appears to be safe in patients with cardiac metastasis from NET.Figure 1AFigure 1B

Outcome of selective pulmonary valve management in patients undergoing surgery for carcinoid heart disease

Outcome of selective pulmonary valve management in patients undergoing surgery for carcinoid heart disease

The Role of Transthoracic Echocardiography for Assessment of Mortality in Patients with Carcinoid Heart Disease Undergoing Valve Replacement

Patients with carcinoid heart disease (CHD) are referred for valve replacement if they have severe symptomatic disease or evidence of right ventricular (RV) failure and an anticipated survival of at least 12 months. Data are lacking, however, on the role of transthoracic echocardiography in predicting outcomes. We carried out a retrospective, single-centre cohort study of patients with a biopsy-confirmed neuroendocrine tumour (NET) and CHD undergoing valve replacement for severe valve disease and symptoms of right heart failure. The aim was to identify factors associated with postoperative mortality, both within one year of surgery and during long-term follow-up. Of 88 patients with NET, 49 were treated surgically (mean age: 64.4 ± 7.6 years; 55% male), of whom 48 had a bioprosthetic tricuspid valve replacement for severe tricuspid regurgitation; 39 patients had a pulmonary valve replacement. Over a median potential follow-up of 96 months (interquartile range: 56-125), there were 37 deaths, with 30-day and one-year mortality of 14% (n = 7) and 39% (n = 19), respectively. A significant relationship between RV size and one-year mortality was observed, with 57% of those with severe RV dilatation dying within a year of surgery, compared to 33% in those with normal RV size (p = 0.039). This difference remained significant in the time-to-event analysis of long-term survival (p = 0.008). RV size was found to reduce significantly with surgery (p < 0.001). Those with persisting RV dilatation (p = 0.007) or worse RV function (p = 0.001) on postoperative echocardiography had significantly shorter long-term survival. In this single-centre retrospective study of patients undergoing surgery for CHD, increasingly severe RV dilatation on preoperative echocardiography predicted adverse outcomes, yielding a doubling of the one-year mortality rate relative to normal RV size. These data support the possibility that early surgery might deliver greater long-term benefits in this patient cohort.

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.

A 33-year follow-up after valvular surgery for carcinoid heart disease.

Valvular surgery has improved long-term prognosis in severe carcinoid heart disease (CaHD). Experience is limited and uncertainty remains about predictors for survival and strategy regarding single vs. double-valve surgery. The aim was to review survival and echocardiographic findings after valvular surgery for CaHD at our institution. Between 1986 and 2019, 60 consecutive patients, median age 64 years, underwent valve surgery for severe CaHD. Operations involved combined tricuspid valve replacement (TVR) and pulmonary valve replacement (PVR) in 42 cases, and TVR-only or TVR with pulmonary valvotomy (no PVR) in 18 patients. All implanted valves were bioprosthetic. Preoperative echocardiography, creatinine, NT-pro-brain natriuretic peptide (NT-pro-BNP), and 24-h urinary 5-hydroxyindoleacetic acid (5-HIAA) were obtained. 30-Day mortality was 12% (n=7), and 8% for the most recent decade 2010-2019. Median survival was 2.2 years and maximum survival 21 years. Patients undergoing combined TVR and PVR had significantly higher survival compared with operations without PVR (median 3.0 vs. 0.9 years, P = 0.02). Preoperative levels of NT-pro-BNP and 5-HIAA in the top quartile predicted poor survival. On preoperative echocardiograms, pulmonary regurgitation was severe in 51% and indeterminate in 17%. Postoperative echocardiography confirmed relatively good durability of bioprostheses, relative to the patients' limited oncological life expectancy. Valvular surgery in CaHD has an acceptable perioperative risk. Survival for combined TVR and PVR was significantly higher compared with operations without PVR. Bioprosthetic valve replacement appears to have adequate durability. Preoperative echocardiography may underestimate pulmonary pathology. Combined TVR and PVR should be considered in most patients.

33-year follow-up after valvular surgery for carcinoid heart disease

Abstract Background Valvular surgery for carcinoid heart disease (CaHD) has been found to improve long-term prognosis. Experience is limited and questions about single versus double-valve surgery as well as selection of prosthetic valves remain under debate. Purpose We reviewed the surgical experience after valvular surgery for CaHD in our institution, which is a national referral center. Methods We reviewed clinical records and echocardiograms. Perioperative death was defined as mortality within 30 days of operation. Results Sixty consecutive patients (32 men, 62±9 years) underwent valve surgery between 1986 and 2019, of whom 59/60 had an intestinal carcinoid disease with hepatic metastases. Mean time from diagnosis to heart surgery was 3.8±3.7 years. Preoperatively, 6 patients were in NYHA class I/II, the others were in class III or IV. All 60 surgical procedures involved tricuspid valve replacement (TVR). In 47 cases, the pulmonary valve was treated surgically: Early in the series, five patients underwent pulmonary valvotomy or commissurotomy, and more recently, 42 patients received pulmonary valve replacement (PVR). All valves were replaced with bioprostheses, except for one pulmonary homograft. Concomitant significant aortic and mitral regurgitation occurred in 2 patients who received quadruple valve replacement. Two re-operations were performed due to degeneration of bioprostheses. Mean hospital stay was 13±9 days. Overall 30-day mortality was 11.6% (n=7) but was reduced to 8.3% in the last decade. The overall median survival was 2.2 years. Maximal survival free of reoperation was 18 years, and maximal survival for a single patient was 21 years. Median survival for combined TVR and PVR was significantly better than for single TVR or TVR combined with pulmonary valvotomy (3.0 years vs. 0.94 years, respectively, p=0.02; see Figure). Preoperatively, left ventricular ejection fraction (EF) was normal in 83%. Severe tricuspid regurgitation was always present. The right ventricle (RV) was dilated (RVD1 49 mm ± 5 mm) as well as the right atrium (60±16 ml/m2). The TAPSE was in normal range (17 to 29 mm). Pulmonary regurgitation was mild, moderate, severe, or indeterminate in 6%, 26%, 51%, and 17% respectively. In 17% the transpulmonary velocity was &amp;gt;2.5 m/s, indicating stenosis. On last available postoperative echo (mean time 2.1 years) EF was found unchanged. RV diameter was nearly normalized (RVD1 41 mm ± 5 mm). TAPSE was reduced (12 mm ± 4 mm). No significant regurgitation was detected in any TVR. There was significant stenosis (mean gradient &amp;gt;5 mmHg) in 23% of the TVR. No regurgitation of significance was detected in any PVR. A mean gradient &amp;gt;20 mmHg was found in 11% of the PVR. Conclusions While perioperative risk of valve replacement in CaHD remains substantial, this study shows relatively favourable outcomes of surgical valve replacement with bioprostheses. PVR together with TVR had better outcomes than those undergoing TVR only. Figure. Survival by Surgical Treatment. Funding Acknowledgement Type of funding source: Public hospital(s). Main funding source(s): Department of Medical Sciences, Uppsala University. Department of Surgical Sciences, Uppsala University

Timing of peptide receptor radiotargeted therapy in relation to cardiac valve surgery for carcinoid heart disease in patients with neuroendocrine metastases and cardiac syndrome. A single-centre study from a centre of excellence.

Perioperative mortality of patients who undergo heart valve surgery for carcinoid heart valve disease has been observed to be high (5%-10%). We investigated whether peptide receptor radiotherapy with lutetium-177 dotatate can be used safely in patients with neuroendocrine neoplasm carcinoid heart valve disease and if there is associated survival advantage by reducing overall exposure of the valves to high doses of vasoactive peptides. Retrospective case notes review was performed on 18 neuroendocrine neoplasm patients (mean 60 years), who underwent heart valve surgery between 2003 and 2017 for carcinoid heart valve disease, 9 of whom received peptide receptor radiotherapy in addition to surgery. All patients were treated with somatostatin receptor antagonists and underwent cardiac valvular surgery (mean two valves replaced) and three benefitted from additional coronary bypass grafting. Nine patients underwent surgery alone: in this group, the time from surgery to progression was 14 months (mean; SD 13.5 months). Nine were treated with peptide receptor radiotherapy in addition to surgery. Six underwent surgery with peptide receptor radiotherapy on progression. Time to progression from surgery to first peptide receptor radiotherapy was mean 25.1 months (SD 23.6 months). No patients developed peritreatment cardiac complications. There were no deaths within the 30-day postoperative period. Average time from surgery to last follow-up/death was 41 months (6-79) in the surgery + lutetium group and in the surgery only group 17 months (1-24). Nine patients died, five in the surgery + lutetium group and four in the surgery only group, all at greater than 1-year postsurgery. Peptide receptor radiotherapy is safe in the setting of Carcinoid valvular heart disease in patients with controlled heart failure, PPRT can be use in the pre- and post-valve surgery period. There appears to be a survival benefit of having peptide receptor radiotherapy. Further evidence for peptide receptor radiotherapy in the neoadjuvant setting prior to cardiothoracic surgery is required.

Timing of peptide receptor radiotargeted therapy in relation to cardiac valve surgery for carcinoid heart disease in patients with neuroendocrine metastases and cardiac syndrome: a single centre study from a centre of excellence

Introduction Perioperative mortality of patients who undergo heart valve surgery for carcinoid heart valve disease has been observed to be high (5%-10%). We investigated whether peptide receptor radiotherapy with lutetium-177 dotatate can be used safely in patients with neuroendocrine neoplasm carcinoid heart valve disease and if there is associated survival advantage by reducing overall exposure of the valves to high doses of vasoactive peptides. Method Retrospective case notes review was performed on 18 neuroendocrine neoplasm patients (mean 60 years), who underwent heart valve surgery between 2003 and 2017 for carcinoid heart valve disease, 9 of whom received peptide receptor radiotherapy in addition to surgery. Results All patients were treated with somatostatin receptor antagonists and underwent cardiac valvular surgery (mean two valves replaced) and three benefitted from additional coronary bypass grafting. Nine patients underwent surgery alone: in this group, the time from surgery to progression was 14 months (mean; SD 13.5 months). Nine were treated with peptide receptor radiotherapy in addition to surgery. Six underwent surgery with peptide receptor radiotherapy on progression. Time to progression from surgery to first peptide receptor radiotherapy was mean 25.1 months (SD 23.6 months). No patients developed peritreatment cardiac complications. There were no deaths within the 30-day postoperative period. Average time from surgery to last follow-up/death was 41 months (6-79) in the surgery + lutetium group and in the surgery only group 17 months (1-24). Nine patients died, five in the surgery + lutetium group and four in the surgery only group, all at greater than 1-year postsurgery. Discussion Peptide receptor radiotherapy is safe in the setting of Carcinoid valvular heart disease in patients with controlled heart failure, PPRT can be use in the pre- and post-valve surgery period. There appears to be a survival benefit of having peptide receptor radiotherapy. Further evidence for peptide receptor radiotherapy in the neoadjuvant setting prior to cardiothoracic surgery is required.

519P - Carcinoid heart disease (CHD): the CRUSOE-NETs, a prospective cohort study from the French group of endocrine tumours (GTE)

BackgroundNeuroendocrine tumors (NET) represent a heterogeneous group of rare tumors, some of them secreting serotonin resulting in the carcinoid syndrome (CS). CHD is an integral part of this syndrome but remains poorly understood. The GTE is conducting a national survey of patients at risk for CHD aiming at evaluating the occurrence & progression rates of CHD, the frequency & results of cardiac surgery, and patient outcomes as well as the role of clinical characteristics and biomarkers as predictive markers; 600 patients are expected over a 5-year period with a 10-year follow-up. We herein present the study status at one year. MethodsPatients with a metastatic ileum or bronchial NET, or any NET with a CS or 5HIAA levels greater than at least twice the upper normal range, seen by a NET specialist and a referee cardiologist are eligible. Clinical, pathological, biological parameters and previous treatments are collected. A transthoracic echocardiography is realised at inclusion and at least every year. ResultsFrom March 2018 to March 2019, 167 patients from 8 centers were included. Median [range] time from NET diagnosis to study inclusion was 56 months [0-501]. Median age was 66 years [34-86] with a male preponderance (53%); 85% had ileum NETs, 8% lung NETs, and 7% other NETs with CS or elevated 5HIAA; 100% had metastatic disease. Most of them have been pretreated: somatostatin analogs 96%, surgery of primary tumor 81% or metastasis 34%, liver embolization 24%, peptide receptor radionuclide therapy 19%, chemotherapy 16%, targeted therapy 12%, radiofrequency ablation 6%, interferon 2%. At inclusion, 81 patients (49%) had a CS, 67% with flushing, 68% with diarrhea. CHD was documented in 22 (13%) patients, all of them had tricuspid disease and simultaneous pulmonary, mitral and/or aortic disease for respectively 13, 3 and 1 of them; 14 patients with CHD had a CS. Seven patients underwent cardiac surgery for CHD. ConclusionsThe first large prospective multicentric study about CHD is ongoing. Preliminary results confirm the feasibility of the study with nearly 170 patients included in one year and 13% of CHD. The inclusion of new patients and 10-year follow-up will allow a better knowledge of the current incidence, progression and prognosis of CHD. Clinical trial identificationNCT03498040. Legal entity responsible for the studyFrench Group of Endocrine Tumors (GTE). FundingIPSEN. DisclosureG. Cadiot: Advisory / Consultancy: Novartis; Advisory / Consultancy: Ipsen; Advisory / Consultancy: AAA; Advisory / Consultancy: Keocyt. P. Nazeyrollas: Honoraria (self), Honoraria (institution), Travel / Accommodation / Expenses, Non-remunerated activity/ies: Novartis; Honoraria (self), Non-remunerated activity/ies: MSD; Honoraria (institution): Sanofi; Honoraria (institution): novo-nordisk; Travel / Accommodation / Expenses, Non-remunerated activity/ies: actelion; Travel / Accommodation / Expenses, Non-remunerated activity/ies: Bayer; Non-remunerated activity/ies: Servier; Non-remunerated activity/ies: bms. P. Afchain: Travel / Accommodation / Expenses: Novartis; Travel / Accommodation / Expenses: Ipsen. F. Delelis: Honoraria (self): Novartis; Honoraria (self): boehringer. J. Forestier: Honoraria (self): Amgen; Honoraria (self), Advisory / Consultancy, Speaker Bureau / Expert testimony, Research grant / Funding (institution): Bayer; Honoraria (self), Speaker Bureau / Expert testimony, Research grant / Funding (institution): Ipsen; Honoraria (self), Advisory / Consultancy, Travel / Accommodation / Expenses: Sanofi; Honoraria (self), Advisory / Consultancy, Speaker Bureau / Expert testimony: Merck; Honoraria (self): Servier. C. Lombard Bohas: Advisory / Consultancy: Ipsen; Advisory / Consultancy: Novartis; Advisory / Consultancy: Pfizer; Advisory / Consultancy: AAA. All other authors have declared no conflicts of interest.

Early Outcomes of Surgery for Carcinoid Heart Disease

The prognosis of patients with metastatic neuroendocrine neoplasms (NEN) continues to improve with modern oncological therapy. In the subgroup of patients with carcinoid syndrome, the development of carcinoid heart disease (CaHD) severely impacts long term survival. Valve surgery has been demonstrated to improve survival and symptoms in patients with CaHD. We sought to assess the outcomes of surgery for CaHD from a single Neuroendocrine Service. We retrospectively reviewed outcomes of patients with CaHD and metastatic NEN who underwent valvular surgery over a 4-year period (2012-2016). Twenty (20) patients (mean age 64 years, range 29-77 years), all with metastatic small intestinal NEN treated with somatostatin analogues, underwent surgery. Tumour grade was: G1 (n=8), G2 (n=9), and unknown (n=3). Preoperative New York Heart Association (NYHA) class was III/IV in 15 patients (75%). The valves affected were: tricuspid (n=20; 19 replace, 1 repair), pulmonary (n=14; 14 replace), mitral (n=2; two replace) and aortic valve (n=2; two replace). Concomitant procedures included patent foramen ovale closure (n=9), right ventricular outflow tract (RVOT) (n=4) augmentation and coronary artery bypass grafting (n=3). There were two operative deaths (10%) due to right heart and liver failure. At 6 weeks, all surviving patients had symptom improvement (NYHA I/II). Median follow-up was 2±1.5 years (<1 month to 5 years). One asymptomatic patient developed RVOT obstruction after pulmonary replacement. Two patients had valvular recurrence. One and 2-year survival were 74% and 48% respectively. Of 13 late deaths, 12 were cancer-related (two with uncorrected progressive pulmonary regurgitation). Surgery for CaHD can be performed with satisfactory early results, leading to an improvement of cardiac symptoms, survival and enabling subsequent oncologic treatment. Further studies are required to improve longer term outcomes in these complex patients with CaHD.

Predictors of mortality following cardiac surgery for carcinoid heart disease.

e15692 Background: Carcinoid Heart Disease (CHD) complicates 50% of carcinoid syndrome cases and represents a major cause of mortality in these patients. Surgery continues to be the only strategy to improve survival in CHD. We aimed to evaluate predictors of mortality following surgery for CHD. Methods: We retrospectively analyzed patients with CHD and a history of valve surgery presenting at a tertiary cancer center between November 2005 and March 2018. Data regarding symptoms, clinical findings, and CHD treatment were collected. Overall survival (OS, time interval from heart surgery to death) was calculated. Univariate Cox proportional hazards regression analyses were conducted to identify variables that were associated with OS. Results: We identified 25 patients with CHD who underwent surgical replacement with bioprosthetic valves: 10 (40%) the tricuspid valve (TV), 11 (44%) TV and pulmonary valve (PV), 3 (12%) TV, PV, and aortic valve, and 1 (4%) all 4 valves. Among them, 19 (76%) survived at least 12 months after the surgery. Clinical factors associated with decreased OS are presented in Table 1. Conclusions: OS following surgery is dictated by the severity of CHD at the time of surgery. Worse outcomes are predicted by a history of congestive heart failure, the number of symptoms, and ascites. Better selection criteria are needed in order to choose the candidates for surgery who will benefit the most. [Table: see text]

Successful anesthetic management of a 14 year old boy undergoing tricuspid valvular surgery for carcinoid heart disease

Successful anesthetic management of a 14 year old boy undergoing tricuspid valvular surgery for carcinoid heart disease

Improving outcome of valve replacement for carcinoid heart disease

ObjectiveCarcinoid heart disease is characterized by tricuspid valve regurgitation and varying degrees of pulmonary valve regurgitation or stenosis. Valve replacement procedures may be complicated by systemic effects of carcinoid syndrome, as well as hepatic dysfunction and right heart failure. This study was performed to identify factors that might be associated with improving early mortality rates and late outcomes. MethodsBetween November 1985 and January 2018, 240 adult patients underwent surgery for carcinoid heart disease at the Mayo Clinic. We analyzed the association of multiple clinical and echocardiographic variables on early mortality and late survival. ResultsThe median (interquartile range) age of patients was 63 years (55-69), and 117 patients (49%) were male. Before operation, 157 patients (70%) had New York Heart Association class III or IV limitation. Somatostatin analogs were used in 221 patients (92%), and long-acting somatostatins were used in 130 patients (54%). Loop diuretic therapy was used preoperatively in 125 patients (52%). Early mortality rate was 29% (9/22) between 1985 and 1994, but decreased to 7% (6/81) during 1995 to 2004, and to 5% (7/128) from 2005 onward. Overall survival estimates at 1, 3, and 5 years were 69%, 48%, and 34%, respectively. Older age, advanced New York Heart Association class, and a nonlinear effect of creatinine were independently associated with overall mortality. ConclusionsValve replacement for carcinoid heart disease has acceptable short-term mortality, and early risk has decreased in the current era. Earlier intervention may improve overall survival.

Early Outcomes of Surgery for Carcinoid Heart Disease

Early Outcomes of Surgery for Carcinoid Heart Disease

Patient outcomes after cardiac surgery for carcinoid heart disease are dependant upon successful cytoreductive multimodal treatment and control of metastatic neuroendocrine disease

Searchable abstracts of presentations at key conferences in endocrinology ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Prognosis of patients with carcinoid heart disease after valvular surgery.

A best evidence topic in cardiac surgery was written according to a structured protocol. We addressed the following question: in patients who are diagnosed with carcinoid heart disease (CHD), do valvular surgeries improve their prognosis? Fifty percent of the patients with clinically diagnosed carcinoid syndrome had cardiac involvement which was present either as valvular dysfunction or as cardiac metastases. These patients often require surgery due to their heightened risk of cardiac disease. Altogether 217 relevant papers were identified as a result of the below-mentioned search, of which 10 papers represented the best evidence to answer the question. The author, journal, date and country of publication, patient group studied, study type, relevant outcomes, results and study weaknesses were tabulated. Of the patients who were identified to have carcinoid heart disease in different studies, 193 patients had valve procedure, mainly replacements at tricuspid, mitral and aortic valve positions and either valvuloplasty or replacement at pulmonary valve. Tricuspid and pulmonary valves represented the majority of the excised valves among patients undergoing valvular surgery for CHD. The pathology of carcinoid valve was attributed to the presence of plaque, causing thickening and retraction. Pure regurgitation was the most common finding in all the valves except pulmonary valve which had both stenosis and insufficiency. Thirty-day mortality was 17% (range 1-63%) and long-term survivors were reported to be alive at an average of 58 months (28-80 months) after the valve surgery. The evidence demonstrates that surgical intervention can lead to improved prognosis and reduce the symptoms of heart failure. Postoperative mortality was mainly due to the carcinoid disease itself and not as a complication of the surgery. Therefore, surgery could be considered for symptomatic palliation in carefully selected individuals.

Early Diffuse Coronary Artery Spasm After Heart Valve Surgery in the Carcinoid Syndrome

Coronary spasm has been described in the context of carcinoid heart disease, but few information are available over perioperative coronary spasm. 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. 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. 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.

Cardiac surgery for carcinoid heart disease

Cardiac surgery for carcinoid heart disease

Cardiac surgery for carcinoid heart disease in 12 cases

Cardiac involvement is now a major source of morbidity and mortality in patients with carcinoid tumors. We reviewed patients with carcinoid heart disease who underwent valvular surgery in our center. Twelve patients with carcinoid heart diseases underwent cardiac surgery between 2000 and 2008. Patients were divided into two groups: group A (n = 6) comprised patients who survived more than 6 months after cardiac surgery, and group D (n = 6) comprised those who died within 6 months. Preoperative factors were compared between the groups. All the 12 patients with carcinoid heart disease underwent tricuspid valve surgery (3 had tricuspid repair and 9 had tricuspid replacement with a bioprosthetic valve). Postoperative 30-day mortality was 16.7% and 2-year actuarial survival was 50.0%. Median survival after the first diagnosis of carcinoid disease was 4.4 years that from first diagnosis of carcinoid heart disease was 2.7 years. Preoperative median left ventricular ejection fraction in group D (52.5%) was significantly lower than that in group A (67.2%, P < 0.05). There were no statistically significant differences between the groups in other parameters. Postoperative prognosis may be worse when preoperative left ventricular ejection fraction is borderline, even if it is within the normal limits. Cardiac evaluation is needed in all patients with carcinoid disease from the earliest time of medical and oncological therapy to improve patient outcome.

Outcomes, risks and complications of cardiac surgery for carcinoid heart disease

The development of carcinoid heart disease causes significant valvular dysfunction, eventually leading to symptomatic right heart failure and impaired survival. Data regarding cardiovascular surgery are limited. We sought to identify outcomes, risks and complications of valve surgery for carcinoid heart disease. Twenty-two patients with carcinoid heart disease underwent cardiac valve surgery between 2006 and 2010. Patients were considered for surgery if (1) they had stable carcinoid tumour, (2) they had severe valvular dysfunction and (3) were symptomatic and (4) they had no other significant co-morbidities. Three patients underwent tricuspid valve replacement alone, 15 patients underwent tricuspid and pulmonary valve replacement, two patients underwent tricuspid, pulmonary and mitral valve replacement and two patients underwent quadruple valve replacement. Overall 30-day mortality was 18%. Causes of death were right ventricular dysfunction (two patients), carcinoid crises (one patient) and pneumonia (one patient). Four patients required permanent pacemaker for complete heart block. Of those that survived initial surgery, median follow-up was 26 months (interquartile range 8-42); 1- and 2-year survival rates were 56% and 44%, respectively. There was no significant difference in survival between those patients in the New York Heart Association (NYHA) class I/II, mild/moderate right ventricular dilatation or N-terminal brain natriuretic peptide (NT-proBNP) <1245 pg ml(-1) compared with those in NYHA class III/IV, severe right ventricular enlargement or NT-proBNP > 1245 pg ml(-1), respectively. Long-term causes of death were related to advanced metastatic carcinoid tumour. No patient required re-operation for bioprosthetic degeneration. Valve surgery for carcinoid heart disease is of higher risk compared with most other forms of valvular surgery. However, in those that survive the operation significant improvement in functional class occurs. Most long-term complications were related to the tumour itself rather than cardiac complications.