Surgery For Carcinoid Heart Disease Research Articles

Surgery for Carcinoid Heart Disease: Current outcomes, Concerns and Controversies

Cardiac involvement in patients with carcinoid disease is a well-recognized pathology. Early reports described right-sided heart involvement. More recently, both right- and left-sided heart disease have been reported. Progress of the treatment of carcinoid disease is leading to an increase in the number of patients presenting for surgery to treat valvular dysfunction. owing to improvements in the prognosis of the basic disease, valve replacement surgery is now a reasonable option in patients with severe valvular dysfunction. This article provides an overview of carcinoid heart disease with an emphasis on current outcomes, concerns and controversies associated with the surgical management of this disease.

Management of patients undergoing multivalvular surgery for carcinoid heart disease: the role of the anaesthetist

BackgroundThe 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. MethodsWe 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. ResultsAll patients received octreotide intraoperatively [650 (300–1050) μg] 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) μg]. Similarly, the use of greater doses of aprotinin (>5 KIU) was associated with greater requirements for octreotide [475 (300–700) vs 750 (320–1050) μg] and higher glucose levels (≥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. ConclusionsCarcinoid 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.

Intraoperative Management of Patients with Carcinoid Heart Disease Having Valvular Surgery: A Review of One Hundred Consecutive Cases

Cardiac surgery for carcinoid heart disease is complicated by hemodynamic instability secondary to carcinoid crises, cardiovascular dysfunction, and blood loss. The safety of vasopressors and the benefit of aprotinin during concomitant octreotide administration are uncertain. We reviewed the effects of vasopressors and aprotinin on octreotide administration and mortality by univariate analysis in 100 consecutive cases of cardiac surgery for carcinoid heart disease from 1985 to 2003. Because mortality declines were temporally related to the introduction of aprotinin, bivariate analyses were performed to identify other factors associated with mortality. Carcinoid symptoms and hypotension were treated with octreotide (n = 89) and/or vasopressors (n = 93). Vasopressors were not associated with increased octreotide administration. Patients requiring epinephrine had higher mortality but also had worse preoperative New York Heart Association class, higher urinary 5-hydroxyindoleacetic acid levels, and increased blood transfusion requirements. Aprotinin (n = 54) was associated with decreased blood transfusion requirements, increased octreotide administration, but not mortality. Overall mortality was 13%, declining from 28% between 1985 and 1994 to 6% between 1995 and 2003. Mortality was associated with greater blood transfusion requirements and longer duration of cardiopulmonary bypass. Vasopressors may be used in conjunction with octreotide in carcinoid patients. The increased mortality associated with epinephrine likely reflects selection bias rather than a primary adverse effect. The improved survival over time in carcinoid patients is multifactorial and unrelated to aprotinin administration, suggesting further inhibition of the kallikrein-kinin system has little added benefit for this outcome in the presence of octreotide.

Carcinoid Heart Disease

HomeCirculationVol. 113, No. 7Carcinoid Heart Disease Free AccessReview ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplementary MaterialsFree AccessReview ArticlePDF/EPUBCarcinoid Heart Disease Wissam A. Jaber, MD and Kyle W. Klarich, MD Wissam A. JaberWissam A. Jaber From the Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minn. Search for more papers by this author and Kyle W. KlarichKyle W. Klarich From the Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minn. Search for more papers by this author Originally published21 Feb 2006https://doi.org/10.1161/CIRCULATIONAHA.105.509471Circulation. 2006;113:e160–e161A 57-year-old man presented with peripheral edema, fatigue, and dyspnea on exertion. He also had diarrhea and occasional flushing for 4 months. On physical examination he had elevated neck veins at 9 cm with a dominant v-wave, sternal lift with 2/6 systolic ejection murmur at the left upper sternal border and holosystolic murmur at the left lower sternal border, and a moderate lower extremity edema. His echocardiogram is shown (Figure 1). In a 24-hour urine collection, 5-hydroxyindolacetic acid was measured as 182 mg (normal, 2 to 8 mg). A computed tomography scan of the abdomen (Figure 2) showed multiple liver lesions consistent with metastatic carcinoid. Treatment with octreotide, a somatostatin analogue, was initiated. Both the tricuspid and pulmonary valves (Figure 3 and Figure 4) were replaced with bioprosthetic valves. The pathology of his native valves showed fibrous intimal thickenings on the inside surfaces of the valvular leaflets consistent with carcinoid heart disease (Figure 5 and Figure 6). The patient recovered well from surgery, and his symptoms of heart failure improved. Download figureDownload PowerPointFigure 1. A continuous wave Doppler signal through the pulmonary valve shows increased systolic peak velocity consistent with stenosis and evidence of pulmonary regurgitation. There is rapid dampening of the regurgitant signal with late diastolic reversal of flow (arrow) consistent with pulmonary stenosis and elevated right ventricular pressure. See Movie I through Movie III.Download figureDownload PowerPointFigure 2. Computed tomography scan of the abdomen showing multiple masses in the liver (arrows).Download figureDownload PowerPointFigure 3. Surgically excised tricuspid valve appears white, with thickened leaflets and chordae. Courtesy of William D. Edwards, MD, Mayo Clinic, Rochester, Minn.Download figureDownload PowerPointFigure 4. Excised pulmonary valve cusps. Courtesy of William D. Edwards, MD, Mayo Clinic, Rochester, Minn.Download figureDownload PowerPointFigure 5. Microscopic examination of the tricuspid valve demonstrating typical fibrous tissue deposition (lower lighter layer) on the ventricular surface of the valve, with preservation of the native valve morphology (upper darker layer). Preserved chords are seen coursing through the carcinoid plaque in the right lower corner. Courtesy of William D. Edwards, MD, Mayo Clinic, Rochester, Minn.Download figureDownload PowerPointFigure 6. Histopathological examination of the tricuspid valve. Note the clear demarcation between the upper normal valvular tissue and the lower fibrous carcinoid deposition. Courtesy of William D. Edwards, MD, Mayo Clinic, Rochester, Minn.The online-only Data Supplement, which contains Movie I through Movie III, is available at http://cir.ahajournals.org/cgi/content/full/e160/DC1.FootnotesCorrespondence to Wissam Jaber, Division of Cardiovascular Diseases, Mayo Clinic, 201 West Center St, Rochester, MN 55905. E-mail [email protected] Previous Back to top Next FiguresReferencesRelatedDetailsCited By Nardozza M, Mele D and Ferrari R (2016) Noninvasive evaluation of right hemodynamics in carcinoid heart disease: A case report, Journal of Clinical Ultrasound, 10.1002/jcu.22413, 45:6, (355-361), Online publication date: 8-Jul-2017. Elsayes K, Menias C, Bowerson M, Osman O, Alkharouby A and Hillen T (2011) Imaging of Carcinoid Tumors, Journal of Computer Assisted Tomography, 10.1097/RCT.0b013e3181fd2044, 35:1, (72-80), Online publication date: 1-Jan-2011. Komoda S, Komoda T, Pavel M, Morawietz L, Wiedenmann B, Hetzer R and Lehmkuhl H (2011) Cardiac surgery for carcinoid heart disease in 12 cases, General Thoracic and Cardiovascular Surgery, 10.1007/s11748-010-0758-9, 59:12, (780-785), Online publication date: 1-Dec-2011. Martos R, Ridge C, Quinn M and Dodd J (2009) Cardiac carcinoid: tricuspid delayed hyperenhancement on cardiac 64-slice multidetector CT and magnetic resonance imaging, Irish Journal of Medical Science, 10.1007/s11845-008-0273-5, 179:3, (447-449), Online publication date: 1-Sep-2010. February 21, 2006Vol 113, Issue 7 Advertisement Article InformationMetrics https://doi.org/10.1161/CIRCULATIONAHA.105.509471PMID: 16490828 Originally publishedFebruary 21, 2006 PDF download Advertisement SubjectsCongenital Heart DiseaseEchocardiographyValvular Heart Disease

Acute Renal Failure After Cardiac Surgery for Carcinoid Heart Disease: Incidence, Risk Factors, and Prognosis

Cardiac surgery for carcinoid heart disease may be associated with acute renal failure (ARF) and multiorgan dysfunction postoperatively. This study was performed to determine the incidence, risk factors, and prognosis of ARF after cardiac surgery for carcinoid heart disease. This is a case-control study of 86 consecutive patients who underwent cardiac surgery for carcinoid heart disease. ARF is defined as hemodialysis requirement or serum creatinine level 50% greater than baseline, resulting in an estimated creatinine clearance less than 40 mL/min (0.67 mL/s). Preoperative, operative, and postoperative characteristics were examined and compared between groups to determine risk factors and prognosis of ARF. ARF occurred in 22% of cases (19 of 86 patients). Preoperative characteristics and type of procedure did not differ between groups. Operative variables associated with ARF by means of univariate analysis included longer surgical and bypass times, intra-aortic balloon pump use, and perioperative epinephrine requirement. Postoperatively, ARF was associated with epinephrine use, prolonged mechanical ventilation, prolonged intensive care unit admission, and higher Acute Physiology and Chronic Health Evaluation II scores. Perioperative mortality in the ARF group was 47% versus 4.5% in the control group (odds ratio, 36.1; 95% confidence interval, 8.0 to 261.8). ARF developed in a relatively high proportion of this cohort, but traditional preoperative risks failed to predict post-cardiac surgery ARF. Variables closely related to perioperative hemodynamic compromise were associated with ARF. ARF portends a particularly poor prognosis in this cohort that is explained largely by multiorgan failure syndrome.

Carcinoid heart disease: impact of pulmonary valve replacement in right ventricular function and remodeling

Carcinoid heart disease characteristically affects tricuspid (TV) and pulmonary valves (PV), and TV replacement is helpful in selected patients. There is uncertainty, however, regarding optimal surgical management of PV regurgitation. We reviewed 22 patients having operation for carcinoid heart disease and compared those having TV and PV replacement (n=12), to those who underwent TV replacement and excision of the PV (n=10). Pre- and postoperative right ventricular (RV) size and dysfunction were assessed by consensus of 2 echocardiographers blinded to type of surgical treatment. RV dysfunction was graded as none (0), mild (1), moderate (2), or severe (3). RV size was graded as normal (0), or mild (1), moderate (2), or severe (3) enlargement. Preoperatively, RV size (2.2+/-0.8 [no PVR]versus 2.7+/-0.6 [with PVR], P=0.15), RV dysfunction (0.9+/-0.9 [no PVR]versus 1.4+/-0.7 [with PVR], P=0.14), and NYHA class were similar in the 2 groups. Postop RV size decreased inpatients with PVR, 2.7+/-0.6 to 1.7+/-1.0 (P=0.008), but did not change appreciably in those without PVR, 2.2+/-0.8 to 2.3+/-0.8 (P=0.67). There was no significant change in RV dysfunction after surgery, 1.4+/-0.7 to 1.8+/-0.9 with PVR (P=0.26) and 0.9+/-0.9 to 1.6+/-0.9 without PVR (P=0.07). PV replacement appears to have a beneficial effect on RV size in patients after surgery for carcinoid heart disease. This may have important implications for RV remodeling after PV replacement.

Carcinoid Heart Disease: Impact of Pulmonary Valve Replacement in Right Ventricular Function and Remodeling

Background Carcinoid heart disease characteristically affects tricuspid (TV) and pulmonary valves (PV), and TV replacement is helpful in selected patients. There is uncertainty, however, regarding optimal surgical management of PV regurgitation. Methods and Results We reviewed 22 patients having operation for carcinoid heart disease and compared those having TV and PV replacement (n=12), to those who underwent TV replacement and excision of the PV (n=10). Pre- and postoperative right ventricular (RV) size and dysfunction were assessed by consensus of 2 echocardiographers blinded to type of surgical treatment. RV dysfunction was graded as none (0), mild (1), moderate (2), or severe (3). RV size was graded as normal (0), or mild (1), moderate (2), or severe (3) enlargement. Preoperatively, RV size (2.2±0.8 [no PVR]versus 2.7±0.6 [with PVR], P =0.15), RV dysfunction (0.9±0.9 [no PVR]versus 1.4±0.7 [with PVR], P =0.14), and NYHA class were similar in the 2 groups. Postop RV size decreased inpatients with PVR, 2.7±0.6 to 1.7±1.0 ( P =0.008), but did not change appreciably in those without PVR, 2.2±0.8 to 2.3±0.8 ( P =0.67). There was no significant change in RV dysfunction after surgery, 1.4±0.7 to 1.8±0.9 with PVR ( P =0.26) and 0.9±0.9 to 1.6±0.9 without PVR ( P =0.07). Conclusions PV replacement appears to have a beneficial effect on RV size in patients after surgery for carcinoid heart disease. This may have important implications for RV remodeling after PV replacement.

Surgical Pathology of Carcinoid Heart Disease: A Study of 139 Valves From 75 Patients Spanning 20 Years

Objective To quantitate the pathologic features of carcinoid plaques in a relatively large number of surgical specimens from a single institution. Patients and Methods Medical records, operative reports, and surgical specimens were reviewed from all patients with carcinoid heart disease who underwent cardiac valvular surgery at Mayo Clinic, Rochester, Minn, between 1980 and 2000. Results The study group included 75 patients (45 men, 30 women) who ranged in age from 26 to 78 years (mean, 59 years). From these 75 patients, 139 valves had been excised surgically (73 tricuspid, 55 pulmonary, 6 mitral, 5 aortic). Pure regurgitation was the most common dysfunctional state of the tricuspid valve (80% [60/75]), mitral valve (97% [32/33]), and aortic valve (96% [23/24]). The pulmonary valve was more often both stenotic and insufficient (52% [37/71]) than purely regurgitant (30% [21/71]). In all cases, valve dysfunction was attributed to the presence of carcinoid plaques, which caused both thickening and retraction. Thickening was the result of both cellular proliferation and deposition of extracellular matrix. Proliferation of myofibroblasts was observed in all plaques and was mild in 49% (68/139) and moderate or severe in 51% (71/139). Extracellular matrix included collagen (in 99% of the 139 valves), myxoid ground substance (98% [136/139]), and elastin (20% [28/139]). Carcinoid plaques were also involved by neovascularization (94% [131/139]), chronic inflammation (94% [131/139]), and mast cell infiltration (64% [89/139]). Severe thickening was attributable primarily to collagen deposition in tricuspid valves and to myofibroblast proliferation and myxoid matrix in pulmonary valves. Conclusions Among patients undergoing valvular surgery for carcinoid heart disease, tricuspid and pulmonary valves represented 92% of the excised valves (128/139). Although numerous cellular and extracellular features were common to the carcinoid plaques, variability in their relative expression produced appreciable differences in the histologic appearance among the plaques. To quantitate the pathologic features of carcinoid plaques in a relatively large number of surgical specimens from a single institution. Medical records, operative reports, and surgical specimens were reviewed from all patients with carcinoid heart disease who underwent cardiac valvular surgery at Mayo Clinic, Rochester, Minn, between 1980 and 2000. The study group included 75 patients (45 men, 30 women) who ranged in age from 26 to 78 years (mean, 59 years). From these 75 patients, 139 valves had been excised surgically (73 tricuspid, 55 pulmonary, 6 mitral, 5 aortic). Pure regurgitation was the most common dysfunctional state of the tricuspid valve (80% [60/75]), mitral valve (97% [32/33]), and aortic valve (96% [23/24]). The pulmonary valve was more often both stenotic and insufficient (52% [37/71]) than purely regurgitant (30% [21/71]). In all cases, valve dysfunction was attributed to the presence of carcinoid plaques, which caused both thickening and retraction. Thickening was the result of both cellular proliferation and deposition of extracellular matrix. Proliferation of myofibroblasts was observed in all plaques and was mild in 49% (68/139) and moderate or severe in 51% (71/139). Extracellular matrix included collagen (in 99% of the 139 valves), myxoid ground substance (98% [136/139]), and elastin (20% [28/139]). Carcinoid plaques were also involved by neovascularization (94% [131/139]), chronic inflammation (94% [131/139]), and mast cell infiltration (64% [89/139]). Severe thickening was attributable primarily to collagen deposition in tricuspid valves and to myofibroblast proliferation and myxoid matrix in pulmonary valves. Among patients undergoing valvular surgery for carcinoid heart disease, tricuspid and pulmonary valves represented 92% of the excised valves (128/139). Although numerous cellular and extracellular features were common to the carcinoid plaques, variability in their relative expression produced appreciable differences in the histologic appearance among the plaques.

Surgical Pathology of Carcinoid Heart Disease: A Study of 139 Valves From 75 Patients Spanning 20 Years

To quantitate the pathologic features of carcinoid plaques in a relatively large number of surgical specimens from a single institution. Medical records, operative reports, and surgical specimens were reviewed from all patients with carcinoid heart disease who underwent cardiac valvular surgery at Mayo Clinic, Rochester, Minn, between 1980 and 2000. The study group included 75 patients (45 men, 30 women) who ranged in age from 26 to 78 years (mean, 59 years). From these 75 patients, 139 valves had been excised surgically (73 tricuspid, 55 pulmonary, 6 mitral, 5 aortic). Pure regurgitation was the most common dysfunctional state of the tricuspid valve (80% [60/75]), mitral valve (97% [32/33]), and aortic valve (96% [23/24]). The pulmonary valve was more often both stenotic and insufficient (52% [37/71]) than purely regurgitant (30% [21/71]). In all cases, valve dysfunction was attributed to the presence of carcinoid plaques, which caused both thickening and retraction. Thickening was the result of both cellular proliferation and deposition of extracellular matrix. Proliferation of myofibroblasts was observed in all plaques and was mild in 49% (68/139) and moderate or severe in 51% (71/139). Extracellular matrix included collagen (in 99% of the 139 valves), myxoid ground substance (98% [136/139]), and elastin (20% [28/139]). Carcinoid plaques were also involved by neovascularization (94% [131/139]), chronic inflammation (94% [131/139]), and mast cell infiltration (64% [89/139]). Severe thickening was attributable primarily to collagen deposition in tricuspid valves and to myofibroblast proliferation and myxoid matrix in pulmonary valves. Among patients undergoing valvular surgery for carcinoid heart disease, tricuspid and pulmonary valves represented 92% of the excised valves (128/ 139). Although numerous cellular and extracellular features were common to the carcinoid plaques, variability in their relative expression produced appreciable differences in the histologic appearance among the plaques.

Regression of metastatic carcinoid tumor after valvular surgery for carcinoid heart disease

BACKGROUND The carcinoid syndrome is a common sequela in patients with carcinoid tumor metastatic to the liver. Cardiac involvement occurs in 19-56% of patients with symptomatic carcinoid syndrome and, in some patients, leads to valvular surgery to relieve symptoms due to progressive right-sided heart failure. Reports of these patients have emphasized amelioration of cardiac symptoms, but postoperative tumor status rarely has been discussed. METHODS This report describes four patients who underwent valvular heart surgery for severe carcinoid heart disease and had regression of their metastatic carcinoid tumor postoperatively. RESULTS All four patients had definite clinical improvement in cardiac function and relief of symptoms related to congestive heart failure postoperatively. Unexpectedly, they also had regression of their metastatic disease, as reflected in decreased levels of urinary 5-hydroxyindoleacetic acid, and objective evidence of a reduction in the size of hepatic metastases. CONCLUSIONS To the authors' knowledge, these four patients represent the first reported cases of metastatic disease regression after valvular surgery for carcinoid heart disease. Further descriptions of tumor status in patients having undergone a heart operation for this disease would be valuable in determining the clinical significance of this finding. Cancer 1997; 79:605-11. © 1997 American Cancer Society.

Regression of metastatic carcinoid tumor after valvular surgery for carcinoid heart disease

The carcinoid syndrome is a common sequela in patients with carcinoid tumor metastatic to the liver. Cardiac involvement occurs in 19-56% of patients with symptomatic carcinoid syndrome and, in some patients, leads to valvular surgery to relieve symptoms due to progressive right-sided heart failure. Reports of these patients have emphasized amelioration of cardiac symptoms, but postoperative tumor status rarely has been discussed. This report describes four patients who underwent valvular heart surgery for severe carcinoid heart disease and had regression of their metastatic carcinoid tumor postoperatively. All four patients had definite clinical improvement in cardiac function and relief of symptoms related to congestive heart failure postoperatively. Unexpectedly, they also had regression of their metastatic disease, as reflected in decreased levels of urinary 5-hydroxyindoleacetic acid, and objective evidence of a reduction in the size of hepatic metastases. To the authors' knowledge, these four patients represent the first reported cases of metastatic disease regression after valvular surgery for carcinoid heart disease. Further descriptions of tumor status in patients having undergone a heart operation for this disease would be valuable in determining the clinical significance of this finding.

Outcome of cardiac surgery for carcinoid heart disease

Objectives.The hypothesis was that cardiac surgery for symptomatic carcinoid heart disease in conjunction with adjunctive therapy could improve the long-term outlook of patients with carcinoid heart disease.Background.Patients with carcinoid heart disease have a dismal prognosis; most die of progressive right heart failure within 1 year after onset of symptoms. Improved therapies for the systemic manifestations of the carcinoid syndrome have resulted in symptomatic improvement and prolonged survival in patients without heart disease.Methods.Twenty-six patients with symptomatic carcinoid heart disease underwent valvular surgery. Preoperative clinical, laboratory, Doppler echocardiographic and hemodynamic factors were evaluated. The survival of the surgical group was compared with that of a control group of 40 medically treated patients.Results.There were nine perioperative deaths (35%), primarily from postoperative bleeding and right ventricular failure. Of the 17 surgical survivors, 8 were alive at a mean of 28 months of follow-up. The postoperative functional class of the eight surviving patients was substantially improved. Late deaths were primarily due to hepatic dysfunction caused by metastatic disease. The only predictor of operative mortality (p = 0.03) was low voltage on preoperative electrocardiography (limb lead voltage ≤ 5 mm). Predictors of late survival included a lower preoperative somatostatin requirement and a lower preoperative urinary 5-hydroxy-indoleacetic acid level. There was a trend toward increased survival for the surgical group compared with the control group.Conclusions.Because new therapies have improved survival in patients with the malignant carcinoid syndrome, cardiac involvement has become a major cause of morbidity and mortality. Valve surgery is the only definitive treatment. Although cardiac surgery carries a high perioperative mortality, marked symptomatic improvement occurs in survivors. Surgical intervention should therefore be considered when cardiac symptoms become severe.